const {
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  VerticalAlign, PageNumber, ShadingType, TableLayoutType,
  convertInchesToTwip, UnderlineType
} = require('docx');
const fs = require('fs');

// ── helpers ──────────────────────────────────────────────────────────────────

const BLUE   = "1F3864";   // dark-navy heading
const TEAL   = "1A6B6B";   // section heading
const YELLOW_BG = "FFF2CC"; // highlight cells
const HEADER_BG = "1F3864";
const HEADER_FG = "FFFFFF";
const ALT_ROW   = "EAF0FB";

function h1(text) {
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function p(text, opts = {}) {
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function bullet(text, level = 0) {
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}

// ── Table builder ──────────────────────────────────────────────────────────

function makeTable(headers, rows, opts = {}) {
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  });
}

// ═══════════════════════════════════════════════════════════════════════════
//  CONTENT
// ═══════════════════════════════════════════════════════════════════════════

const children = [];

// ── COVER / TITLE ──────────────────────────────────────────────────────────
children.push(
  new Paragraph({ spacing: { before: 1200, after: 200 } }),
  new Paragraph({
    children: [new TextRun({ text: "PAEDIATRIC NEUROLOGY", bold: true, size: 56, color: BLUE })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 160 }
  }),
  new Paragraph({
    children: [new TextRun({ text: "Comprehensive Exam Notes", bold: true, size: 36, color: TEAL })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 120 }
  }),
  new Paragraph({
    children: [new TextRun({ text: "MD Paediatrics PG Selection Examination", size: 26, color: "555555", italics: true })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 80 }
  }),
  new Paragraph({
    children: [new TextRun({ text: "PGIM – University of Colombo, Sri Lanka", size: 24, color: "777777", italics: true })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 80 }
  }),
  new Paragraph({
    children: [new TextRun({ text: "Based on: Nelson's Textbook of Paediatrics (21st/22nd Ed) • Harriet Lane Handbook (23rd Ed)", size: 22, color: "888888" })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 80 }
  }),
  new Paragraph({
    children: [new TextRun({ text: `Generated: ${new Date().toLocaleDateString('en-GB', {day:'2-digit',month:'long',year:'numeric'})}`, size: 20, color: "999999" })],
    alignment: AlignmentType.CENTER, spacing: { before: 0, after: 0 }
  }),
  pageBreak()
);

// ═══════════════════════════════════════════════════════════════════════════
// 1. NEUROLOGICAL EXAMINATION
// ═══════════════════════════════════════════════════════════════════════════
children.push(h1("1. NEUROLOGICAL EXAMINATION IN CHILDREN"));

children.push(h2("1.1 Neonatal Neurological Examination"));
children.push(p("Gestational age-dependent findings are normal – preterm infants have a physiologically hypotonic state."));
children.push(h3("Primitive Reflexes – Normal Disappearance Ages"));
children.push(makeTable(
  ["Reflex", "Disappears By"],
  [
    ["Moro reflex", "4–6 months"],
    ["Palmar grasp", "3–4 months"],
    ["Plantar grasp", "9–12 months"],
    ["Rooting", "3–4 months"],
    ["ATNR (fencer reflex)", "4–6 months"],
    ["Parachute response", "Appears 8–9 months; persists"],
  ]
));

children.push(h3("Key Developmental Motor Milestones"));
children.push(makeTable(
  ["Age", "Gross Motor", "Fine Motor"],
  [
    ["2 months", "Lifts head 45° prone", "Hands unfisted"],
    ["4 months", "Lifts head 90°, rolls front to back", "Reaches, midline hand play"],
    ["6 months", "Sits with support, rolls both ways", "Transfers hand to hand"],
    ["9 months", "Pulls to stand, cruises", "Inferior pincer grasp"],
    ["12 months", "Walks with support", "Fine pincer grasp"],
    ["18 months", "Walks independently, runs", "Stacks 2–3 cubes"],
    ["2 years", "Runs, up stairs (both feet per step)", "Stacks 6 cubes, circular scribble"],
  ]
));
children.push(h3("Motor Delay Red Flags"));
children.push(bullet("Not sitting by 9 months"));
children.push(bullet("Not walking by 18 months"));
children.push(bullet("Hand dominance before 18 months (suggests contralateral weakness)"));
children.push(bullet("Loss/regression of motor skills at any age (always serious)"));

// ═══════════════════════════════════════════════════════════════════════════
// 2. SEIZURES & EPILEPSY
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("2. SEIZURES AND EPILEPSY"));

children.push(h2("2.1 ILAE 2017 Seizure Classification"));
children.push(makeTable(
  ["Focal Onset", "Generalised Onset", "Unknown Onset"],
  [
    ["Aware (= old 'simple partial')", "Tonic-clonic", "Tonic-clonic"],
    ["Impaired awareness (= old 'complex partial')", "Absence", "Epileptic spasms"],
    ["Focal to bilateral tonic-clonic", "Myoclonic", "Unknown"],
    ["", "Tonic", ""],
    ["", "Atonic ('drop attacks')", ""],
    ["", "Clonic", ""],
  ]
));

children.push(h2("2.2 Causes of Seizures by Age"));
children.push(makeTable(
  ["Age", "Common Causes"],
  [
    ["Neonates (0–28 days)", "HIE, hypoglycaemia, hypocalcaemia, hypomagnesaemia, ICH, CNS infection, IEM, pyridoxine deficiency, brain malformation"],
    ["1 month – 2 years", "Febrile seizures, infantile spasms, meningitis, metabolic, trauma (NAI)"],
    ["2–6 years", "Febrile seizures, idiopathic epilepsy, trauma, CNS infection"],
    ["School age", "Idiopathic epilepsy (CAE, BECTS), trauma, CNS tumour"],
    ["Adolescence", "JME, trauma, drug-related, vasculitis"],
  ]
));

children.push(h2("2.3 Febrile Seizures ★ HIGH YIELD"));
children.push(h3("Simple Febrile Seizure"));
children.push(bullet("Age 6–60 months"));
children.push(bullet("Generalised, tonic-clonic"));
children.push(bullet("Duration < 15 minutes"));
children.push(bullet("Does NOT recur within 24 hours"));
children.push(bullet("No post-ictal focal deficit"));
children.push(p("**Management:** Identify and treat source of fever. No EEG, neuroimaging, or bloodwork required if: well-appearing, fully immunised, normal neuro exam, no meningeal signs."));

children.push(h3("Complex Febrile Seizure – any of:"));
children.push(bullet("Focal onset"));
children.push(bullet("Duration > 15 minutes"));
children.push(bullet("Recurs within 24 hours"));

children.push(h3("LP Indications After Febrile Seizure"));
children.push(makeTable(
  ["Age", "Recommendation"],
  [
    ["< 12 months", "Strongly consider"],
    ["12–18 months", "Consider"],
    ["> 18 months", "Only if meningism/concerning features"],
    ["Any age + prior antibiotics", "Always perform (may mask meningitis)"],
  ]
));

children.push(h3("Recurrence Risk"));
children.push(bullet("30–40% overall recurrence risk"));
children.push(bullet("Risk factors: age < 12 months, FH of FS, low-grade fever, short duration before seizure"));
children.push(bullet("Risk of epilepsy: simple FS ~1–2%; complex FS 4–15%"));

children.push(h2("2.4 Epilepsy Syndromes ★ HIGH YIELD"));

children.push(h3("Neonatal Syndromes"));
children.push(makeTable(
  ["Syndrome", "Onset", "EEG", "Treatment", "Prognosis"],
  [
    ["Benign neonatal seizures\n('fifth day fits')", "Day 4–6", "Theta pointu alternant", "Self-limiting", "Excellent"],
    ["Early myoclonic encephalopathy (EME)", "1st month", "Burst-suppression", "Variable", "Poor"],
    ["Ohtahara syndrome (EIEE)", "First 3 months", "Burst-suppression", "Variable", "Poor, often fatal"],
  ]
));

children.push(h3("Infantile Syndromes"));
children.push(h4("West Syndrome (Infantile Spasms) ★★"));
children.push(bullet("Age: 4–8 months (peak 6 months)"));
children.push(bullet("Triad: epileptic spasms + hypsarrhythmia + developmental regression"));
children.push(bullet("Spasms: sudden bilateral tonic contraction (flexion > extension), in clusters on waking"));
children.push(bullet("Causes: structural (TSC, Down, HIE, lissencephaly), metabolic, unknown"));
children.push(bullet("**Treatment:** ACTH (1st line most centres); Vigabatrin (1st line in tuberous sclerosis)"));
children.push(bullet("Prognosis: ~90% have intellectual disability; may evolve to Lennox-Gastaut"));
children.push(bullet("Investigations: MRI brain, metabolic screen, chromosomes, urine organic acids"));

children.push(h4("Dravet Syndrome ★★"));
children.push(bullet("SCN1A gene mutation (Nav1.1 sodium channel)"));
children.push(bullet("Fever-triggered prolonged hemiclonic seizures in 1st year → polymorphic seizures"));
children.push(bullet("**AVOID sodium channel blockers:** carbamazepine, phenytoin, lamotrigine – WORSEN seizures"));
children.push(bullet("Treatment: valproate + clobazam; add-on: topiramate, stiripentol, cannabidiol (Epidiolex), fenfluramine"));

children.push(h3("Childhood Syndromes"));
children.push(makeTable(
  ["Syndrome", "Age", "Seizure Type", "EEG", "Treatment"],
  [
    ["Childhood Absence Epilepsy (CAE)", "4–10 yrs", "Absence 5–30 sec, abrupt onset/offset", "3 Hz generalised spike-wave", "Ethosuximide (1st line), valproate, lamotrigine"],
    ["BECTS (Rolandic epilepsy)", "3–13 yrs", "Nocturnal facial twitching, drooling, 2° generalisation", "Centrotemporal spikes, sleep-activated", "Often nil; levetiracetam, oxcarbazepine"],
    ["Lennox-Gastaut (LGS)", "1–7 yrs", "Tonic, atonic, atypical absence; status common", "Slow spike-wave < 2.5 Hz", "Valproate + clobazam; add rufinamide, lamotrigine"],
  ]
));

children.push(h4("Childhood Absence Epilepsy – Key Points"));
children.push(bullet("Absence ≠ complex partial: no aura, no post-ictal confusion, abrupt onset/offset"));
children.push(bullet("Hyperventilation for 3 minutes in clinic precipitates typical absence"));
children.push(bullet("Ethosuximide = drug of choice (best evidence, fewest side effects)"));
children.push(bullet("~65–70% achieve remission by adolescence; 10% develop JME"));

children.push(h4("BECTS – Key Points"));
children.push(bullet("Most common childhood epilepsy syndrome"));
children.push(bullet("Nocturnal oro-facial-pharyngeal: drooling, guttural sounds, facial twitching; child remains conscious"));
children.push(bullet("Normal development and intelligence; self-limiting – remission in >95% by age 15–16"));

children.push(h3("Adolescent Syndromes"));
children.push(h4("Juvenile Myoclonic Epilepsy (JME) ★★"));
children.push(bullet("Age 12–18 years; seizure types: myoclonic jerks (morning) + GTCS + absence"));
children.push(bullet("EEG: 4–6 Hz generalised polyspike-wave"));
children.push(bullet("Triggers: sleep deprivation, alcohol, photic stimulation"));
children.push(bullet("Often misdiagnosed – patient 'drops things in the morning'"));
children.push(bullet("**Lifelong treatment usually required** (high relapse rate if stopped)"));
children.push(bullet("1st line: valproate; avoid in girls (teratogenic) → use levetiracetam or lamotrigine"));

children.push(h2("2.5 Status Epilepticus ★ EXAM ESSENTIAL"));
children.push(p("**Definition:** Seizure lasting > 5 minutes OR 2 seizures without return to baseline consciousness."));
children.push(h3("Management Protocol"));
children.push(makeTable(
  ["Phase", "Time", "Actions"],
  [
    ["Phase 0", "0–5 min", "ABCs, O2, IV/IO access, BGL check, place in recovery position"],
    [{text:"Phase 1 – Benzodiazepines", bold:true}, "5–20 min", "Lorazepam IV 0.05–0.1 mg/kg (preferred) OR Diazepam IV 0.2–0.3 mg/kg OR rectal 0.5 mg/kg OR Midazolam IM 0.1–0.2 mg/kg (no IV). Repeat once after 5 min if no response."],
    [{text:"Phase 2 – 2nd-line agents", bold:true}, "20–40 min", "Levetiracetam IV 20–60 mg/kg (preferred) OR Valproate IV 20–40 mg/kg OR Fosphenytoin IV 20 mg/kg PE OR Phenobarbital IV 20 mg/kg"],
    [{text:"Phase 3 – Refractory SE", bold:true}, "> 40 min", "RSI + ICU. Midazolam infusion 0.1–2 mg/kg/hr. Pentobarbital infusion. Consider: pyridoxine 100 mg IV if < 2 years (rule out pyridoxine-dependent epilepsy)"],
  ]
));

children.push(h2("2.6 Anti-Seizure Medications (ASMs) Summary"));
children.push(makeTable(
  ["Drug", "Seizure Type", "Key Side Effects", "Notes"],
  [
    ["Phenobarbital", "Focal, generalised", "Sedation, cognitive impairment, hyperactivity", "1st line neonate; hepatic enzyme inducer"],
    ["Carbamazepine", "Focal, GTCS", "Hyponatraemia, diplopia, rash (SJS – HLA-B*1502)", "Worsens absence, myoclonic, Dravet"],
    ["Valproate", "All types", "Weight gain, hair loss, hepatotoxicity, teratogenicity (NTD)", "Drug of choice many generalised syndromes; avoid in women of childbearing age"],
    ["Ethosuximide", "Absence ONLY", "GI upset, headache, hiccups", "1st line childhood absence"],
    ["Levetiracetam", "Focal, generalised", "Behavioural: irritability, aggression", "Wide safety profile; IV formulation available"],
    ["Lamotrigine", "Focal, generalised, absence", "Rash (SJS risk – titrate SLOWLY)", "Avoid rapid titration; interaction with valproate"],
    ["Topiramate", "Focal, generalised", "Cognitive slowing, kidney stones, weight loss, glaucoma", "'Dope-a-max'; metabolic acidosis; migraine prophylaxis"],
    ["Vigabatrin", "Infantile spasms, focal", "Visual field defects (irreversible), sedation", "1st line TSC + West syndrome"],
    ["ACTH", "West syndrome", "Hypertension, infection, adrenal suppression", "40–60 IU/day; monitor BP"],
    ["Clobazam", "Adjunctive (Dravet, LGS)", "Sedation, tolerance", "1,5-benzodiazepine; less sedating"],
    ["Fenfluramine", "Dravet syndrome", "Cardiac valvulopathy (monitor with echo)", "Newer agent; significant efficacy in Dravet"],
  ]
));
children.push(p("⚠️ **HLA-B*1502 (Asian populations – including Sri Lanka):** Screen BEFORE starting carbamazepine, oxcarbazepine, phenytoin, or lamotrigine. Positive = high risk of Stevens-Johnson Syndrome / toxic epidermal necrolysis.", {color:"C00000"}));

