mkdir -p /home/daytona/workspace/bibrachial-mnd && ls /home/daytona/workspace/bi…

~/bibrachial-mnd/build_pptx.js

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// ─── SLIDE 2: DEFINITION & OVERVIEW ─────────────────────────────────────────
contentSlide(
  "Definition & Overview",
  [
    "Bibrachial MND (Flail Arm Syndrome / Brachial Amyotrophic Diplegia) is a regional variant of ALS",
    "Selective degeneration of LOWER MOTOR NEURONS supplying both upper extremities",
    "Also called 'Hanging Man Syndrome' or 'Man-in-a-Barrel Syndrome'",
    "Accounts for ~10% of all ALS cases",
    "Predominantly affects men (male:female ratio ~5–9:1)",
    "Mean age at onset: 55–65 years",
    "Longer median survival vs typical ALS (median >4 years)",
    "##Key distinction",
    "Proximal shoulder-girdle muscles affected early → distal spread",
    "Legs and bulbar muscles relatively SPARED until late",
    "UMN signs in arms may be mild or absent initially; LMN signs dominate",
  ],
  [
    "##ALS Variant Spectrum",
    "Classical ALS — UMN + LMN, limbs + bulbar",
    "Progressive Muscular Atrophy (PMA) — LMN only",
    "Progressive Bulbar Palsy (PBP) — bulbar only",
    "Primary Lateral Sclerosis (PLS) — UMN only",
    "Flail Arm Syndrome (FAS) — proximal UE predominant",
    "Flail Leg Syndrome (FLS) — LE predominant",
    "##Eponym",
    "Originally described as 'Brachial Amyotrophic Diplegia' by Vulpian (1886)",
    "Re-described as 'Man-in-a-Barrel Syndrome' — bilateral arm weakness with intact legs",
  ],
  "Variant Spectrum"
);

// ─── SLIDE 3: PATHOPHYSIOLOGY OVERVIEW ───────────────────────────────────────
sectionSlide("PATHOPHYSIOLOGY", "How does Bibrachial MND occur? — Proximal & Distal mechanisms");

// ─── SLIDE 4: PATHOPHYSIOLOGY — PROXIMAL ─────────────────────────────────────
contentSlide(
  "Pathophysiology — Proximal Pattern (Shoulder-Girdle Onset)",
  [
    "##Site of primary LMN degeneration",
    "Anterior horn cells at C5–C6 spinal segments (shoulder/arm)",
    "Motor cortex neurons projecting via corticospinal tract",
    "##Mechanism",
    "TDP-43 / FUS protein aggregation in motor neurons → toxic gain-of-function",
    "Glutamate excitotoxicity → calcium influx → mitochondrial dysfunction",
    "SOD1 mutations (20% familial) → oxidative stress → cell death",
    "C9ORF72 GGGGCC repeat expansion → most common genetic cause",
    "##Why shoulder-girdle first?",
    "Proximal C5–C6 neurons may be selectively vulnerable due to:",
    "Higher metabolic demand (large motor units innervating deltoid, biceps)",
    "Neurofilament accumulation → impaired axonal transport in long neurons",
    "Local astrocyte dysfunction: SOD1-mutant astrocytes toxic to motor neurons",
  ],
  [
    "##Gross pathology",
    "Atrophy of precentral gyrus (motor cortex)",
    "Sclerosis and pallor of corticospinal tracts",
    "Thinning of ventral nerve roots C5–C6",
    "Atrophy of deltoid, infraspinatus, biceps, supraspinatus",
    "##Microscopy",
    "50% loss of spinal motor neurons at cervical levels",
    "Diffuse astrocytic gliosis in spinal gray matter",
    "TDP-43 immunoreactive inclusions in surviving neurons",
    "Ubiquitin-positive cytoplasmic inclusions",
    "##Spared structures",
    "Onuf nucleus (sacral — pelvic floor) → bladder control intact",
    "CN III, IV, VI nuclei → eye movements intact",
    "Sensory neurons (posterior horn) → no sensory loss",
  ],
  "Pathology"
);

