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

~/bibrachial-mnd-ppt/build.js

const pptxgen = require("pptxgenjs");

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pres.title = "Bibrachial MND";
pres.author = "Medical Education";

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// ═══════════════════════════════════════════════════════════════
//  SLIDE 1 – TITLE
// ═══════════════════════════════════════════════════════════════
titleSlide(
  "Bibrachial MND",
  "Flail Arm Syndrome — Proximal & Distal Mechanisms"
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 2 – WHAT IS BIBRACHIAL MND?
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "What is Bibrachial MND?",
  [
    "A segmental variant of Amyotrophic Lateral Sclerosis (ALS) — also called Motor Neuron Disease (MND)",
    "Characterized by bilateral flaccid weakness, wasting, and fasciculations predominantly confined to both arms and shoulder girdle",
    'Also known as: "Flail Arm Syndrome" (FAS), "Dangling Arm Syndrome", "Man-in-a-Barrel Syndrome", or "Bibrachial Palsy"',
    "Accounts for up to 10% of all ALS cases; far more common in men (M:F ratio ~9:1)",
    "Carries a significantly better prognosis than classic ALS — longer median survival (≥4 years vs ~3 years)",
    "Brain (1969) coined the term Motor Neuron Disease specifically for combined UMN + LMN degeneration",
    "In Bibrachial MND: LMN degeneration dominates in bilateral cervical anterior horn cells (C5–C8)",
  ]
);

// ═══════════════════════════════════════════════════════════════
//  SECTION BANNER
// ═══════════════════════════════════════════════════════════════
sectionBanner("Pathophysiology\nHow Does It Occur?");

// ═══════════════════════════════════════════════════════════════
//  SLIDE 3 – UPPER vs LOWER MOTOR NEURON
// ═══════════════════════════════════════════════════════════════
twoColSlide(
  "UMN vs LMN Anatomy — The Foundation",
  "Upper Motor Neuron (UMN)",
  [
    "Cell body in primary motor cortex (Brodmann area 4) and premotor areas (area 6)",
    "Betz cells: giant pyramidal neurons in layer 5",
    "Axons form corticospinal tract — decussate at medullary pyramids",
    "Descend in lateral funiculus of spinal cord",
    "Synapse on anterior horn cells (LMNs)",
    "UMN signs: spasticity, hyperreflexia, Babinski, clonus",
    "In bibrachial MND: UMN signs are minimal — predominant LMN picture",
  ],
  "Lower Motor Neuron (LMN)",
  [
    "Cell bodies in anterior horn of spinal cord (C4–T1 for upper limbs)",
    "Directly innervate skeletal muscles via neuromuscular junction",
    "Organized into motor columns by somatotopic map",
    "Cervical enlargement (C5–C8) governs shoulder & arm muscles",
    "LMN signs: flaccid weakness, atrophy, fasciculations, areflexia",
    "In bibrachial MND: selective degeneration of C5–C8 anterior horn cells",
    "Legs are relatively spared (lumbar LMNs intact early in disease)",
  ]
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 4 – PATHOMECHANISM
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Pathomechanism of Bibrachial MND",
  [
    "SELECTIVE ANTERIOR HORN DEGENERATION: Loss of lower motor neurons in the cervical cord (C4–T1) bilaterally with relative sparing of lumbar LMNs",
    "PROTEIN AGGREGATION: TDP-43 (Tar DNA binding protein) forms cytoplasmic inclusions in anterior horn cells — hallmark of ~97% of sporadic ALS",
    "OXIDATIVE STRESS: SOD1 (Superoxide Dismutase-1) mutations → accumulation of toxic superoxide radicals → motor neuron death (familial cases)",
    "EXCITOTOXICITY: Glutamate-mediated over-activation of AMPA/NMDA receptors → calcium influx → mitochondrial dysfunction → apoptosis",
    "AXONAL TRANSPORT FAILURE: Mutations in dynactin (p150), KIF5A disrupt retrograde transport → organelle accumulation → neuron death",
    "NEUROINFLAMMATION: Microglial activation and astrocyte dysfunction amplify motor neuron injury",
    "SEGMENTAL SPECIFICITY: Cervical cord neurons may be uniquely vulnerable due to high metabolic demands of long axons innervating arms",
  ],
  { fontSize: 14 }
);

// ═══════════════════════════════════════════════════════════════
//  SECTION BANNER
// ═══════════════════════════════════════════════════════════════
sectionBanner("Proximal vs Distal\nPatterns of Involvement");