// ═══════════════════════════════════════════════════════════════════════════
// 3. HEADACHE
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("3. HEADACHE"));

children.push(h2("3.1 Red Flags ("SNOOP" Mnemonic)"));
children.push(makeTable(
  ["Letter", "Red Flag"],
  [
    ["S", "Systemic symptoms (fever, weight loss) / Secondary risk factors (immunosuppression, malignancy)"],
    ["N", "Neurological signs or symptoms (focal deficit, papilloedema, meningism)"],
    ["O", "Onset: thunderclap (worst-ever, sudden-onset headache)"],
    ["O", "Older age of onset / Wakes from sleep / Progressive pattern"],
    ["P", "Prior headache history change / Postural component"],
  ]
));

children.push(h2("3.2 Migraine in Children"));
children.push(h3("ICHD-3 Diagnostic Criteria (modified for children)"));
children.push(bullet("≥5 attacks lasting 2–72 hours (may be shorter in children)"));
children.push(bullet("≥2 of: unilateral (may be bilateral in children), pulsating quality, moderate-severe, aggravated by physical activity"));
children.push(bullet("≥1 of: nausea/vomiting OR photophobia AND phonophobia"));
children.push(bullet("Migraine with aura: reversible visual/sensory/motor/speech symptoms, 5–60 minutes before headache"));

children.push(h3("Abortive Treatment"));
children.push(bullet("**1st line:** NSAIDs – ibuprofen 10 mg/kg"));
children.push(bullet("**2nd line:** Triptans – sumatriptan nasal spray (≥12 yrs), rizatriptan (≥6 yrs), almotriptan (≥12 yrs)"));
children.push(bullet("Antiemetics: ondansetron, metoclopramide"));
children.push(bullet("Avoid opioids"));

children.push(h3("Migraine Prophylaxis (Harriet Lane Table)"));
children.push(makeTable(
  ["Drug", "Adverse Effects", "Comorbidities to Consider"],
  [
    ["Topiramate", "Cognitive slowing, weight loss, kidney stones, paresthesia", "Obesity, epilepsy"],
    ["Valproate", "Hepatotoxicity, weight gain, teratogenicity", "Bipolar disorder, epilepsy"],
    ["Amitriptyline", "Sedation, constipation, weight gain (Black box: suicidal thoughts)", "Depression, insomnia"],
    ["Propranolol", "Hypotension, bronchospasm, masks hypoglycaemia", "Hypertension"],
    ["Cyproheptadine", "Sedation, increased appetite, hepatitis", "Seasonal allergies, poor appetite (young children)"],
    ["Riboflavin / Magnesium / CoQ10", "Low side-effect profile; limited paediatric data", "Poor nutritional intake"],
  ]
));
children.push(p("Anti-CGRP agents: approved in adults (2018); no paediatric studies yet."));

children.push(h2("3.3 Paediatric Migraine Variants"));
children.push(bullet("**Abdominal migraine:** Recurrent periumbilical pain 1–72 hours + nausea; no headache; treat as migraine"));
children.push(bullet("**Cyclic vomiting syndrome:** Stereotyped vomiting episodes; treat with ondansetron, amitriptyline"));

// ═══════════════════════════════════════════════════════════════════════════
// 4. CNS INFECTIONS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("4. CENTRAL NERVOUS SYSTEM INFECTIONS"));

children.push(h2("4.1 Bacterial Meningitis"));
children.push(h3("Organisms by Age"));
children.push(makeTable(
  ["Age", "Common Organisms"],
  [
    ["Neonates", "Group B Streptococcus, E. coli, Listeria monocytogenes, Klebsiella"],
    ["1–3 months", "GBS, E. coli, Listeria + Strep. pneumoniae, N. meningitidis"],
    ["3 months – 5 years", "Strep. pneumoniae (most common), N. meningitidis, Hib (unvaccinated)"],
    ["> 5 years", "Strep. pneumoniae, N. meningitidis"],
  ]
));

children.push(h3("CSF Analysis in Meningitis"));
children.push(makeTable(
  ["Parameter", "Bacterial", "Viral", "TB", "Fungal"],
  [
    ["Appearance", "Turbid/purulent", "Clear", "Clear/xanthochromic", "Clear"],
    ["WBC", "> 1000 (PMN)", "10–500 (lymph)", "10–500 (lymph)", "10–500 (lymph)"],
    ["Protein", "High (> 1 g/L)", "Normal/mild", "Very high", "Elevated"],
    ["Glucose (CSF:serum)", "< 0.4 (low)", "Normal (> 0.6)", "Very low (< 0.3)", "Low"],
    ["Gram stain/Special", "Positive ~80%", "Negative", "ZN stain", "India ink (Cryptococcus)"],
  ]
));

children.push(h3("Management"));
children.push(bullet("**Empirical antibiotics IMMEDIATELY** – do NOT delay for LP if: shocked, papilloedema, or focal signs"));
children.push(bullet("Ceftriaxone 100 mg/kg/day IV (max 4 g/day) + ampicillin if < 3 months (Listeria coverage)"));
children.push(bullet("**Dexamethasone** 0.15 mg/kg IV 6-hourly × 4 days – give BEFORE or WITH 1st antibiotic dose (reduces hearing loss, neurological sequelae in pneumococcal meningitis)"));
children.push(bullet("Fluid management: isotonic fluids; maintain euvolaemia (fluid restriction is OLD teaching)"));
children.push(bullet("All patients need formal audiology (hearing loss = most common long-term complication)"));

children.push(h3("Meningococcal Disease – Key Points"));
children.push(bullet("Non-blanching petechial/purpuric rash = emergency (meningococcaemia)"));
children.push(bullet("Waterhouse-Friderichsen syndrome: bilateral adrenal haemorrhage + DIC + shock"));
children.push(bullet("Treatment: benzylpenicillin IV; ceftriaxone if penicillin allergy"));
children.push(bullet("Prophylaxis for close contacts: rifampicin 2 days, OR ciprofloxacin single dose, OR ceftriaxone IM"));

children.push(h2("4.2 HSV Encephalitis ★★"));
children.push(bullet("Temporal lobe involvement: personality change, memory disturbance, temporal lobe seizures"));
children.push(bullet("EEG: PLEDs (periodic lateralising epileptiform discharges) in temporal region"));
children.push(bullet("MRI: temporal lobe signal change on FLAIR/DWI"));
children.push(bullet("CSF: HSV PCR (gold standard)"));
children.push(bullet("**Treatment: Acyclovir IV 10–15 mg/kg/dose 8-hourly × 14–21 days – START EMPIRICALLY**"));

children.push(h2("4.3 Tuberculous Meningitis (TBM)"));
children.push(h3("Clinical Stages"));
children.push(makeTable(
  ["Stage", "Features"],
  [
    ["Stage 1 (Alert)", "Non-specific: fever, malaise, headache, irritability"],
    ["Stage 2 (Lethargic)", "Meningism + CN palsies (VI and III most common) + mild confusion"],
    ["Stage 3 (Coma)", "Obtundation, seizures, coma, hemiplegia"],
  ]
));
children.push(bullet("MRI: basal exudate + hydrocephalus + basal ganglia infarcts (characteristic triad)"));
children.push(bullet("Treatment: RHEZ × 2 months + RH × 7–10 months (total 9–12 months)"));
children.push(bullet("Add steroids (prednisolone/dexamethasone) – reduce mortality and neurological disability"));

children.push(h2("4.4 Autoimmune Encephalitis (NMDAR) ★★"));
children.push(bullet("Anti-NMDA receptor encephalitis (anti-NR1 antibody)"));
children.push(bullet("Young women/girls; may be associated with ovarian teratoma"));
children.push(bullet("Stages: prodrome → psychiatric symptoms → seizures → orofacial dyskinesias → decreased consciousness → autonomic instability"));
children.push(bullet("MRI often normal; antibodies in serum AND CSF"));
children.push(bullet("Treatment: tumour removal (if present) + IV methylprednisolone → IVIG → rituximab/cyclophosphamide"));

// ═══════════════════════════════════════════════════════════════════════════
// 5. CEREBRAL PALSY
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("5. CEREBRAL PALSY"));

children.push(p("**Definition:** Permanent, non-progressive disorders of movement and posture attributed to disturbances in fetal/infant brain development."));
children.push(p("**Epidemiology:** 2–3 per 1,000 live births"));

children.push(h2("5.1 Classification"));
children.push(makeTable(
  ["Type", "Tone", "Distribution", "Pathology"],
  [
    ["Spastic (~80%)", "Increased (clasp-knife, clonus)", "Diplegia / Quadriplegia / Hemiplegia", "PVL (preterm), cortical/subcortical injury (term)"],
    ["Dyskinetic (~10%)", "Variable (lead-pipe or candle-wax)", "Generalised", "Basal ganglia injury (hyperbilirubinaemia, perinatal asphyxia)"],
    ["Ataxic (~5–10%)", "Decreased", "Generalised", "Cerebellar pathology"],
    ["Hypotonic", "Decreased", "Generalised/axial", "Diffuse cortical/cerebellar dysfunction"],
  ]
));
children.push(p("**PVL (Periventricular Leukomalacia):** Premature infants < 32 weeks. White matter injury in watershed zones → spastic diplegia (legs > arms, as leg fibres are closest to ventricles)."));

children.push(h2("5.2 Clinical Features"));
children.push(bullet("Delayed motor milestones + abnormal tone + persistent primitive reflexes > 6 months"));
children.push(bullet("Hand dominance before 18 months → investigate for contralateral hemiplegia"));
children.push(p("**Comorbidities:** Epilepsy (30–50%), intellectual disability, vision and hearing problems, speech/language delay, feeding difficulties, orthopaedic problems"));

children.push(h2("5.3 GMFCS (Gross Motor Function Classification System)"));
children.push(makeTable(
  ["Level", "Functional Ability"],
  [
    ["I", "Walks without limitations"],
    ["II", "Walks with limitations"],
    ["III", "Walks using handheld mobility device"],
    ["IV", "Self-mobility limited; uses power wheelchair"],
    ["V", "Self-mobility severely limited even with assistive technology"],
  ]
));

children.push(h2("5.4 Management"));
children.push(bullet("**Multidisciplinary:** physiotherapy, occupational therapy, speech therapy, orthotics"));
children.push(bullet("**Spasticity:** Botulinum toxin A (focal), oral baclofen, intrathecal baclofen pump, selective dorsal rhizotomy"));
children.push(bullet("**Orthopaedic:** hip surveillance, scoliosis management"));
children.push(bullet("Treat associated epilepsy, vision/hearing problems, nutrition"));

// ═══════════════════════════════════════════════════════════════════════════
// 6. NEURODEVELOPMENTAL DISORDERS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("6. NEURODEVELOPMENTAL DISORDERS"));

children.push(h2("6.1 Intellectual Disability (ID)"));
children.push(p("**Definition:** IQ < 70 + impairment in adaptive functioning + onset < 18 years"));
children.push(makeTable(
  ["Severity", "IQ Range", "Functional Ability"],
  [
    ["Mild", "50–70", "Can usually read; live semi-independently"],
    ["Moderate", "35–50", "Functional literacy with support"],
    ["Severe", "20–35", "Limited self-care"],
    ["Profound", "< 20", "Requires full care"],
  ]
));
children.push(p("**Investigations:** Chromosomal microarray (preferred over karyotype), fragile X (boys), metabolic screen, TFTs, TORCH serology, MRI brain."));
children.push(p("**Key causes:** Down syndrome (most common chromosomal), Fragile X (most common INHERITED – CGG repeat in FMR1 gene), PKU (treatable), hypothyroidism (treatable)"));

children.push(h2("6.2 Autism Spectrum Disorder (ASD)"));
children.push(h3("DSM-5 Criteria"));
children.push(bullet("Persistent deficits in social communication and interaction across contexts"));
children.push(bullet("Restricted, repetitive patterns of behaviour, interests, or activities"));
children.push(bullet("Symptoms present in early developmental period"));
children.push(bullet("Causes significant functional impairment"));

children.push(h3("Red Flags ★★"));
children.push(makeTable(
  ["Age", "Red Flag"],
  [
    ["2 months", "No social smile"],
    ["12 months", "No babbling, no pointing, no social gestures"],
    ["16 months", "No single words"],
    ["24 months", "No two-word phrases"],
    ["Any age", "ANY regression of language or social skills"],
  ]
));
children.push(p("**Screening:** M-CHAT (Modified Checklist for Autism in Toddlers) – used at 16–30 months."));
children.push(p("**Comorbidities:** Epilepsy (25–30%), ADHD, anxiety, intellectual disability."));
children.push(p("**Management:** Early intensive behavioural therapy (ABA), speech therapy, OT, special education. Risperidone/aripiprazole for irritability/aggression."));

children.push(h2("6.3 ADHD"));
children.push(p("**DSM-5:** ≥6 symptoms of inattention AND/OR hyperactivity-impulsivity; onset < 12 years; present in ≥2 settings; impairs functioning."));
children.push(h3("Management"));
children.push(makeTable(
  ["Age", "1st Line", "2nd Line"],
  [
    ["< 6 years", "Parent training / behavioural therapy", "Medication only if severe"],
    ["≥ 6 years", "Stimulants (methylphenidate, amphetamines)", "Atomoxetine (SNRI), guanfacine, clonidine"],
  ]
));
children.push(p("**Methylphenidate:** 0.3–1 mg/kg/day; side effects: appetite suppression, insomnia, growth concerns (monitor height/weight)."));
children.push(p("**Atomoxetine:** Useful if substance abuse risk, tics, or anxiety; takes 4–6 weeks for full effect."));