// ─── SLIDE 5: PATHOPHYSIOLOGY — DISTAL SPREAD ────────────────────────────────
contentSlide(
  "Pathophysiology — Distal Spread & Rostro-Caudal Pattern",
  [
    "##Distal arm involvement (C7–T1 segment)",
    "After shoulder-girdle atrophy: spread to forearm flexors/extensors, intrinsics",
    "Wrist drop, finger weakness, thenar/hypothenar wasting follow",
    "Fasciculations appear distally as motor units become unstable",
    "##Why 'flail arm'?",
    "Combined proximal (C5-C6) + distal (C7-T1) LMN loss",
    "Arms hang limp, adducted, internally rotated — 'dangling arms'",
    "Patient cannot lift, abduct, or flex elbow against gravity",
    "##Temporal sequence",
    "1. Proximal weakness (shoulder abduction, elbow flexion) → months",
    "2. Distal weakness (wrist/fingers) → months to 1–2 years later",
    "3. Contralateral arm involved (hence 'bilateral')",
    "4. Legs involved much later (>18–24 months after onset)",
    "5. Bulbar involvement last (dysphagia, dysarthria)",
  ],
  [
    "##UMN component in distal pattern",
    "Corticospinal neurons to cervical cord degenerate in parallel",
    "Initially: LMN signs dominate (atrophy + hyporeflexia)",
    "Later: UMN signs may emerge (brisk knee jerks, Babinski)",
    "Reflex dissociation: absent biceps jerk (C5/C6 LMN) but preserved knee jerk",
    "##Molecular basis of spread",
    "Prion-like propagation of misfolded TDP-43 / SOD1 aggregates",
    "Anterograde/retrograde axonal spread via neuromuscular synapses",
    "Corticomotoneuronal hyperexcitability drives LMN degeneration",
    "##Why legs spared longer?",
    "Lumbar anterior horn cells less vulnerable initially",
    "Possible 'dying-back' upper motor neuron pattern from cervical to lumbar",
    "FAS shows preferential cervical neuronal vulnerability vs. typical ALS",
  ],
  "UMN Component & Spread"
);

// ─── SLIDE 6: CLINICAL FEATURES ──────────────────────────────────────────────
sectionSlide("CLINICAL FEATURES", "Signs, Symptoms & Disease Course");

// ─── SLIDE 7: CLINICAL FEATURES — SYMPTOMS ───────────────────────────────────
contentSlide(
  "Clinical Features — Presenting Symptoms",
  [
    "##Cardinal Presenting Complaint",
    "Bilateral proximal arm weakness — cannot raise arms above head",
    "Initially UNILATERAL, then spreads to contralateral arm (weeks–months)",
    "Difficulty combing hair, reaching shelves, lifting objects",
    "Shoulder-girdle wasting noticed in mirror",
    "##Early phase (months 1–12)",
    "Arm hangs limply at side — adducted, internally rotated",
    "Progressive weight loss in arm musculature",
    "Coarse fasciculations visible in deltoid, biceps, triceps, forearm",
    "Cramps common especially at night",
    "NO sensory symptoms (paraesthesia, numbness) — key negative",
    "NO sphincter disturbance",
  ],
  [
    "##Late phase",
    "Bilateral flail arms — completely dependent arms",
    "Distal weakness: wrist drop, grip weakness, claw hand",
    "Respiratory embarrassment: paradoxical breathing if diaphragm involved",
    "Mild dysarthria / dysphagia may appear late",
    "##Negative features (help confirm diagnosis)",
    "No bladder/bowel dysfunction",
    "No sensory loss",
    "No eye movement disorder (CN III/IV/VI spared)",
    "Cognitive function usually intact (rare FTD-ALS overlap)",
    "No pain in early stages",
    "##Progression",
    "Median survival: 4–5 years (better than classic ALS 2–3 years)",
    "Death usually from respiratory failure or aspiration pneumonia",
  ],
  "Late & Negative Features"
);

// ─── SLIDE 8: CLINICAL FEATURES — SIGNS ──────────────────────────────────────
contentSlide(
  "Clinical Features — Neurological Signs",
  [
    "##LMN signs (dominant, bilateral arms)",
    "Wasting and atrophy — deltoid, biceps, brachioradialis, intrinsics",
    "Fasciculations — coarse, visible; deltoid, biceps, forearm",
    "Hypotonia of upper limbs",
    "Absent / diminished biceps (C5/C6) and brachioradialis (C5/C6) reflexes",
    "Absent triceps jerk (C7) as disease progresses distally",
    "##Reflex dissociation (pathognomonic clue)",
    "Absent biceps + brachioradialis jerk (LMN arm)",
    "PRESERVED / brisk knee jerk (UMN leg)",
    "Absent jaw jerk or brisk jaw jerk depending on bulbar involvement",
    "Extensor plantar response (Babinski) may appear with leg UMN involvement",
  ],
  [
    "##UMN signs",
    "Spasticity in legs (if involved late)",
    "Brisk knee and ankle jerks",
    "Bilateral Babinski signs",
    "Hoffman's sign may be present or absent in arms",
    "##Bulbar signs (late)",
    "Tongue fasciculations and wasting",
    "Dysarthria (spastic + flaccid — 'hot potato' voice)",
    "Dysphagia (solids first, then liquids)",
    "Drooling / excessive salivation",
    "Emotional lability (pseudobulbar affect)",
    "##Respiratory signs",
    "Paradoxical diaphragmatic movement",
    "Orthopnoea / exertional dyspnoea",
    "Use of accessory muscles of respiration",
  ],
  "UMN / Bulbar / Respiratory"
);