// ═══════════════════════════════════════════════════════════════
//  SLIDE 5 – PROXIMAL PATTERN
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Proximal Pattern — Shoulder Girdle Onset",
  [
    "MUSCLES AFFECTED: Deltoid, supraspinatus, infraspinatus, serratus anterior, rhomboids, trapezius, biceps, triceps",
    "NEUROANATOMY: Degeneration of C5–C6 anterior horn cells; innervation of shoulder girdle by upper cervical myotomes",
    "CLINICAL FEATURES: Inability to raise arms above the head; scapular winging; dropped-shoulder appearance",
    "FASCICULATIONS: Prominently visible in deltoid and biceps in early stages",
    "ONSET PATTERN: Arms appear 'dangling' at the sides — the classic 'dangling arm sign'; patients use gravity to swing arms for tasks",
    "REFLEXES: Biceps and brachioradialis reflexes absent or reduced (LMN at C5–C6); paradoxically, triceps (C7) may be preserved early",
    "EMG FINDING: Denervation (fibrillations, positive sharp waves) and reinnervation (giant MUPs) in proximal shoulder muscles with reduced interference pattern",
    "PROGNOSIS: Proximal pattern — slower disease progression compared to bulbar or distal onset",
  ],
  { fontSize: 13.5 }
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 6 – DISTAL PATTERN
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Distal Pattern — Hand & Forearm Onset",
  [
    "MUSCLES AFFECTED: Thenar eminence (APB, FPB, OP), hypothenar (ADM, FDM), dorsal & palmar interossei, lumbricals, forearm flexors",
    "NEUROANATOMY: C7–C8/T1 anterior horn cell degeneration; thenar wasting = classical early distal sign in ALS",
    "CLINICAL FEATURES: Weakness and wasting of intrinsic hand muscles; difficulty with fine motor tasks (buttons, writing); claw hand appearance",
    "SPLIT-HAND SIGN: Thenar (median-innervated) muscles waste preferentially over hypothenar (ulnar-innervated) muscles — a characteristic ALS finding",
    "FASCICULATIONS: Small-amplitude fasciculations in intrinsic hand muscles and forearm",
    "REFLEXES: Absent finger and wrist reflexes; note — triceps reflex may paradoxically be preserved or brisk (reflecting cervical UMN component)",
    "EMG: Active denervation in first dorsal interosseous and APB; abnormal recruitment in ulnar and median nerve-innervated muscles",
    "SPREAD: Disease typically progresses contiguously — from distal to proximal within the same limb, then crosses to the contralateral side",
  ],
  { fontSize: 13.5 }
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 7 – COMPARISON TABLE
// ═══════════════════════════════════════════════════════════════
twoColSlide(
  "Proximal vs Distal Bibrachial MND — Comparison",
  "PROXIMAL (C5–C6 Dominant)",
  [
    "Onset: Shoulder girdle, upper arm",
    "Key muscles: Deltoid, biceps, supraspinatus",
    "Myotomes: C5–C6 anterior horn",
    "Absent reflexes: Biceps, brachioradialis",
    "Clinical sign: Dangling arm, scapular winging",
    "EMG: Denervation in deltoid/biceps",
    "Progression: Tends to spread distally over months",
    "Swallowing: Spared longer",
    "Prognosis: Relatively slower progression",
  ],
  "DISTAL (C7–T1 Dominant)",
  [
    "Onset: Hands, intrinsic muscles",
    "Key muscles: Thenar, interossei, ADM",
    "Myotomes: C7–C8–T1 anterior horn",
    "Absent reflexes: Finger jerks, wrist flexors",
    "Clinical sign: Claw hand, split-hand pattern",
    "EMG: Denervation in FDI, APB, ADM",
    "Progression: Spreads proximally then bilaterally",
    "Fasciculations: Fine, in interossei",
    "Prognosis: May progress to legs eventually",
  ]
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 8 – CLINICAL FEATURES
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Clinical Features of Bibrachial MND",
  [
    "DEMOGRAPHICS: Middle-aged to older adults; male predominance (M:F ~9:1); mean age of onset ~65 years",
    "PRESENTATION: Gradual bilateral symmetric arm weakness; legs and bulbar function relatively preserved for months–years",
    "MOTOR EXAM: Profound atrophy + fasciculations of arm/shoulder; flaccid tone; reduced/absent upper limb reflexes",
    "PARADOXICAL REFLEXES: Despite predominant LMN picture, brisk jaw jerk or lower limb hyperreflexia may indicate coexisting UMN involvement",
    "SENSORY EXAM: Normal — absence of sensory signs distinguishes MND from cervical myelopathy and neuropathy",
    "BULBAR FUNCTION: Preserved early; speech and swallowing typically intact until late disease",
    "COGNITION: Generally preserved; but up to 50% may show subtle frontal executive dysfunction without frank dementia",
    "RESPIRATORY: Diaphragmatic weakness develops later — orthopnea, reduced FVC in supine, morning headaches from CO2 retention",
  ],
  { fontSize: 13.5 }
);

// ═══════════════════════════════════════════════════════════════
//  SECTION BANNER
// ═══════════════════════════════════════════════════════════════
sectionBanner("Genetics & Pathology");