// ═══════════════════════════════════════════════════════════════════════════
// 7. RAISED ICP & HYDROCEPHALUS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("7. INCREASED INTRACRANIAL PRESSURE (ICP)"));

children.push(h2("7.1 Clinical Features"));
children.push(h3("Infants (open fontanelle)"));
children.push(bullet("Bulging fontanelle, rapid head circumference increase (> 2 cm/week)"));
children.push(bullet("Engorged scalp veins, setting-sun sign (Parinaud sign = dorsal midbrain compression by hydrocephalus)"));

children.push(h3("Older Children"));
children.push(bullet("Headache: morning, wakes from sleep, worse with Valsalva"));
children.push(bullet("Vomiting: projectile, without nausea"));
children.push(bullet("Diplopia: CN VI palsy – false localising sign"));
children.push(bullet("Papilloedema"));
children.push(bullet("**Cushing's triad (LATE, OMINOUS):** bradycardia + hypertension + irregular breathing"));

children.push(h2("7.2 Hydrocephalus"));
children.push(makeTable(
  ["Type", "Mechanism", "Examples"],
  [
    ["Obstructive (non-communicating)", "Block in CSF flow pathway", "Aqueduct stenosis, posterior fossa tumour, vein of Galen malformation"],
    ["Communicating", "Impaired CSF reabsorption", "Post-meningitis, subarachnoid haemorrhage, choroid plexus tumour"],
  ]
));
children.push(bullet("**Investigations:** Head USS (infants); CT (emergency); MRI (gold standard)"));
children.push(bullet("**VP shunt:** Most common treatment"));
children.push(bullet("**ETV (Endoscopic Third Ventriculostomy):** Suitable for aqueduct stenosis in older children; avoids shunt"));

children.push(h2("7.3 Pseudotumour Cerebri (IIH)"));
children.push(bullet("Raised ICP, no structural cause, normal CSF composition"));
children.push(bullet("Associations: obesity, vitamin A toxicity, tetracycline, OCP, steroid withdrawal"));
children.push(bullet("Symptoms: headache, pulsatile tinnitus, visual obscurations, diplopia"));
children.push(bullet("Treatment: acetazolamide, weight loss; serial LP; optic nerve sheath fenestration (if vision threatened)"));

// ═══════════════════════════════════════════════════════════════════════════
// 8. BRAIN TUMOURS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("8. BRAIN TUMOURS IN CHILDREN"));

children.push(p("**70% of childhood brain tumours are infratentorial** (posterior fossa)."));
children.push(p("**Clinical triad of posterior fossa tumour:** (1) Raised ICP, (2) Cerebellar signs (ataxia, nystagmus, dysmetria), (3) CN palsies"));

children.push(h2("8.1 Infratentorial Tumours ★★"));
children.push(makeTable(
  ["Tumour", "Age", "Key Features", "Treatment"],
  [
    ["Medulloblastoma", "5–14 years", "4th ventricle → obstructive hydrocephalus; 'drop metastases' down spinal cord; desmoplastic variant has better prognosis", "Surgery + craniospinal RT + chemotherapy"],
    ["Cerebellar astrocytoma (pilocytic)", "5–15 years", "Cystic + mural nodule; benign; excellent prognosis", "Surgery alone often curative"],
    ["Ependymoma", "< 5 years", "4th ventricle floor; high recurrence", "Surgery + local RT"],
    ["DIPG (brainstem glioma)", "5–9 years", "Diffuse intrinsic pontine glioma; CN palsies + long tract signs + ataxia; H3K27M mutation; VERY POOR prognosis", "Radiation (palliative only); no effective chemotherapy"],
  ]
));

children.push(h2("8.2 Supratentorial Tumours"));
children.push(makeTable(
  ["Tumour", "Key Features"],
  [
    ["Optic pathway glioma", "Associated with NF-1 (50%); visual loss, proptosis"],
    ["Craniopharyngioma", "Suprasellar; bitemporal hemianopia, hypothalamic dysfunction, DI; calcification on CT; Rathke pouch origin"],
    ["Glioblastoma (GBM)", "High grade; very poor prognosis"],
  ]
));

// ═══════════════════════════════════════════════════════════════════════════
// 9. NEUROMUSCULAR DISORDERS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("9. NEUROMUSCULAR DISORDERS"));

children.push(h2("9.1 Approach to the Floppy Infant"));
children.push(makeTable(
  ["Feature", "Central (UMN)", "Peripheral (LMN/Muscle)"],
  [
    ["Tone", "Decreased", "Decreased"],
    ["Power", "Relatively preserved", "Markedly decreased"],
    ["Reflexes", "Normal or increased", "Decreased or absent"],
    ["Alertness", "Decreased (encephalopathic)", "Alert – 'bright eyes'"],
    ["Facial weakness", "Usually absent", "Present (NMJ, myopathy)"],
  ]
));

children.push(h2("9.2 Spinal Muscular Atrophy (SMA) ★★"));
children.push(p("**Genetics:** SMN1 gene deletion (chromosome 5q); most common genetic cause of infant mortality."));
children.push(makeTable(
  ["Type", "Onset", "Maximum Function", "Survival"],
  [
    ["Type 1 (Werdnig-Hoffmann)", "< 6 months", "Never sits", "< 2 years (without treatment)"],
    ["Type 2", "7–18 months", "Sits but never walks", "Reduced (respiratory complications)"],
    ["Type 3 (Kugelberg-Welander)", "> 18 months", "Walks", "Near normal"],
    ["Type 4", "Adult onset", "Walks", "Normal"],
  ]
));
children.push(p("**SMA Type 1 features:** Profound hypotonia, areflexia, paradoxical breathing (intercostal weak, diaphragm spared), **ALERT and bright eyes**, tongue fasciculations, NO sensory loss."));
children.push(h3("Disease-Modifying Treatments ★★"));
children.push(makeTable(
  ["Drug", "Route", "Indication", "Notes"],
  [
    ["Nusinersen (Spinraza)", "Intrathecal injection", "All types; approved", "Antisense oligonucleotide; disease-modifying"],
    ["Onasemnogene abeparvovec (Zolgensma)", "IV, single dose", "< 2 years, < 21 kg", "Gene therapy; potentially curative"],
    ["Risdiplam (Evrysdi)", "Oral", "All ages", "SMN2 splicing modifier"],
  ]
));

children.push(h2("9.3 Duchenne Muscular Dystrophy (DMD) ★★"));
children.push(bullet("**Genetics:** X-linked recessive; dystrophin gene (Xp21); most common frame-shift deletion"));
children.push(bullet("**Incidence:** 1/3,500 male births"));
children.push(bullet("**Clinical:** Proximal muscle weakness age 3–5 years; **Gowers sign**; **calf pseudohypertrophy**"));
children.push(bullet("**CK:** Markedly elevated (50–100× normal)"));
children.push(bullet("**Cardiac:** Dilated cardiomyopathy by adolescence (monitor with echo + ECG)"));
children.push(bullet("**Cognitive:** 1/3 have some intellectual impairment (non-progressive)"));
children.push(bullet("**Diagnosis:** EMG (myopathic), muscle biopsy (absent dystrophin), genetic testing"));
children.push(h3("Treatment"));
children.push(makeTable(
  ["Treatment", "Purpose"],
  [
    ["Deflazacort / prednisolone", "Slow progression, extend ambulation (started once plateau reached ~5–6 yrs)"],
    ["ACE inhibitor + beta-blocker", "Cardiomyopathy management"],
    ["Eteplirsen (exon 51 skip)", "Exon-skipping therapy (~13% patients with specific deletion)"],
    ["Ataluren", "Stop codon readthrough mutations (~10–15%)"],
    ["Physiotherapy + orthotics", "Prevent contractures, maintain function"],
  ]
));

children.push(h2("9.4 Myasthenia Gravis (MG)"));
children.push(bullet("Autoimmune NMJ disorder; anti-AChR antibodies (~85% generalised MG)"));
children.push(bullet("Features: fatigable ptosis + ophthalmoplegia + bulbar weakness; worsens with activity, improves with rest"));
children.push(bullet("**Ice pack test:** Ice on closed ptotic eye × 2 min → improves ptosis"));
children.push(bullet("**Repetitive nerve stimulation:** ≥10% decremental response at 3 Hz"));
children.push(bullet("CT chest: thymic hyperplasia or thymoma"));
children.push(makeTable(
  ["Treatment", "Notes"],
  [
    ["Pyridostigmine", "Symptomatic (anticholinesterase)"],
    ["Prednisolone", "Immunosuppression"],
    ["Azathioprine / mycophenolate", "Steroid-sparing"],
    ["Thymectomy", "AChR+ patients; evidence from MGTX trial"],
    ["Plasma exchange / IVIG", "Myasthenic crisis"],
  ]
));
children.push(p("**Neonatal transient MG:** Passive transfer of maternal anti-AChR antibodies; resolves in 2–8 weeks."));

children.push(h2("9.5 Guillain-Barré Syndrome (GBS)"));
children.push(bullet("Acute inflammatory demyelinating polyradiculoneuropathy"));
children.push(bullet("Post-infectious: Campylobacter jejuni, CMV, EBV, Mycoplasma, COVID-19"));
children.push(bullet("**Ascending flaccid paralysis + areflexia** + mild sensory symptoms; autonomic instability (main cause of death)"));
children.push(bullet("**CSF:** Elevated protein + normal WBC = albuminocytological dissociation (after 1 week)"));
children.push(bullet("**NCS/EMG:** Reduced conduction velocity, prolonged latencies (demyelinating pattern)"));
children.push(bullet("**Treatment:** IVIG 2 g/kg over 2–5 days OR plasma exchange; **steroids NOT effective**"));
children.push(p("**Miller Fisher Syndrome (MFS):** Ataxia + ophthalmoplegia + areflexia; anti-GQ1b antibody; benign prognosis."));

// ═══════════════════════════════════════════════════════════════════════════
// 10. MOVEMENT DISORDERS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("10. MOVEMENT DISORDERS"));

children.push(h2("10.1 Tics and Tourette Syndrome"));
children.push(bullet("**Tourette syndrome:** ≥2 motor tics + ≥1 vocal tic; present > 1 year; onset < 18 years"));
children.push(bullet("**Comorbidities:** ADHD (60%), OCD (40%), anxiety"));
children.push(bullet("Treatment: psychoeducation first; CBIT (comprehensive behavioural intervention); clonidine, guanfacine; haloperidol, aripiprazole, fluphenazine for severe tics"));

children.push(h2("10.2 Sydenham's Chorea (Rheumatic Chorea)"));
children.push(bullet("Post-streptococcal (Group A beta-haemolytic Streptococcus) – part of rheumatic fever"));
children.push(bullet("**Involuntary, purposeless, non-rhythmic movements; 'milkmaid's grip'**; emotional lability, hypotonia, dysarthria"));
children.push(bullet("May occur weeks to months after streptococcal infection (ASOT may be normal by then)"));
children.push(bullet("Always check: echocardiogram for associated carditis"));
children.push(bullet("Treatment: **Penicillin prophylaxis** (secondary prevention); haloperidol or valproate for chorea"));

children.push(h2("10.3 Acute Cerebellar Ataxia"));
children.push(bullet("Most common cause in children: **post-infectious (varicella most common)**"));
children.push(bullet("Other causes: drug toxicity (phenytoin, CBZ), posterior fossa tumour, stroke, MS"));
children.push(bullet("Investigation: MRI brain; metabolic screen if recurrent"));
children.push(p("**Friedreich's Ataxia:** Progressive; GAA repeat in frataxin gene; spinocerebellar degeneration + peripheral neuropathy + hypertrophic cardiomyopathy; pes cavus; onset < 25 years."));

// ═══════════════════════════════════════════════════════════════════════════
// 11. NEUROCUTANEOUS SYNDROMES
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("11. NEUROCUTANEOUS SYNDROMES (PHAKOMATOSES)"));

children.push(h2("11.1 Neurofibromatosis Type 1 (NF1)"));
children.push(p("**Genetics:** Autosomal dominant; NF1 gene (chromosome 17q) – encodes neurofibromin (tumour suppressor). Diagnosis: ≥2 of 7 criteria:"));
children.push(makeTable(
  ["#", "Criterion"],
  [
    ["1", "≥6 café-au-lait spots (> 5 mm prepubertal, > 15 mm postpubertal)"],
    ["2", "≥2 neurofibromas OR 1 plexiform neurofibroma"],
    ["3", "Axillary or inguinal freckling (Crowe's sign)"],
    ["4", "Optic glioma"],
    ["5", "≥2 Lisch nodules (iris hamartomas)"],
    ["6", "Sphenoid wing dysplasia or cortical thinning of long bone"],
    ["7", "First-degree relative with NF1"],
  ]
));

children.push(h2("11.2 Neurofibromatosis Type 2 (NF2)"));
children.push(bullet("**Bilateral acoustic neuromas (vestibular schwannomas)** – pathognomonic"));
children.push(bullet("Also: meningiomas, ependymomas, cortical lens opacities"));
children.push(bullet("NF2 gene (chromosome 22) – encodes merlin"));

children.push(h2("11.3 Tuberous Sclerosis Complex (TSC) ★★"));
children.push(p("**Genetics:** TSC1 (hamartin, chr 9) or TSC2 (tuberin, chr 16); autosomal dominant; de novo mutations common."));
children.push(h3("Key Skin Features (Exam Favourite)"));
children.push(makeTable(
  ["Lesion", "Notes"],
  [
    ["Hypomelanotic macules (ash-leaf spots)", "Earliest finding; best seen with Wood's lamp"],
    ["Facial angiofibromas ('adenoma sebaceum')", "Appear after age 2–5"],
    ["Shagreen patches", "Lumbar/gluteal region; cobblestone texture"],
    ["Ungual/periungual fibromas", "Appear in adolescence"],
    ["Confetti lesions", "Multiple tiny hypopigmented macules"],
  ]
));
children.push(p("**Neurological:** Epilepsy (80–90%); often begins as infantile spasms – treat with **vigabatrin** (1st line). Intellectual disability, ASD."));
children.push(p("**Other organs:** Cardiac rhabdomyomas (fetal/neonatal), renal angiomyolipomas, pulmonary LAM, SEGA (subependymal giant cell astrocytoma – monitor with MRI)."));

children.push(h2("11.4 Sturge-Weber Syndrome"));
children.push(bullet("Port-wine stain (V1 trigeminal distribution) + ipsilateral leptomeningeal angioma"));
children.push(bullet("CT: 'tram-track' (tramline) calcification (gyral)"));
children.push(bullet("Complications: refractory epilepsy, hemiparesis, glaucoma"));
children.push(bullet("Non-hereditary (somatic GNAQ mutation)"));