// ─── SLIDE 9: DIFFERENTIAL DIAGNOSIS ─────────────────────────────────────────
sectionSlide("DIFFERENTIAL DIAGNOSIS", "Conditions that mimic Bibrachial MND");

// ─── SLIDE 10: DD TABLE ───────────────────────────────────────────────────────
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    [{ text:"Cervical Myelopathy + Radiculopathy", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Pain; sensory symptoms; UMN signs caudal to LMN; bladder symptoms; precipitated by neck movement", options:{color:TEXT,fontSize:11} }, { text:"MRI cervical spine; nerve conduction studies", options:{color:TEXT,fontSize:11} }],
    [{ text:"Brachial Neuritis (Parsonage-Turner)", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Acute onset severe shoulder pain followed by weakness; patchy unilateral; usually monophasic", options:{color:TEXT,fontSize:11} }, { text:"Spontaneous partial recovery; EMG/NCS; MRI brachial plexus", options:{color:TEXT,fontSize:11} }],
    [{ text:"Multifocal Motor Neuropathy (MMN)", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Pure LMN; asymmetric; conduction block on NCS; anti-GM1 antibodies raised; responds to IVIG", options:{color:TEXT,fontSize:11} }, { text:"NCS: conduction block; anti-GM1 Ab; IVIG response", options:{color:TEXT,fontSize:11} }],
    [{ text:"Inclusion Body Myositis (IBM)", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Finger flexor + quadriceps weakness pattern; CK moderately raised; muscle biopsy diagnostic", options:{color:TEXT,fontSize:11} }, { text:"EMG: myopathic; biopsy: rimmed vacuoles, IBM deposits", options:{color:TEXT,fontSize:11} }],
    [{ text:"Hirayama Disease (Monomelic Amyotrophy)", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Young males; unilateral or asymmetric distal upper limb; self-limiting; cervical flexion MRI shows dural shift", options:{color:TEXT,fontSize:11} }, { text:"Flexion MRI cervical; arrested progression", options:{color:TEXT,fontSize:11} }],
    [{ text:"Progressive Muscular Atrophy (PMA)", options:{bold:true,color:TEXT,fontSize:11} }, { text:"Pure LMN variant of ALS; symmetric; generalized; no regional predilection; no UMN signs", options:{color:TEXT,fontSize:11} }, { text:"Generalized vs. arm-predominant distribution; EMG", options:{color:TEXT,fontSize:11} }],
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    [{ text:"Radiation Myelopathy", options:{bold:true,color:TEXT,fontSize:11} }, { text:"History of prior neck/thorax radiotherapy (e.g., Hodgkin lymphoma); onset years after treatment", options:{color:TEXT,fontSize:11} }, { text:"Radiation history; MRI cord changes in radiation field", options:{color:TEXT,fontSize:11} }],
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// ─── SLIDE 11: MORE DD ────────────────────────────────────────────────────────
contentSlide(
  "Differential Diagnosis — Additional Conditions",
  [
    "##Neuromuscular Junction disorders",
    "Myasthenia Gravis — fatigable weakness; ptosis; diplopia; positive Tensilon test; AChR antibodies",
    "Lambert-Eaton Syndrome — proximal LL > UL; autonomic features; anti-VGCC Ab; incremental NCS",
    "##Hereditary Motor Neuropathies (CMT)",
    "Family history; distal wasting; foot deformity; slow progressive; abnormal NCS",
    "##Spinal Muscular Atrophy (Adult SMA type 3/4)",
    "Symmetric proximal weakness; SMN1 gene deletion; purely LMN; slow progression",
    "##Post-Polio Syndrome",
    "History of acute poliomyelitis decades earlier; fatigue + new weakness; no active denervation on EMG",
    "##Lead / Heavy Metal Neuropathy",
    "Exposure history; wrist drop; pure motor; blood lead level elevated; basophilic stippling on smear",
  ],
  [
    "##Paraneoplastic Motor Neuron Syndrome",
    "Underlying malignancy (lymphoma, lung, breast); anti-amphiphysin / anti-Hu antibodies",
    "##Bilateral Brachial Plexopathy",
    "Trauma / infiltration / radiation; imaging shows plexus lesion; sensory abnormalities usually present",
    "##Cervical Cord Infarction (Bibrachial Pattern)",
    "Acute onset; bilateral arm weakness + sensory level; anterior spinal artery territory",
    "MRI spine: diffuse cord T2 signal; 'owl-eye' or 'snake-eye' sign",
    "##Sjogren Syndrome Motor Neuropathy",
    "Non-motor features (dry eyes, dry mouth, parotid enlargement); anti-Ro/La antibodies",
    "##Thyrotoxicosis with Myopathy",
    "Systemic features of hyperthyroidism; proximal weakness; TFTs abnormal; CK mild rise",
  ],
  "NMJ & Hereditary"
);