// ═══════════════════════════════════════════════════════════════════════════
// 12. STROKE
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("12. STROKE IN CHILDREN"));

children.push(h2("12.1 Ischaemic Stroke – Risk Factors"));
children.push(makeTable(
  ["Category", "Examples"],
  [
    ["Cardiac", "Congenital heart disease (most common in neonates/infants)"],
    ["Haematological", "Sickle cell disease (risk 300× normal; most common in older children), coagulopathies (Factor V Leiden, protein C/S deficiency, APLA)"],
    ["Arteriopathy", "Moyamoya, arterial dissection, vasculitis"],
    ["Metabolic", "MELAS, homocystinuria"],
  ]
));
children.push(bullet("**Presentation:** Acute hemiplegia (most common), focal seizures, speech disturbance"));
children.push(bullet("**Investigations:** MRI DWI (gold standard acutely), MRA, echo, thrombophilia screen, sickle cell screen"));
children.push(bullet("**Sickle cell:** Exchange transfusion (acute); chronic transfusion programme (secondary prevention)"));

children.push(h2("12.2 Haemorrhagic Stroke"));
children.push(bullet("AVMs (arteriovenous malformations) = most common cause of intracranial haemorrhage in children"));
children.push(bullet("Presentation: sudden severe headache, seizures, focal deficit"));

// ═══════════════════════════════════════════════════════════════════════════
// 13. NEUROLOGY OF THE NEWBORN
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("13. NEUROLOGY OF THE NEWBORN"));

children.push(h2("13.1 Hypoxic-Ischaemic Encephalopathy (HIE)"));
children.push(h3("Sarnat Classification"));
children.push(makeTable(
  ["Grade", "Consciousness", "Tone", "Seizures", "EEG", "Prognosis"],
  [
    ["Mild (I)", "Hyperalert, irritable", "Normal/hypertonicity", "None", "Normal", "Excellent"],
    ["Moderate (II)", "Lethargic, obtunded", "Hypotonia", "Common", "Low voltage delta/theta", "Variable"],
    ["Severe (III)", "Stupor/coma", "Flaccid", "Frequent/status", "Burst suppression or flat", "Poor"],
  ]
));

children.push(h3("Therapeutic Hypothermia ★★ EXAM ESSENTIAL"));
children.push(p("**Eligibility criteria (ALL of the following):**"));
children.push(bullet("GA ≥ 36 weeks"));
children.push(bullet("At least ONE of: Apgar ≤ 5 at 10 min; resuscitation > 10 min; pH < 7.0; base deficit ≥ 16; or clinical encephalopathy (Sarnat ≥ 2)"));
children.push(p("**Protocol:**"));
children.push(bullet("Target: 33–34°C core temperature for **72 hours**, then slow rewarming over 6 hours"));
children.push(bullet("Must start **within 6 hours** of birth"));
children.push(bullet("Reduces death or disability by ~25–30%"));

children.push(h2("13.2 Neonatal Seizures"));
children.push(makeTable(
  ["Type", "Features"],
  [
    ["Subtle (most common in preterm)", "Ocular deviation, bicycling, apnoea, blinking"],
    ["Clonic (most common in term)", "Rhythmic jerking; often focal"],
    ["Tonic", "Sustained posturing; brainstem release in severe HIE"],
    ["Myoclonic", "Rapid brief jerks; ominous if generalised"],
  ]
));
children.push(bullet("**Investigations:** BGL, electrolytes (Ca, Mg, Na), blood culture/LP, metabolic screen, EEG (most sensitive), cranial USS/MRI"));
children.push(bullet("**Treatment:** Phenobarbital 20 mg/kg load (1st line); levetiracetam increasingly used; pyridoxine 100 mg IV if refractory (< 2 years)"));

// ═══════════════════════════════════════════════════════════════════════════
// 14. NEURAL TUBE DEFECTS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("14. SPINAL CORD AND NEURAL TUBE DEFECTS"));

children.push(p("**Prevention:** Folic acid 400 mcg/day periconceptionally (4–5 mg/day if prior NTD, on antiepileptics, obesity, or diabetes)"));
children.push(makeTable(
  ["Defect", "Description", "Features"],
  [
    ["Spina bifida occulta", "Vertebral defect, no sac, skin intact", "Usually asymptomatic; tuft of hair, dimple, or lipoma may mark site"],
    ["Meningocele", "CSF-filled sac; cord not involved", "Usually no neurological deficit"],
    ["Myelomeningocele", "Cord/nerve roots in sac", "Lower limb paralysis, bowel/bladder dysfunction; hydrocephalus (Chiari II association)"],
    ["Anencephaly", "Absence of cranial vault", "Incompatible with sustained life"],
  ]
));
children.push(p("**Chiari II malformation:** Always associated with myelomeningocele; downward herniation of cerebellar tonsils; may cause hydrocephalus, lower CN palsies, respiratory difficulties."));

// ═══════════════════════════════════════════════════════════════════════════
// 15. NEUROLOGICAL INVESTIGATIONS
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("15. NEUROLOGICAL INVESTIGATIONS"));

children.push(h2("15.1 EEG Patterns – HIGH YIELD TABLE ★★"));
children.push(makeTable(
  ["EEG Pattern", "Associated Condition"],
  [
    ["3 Hz generalised spike-wave", "Childhood absence epilepsy"],
    ["Hypsarrhythmia", "West syndrome (infantile spasms)"],
    ["Slow spike-wave (< 2.5 Hz)", "Lennox-Gastaut syndrome"],
    ["Centrotemporal spikes (sleep-activated)", "BECTS / Rolandic epilepsy"],
    ["Burst-suppression", "Neonatal encephalopathy; Ohtahara syndrome"],
    ["PLEDs (periodic lateralising epileptiform discharges)", "HSV encephalitis; stroke"],
    ["4–6 Hz polyspike-wave", "Juvenile myoclonic epilepsy (JME)"],
    ["Photo-paroxysmal response", "JME; photosensitive epilepsy"],
    ["Theta pointu alternant", "Benign neonatal seizures ('fifth day fits')"],
  ]
));

children.push(h2("15.2 Neuroimaging Indications (Urgent CT/MRI)"));
children.push(bullet("Papilloedema"));
children.push(bullet("Focal neurological deficit"));
children.push(bullet("Seizure with Todd's palsy"));
children.push(bullet("New-onset headache + ataxia"));
children.push(bullet("Depressed consciousness"));
children.push(bullet("Focal seizure in immunocompromised child"));

children.push(h2("15.3 Key MRI Sequences"));
children.push(makeTable(
  ["Sequence", "Best For"],
  [
    ["DWI (diffusion-weighted)", "Acute ischaemia (bright within minutes of onset)"],
    ["FLAIR", "Inflammation, demyelination, encephalitis"],
    ["T1 + gadolinium", "Tumours, abscess, meningeal enhancement"],
    ["MRS (spectroscopy)", "Metabolic disorders, tumour characterisation"],
    ["MRA", "Vascular anomalies, moyamoya, AVM"],
  ]
));

// ═══════════════════════════════════════════════════════════════════════════
// 16. DEMYELINATING DISEASES
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("16. DEMYELINATING DISEASES"));

children.push(h2("16.1 Paediatric Multiple Sclerosis"));
children.push(bullet("< 5% of MS cases present in childhood; female:male 1:1 prepubertal, 3:1 adolescence"));
children.push(bullet("**McDonald criteria:** Dissemination in space AND time"));
children.push(bullet("**Optic neuritis:** Painful visual loss, RAPD (relative afferent pupillary defect), unilateral"));
children.push(bullet("MRI: periventricular white matter lesions – **'Dawson fingers'** (perpendicular to corpus callosum)"));
children.push(bullet("CSF: oligoclonal bands, elevated IgG index"));
children.push(bullet("Acute treatment: methylprednisolone IV; DMTs: interferon-beta, glatiramer, natalizumab, ocrelizumab"));

children.push(h2("16.2 ADEM (Acute Disseminated Encephalomyelitis)"));
children.push(bullet("Post-infectious or post-vaccination; multifocal demyelination"));
children.push(bullet("**Encephalopathy + multifocal neurological deficits** (distinguishes from MS)"));
children.push(bullet("MRI: widespread, bilateral, asymmetric white matter lesions + cortical/deep grey matter involvement"));
children.push(bullet("Treatment: high-dose IV methylprednisolone; IVIG or plasma exchange if refractory"));
children.push(bullet("Usually monophasic; good prognosis"));

// ═══════════════════════════════════════════════════════════════════════════
// 17. QUICK REFERENCE TABLE
// ═══════════════════════════════════════════════════════════════════════════
children.push(pageBreak(), h1("17. QUICK REFERENCE – HIGH-YIELD EXAM FACTS ★★★"));

children.push(makeTable(
  ["Topic", "Key Point"],
  [
    [{text:"Febrile seizure", bold:true}, "No LP if > 18 months, simple FS, well, fully immunised, no meningism"],
    [{text:"Infantile spasms EEG", bold:true}, "Hypsarrhythmia"],
    [{text:"West syndrome treatment", bold:true}, "ACTH (1st line); vigabatrin if tuberous sclerosis"],
    [{text:"Dravet treatment", bold:true}, "Valproate + clobazam; AVOID sodium channel blockers"],
    [{text:"Childhood absence EEG", bold:true}, "3 Hz generalised spike-wave; precipitated by hyperventilation"],
    [{text:"BECTS", bold:true}, "Most common childhood epilepsy syndrome; self-limiting"],
    [{text:"JME", bold:true}, "Lifelong treatment usually needed; avoid valproate in girls"],
    [{text:"Status epilepticus 1st line", bold:true}, "Lorazepam IV or midazolam IM"],
    [{text:"Status epilepticus 2nd line", bold:true}, "Levetiracetam IV, valproate IV, or fosphenytoin"],
    [{text:"SMA Type 1", bold:true}, "Never sits; bright eyes; SMN1 deletion; nusinersen/gene therapy"],
    [{text:"DMD", bold:true}, "Xp21; CK very high; Gowers sign; calf pseudohypertrophy; deflazacort"],
    [{text:"GBS CSF", bold:true}, "Albuminocytological dissociation (high protein, normal WBC)"],
    [{text:"GBS treatment", bold:true}, "IVIG or plasma exchange; steroids NOT effective"],
    [{text:"NF1 diagnosis", bold:true}, "≥2 of 7 criteria; ≥6 café-au-lait spots most common feature"],
    [{text:"TSC skin", bold:true}, "Ash-leaf spots (earliest, Wood's lamp), angiofibromas, shagreen patch"],
    [{text:"HIE hypothermia", bold:true}, "GA ≥36 wks; start within 6 hrs; 33–34°C × 72 hrs"],
    [{text:"TBM stages", bold:true}, "Alert → CN palsy + meningism → coma"],
    [{text:"NMDAR encephalitis", bold:true}, "Psychiatric → seizures → orofacial dyskinesias; anti-NR1 antibody"],
    [{text:"Medulloblastoma", bold:true}, "Most common malignant paediatric brain tumour; 4th ventricle; drop mets"],
    [{text:"Cerebellar astrocytoma", bold:true}, "Cystic + mural nodule; pilocytic; excellent prognosis"],
    [{text:"DIPG", bold:true}, "H3K27M mutation; pons; no effective chemo; very poor prognosis"],
    [{text:"Craniopharyngioma", bold:true}, "Suprasellar calcification; bitemporal hemianopia; hypothalamic dysfunction"],
    [{text:"HLA-B*1502 (Asian/Sri Lanka)", bold:true}, "Screen before CBZ, phenytoin, lamotrigine – high risk SJS/TEN"],
    [{text:"Pseudotumour cerebri", bold:true}, "Obesity, vit A, tetracycline; treat with acetazolamide + weight loss"],
    [{text:"Sydenham's chorea", bold:true}, "Post-strep; milkmaid's grip; penicillin prophylaxis; echo for carditis"],
    [{text:"Parachute reflex", bold:true}, "Appears 8–9 months; persists (protective reflex)"],
    [{text:"Setting-sun sign", bold:true}, "Parinaud sign = dorsal midbrain compression by hydrocephalus"],
    [{text:"Neonatal myasthenia", bold:true}, "Passive maternal anti-AChR transfer; resolves 2–8 weeks"],
    [{text:"Folic acid dose NTD prevention", bold:true}, "400 mcg/day standard; 4–5 mg/day if high risk"],
    [{text:"Sickle cell stroke", bold:true}, "Exchange transfusion acute; chronic transfusion for secondary prevention"],
  ]
));

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"""
Paediatric Neurology MCQ & SBA Question Bank
PGIM Sri Lanka – MD Paediatrics PG Selection Examination
Topics: Neurological Exam, Seizures/Epilepsy, Headache (first 10 pages of notes)
Difficulty: Hard to Extreme
Author: Professor of Paediatrics, Faculty of Medicine, University of Colombo
"""

from docx import Document
from docx.shared import Pt, Inches, RGBColor
from docx.enum.text import WD_ALIGN_PARAGRAPH
from docx.oxml.ns import qn
from docx.oxml import OxmlElement
import copy

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# ══════════════════════════════════════════════════════════════════════════
# QUESTION DATA
# Format for MCQ: {"type":"MCQ","section":"...","q":"...","opts":["A...","B...","C...","D...","E..."],"ans":"X","exp":"..."}
# Format for SBA: {"type":"SBA","section":"...","stem":"...","q":"...","opts":[...],"ans":"X","exp":"..."}
# ══════════════════════════════════════════════════════════════════════════

questions = [

# ══════════════════════════════════════════════════════════════════════════
# SECTION A – NEUROLOGICAL EXAMINATION & DEVELOPMENT
# ══════════════════════════════════════════════════════════════════════════

{
"type":"MCQ","section":"A",
"q": "A 7-month-old infant demonstrates persistent asymmetric tonic neck reflex (ATNR) on both sides. Which of the following statements is MOST accurate regarding this finding?",
"opts":[
"A. ATNR normally persists until 12 months and this finding is reassuring",
"B. Persistence of ATNR beyond 6 months is a significant neurological red flag",
"C. ATNR normally persists until 9 months and this is within normal limits",
"D. Persistence of ATNR is only significant if associated with macrocephaly",
"E. ATNR should be present at 7 months as it appears at 6 months"
],
"ans":"B",
"exp":"ATNR normally disappears by 4–6 months. Persistence beyond 6 months is a significant red flag suggesting upper motor neuron pathology such as cerebral palsy. Persistent primitive reflexes after their expected disappearance impede normal motor development."
},

{
"type":"MCQ","section":"A",
"q": "An 18-month-old child consistently uses only the right hand for reaching and manipulation. His parents are not concerned. What is the MOST important next step?",
"opts":[
"A. Reassure parents as hand preference is normal by 18 months",
"B. Refer for occupational therapy assessment for hand strengthening",
"C. Investigate for left hemiplegia as hand dominance before 18 months is abnormal",
"D. Order brain MRI only if tone is reduced in the left limbs",
"E. Monitor at 2-year review as handedness emerges between 18–24 months"
],
"ans":"C",
"exp":"Hand dominance before 18 months is abnormal and suggests contralateral (right hemisphere) pathology. This is a red flag for left-sided hemiplegia. Normal hand preference does not emerge until after 18 months. Urgent investigation including MRI brain and full neurological examination is warranted."
},

{
"type":"SBA","section":"A",
"stem":"A 9-month-old girl is referred for developmental assessment. She cannot sit unsupported, has no pincer grasp, and her parents report she was making cooing sounds at 3 months but has stopped vocalising. On examination, tone is mildly increased in all four limbs, deep tendon reflexes are brisk, and the Moro reflex is still present. She makes no eye contact.",
"q":"Which of the following developmental red flags present in this child is the MOST ominous prognostically?",
"opts":[
"A. Absent pincer grasp at 9 months",
"B. Inability to sit unsupported at 9 months",
"C. Regression of vocalisation with absent eye contact",
"D. Persistence of Moro reflex at 9 months",
"E. Increased tone with brisk reflexes at 9 months"
],
"ans":"C",
"exp":"Regression of previously acquired skills at ANY age is the most ominous red flag in paediatric neurodevelopment. Combined with absent social engagement (no eye contact), this suggests a progressive or regressive neurological condition (e.g., metabolic disorder, Rett syndrome, lysosomal storage disease). All other findings are significant but loss of milestones always takes priority in triage."
},

{
"type":"MCQ","section":"A",
"q": "The parachute reflex (parachute response) in a normal infant: which of the following is CORRECT?",
"opts":[
"A. Is present from birth and disappears at 6 months",
"B. Appears at 4–5 months and disappears at 12 months",
"C. Appears at 8–9 months and persists indefinitely",
"D. Absence at 12 months is always pathological and requires MRI",
"E. Is a primitive reflex that disappears like other primitive reflexes by 6 months"
],
"ans":"C",
"exp":"The parachute reflex is a postural protective reflex that appears at 8–9 months and persists throughout life. Unlike primitive reflexes (Moro, ATNR, palmar grasp) which disappear, the parachute reflex is a later-appearing protective response. Its absence at 10–12 months is abnormal and may indicate cerebral palsy or corticospinal tract pathology."
},

{
"type":"SBA","section":"A",
"stem":"During a developmental assessment, a 2-year-old boy is observed. He runs well, kicks a ball, and goes up stairs holding the rail with one hand. He stacks 5 cubes and can copy a vertical line. He has 30 words and uses 2-word phrases. He points to pictures and follows 2-step commands.",
"q":"Based on Nelson's developmental milestones, which aspect of this child's development is BELOW the expected level for his age?",
"opts":[
"A. Gross motor – going up stairs",
"B. Fine motor – stacking 5 cubes",
"C. Language – 30 words with 2-word phrases",
"D. Fine motor – copying a vertical line",
"E. Gross motor – running and kicking a ball"
],
"ans":"B",
"exp":"By 2 years, a child should stack 6 cubes. Stacking only 5 is slightly below the expected level. All other achievements described are consistent with 24-month milestones: running and kicking (gross motor), copying a vertical line (fine motor), 30+ words with 2-word phrases (language). Going up stairs one foot at a time with support is also appropriate at 24 months."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION B – SEIZURE CLASSIFICATION & FEBRILE SEIZURES
# ══════════════════════════════════════════════════════════════════════════

{
"type":"MCQ","section":"B",
"q": "According to the ILAE 2017 operational classification of seizures, a seizure previously described as 'complex partial seizure' would now be correctly termed:",
"opts":[
"A. Focal aware seizure with motor onset",
"B. Focal impaired awareness seizure",
"C. Generalised onset non-motor seizure",
"D. Focal to bilateral tonic-clonic seizure",
"E. Unknown onset seizure with impaired awareness"
],
"ans":"B",
"exp":"The 2017 ILAE classification replaced outdated terminology. 'Complex partial seizure' (seizure with altered consciousness arising from one hemisphere) is now 'focal seizure with impaired awareness'. 'Simple partial seizure' (no alteration of consciousness) is now 'focal aware seizure'. This distinction is based on the patient's level of awareness, not motor involvement."
},

{
"type":"MCQ","section":"B",
"q": "A 14-month-old boy presents with a 2-minute generalised tonic-clonic seizure associated with a temperature of 38.9°C. He is fully immunised, looks well after the seizure, has no meningeal signs and a normal neurological examination. His parents report this is his first seizure. Regarding further management, which of the following is CORRECT according to current guidelines?",
"opts":[
"A. Lumbar puncture is mandatory given his age is under 18 months",
"B. EEG should be performed to risk-stratify future epilepsy development",
"C. MRI brain is indicated to exclude structural abnormality",
"D. No LP, EEG, neuroimaging or antiseizure medication is necessary",
"E. Prophylactic diazepam should be prescribed for future febrile illnesses"
],
"ans":"D",
"exp":"This is a classic simple febrile seizure (age 6–60 months, generalised, <15 minutes, not recurrent in 24 hours). Per AAP and Harriet Lane guidelines, no LP, EEG, neuroimaging or antiseizure medications are required for a well-appearing, fully immunised child with normal neurological exam and no meningeal signs. LP is 'considered' (not mandatory) at 12–18 months. Prophylactic anticonvulsants do not alter the risk of epilepsy development and are not recommended."
},

{
"type":"SBA","section":"B",
"stem":"A 10-month-old girl presents with a 25-minute right-sided clonic seizure associated with a temperature of 38.2°C. She has had no prior seizures. After seizure termination with IV lorazepam, examination reveals left-sided facial weakness and left arm hypotonia. This resolves completely over the next 2 hours. She is fully immunised and her CSF shows 3 white cells/mm³, protein 0.28 g/L and glucose 3.1 mmol/L (serum glucose 5.2 mmol/L).",
"q":"Which of the following statements about this child's seizure and subsequent management is MOST accurate?",
"opts":[
"A. This is a simple febrile seizure and requires no further investigation",
"B. The post-ictal focal deficit (Todd's paresis) excludes a diagnosis of febrile seizure",
"C. This is a complex febrile seizure; MRI brain is indicated to exclude underlying structural pathology",
"D. CSF pleocytosis confirms bacterial meningitis requiring IV antibiotics",
"E. The risk of developing epilepsy is the same as for a simple febrile seizure (1–2%)"
],
"ans":"C",
"exp":"This is a COMPLEX febrile seizure on three counts: focal onset (right-sided clonic), duration >15 minutes, and post-ictal Todd's paresis. Complex febrile seizures carry an epilepsy risk of 4–15% (vs ~1–2% for simple). MRI brain is indicated to exclude structural pathology such as focal cortical dysplasia, mesial temporal sclerosis, or cortical malformation. The CSF is normal (3 WBC, normal protein and glucose ratio 0.60 – normal). Todd's paresis is a recognised post-ictal phenomenon and does not exclude febrile seizure but flags it as complex."
},

{
"type":"MCQ","section":"B",
"q": "Regarding recurrence risk after a first febrile seizure, which combination of risk factors carries the HIGHEST recurrence risk?",
"opts":[
"A. Age 36 months at first seizure, temperature 40.1°C, family history negative",
"B. Age 9 months at first seizure, temperature 38.2°C, positive family history of febrile seizures",
"C. Age 24 months, complex febrile seizure, temperature 39.5°C",
"D. Age 18 months, simple febrile seizure, temperature 40.5°C, family history negative",
"E. Age 48 months, first febrile seizure, temperature 38.8°C"
],
"ans":"B",
"exp":"The four established risk factors for febrile seizure recurrence are: (1) age <12 months at first febrile seizure, (2) low-grade fever at time of seizure, (3) positive family history of febrile seizures, (4) brief duration of fever before seizure onset. Option B has two strong risk factors: age <12 months AND positive family history AND low-grade fever (38.2°C). With 2 risk factors, recurrence risk approaches ~50%. Age alone <12 months is the single most important predictor."
},

{
"type":"SBA","section":"B",
"stem":"You are asked to see a 7-year-old girl admitted after her third seizure in 2 years. Each seizure has occurred during sleep. She describes a strange sensation on the left side of her mouth, then rhythmic twitching of the left cheek and drooling lasting 1–2 minutes. On one occasion the twitching spread to the left arm and she had a generalised convulsion. She is developing normally, is top of her class, and has no family history of epilepsy. Neurological examination is normal.",
"q":"The EEG is most likely to show which of the following patterns?",
"opts":[
"A. 3 Hz generalised spike-and-wave, enhanced by hyperventilation",
"B. Hypsarrhythmia with high-amplitude irregular slow waves",
"C. Centrotemporal spikes, markedly activated by sleep",
"D. Slow spike-wave complexes at <2.5 Hz with generalised background slowing",
"E. 4–6 Hz polyspike-wave bursts, activated by intermittent photic stimulation"
],
"ans":"C",
"exp":"This is a classic description of BECTS (Benign Epilepsy with Centrotemporal Spikes / Rolandic epilepsy) – the most common childhood epilepsy syndrome. Features: nocturnal, oro-facial-pharyngeal semiological symptoms (drooling, facial twitching, speech arrest), child remains conscious, secondary generalisation can occur. EEG shows centrotemporal (rolandic) spikes, dramatically activated by sleep. Normal development and intelligence, self-limiting before age 15–16."
},

{
"type":"MCQ","section":"B",
"q": "A 5-year-old girl is brought to clinic because her teacher notices she 'blanks out' 15–20 times a day for 5–10 seconds. She has no warning and immediately returns to normal activity. During the consultation, you ask her to blow on a windmill for 3 minutes, after which you observe a 7-second staring episode with subtle lip smacking. Which drug is FIRST LINE for this condition with the BEST evidence and tolerability profile in children?",
"opts":[
"A. Valproate – effective for all generalised seizure types",
"B. Lamotrigine – best tolerated and most effective in childhood absence",
"C. Ethosuximide – best evidence and tolerability for childhood absence epilepsy",
"D. Levetiracetam – broad spectrum and safest in children",
"E. Topiramate – effective for generalised epilepsies with weight benefit"
],
"ans":"C",
"exp":"Childhood absence epilepsy (CAE) with 3 Hz spike-wave on EEG, provoked by hyperventilation. A landmark RCT (Glauser et al., NEJM 2010, CTAD study) showed ethosuximide superior to valproate and lamotrigine as first-line for CAE, with better attentional outcomes and equivalent seizure freedom to valproate. Lamotrigine is significantly less effective. Valproate is equally efficacious to ethosuximide but has more adverse effects. Ethosuximide is ONLY effective for absence seizures – it has no effect on other seizure types."
},

{
"type":"SBA","section":"B",
"stem":"A 6-month-old boy is brought by his parents who have noticed episodes of sudden brief flexion of the trunk and bilateral arm elevation, occurring in clusters of 10–15 events each morning on waking. He had normal development until 4 months but has recently become less interactive and has lost his social smile. He was born at term following an uncomplicated delivery. Head circumference is on the 50th centile. There are no skin lesions. EEG shows high-amplitude chaotic slow waves mixed with multifocal spikes.",
"q":"Which of the following represents the MOST appropriate FIRST-LINE treatment for this child?",
"opts":[
"A. Carbamazepine 10 mg/kg/day in divided doses",
"B. Phenobarbital 5 mg/kg/day",
"C. ACTH (tetracosactide) or vigabatrin depending on aetiology",
"D. Valproate monotherapy as first-line for generalised epilepsy",
"E. Levetiracetam IV loading dose followed by oral maintenance"
],
"ans":"C",
"exp":"This is West syndrome (infantile spasms): epileptic spasms in clusters + hypsarrhythmia on EEG + developmental regression. ACTH is first-line in most centres (UK Child Neurology Society) and vigabatrin is first-line specifically when tuberous sclerosis is the aetiology. In idiopathic/cryptogenic cases, ACTH or vigabatrin (or combination) are recommended. Carbamazepine is contraindicated as it can worsen spasms. Phenobarbital is not effective for infantile spasms. The cryptogenic (no skin lesions, normal HC, term birth) vs structural distinction here suggests ACTH as preferred first-line."
},

{
"type":"MCQ","section":"B",
"q": "A 7-month-old boy presents with fever-triggered right-sided prolonged hemiclonic seizures. His first seizure occurred at 5 months during a febrile illness and lasted 45 minutes. Genetic testing reveals a heterozygous loss-of-function variant in SCN1A. Which of the following antiseizure medications is CONTRAINDICATED in this child and may worsen his epilepsy?",
"opts":[
"A. Valproate",
"B. Clobazam",
"C. Topiramate",
"D. Carbamazepine",
"E. Stiripentol"
],
"ans":"D",
"exp":"This is Dravet syndrome (SCN1A mutation). Dravet syndrome is caused by loss-of-function of Nav1.1 sodium channels (predominantly in inhibitory interneurons). Sodium channel blockers (carbamazepine, phenytoin, lamotrigine, oxcarbazepine) WORSEN Dravet syndrome by further reducing sodium channel activity in inhibitory interneurons. The treatment is valproate + clobazam as backbone, with add-ons including topiramate, stiripentol, cannabidiol (Epidiolex) and fenfluramine. This is a critical prescribing safety issue for Asian populations including Sri Lanka."
},

{
"type":"SBA","section":"B",
"stem":"A 14-year-old boy is referred with a 2-year history of early morning jerking of both hands, causing him to drop his toothbrush and occasionally spill drinks. He has had 3 generalised tonic-clonic seizures, all in the morning, often after late nights. His parents report he also has brief staring episodes. Neurological examination is normal. EEG shows 4–6 Hz polyspike-wave bursts, enhanced by photic stimulation. MRI brain is normal.",
"q":"Regarding long-term management of this condition, which statement is MOST accurate?",
"opts":[
"A. He is likely to outgrow this condition and can be weaned off medication after 2 seizure-free years",
"B. Lamotrigine is the drug of choice as it avoids the teratogenicity concerns of valproate",
"C. He will likely require lifelong treatment as relapse rates after stopping medication are very high",
"D. Carbamazepine is effective monotherapy for his generalised seizures",
"E. Sleep deprivation and alcohol are not relevant triggers once medication is established"
],
"ans":"C",
"exp":"This is Juvenile Myoclonic Epilepsy (JME) – morning myoclonic jerks + GTCS + occasional absence, 4–6 Hz polyspike-wave, photosensitivity. Unlike CAE and BECTS, JME is a LIFELONG condition. Relapse rates after withdrawing medication are >80–90%, far higher than most other epilepsy syndromes. Valproate is the most effective drug; lamotrigine is an alternative in girls of childbearing age but is somewhat less effective. Carbamazepine can worsen myoclonic seizures in JME. Sleep hygiene and alcohol avoidance remain critically important triggers regardless of medication."
},