// ─── SLIDE 12: INVESTIGATIONS ────────────────────────────────────────────────
sectionSlide("INVESTIGATIONS", "Diagnosis & Work-up of Flail Arm Syndrome");

// ─── SLIDE 13: INVESTIGATIONS ────────────────────────────────────────────────
contentSlide(
  "Investigations — Electrophysiology & Blood Tests",
  [
    "##EMG (most important test)",
    "Active denervation: fibrillations + positive sharp waves in multiple cervical myotomes",
    "Fasciculation potentials in affected muscles (deltoid, biceps, first dorsal interosseous)",
    "Large-amplitude, long-duration, polyphasic motor unit potentials (chronic denervation)",
    "Reduced recruitment pattern bilaterally in upper limb muscles",
    "EMG must show changes in ≥2 of 4 body regions (Awaji-Shima / El Escorial criteria)",
    "##Nerve Conduction Studies (NCS)",
    "Motor NCS: normal conduction velocity (axonal loss only, no demyelination)",
    "CMAP amplitude reduced in median, ulnar, radial nerves (axonal loss)",
    "Sensory NCS: NORMAL — distinguishes from sensory neuropathies",
    "NO conduction block — distinguishes from Multifocal Motor Neuropathy (MMN)",
  ],
  [
    "##Blood Tests",
    "FBC, ESR, CRP — usually normal; rule out inflammatory/infective causes",
    "Serum CK — mildly elevated (2–4× normal) due to denervation atrophy",
    "TFTs — exclude thyrotoxic myopathy",
    "Serum electrolytes — hypokalaemia (periodic paralysis)",
    "Vitamin B12, folate — exclude deficiency neuropathy",
    "Anti-GM1 antibodies — if raised, suggests MMN (treatable!)",
    "Anti-Hu, Anti-amphiphysin — paraneoplastic screen",
    "Androgen receptor (AR) CAG repeat — if Kennedy disease suspected",
    "Heavy metal screen (lead, mercury) — if exposure history",
    "HIV serology — HIV-associated brachial amyotrophic diplegia described",
    "SMN1 gene analysis — rule out adult SMA",
  ],
  "Blood Tests"
);

// ─── SLIDE 14: IMAGING & CRITERIA ────────────────────────────────────────────
contentSlide(
  "Investigations — Neuroimaging & Diagnostic Criteria",
  [
    "##MRI Brain",
    "Bilateral T2 hypointensity along corticospinal tracts (iron deposition)",
    "Atrophy of precentral gyrus bilaterally in established disease",
    "T1 cortical thinning in motor strip",
    "Important to EXCLUDE mimics: brainstem tumours, cortical lesions",
    "##MRI Cervical Spine",
    "Essential to exclude structural causes (disc herniation, osteophytes, tumour)",
    "May show mild cord atrophy at C5–C6 in FAS",
    "Cervical flexion MRI: anterior dural shift in Hirayama — differentiates",
    "##MRI Brachial Plexus",
    "When unilateral, asymmetric, or pain is present — exclude plexopathy",
    "T2 STIR: increased signal in affected roots in CIDP or Parsonage-Turner",
  ],
  [
    "##Diagnostic Criteria (El Escorial / Awaji-Shima)",
    "Definite ALS: UMN + LMN signs in 3 regions",
    "Probable ALS: UMN + LMN in 2 regions; UMN signs rostral to LMN",
    "Possible ALS: UMN + LMN in 1 region",
    "FAS often enters as 'possible' or 'probable' ALS early on",
    "##Pulmonary Function Tests",
    "Forced Vital Capacity (FVC) <50% predicted — trigger for NIV",
    "Sniff Nasal Inspiratory Pressure (SNIP) — diaphragm assessment",
    "Nocturnal oximetry — detect nocturnal hypoventilation",
    "##CSF Analysis",
    "Usually NORMAL — helps exclude inflammatory/infective causes",
    "May show mildly elevated protein (non-specific)",
    "##Muscle Biopsy",
    "If IBM or inflammatory myopathy suspected — shows denervation features in MND",
    "Grouped atrophy, type I and II fibre grouping, angular fibres",
  ],
  "Criteria & PFTs"
);