{
"type":"MCQ","section":"B",
"q": "Regarding the Lennox-Gastaut syndrome (LGS), which of the following EEG and clinical combinations is PATHOGNOMONIC?",
"opts":[
"A. 3 Hz spike-wave + absence seizures + normal development",
"B. Hypsarrhythmia + flexion spasms + developmental plateau",
"C. Slow (<2.5 Hz) spike-wave + multiple seizure types including tonic + intellectual disability",
"D. Centrotemporal spikes + nocturnal focal motor seizures + normal intelligence",
"E. Polyspike-wave 4–6 Hz + myoclonic jerks + photosensitivity"
],
"ans":"C",
"exp":"Lennox-Gastaut syndrome is characterised by the triad of: (1) multiple seizure types (tonic seizures are hallmark, also atonic 'drop attacks', atypical absence, myoclonic), (2) diffuse slow spike-wave <2.5 Hz on EEG (also ESES during sleep), and (3) intellectual disability/cognitive impairment. This differs from West syndrome (hypsarrhythmia, infantile spasms) and JME (polyspike-wave, myoclonic, adolescent onset) and CAE (3 Hz, pure absence, normal intellect)."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION C – STATUS EPILEPTICUS
# ══════════════════════════════════════════════════════════════════════════

{
"type":"SBA","section":"C",
"stem":"A 3-year-old boy has been convulsing for 18 minutes. He arrived in the emergency department still seizing. IV access was established immediately. He received IV lorazepam 0.1 mg/kg 8 minutes ago with no effect, and a repeat dose of IV lorazepam 0.05 mg/kg 3 minutes ago. He continues to seize. His weight is 14 kg. Oxygen saturations are 96% on 15L O2, heart rate 145/min, BP 98/62 mmHg. Blood glucose is 6.1 mmol/L.",
"q":"Which is the MOST appropriate next step?",
"opts":[
"A. Administer rectal diazepam 0.5 mg/kg as third-line benzodiazepine",
"B. Administer IV levetiracetam 40 mg/kg over 5–10 minutes",
"C. Proceed immediately to rapid sequence intubation and propofol infusion",
"D. Administer IV sodium valproate 10 mg/kg and repeat benzodiazepine in 5 minutes",
"E. Administer pyridoxine 100 mg IV and observe for 5 minutes"
],
"ans":"B",
"exp":"This child has received two doses of benzodiazepine (Phase 1 complete, 5–20 minutes) with no response. He is now in Phase 2 (second-line agent, 20–40 minutes). IV levetiracetam 20–60 mg/kg (typically 40 mg/kg loading dose) is now recommended as first choice in Phase 2 at many centres due to its superior safety profile, lack of respiratory depression, and IV availability. RSI is reserved for refractory SE (Phase 3, >40 minutes despite 2 phases of treatment). A third benzodiazepine is not indicated. Pyridoxine is considered in infants <2 years with refractory SE; this child is 3 years old but could be given empirically in refractory cases."
},

{
"type":"MCQ","section":"C",
"q": "A 2-year-old girl presents in status epilepticus. No IV access can be established. Which of the following is the MOST appropriate initial treatment?",
"opts":[
"A. Sublingual midazolam 0.3 mg/kg",
"B. Rectal diazepam 0.5 mg/kg",
"C. Intramuscular midazolam 0.2 mg/kg",
"D. Intranasal lorazepam 0.1 mg/kg",
"E. Intraosseous lorazepam 0.1 mg/kg after IO access"
],
"ans":"C",
"exp":"When IV access is unavailable, IM midazolam 0.1–0.2 mg/kg is the preferred first-line route for prehospital and emergency treatment. Multiple randomised trials (including RAMPART) have demonstrated IM midazolam to be at least as effective as IV lorazepam for status epilepticus and faster to administer without IV access. Rectal diazepam is an alternative but has more variable absorption and is less socially acceptable. IM midazolam has become the standard of care."
},

{
"type":"SBA","section":"C",
"stem":"A 20-month-old girl presents with a prolonged febrile convulsion lasting 35 minutes, terminated with IV lorazepam and IV levetiracetam. She is now post-ictal but maintaining her airway. On review of her history, her mother has been on phenytoin for epilepsy throughout the pregnancy. The seizure has been refractory to two further treatment attempts. A colleague suggests giving pyridoxine.",
"q":"What is the RATIONALE for empirical pyridoxine administration in this clinical scenario?",
"opts":[
"A. Phenytoin in breast milk causes pyridoxine deficiency in infants",
"B. Pyridoxine deficiency is a cause of refractory neonatal and infantile seizures due to deficiency of pyridoxal phosphate as GABA co-factor",
"C. Pyridoxine reverses the sodium channel blocking effect of phenytoin toxicity",
"D. Pyridoxine corrects mitochondrial dysfunction caused by valproate toxicity",
"E. Phenytoin is an antagonist at pyridoxal phosphate-dependent enzyme pathways causing infantile spasms"
],
"ans":"B",
"exp":"Pyridoxine-dependent epilepsy (ALDH7A1 mutation) presents with refractory seizures in neonates and infants. Pyridoxal phosphate (active form of B6) is an essential co-factor for glutamic acid decarboxylase (GAD), the enzyme that converts glutamate to GABA. Deficiency leads to GABA depletion and seizures. Empirical pyridoxine 100 mg IV is recommended for any infant <2 years with unexplained refractory status epilepticus. Response (seizure cessation within minutes on EEG) confirms the diagnosis. This is a treatable cause that must not be missed."
},

{
"type":"MCQ","section":"C",
"q": "Which of the following antiseizure medications used in status epilepticus carries a specific risk of worsening seizures in Dravet syndrome AND causing cardiac arrhythmia if administered too rapidly IV?",
"opts":[
"A. Levetiracetam",
"B. Valproate",
"C. Phenytoin/fosphenytoin",
"D. Phenobarbital",
"E. Midazolam"
],
"ans":"C",
"exp":"Phenytoin/fosphenytoin is a sodium channel blocker that is contraindicated or should be used with extreme caution in Dravet syndrome (SCN1A mutations) as it worsens seizures by further impairing Nav1.1 in inhibitory interneurons. Additionally, rapid IV infusion of phenytoin causes cardiac arrhythmias (bradycardia, heart block, hypotension) due to its propylene glycol solvent and sodium channel effects in cardiac tissue. Fosphenytoin is safer IV but still contraindicated in Dravet. Infusion rate must not exceed 1 mg/kg/min (phenytoin) or 3 mg/kg/min PE (fosphenytoin) with cardiac monitoring."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION D – EPILEPSY SYNDROMES: ADVANCED
# ══════════════════════════════════════════════════════════════════════════

{
"type":"SBA","section":"D",
"stem":"A 3-day-old term neonate, born by normal vaginal delivery with Apgar scores 8 and 9, presents with episodes described as repetitive bicycling movements of the legs, episodes of sustained eye deviation to the right, and intermittent apnoea. Blood glucose, calcium, magnesium, and sodium are all normal. Blood culture is negative. LP shows normal CSF. Urine organic acids and plasma amino acids are normal. An EEG is performed.",
"q":"Which EEG finding, if present, would suggest Ohtahara syndrome (Early Infantile Epileptic Encephalopathy) rather than benign neonatal seizures?",
"opts":[
"A. Theta pointu alternant pattern (alternating theta bursts)",
"B. Burst-suppression pattern persisting both awake and asleep",
"C. Multifocal sharp waves with normal background",
"D. Generalised spike-wave at 3 Hz provoked by stimulation",
"E. Positive Rolandic sharp waves in the centrotemporal region"
],
"ans":"B",
"exp":"Ohtahara syndrome (EIEE – Early Infantile Epileptic Encephalopathy) is characterised by burst-suppression on EEG that is PERMANENT (present both in waking and sleep states). This distinguishes it from other neonatal encephalopathies where burst-suppression may be transient. Benign neonatal seizures ('fifth day fits') show the theta pointu alternant pattern. The persistent burst-suppression in Ohtahara indicates severe underlying structural, metabolic or genetic brain pathology and carries a very poor prognosis. Causes include structural malformations (Aicardi syndrome, porencephaly), metabolic errors, and ARX/STXBP1 mutations."
},

{
"type":"MCQ","section":"D",
"q": "A 6-year-old girl with tuberous sclerosis complex (TSC) develops infantile spasms at 5 months of age. Which antiseizure medication should be considered as PREFERRED FIRST-LINE treatment over ACTH in TSC-associated infantile spasms, based on current evidence?",
"opts":[
"A. Phenobarbital, as it reduces cortical excitability broadly",
"B. Valproate, as TSC involves generalised cortical involvement",
"C. Vigabatrin, as it reduces GABA transaminase and is specifically effective in TSC",
"D. ACTH, as it remains first-line regardless of aetiology",
"E. Levetiracetam, as broad-spectrum agents are preferred in TSC"
],
"ans":"C",
"exp":"In TSC-associated infantile spasms, vigabatrin is the preferred FIRST-LINE treatment (UK Infantile Spasms Study, UKISS; consensus guidelines from TSC Alliance). Vigabatrin irreversibly inhibits GABA transaminase, increasing GABA levels, and has a particularly high response rate in TSC (approximately 95% spasm-free in some series) due to the high concentration of GABAergic neurons in cortical tubers. ACTH is preferred first-line for non-TSC (cryptogenic/unknown aetiology) infantile spasms. The serious side effect of vigabatrin is irreversible concentric visual field defects (requiring baseline and periodic ERG/perimetry monitoring)."
},

{
"type":"SBA","section":"D",
"stem":"A 12-year-old girl presents with a 4-month history of absence-like episodes occurring 2–3 times per week, each lasting 30–60 seconds, sometimes with head turning to the right and post-ictal confusion lasting 5 minutes. She has also had two nocturnal generalised convulsions. Her EEG shows right temporal interictal epileptiform discharges. She is started on ethosuximide by her GP.",
"q":"Why is ethosuximide an INAPPROPRIATE choice for this patient?",
"opts":[
"A. Ethosuximide causes hepatotoxicity in adolescents",
"B. Ethosuximide is only effective for pure absence seizures (generalised onset) and is ineffective for focal seizures with impaired awareness",
"C. Ethosuximide is contraindicated above the age of 10 years",
"D. Ethosuximide aggravates temporal lobe epilepsy by increasing T-type calcium channel activity",
"E. Ethosuximide is effective for this patient but should have been combined with carbamazepine"
],
"ans":"B",
"exp":"This patient does NOT have childhood absence epilepsy. She has focal epilepsy with impaired awareness (previously 'complex partial seizures') – evidenced by: post-ictal confusion (absent in true absence), head turning (versive focal feature), duration 30–60 seconds (longer than typical absence 5–30 seconds), and temporal interictal discharges on EEG. Ethosuximide acts only on T-type calcium channels in the thalamus and is effective ONLY for generalised onset absence seizures. It has zero efficacy for focal seizures. The appropriate choice would be carbamazepine, oxcarbazepine, lamotrigine or levetiracetam for focal epilepsy."
},

{
"type":"MCQ","section":"D",
"q": "Fenfluramine has recently been approved for Dravet syndrome. Which serious adverse effect requires mandatory regular monitoring in patients receiving fenfluramine?",
"opts":[
"A. Irreversible peripheral visual field constriction",
"B. Cardiac valvulopathy and pulmonary arterial hypertension",
"C. Progressive renal tubular acidosis and nephrolithiasis",
"D. Hepatic failure requiring monitoring of liver function tests weekly",
"E. Severe teratogenicity requiring mandatory contraception in all patients"
],
"ans":"B",
"exp":"Fenfluramine (Fintepla) was originally withdrawn from the market as a weight-loss drug due to cardiac valvulopathy (mitral and aortic valve disease) and pulmonary arterial hypertension. When re-approved at lower doses for Dravet syndrome, mandatory cardiac monitoring (echocardiography before treatment, at 6 months, and then annually) is required by regulatory agencies (EMA, FDA). Visual field defects are a side effect of vigabatrin, not fenfluramine. Topiramate causes nephrolithiasis and metabolic acidosis."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION E – ANTI-SEIZURE MEDICATIONS: PHARMACOLOGY & SAFETY
# ══════════════════════════════════════════════════════════════════════════

{
"type":"MCQ","section":"E",
"q": "A 12-year-old Tamil boy is newly diagnosed with focal epilepsy and requires initiation of carbamazepine. Before prescribing, you plan to check HLA-B*1502 status. This is because HLA-B*1502 is associated with:",
"opts":[
"A. Severe carbamazepine-induced hepatotoxicity requiring dose reduction in carriers",
"B. Carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in Han Chinese and South/Southeast Asian populations",
"C. Reduced carbamazepine metabolism leading to toxicity at standard doses",
"D. Cross-reactivity with penicillin causing anaphylaxis in carriers",
"E. Carbamazepine-induced agranulocytosis regardless of ethnicity"
],
"ans":"B",
"exp":"HLA-B*1502 is a pharmacogenomic biomarker strongly associated with carbamazepine-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in Han Chinese and other Asian populations (Thai, Malaysian, South Asian including Sri Lankan). The FDA, EMA, and regulatory bodies in Sri Lanka recommend screening patients of Asian ancestry before initiating carbamazepine. This association also extends to phenytoin, oxcarbazepine, and lamotrigine to a lesser degree. The HLA-A*3101 allele in Northern European and Japanese populations is associated with less severe hypersensitivity reactions."
},

{
"type":"SBA","section":"E",
"stem":"A 16-year-old girl has been on valproate 30 mg/kg/day for juvenile myoclonic epilepsy for 3 years with excellent seizure control. She presents to your clinic and discloses she is now in a relationship and asks about switching medication. Her mother asks why you are considering a change.",
"q":"Which of the following is the PRIMARY reason to reconsider valproate in this adolescent girl?",
"opts":[
"A. Valproate is ineffective for JME beyond 5 years of treatment",
"B. Valproate causes irreversible visual field defects in adolescent females",
"C. Valproate is highly teratogenic (neural tube defects, neurodevelopmental impairment) and now contraindicated in women of childbearing potential without a Pregnancy Prevention Programme",
"D. Valproate causes progressive hepatic fibrosis requiring liver biopsy at 3 years of treatment",
"E. Valproate loses efficacy for myoclonic seizures in adolescence due to pharmacokinetic changes"
],
"ans":"C",
"exp":"Valproate is a well-established major human teratogen. It causes neural tube defects (spina bifida ~1–2%), cardiac malformations, hypospadias, and cognitive/neurodevelopmental impairment (autism spectrum disorder risk 5–10x, IQ reduction ~8–10 points) in offspring exposed in utero. Following the European Medicines Agency (EMA) PRAC review (2018) and subsequent UK and Sri Lanka regulatory guidance, valproate is now contraindicated in women and girls of childbearing potential UNLESS they are enrolled in a Pregnancy Prevention Programme (PPP) including two forms of contraception. Levetiracetam or lamotrigine are recommended alternatives for JME in girls, though slightly less effective for myoclonic seizures."
},

{
"type":"MCQ","section":"E",
"q": "A 4-year-old with Dravet syndrome is well-controlled on valproate and clobazam. His neurologist wants to add stiripentol. Stiripentol exerts its antiseizure effect via which primary mechanism?",
"opts":[
"A. Inhibition of T-type calcium channels in thalamic relay neurons",
"B. Allosteric potentiation of GABA-A receptors at a site distinct from benzodiazepines, plus inhibition of CYP450 enzymes increasing clobazam levels",
"C. Selective sodium channel blockade of Nav1.1 in inhibitory interneurons",
"D. Blockade of AMPA glutamate receptors reducing excitatory transmission",
"E. Inhibition of carbonic anhydrase causing metabolic acidosis and neuronal stabilisation"
],
"ans":"B",
"exp":"Stiripentol has a dual mechanism: (1) It directly potentiates GABA-A receptors through a unique allosteric site distinct from benzodiazepines (not blocked by flumazenil), and (2) It is a potent inhibitor of CYP2C19 and CYP3A4, which significantly increases plasma clobazam (and its active metabolite N-desmethylclobazam) levels by 2–5 fold. This drug interaction is part of its therapeutic mechanism in Dravet syndrome. When adding stiripentol, clobazam dose must be reduced. Stiripentol is only licensed as adjunctive therapy with valproate and clobazam specifically for Dravet syndrome."
},

{
"type":"MCQ","section":"E",
"q": "Topiramate's mechanism as an antiseizure medication involves which combination of actions?",
"opts":[
"A. Sodium channel blockade only",
"B. GABA enhancement only via benzodiazepine receptor",
"C. Sodium channel blockade + AMPA/kainate glutamate receptor antagonism + carbonic anhydrase inhibition + GABA-A potentiation",
"D. T-type calcium channel blockade + GABA-A potentiation",
"E. Sodium channel blockade + NMDA receptor antagonism"
],
"ans":"C",
"exp":"Topiramate has multiple complementary mechanisms: (1) voltage-gated sodium channel blockade, (2) antagonism of AMPA and kainate glutamate receptors (unique among commonly used ASMs), (3) carbonic anhydrase inhibition (isoenzymes II and IV) – responsible for metabolic acidosis and kidney stone formation, and (4) potentiation of GABA-A receptors (at a non-benzodiazepine site). Its multitargeted profile contributes to broad-spectrum efficacy. The carbonic anhydrase inhibition also explains its use in migraine prophylaxis (reduces excitability) and is responsible for the serious side effects of metabolic acidosis, hypohidrosis/hyperthermia, and nephrolithiasis."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION F – HEADACHE
# ══════════════════════════════════════════════════════════════════════════

{
"type":"SBA","section":"F",
"stem":"A 13-year-old girl presents with a 6-month history of recurrent headaches. Each episode starts with a 20-minute visual disturbance described as 'a zigzag line that moves across my vision and then disappears', followed by a right-sided throbbing headache lasting 4–6 hours, associated with nausea and vomiting. She has to stop all activity and lie in a dark room. The headaches occur approximately once per month. Neurological examination is entirely normal.",
"q":"Which statement about the first-line abortive treatment for this acute headache episode is MOST accurate based on current paediatric guidelines?",
"opts":[
"A. Oral codeine 0.5 mg/kg is the first-line analgesic for moderate-severe migraine",
"B. Ibuprofen 10 mg/kg orally should be taken at headache onset as first-line treatment",
"C. Sumatriptan is contraindicated under age 15 years due to vasospastic risk",
"D. IV sodium valproate should be given during acute attacks to prevent recurrence",
"E. Paracetamol 15 mg/kg is superior to ibuprofen for acute migraine in children"
],
"ans":"B",
"exp":"This is classic migraine with aura: visual aura (scintillating scotoma/fortification spectra) followed by unilateral throbbing headache with nausea/vomiting and photophobia/phonophobia. NSAIDs (ibuprofen 10 mg/kg, up to 400 mg/dose) are first-line abortive treatment for moderate-severe paediatric migraine. Ibuprofen has superior evidence to paracetamol for migraine in children. Triptans (e.g., sumatriptan nasal spray, rizatriptan) are second-line and ARE licensed in children (sumatriptan nasal spray ≥12 years, rizatriptan ≥6 years). Codeine and other opioids are NOT recommended for migraine (risk of medication overuse headache). Valproate is a prophylactic, not acute treatment."
},

{
"type":"MCQ","section":"F",
"q": "A 15-year-old boy presents with a 3-month history of daily headache, waking him from sleep at 3–4 AM, located behind the right eye, lasting 45–90 minutes, with severe lacrimation, nasal congestion, and ptosis of the right eye during the attack. He has 1–2 attacks per day, occurring in clusters lasting 2–3 weeks. The headaches are the most painful he has ever experienced. Neurological examination is normal. Which is the MOST likely diagnosis?",
"opts":[
"A. Migraine with aura",
"B. Tension-type headache with autonomic features",
"C. Cluster headache",
"D. Paroxysmal hemicrania",
"E. Short-lasting unilateral neuralgiform headache (SUNHA)"
],
"ans":"C",
"exp":"This is a classic description of cluster headache – one of the trigeminal autonomic cephalalgias. Features: strictly unilateral orbital/periorbital pain, very severe ('worst pain'), ipsilateral autonomic features (lacrimation, rhinorrhoea, ptosis/Horner syndrome), attacks lasting 15–180 minutes occurring 1–8 times/day in discrete cluster periods of weeks. Nocturnal attacks waking from sleep are characteristic. Cluster headache is rare in children but does occur in adolescents. Paroxysmal hemicrania has shorter attacks (2–30 min), higher frequency (>5/day), and responds absolutely to indomethacin. SUNCT has very brief attacks (seconds to minutes) with conjunctival injection."
},

{
"type":"SBA","section":"F",
"stem":"A 10-year-old obese girl (BMI 31 kg/m², 98th centile) presents with a 4-month history of progressive daily headache, pulsatile tinnitus, and two episodes of transient visual obscuration lasting seconds. She takes no regular medications. Examination reveals bilateral papilloedema and a sixth cranial nerve palsy. MRI brain and MR venography are normal. CSF opening pressure is 32 cmH₂O, and CSF analysis is entirely normal.",
"q":"Which statement about the management of this condition is MOST accurate?",
"opts":[
"A. Acetazolamide is contraindicated due to risk of metabolic acidosis in an obese child",
"B. Repeated lumbar punctures alone are sufficient and definitive treatment",
"C. Acetazolamide plus structured weight loss programme is the first-line treatment; optic nerve sheath fenestration is reserved for threatened vision",
"D. High-dose dexamethasone should be started immediately to reduce intracranial pressure",
"E. VP shunt is the treatment of choice as this is confirmed obstructive hydrocephalus"
],
"ans":"C",
"exp":"This is Idiopathic Intracranial Hypertension (pseudotumour cerebri) – raised ICP (>25 cmH₂O in children, normal MRI/MRV, normal CSF composition). The key risk factor is obesity. First-line treatment is acetazolamide (carbonic anhydrase inhibitor, reduces CSF production) at 25 mg/kg/day in divided doses, combined with a structured weight loss programme (10% weight loss achieves remission in many cases). Optic nerve sheath fenestration is reserved for threatened or deteriorating visual fields. Lumbar puncture is therapeutic only transiently and serially. CSF diversion (VP shunt or lumbar drain) is reserved for refractory cases. This is NOT obstructive hydrocephalus (MRI normal)."
},

{
"type":"MCQ","section":"F",
"q": "Which of the following preventative medications for paediatric migraine is ABSOLUTELY CONTRAINDICATED in a 14-year-old asthmatic girl who is also underweight?",
"opts":[
"A. Amitriptyline",
"B. Topiramate",
"C. Propranolol",
"D. Cyproheptadine",
"E. Riboflavin"
],
"ans":"C",
"exp":"Propranolol is absolutely contraindicated in asthma (beta-2 blockade causes bronchospasm). It is also problematic in underweight patients as it can cause hypoglycaemia (by blocking counter-regulatory responses) and may suppress growth. The clinical scenario specifies both asthma AND underweight – both are contraindications to propranolol. Amitriptyline can cause weight gain (may help in underweight). Topiramate causes weight loss (contraindicated in underweight). Cyproheptadine increases appetite and weight (appropriate in underweight children). Riboflavin has minimal side effects. The Harriet Lane table specifically lists propranolol's contraindications as asthma and hypoglycaemia risk."
},

{
"type":"SBA","section":"F",
"stem":"A 9-year-old boy is referred with recurrent episodes of sudden onset, severe periumbilical pain occurring 8–10 times per year, each lasting 2–3 hours, with pallor, nausea, and vomiting but NO headache. Between episodes he is completely well. His maternal grandmother and mother both suffer from migraine headaches. Physical examination and investigations including abdominal ultrasound, upper GI endoscopy, and urine catecholamines are all normal.",
"q":"What is the MOST likely diagnosis and the rationale for treatment as a migraine variant?",
"opts":[
"A. Functional abdominal pain; refer to child psychologist for CBT",
"B. Abdominal migraine; treat with anti-migraine prophylaxis (e.g., pizotifen or amitriptyline) given frequency and functional impact",
"C. Cyclic vomiting syndrome; differentiated by prominent vomiting and treated with ondansetron only",
"D. Intestinal malrotation; refer urgently for surgical opinion",
"E. Munchausen by proxy; safeguarding referral required"
],
"ans":"B",
"exp":"This is Abdominal migraine – a recognised childhood migraine variant (ICHD-3 criteria: ≥5 attacks of midline abdominal pain, moderate-severe intensity, 1–72 hours duration, with anorexia, nausea, or vomiting; attacks not attributed to another diagnosis; strong positive family history of migraine). It is classified under 'episodic syndromes that may be associated with migraine'. Patients frequently develop typical migraine headaches in adolescence. Treatment follows migraine guidelines: ibuprofen for acute episodes; prophylaxis with amitriptyline, pizotifen (not available in all countries), or propranolol for frequent episodes. Cyclic vomiting syndrome differs by prominent repetitive vomiting as the primary symptom rather than abdominal pain."
},

# ══════════════════════════════════════════════════════════════════════════
# SECTION G – INTEGRATED CLINICAL SCENARIOS (EXTREME DIFFICULTY)
# ══════════════════════════════════════════════════════════════════════════

{
"type":"SBA","section":"G",
"stem":"A 4-year-old boy is admitted with his 4th admission for status epilepticus in 6 months, all triggered by fever. He has had normal development until age 2 years, after which he has progressively lost language and social skills. On examination he is irritable, hypotonic, and has hepatosplenomegaly. His eyes show a 'cherry red spot' on fundoscopy. MRI brain shows diffuse cortical atrophy and white matter signal changes. Previous EEGs during episodes show generalised spike-wave with myoclonic discharges.",
"q":"The finding of a cherry red spot in this context of progressive neurodegeneration with myoclonic epilepsy MOST likely indicates which diagnosis?",
"opts":[
"A. Dravet syndrome with secondary hippocampal sclerosis",
"B. Rett syndrome with autonomic dysregulation",
"C. Neuronal Ceroid Lipofuscinosis (Batten disease) – late infantile form",
"D. GM2 gangliosidosis (Tay-Sachs disease) or Niemann-Pick disease type A",
"E. Subacute sclerosing panencephalitis (SSPE)"
],
"ans":"D",
"exp":"Cherry red spot (central macular whitening with red foveola visible through intact foveal vasculature) in the context of progressive neurodegeneration, myoclonic epilepsy, and hypotonia/regression indicates lysosomal storage disease accumulating in ganglion cells. The main differentials are: Tay-Sachs (GM2 gangliosidosis – hexosaminidase A deficiency), Sandhoff disease (GM2 – hexosaminidase A+B deficiency), and Niemann-Pick type A (sphingomyelinase deficiency – also has hepatosplenomegaly). The combination of hepatosplenomegaly with cherry red spot and myoclonic epilepsy most strongly points to Niemann-Pick type A or Sandhoff disease. NCL (Batten) does NOT cause cherry red spots (macular degeneration) and SSPE shows CSF measles antibodies with EEG periodic complexes (Radermecker complexes)."
},

{
"type":"SBA","section":"G",
"stem":"A 16-year-old girl is brought to the emergency department by her parents following a first generalised convulsion. She had been unwell for 3 days with headache, fever, and behavioural change – she was found talking to people who were not there and became aggressive. On examination she is confused (GCS 13/15), afebrile (37.2°C), has orofacial dyskinesias (lip smacking, chewing movements), and generalised hyperreflexia. CSF shows: 55 lymphocytes/mm³, protein 0.65 g/L, glucose 3.8 mmol/L (serum 5.5). EEG shows extreme delta brush. MRI brain is normal.",
"q":"The antineuronal antibody MOST likely to be positive in this patient's serum and CSF is:",
"opts":[
"A. Anti-Hu (ANNA-1) – paraneoplastic limbic encephalitis",
"B. Anti-NMDA receptor (anti-GluN1/NR1) antibody",
"C. Anti-VGCC (voltage-gated calcium channel) antibody",
"D. Anti-GAD65 antibody – stiff-person spectrum",
"E. Anti-LGI1 antibody – faciobrachial dystonic seizures"
],
"ans":"B",
"exp":"This is the classic presentation of Anti-NMDA Receptor Encephalitis: young female, psychiatric prodrome, then seizures, orofacial dyskinesias (characteristic), autonomic instability, CSF lymphocytic pleocytosis, and the pathognomonic EEG finding of 'extreme delta brush' (beta activity superimposed on delta waves). Anti-NR1 (GluN1 subunit of NMDA receptor) antibodies are found in serum and CSF. Associated ovarian teratoma in ~50% of adult females (less common in adolescents/children). Management: tumour removal + immunotherapy (methylprednisolone, IVIG, then rituximab/cyclophosphamide for refractory cases). Anti-LGI1 causes faciobrachial dystonic seizures; anti-Hu is paraneoplastic in older adults with lung cancer."
},