// ─── SLIDE 15: MANAGEMENT OVERVIEW ───────────────────────────────────────────
contentSlide(
  "Management Overview",
  [
    "##Disease-Modifying",
    "Riluzole 50 mg BD — anti-glutamatergic; modest survival benefit (+3–6 months)",
    "Edaravone (IV) — anti-oxidant; slows functional decline in selected patients",
    "##Respiratory Support",
    "Non-invasive ventilation (NIV/BiPAP) when FVC <50%",
    "Tracheostomy ventilation in selected patients for prolonged survival",
    "Cough assist devices / manually assisted cough",
    "##Nutritional Support",
    "PEG insertion early (when FVC >50%) — prophylactic feeding",
    "High-calorie diet — weight maintenance improves prognosis",
    "##Communication",
    "AAC devices — speech generating devices as dysarthria worsens",
    "Eye-tracking technology for late-stage communication",
  ],
  [
    "##Physiotherapy & OT",
    "Upper limb orthoses — arm slings, balanced forearm orthosis",
    "Shoulder subluxation prevention strapping",
    "Passive ROM exercises to prevent contractures",
    "Wrist/finger splints for functional positioning",
    "##Symptom Management",
    "Sialorrhoea: Glycopyrronium, hyoscine, amitriptyline, Botulinum toxin",
    "Muscle cramps: Quinine, magnesium, mexiletine",
    "Spasticity: Baclofen, tizanidine, botulinum toxin",
    "Pain: Regular paracetamol, NSAIDs, opioids in late stage",
    "Emotional lability: Dextromethorphan/quinidine (Nuedexta), SSRIs",
    "##Prognosis",
    "Median survival FAS: 4–5 years (vs 2–3 years classic ALS)",
    "Better prognosis: younger age, limb onset, longer diagnostic delay",
    "Death from respiratory failure / aspiration pneumonia",
  ],
  "Physiotherapy & Prognosis"
);

// ─── SLIDE 16: SUMMARY ───────────────────────────────────────────────────────
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    { text:"Bibrachial MND = Flail Arm Syndrome = Brachial Amyotrophic Diplegia — a regional ALS variant", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"LMN degeneration at C5–T1 anterior horn cells → proximal (C5–C6) followed by distal (C7–T1) arm weakness", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"Prion-like TDP-43 / SOD1 spread, excitotoxicity, and oxidative stress underlie pathogenesis", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"Bilateral flail arms, fasciculations, wasting — NO sensory loss, NO bladder symptoms", options:{ bullet:true, color:ACCENT, fontSize:14, fontFace:"Calibri", breakLine:true, bold:true } },
    { text:"Reflex dissociation: absent biceps jerk (LMN) + brisk knee jerk (UMN) is a diagnostic clue", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"DD: Kennedy disease, MMN, IBM, Hirayama, brachial plexopathy, cervical myelopathy", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"Investigations: EMG (active denervation, no conduction block), normal sensory NCS, MRI brain + cervical spine", options:{ bullet:true, color:WHITE, fontSize:14, fontFace:"Calibri", breakLine:true } },
    { text:"Median survival 4–5 years; better than classic ALS; death from respiratory failure", options:{ bullet:true, color:ACCENT, fontSize:14, fontFace:"Calibri", breakLine:true, bold:true } },
  ];
  s.addText(bullets, { x:0.8, y:0.85, w:11.7, h:4.5, fontFace:"Calibri" });
}

// ─── WRITE ────────────────────────────────────────────────────────────────────
pres.writeFile({ fileName: "/home/daytona/workspace/bibrachial-mnd/Bibrachial_MND_Flail_Arm_Syndrome.pptx" })
  .then(() => console.log("PPTX written successfully"))
  .catch(e => { console.error(e); process.exit(1); });

cd /home/daytona/workspace/bibrachial-mnd && node build_pptx.js 2>&1

ls -lh /home/daytona/workspace/bibrachial-mnd/Bibrachial_MND_Flail_Arm_Syndrome.…