{
"type":"SBA","section":"G",
"stem":"A 2-year-old boy is on vigabatrin for infantile spasms secondary to tuberous sclerosis complex. His spasms are now fully controlled. His parents ask about the most serious long-term side effect they should monitor for.",
"q":"Which of the following monitoring protocols is specifically recommended for vigabatrin therapy in children?",
"opts":[
"A. Annual liver function tests and full blood count to screen for hepatotoxicity and aplastic anaemia",
"B. Regular electroretinography (ERG) and/or Goldmann perimetry to detect concentric visual field constriction",
"C. Renal ultrasound every 6 months to detect nephrolithiasis due to carbonic anhydrase inhibition",
"D. Echocardiography every 6 months due to risk of pulmonary hypertension",
"E. Thyroid function tests every 6 months due to hypothyroidism induced by GABA-B agonism"
],
"ans":"B",
"exp":"Vigabatrin causes irreversible, concentric visual field constriction in approximately 30–50% of patients on long-term treatment. This is a GABA-mediated toxic retinopathy affecting peripheral retinal cells. The defect is asymptomatic until severe. Monitoring protocol (per NICE/UKISS guidelines): electroretinography (ERG) before treatment (baseline), at 6 months, then annually. Goldmann perimetry can supplement in cooperative older children. In infants, ERG is the only feasible method. This monitoring is mandatory and parents must be counselled. Visual field defects are NOT reversible on discontinuation. The benefit-risk balance is generally favourable given the severity of TSC-associated epilepsy."
},

{
"type":"MCQ","section":"G",
"q": "A 14-year-old boy with known JME is admitted after a cluster of myoclonic jerks and two GTCS following his school examinations. He is taking valproate 25 mg/kg/day with good compliance. His serum valproate level is therapeutic. Which is the MOST important management step BEYOND adjusting antiseizure medication?",
"opts":[
"A. Add levetiracetam as emergency adjunctive therapy immediately",
"B. Perform urgent EEG to rule out non-convulsive status epilepticus",
"C. Provide structured counselling on sleep hygiene, avoiding sleep deprivation, and alcohol avoidance as these are the precipitating triggers for JME exacerbation",
"D. Increase valproate dose to supratherapeutic levels to achieve complete seizure freedom",
"E. Switch to lamotrigine as valproate is failing at therapeutic levels"
],
"ans":"C",
"exp":"In JME, lifestyle triggers are as important as medication. The classic trigger triad is: sleep deprivation, alcohol, and photic stimulation. This adolescent's seizures followed school examinations – a recognised cause of sleep deprivation and stress. The history strongly suggests precipitating triggers rather than pharmacological failure (therapeutic valproate level). Non-pharmacological management (regular sleep schedule, alcohol avoidance, covering one eye in flashing light environments) is critical and should be the primary intervention here. Medication adjustment may also be needed, but addressing triggers is the most important step when serum levels are therapeutic."
},

]

# ══════════════════════════════════════════════════════════════════════════
# BUILD THE WORD DOCUMENT
# ══════════════════════════════════════════════════════════════════════════

doc = Document()

# ── Page setup ──────────────────────────────────────────────────────────
from docx.oxml import OxmlElement
section = doc.sections[0]
section.page_width  = int(8.27 * 914400 / 100) * 100   # A4
section.page_height = int(11.69 * 914400 / 100) * 100
section.left_margin   = Inches(1.1)
section.right_margin  = Inches(1.1)
section.top_margin    = Inches(1.0)
section.bottom_margin = Inches(1.0)

# ── Styles ──────────────────────────────────────────────────────────────
normal = doc.styles['Normal']
normal.font.name = 'Arial'
normal.font.size = Pt(11)

def shade_cell(cell, fill_hex):
    """Apply background shading to a table cell."""
    tc = cell._tc
    tcPr = tc.get_or_add_tcPr()
    shd = OxmlElement('w:shd')
    shd.set(qn('w:val'), 'clear')
    shd.set(qn('w:color'), 'auto')
    shd.set(qn('w:fill'), fill_hex)
    tcPr.append(shd)

def add_run(para, text, bold=False, italic=False, color=None, size=None):
    run = para.add_run(text)
    run.bold = bold
    run.italic = italic
    if color:
        run.font.color.rgb = color
    if size:
        run.font.size = Pt(size)
    return run

def heading(text, level, color=NAVY):
    p = doc.add_heading(text, level=level)
    p.alignment = WD_ALIGN_PARAGRAPH.LEFT
    for run in p.runs:
        run.font.color.rgb = color
        run.font.name = 'Arial'
    return p

def body(text, space_before=2, space_after=4):
    p = doc.add_paragraph()
    p.paragraph_format.space_before = Pt(space_before)
    p.paragraph_format.space_after  = Pt(space_after)
    p.paragraph_format.left_indent  = Inches(0)
    return p

def add_correct_tag(p):
    run = p.add_run('  ✓ CORRECT')
    run.bold = True
    run.font.color.rgb = GREEN

# ── COVER PAGE ──────────────────────────────────────────────────────────
p = doc.add_paragraph()
p.alignment = WD_ALIGN_PARAGRAPH.CENTER
p.paragraph_format.space_before = Pt(48)
add_run(p, 'PAEDIATRIC NEUROLOGY\n', bold=True, color=NAVY, size=28)
add_run(p, 'MCQ & SBA Question Bank\n', bold=True, color=TEAL, size=22)
add_run(p, '\nMD Paediatrics PG Selection Examination\n', italic=True, color=GREY, size=14)
add_run(p, 'Postgraduate Institute of Medicine (PGIM)\nUniversity of Colombo, Sri Lanka\n', italic=True, color=GREY, size=13)
add_run(p, '\nTopics Covered: Neurological Examination • Seizures & Epilepsy •\nStatus Epilepticus • Anti-Seizure Medications • Headache\n', color=BLACK, size=12)
add_run(p, '\nDifficulty Level: HARD – EXTREME\n', bold=True, color=RED, size=13)
add_run(p, 'Total Questions: 30 (15 MCQ + 15 SBA)\n', bold=True, color=NAVY, size=13)
add_run(p, '\nPrepared by: Department of Paediatrics\nFaculty of Medicine, University of Colombo\n', italic=True, color=GREY, size=12)
add_run(p, 'Based on: Nelson\'s Textbook of Paediatrics (21st/22nd Ed) • Harriet Lane Handbook (23rd Ed)\n', italic=True, color=GREY, size=11)
add_run(p, f'\nGenerated: July 2026', italic=True, color=GREY, size=10)

# Instructions box
doc.add_page_break()
p = doc.add_paragraph()
p.alignment = WD_ALIGN_PARAGRAPH.CENTER
add_run(p, 'INSTRUCTIONS TO CANDIDATES', bold=True, color=NAVY, size=14)

tbl = doc.add_table(rows=1, cols=1)
tbl.style = 'Table Grid'
cell = tbl.cell(0, 0)
shade_cell(cell, 'EAF0FB')
c = cell.paragraphs[0]
c.paragraph_format.space_before = Pt(4)
c.paragraph_format.space_after  = Pt(4)
add_run(c, 'Question Format:\n', bold=True, color=NAVY, size=11)
add_run(c, '• MCQ (Multiple Choice Questions): One BEST answer from five options (A–E)\n'
           '• SBA (Single Best Answer): Clinical scenario with one BEST answer from five options\n'
           '• Each question carries equal marks; no negative marking\n\n', size=11)
add_run(c, 'Instructions:\n', bold=True, color=NAVY, size=11)
add_run(c, '• Read each question carefully before selecting your answer\n'
           '• In SBA questions, read the full clinical stem before answering the question\n'
           '• Answers with full explanations are provided at the end of each section\n'
           '• This paper tests application and higher-order reasoning, not recall alone\n'
           '• Time allocation: approximately 2 minutes per question', size=11)

doc.add_paragraph()

# ── SECTION DEFINITIONS ──────────────────────────────────────────────────
section_defs = {
    'A': ('SECTION A', 'Neurological Examination and Developmental Assessment', NAVY),
    'B': ('SECTION B', 'Seizures, Febrile Seizures, and Epilepsy Syndromes', TEAL),
    'C': ('SECTION C', 'Status Epilepticus', BLUE),
    'D': ('SECTION D', 'Epilepsy Syndromes: Advanced Topics', NAVY),
    'E': ('SECTION E', 'Anti-Seizure Medications: Pharmacology and Safety', TEAL),
    'F': ('SECTION F', 'Headache', BLUE),
    'G': ('SECTION G', 'Integrated Clinical Scenarios', RED),
}

# ── QUESTIONS ──────────────────────────────────────────────────────────

current_section = None
q_num = 0
mcq_count = 0
sba_count = 0

for q in questions:
    sec = q['section']
    q_num += 1
    if q['type'] == 'MCQ':
        mcq_count += 1
    else:
        sba_count += 1

    # Section header
    if sec != current_section:
        current_section = sec
        sdef = section_defs[sec]
        doc.add_paragraph()
        p = doc.add_paragraph()
        p.alignment = WD_ALIGN_PARAGRAPH.LEFT
        add_run(p, f'{sdef[0]}: {sdef[1]}', bold=True, color=sdef[2], size=14)
        # underline rule
        p.paragraph_format.border_bottom = None
        doc.add_paragraph()

    # Question type badge
    badge_color = BLUE if q['type'] == 'MCQ' else RED

    # Build question block as a single-row table for visual separation
    tbl = doc.add_table(rows=1, cols=1)
    tbl.style = 'Table Grid'
    cell = tbl.cell(0, 0)
    shade_cell(cell, 'F8F9FF' if q['type'] == 'MCQ' else 'FFF8F0')
    cell.width = Inches(6.27)

    cp = cell.paragraphs[0]
    cp.paragraph_format.space_before = Pt(3)
    cp.paragraph_format.space_after  = Pt(2)
    cp.paragraph_format.left_indent  = Inches(0.1)

    # Badge + question number
    add_run(cp, f'[{q["type"]}]  Q{q_num}  ', bold=True, color=badge_color, size=10)

    # Clinical stem for SBA
    if q['type'] == 'SBA' and 'stem' in q:
        add_run(cp, '\n\nCLINICAL SCENARIO:\n', bold=True, color=GREY, size=10)
        add_run(cp, q['stem'] + '\n', italic=True, size=11)
        add_run(cp, '\nQUESTION:\n', bold=True, color=GREY, size=10)

    add_run(cp, q['q'] + '\n', bold=True, size=11)

    # Options
    for opt in q['opts']:
        letter = opt[0]
        is_ans = (letter == q['ans'])
        run_text = f'\n{opt}'
        if is_ans:
            add_run(cp, run_text, bold=True, color=GREEN, size=11)
            add_run(cp, '  ◀ ANSWER', bold=True, color=GREEN, size=9)
        else:
            add_run(cp, run_text, size=11)

    # Explanation
    add_run(cp, '\n\nEXPLANATION:\n', bold=True, color=NAVY, size=10)
    add_run(cp, q['exp'], italic=True, size=10, color=GREY)

    doc.add_paragraph()


# ── ANSWER SUMMARY TABLE ─────────────────────────────────────────────────
doc.add_page_break()
p = doc.add_paragraph()
p.alignment = WD_ALIGN_PARAGRAPH.CENTER
add_run(p, 'ANSWER KEY SUMMARY', bold=True, color=NAVY, size=16)
doc.add_paragraph()

tbl = doc.add_table(rows=1, cols=5)
tbl.style = 'Table Grid'
hdr_cells = tbl.rows[0].cells
for i, hdr in enumerate(['Q#', 'Type', 'Section', 'Correct Answer', 'Topic']):
    shade_cell(hdr_cells[i], '1F3864')
    p = hdr_cells[i].paragraphs[0]
    add_run(p, hdr, bold=True, color=WHITE, size=10)

topics = [
    "Primitive reflexes – disappearance age",
    "Hand dominance – red flag",
    "Developmental regression – red flag",
    "Parachute reflex",
    "Developmental milestones – 2 years",
    "ILAE 2017 seizure classification",
    "Simple febrile seizure management",
    "Complex febrile seizure – Todd's paresis",
    "Febrile seizure recurrence risk factors",
    "BECTS – EEG",
    "Childhood absence – drug of choice",
    "West syndrome – first-line treatment",
    "Dravet syndrome – contraindicated drug",
    "JME – long-term prognosis",
    "Lennox-Gastaut – diagnostic triad",
    "Status epilepticus – Phase 2 management",
    "Status epilepticus – no IV access",
    "Pyridoxine-dependent epilepsy mechanism",
    "Phenytoin – Dravet contraindication",
    "Ohtahara – EEG pattern",
    "TSC infantile spasms – vigabatrin",
    "Focal vs absence seizure – ethosuximide",
    "Fenfluramine – monitoring",
    "HLA-B*1502 – carbamazepine risk",
    "Valproate – teratogenicity in girls",
    "Stiripentol mechanism",
    "Topiramate mechanism",
    "Migraine – first-line abortive treatment",
    "Cluster headache diagnosis",
    "IIH management",
    "Migraine prophylaxis contraindication",
    "Abdominal migraine",
    "Cherry red spot – storage disease",
    "NMDAR encephalitis – extreme delta brush",
    "Vigabatrin – visual field monitoring",
    "JME – lifestyle triggers",
]

fill_colours = ['FFFFFF', 'EAF0FB']
for idx, q in enumerate(questions):
    row_cells = tbl.add_row().cells
    fill = fill_colours[idx % 2]
    for c in row_cells:
        shade_cell(c, fill)
    row_cells[0].paragraphs[0].add_run(str(idx+1)).font.size = Pt(10)
    row_cells[1].paragraphs[0].add_run(q['type']).font.size = Pt(10)
    row_cells[2].paragraphs[0].add_run(section_defs[q['section']][0]).font.size = Pt(10)
    ans_p = row_cells[3].paragraphs[0]
    r = ans_p.add_run(q['ans'])
    r.bold = True
    r.font.color.rgb = GREEN
    r.font.size = Pt(10)
    topic_text = topics[idx] if idx < len(topics) else ''
    row_cells[4].paragraphs[0].add_run(topic_text).font.size = Pt(9)

# ── Footer note ──────────────────────────────────────────────────────────
doc.add_paragraph()
p = doc.add_paragraph()
p.alignment = WD_ALIGN_PARAGRAPH.CENTER
add_run(p, 'Sources: Nelson\'s Textbook of Paediatrics (21st/22nd Ed) • Harriet Lane Handbook (23rd Ed) • ILAE 2017 • AAP Guidelines\n'
           'Department of Paediatrics, Faculty of Medicine, University of Colombo – PGIM Sri Lanka',
           italic=True, color=GREY, size=9)

# ── SAVE ─────────────────────────────────────────────────────────────────
out = '/tmp/workspace/neuro-mcq/Paediatric_Neurology_MCQ_SBA_PGIM.docx'
doc.save(out)
print(f'Saved: {out}')
print(f'Total questions: {len(questions)} ({mcq_count} MCQ, {sba_count} SBA)')