// ═══════════════════════════════════════════════════════════════
//  SLIDE 9 – GENETICS
// ═══════════════════════════════════════════════════════════════
highlightSlide("Key Genetic Mutations in ALS / Bibrachial MND", [
  { label: "SOD1 (ALS1)", value: "Chr 21q22\nAutosomally dominant\nOxidative stress ↑\n~20% of familial ALS", color: C.teal },
  { label: "TDP-43 (ALS10)", value: "Chr 1q\nRNA-binding protein\nCytoplasmic inclusions\nAD, FTD overlap", color: C.slideAccent },
  { label: "C9orf72", value: "Chr 9p21\nHexanucleotide repeat\nMost common familial\nALS + FTD overlap", color: C.red },
  { label: "FUS/TLS (ALS6)", value: "Chr 16p\nRNA metabolism\nAD (some AR)\nFTD overlap", color: C.teal },
  { label: "TARDBP/TDP-43", value: "Protein aggregation\nPathognomonic of\n~97% sporadic ALS\n& Bibrachial MND", color: C.slideAccent },
  { label: "KIF5A (ALS25)", value: "Chr 12q13.3\nAxonal transport\nMotor kinesin gene\nSlower progression", color: C.green },
]);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 10 – DIAGNOSIS
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Diagnosis of Bibrachial MND",
  [
    "CLINICAL DIAGNOSIS: No specific biomarker; based on history + examination showing progressive bilateral arm LMN weakness without sensory signs",
    "EL ESCORIAL / AWAJI-SHIMA CRITERIA: Requires LMN + UMN evidence in ≥2 regions (bulbar, cervical, thoracic, lumbar) with progressive spread",
    "EMG/NCS: Key investigation — shows denervation (fibrillations, PSWs, fasciculations) and reinnervation (giant MUPs) bilaterally in arm muscles; nerve conductions normal",
    "MRI BRAIN & SPINE: Exclude cervical spondylotic myelopathy, Chiari malformation, cord tumors — important differential. T2 hyperintensity in corticospinal tracts on MRI brain supports ALS",
    "BLOOD TESTS: Exclude mimics — anti-GM1 antibodies (multifocal motor neuropathy), CK (may be mildly elevated), thyroid, B12, VDRL, anti-HMGCR",
    "CSF BIOMARKERS: Elevated neurofilament light (NfL) and phosphorylated neurofilament heavy (pNfH) in CSF and plasma — promising emerging biomarkers",
    "GENETIC TESTING: Offered to all patients — screen for SOD1, C9orf72, TARDBP, FUS mutations",
    "DIFFERENTIAL: Cervical spondylotic myelopathy, multifocal motor neuropathy (MMN), Kennedy disease, hirayama disease, inclusion body myositis",
  ],
  { fontSize: 13 }
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 11 – DIFFERENTIALS
// ═══════════════════════════════════════════════════════════════
twoColSlide(
  "Differential Diagnosis",
  "Must-Exclude Mimics",
  [
    "Cervical spondylotic myelopathy — sensory signs, spinal cord compression on MRI",
    "Multifocal Motor Neuropathy (MMN) — conduction block on NCS; anti-GM1 antibodies; responds to IVIG",
    "Kennedy Disease (SBMA) — X-linked; sensory involvement; gynecomastia; CAG repeat in AR gene",
    "Hirayama Disease — young men; monomelic; neck flexion worsens on MRI (dynamic changes)",
    "Brachial Neuritis (Parsonage-Turner) — acute painful onset; unilateral; self-limiting",
    "Inclusion Body Myositis (IBM) — finger flexor + quadriceps pattern; CK elevated; biopsy diagnostic",
    "Cervical cord infarct — anterior spinal artery; acute onset; bibrachial paralysis possible",
  ],
  "Distinguishing Features",
  [
    "No sensory loss distinguishes MND from polyneuropathy and cord lesions",
    "Normal NCS distinguishes from MMN and polyneuropathy",
    "Bilateral onset distinguishes from Hirayama (monomelic)",
    "Gradual progression distinguishes from Guillain-Barré",
    "Absence of pain distinguishes from brachial neuritis",
    "EMG: widespread denervation in asymptomatic muscles — hallmark of dissemination in MND",
    "No ophthalmopathy/bladder dysfunction unlike MS or spinal lesions",
  ]
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 12 – MANAGEMENT
// ═══════════════════════════════════════════════════════════════
contentSlide(
  "Management of Bibrachial MND",
  [
    "RILUZOLE: Only disease-modifying drug; glutamate antagonist; extends survival by ~2–3 months; 100 mg/day orally; approved for ALS/MND (Cochrane 2012)",
    "EDARAVONE: Free radical scavenger; IV infusion; slows functional decline in select early ALS patients; approved in Japan, USA, Canada",
    "MULTIDISCIPLINARY TEAM: Neurologist + physiotherapist + OT + speech therapist + dietitian + respiratory specialist + palliative care",
    "PHYSIOTHERAPY: Stretching, range-of-motion exercises for arms; orthotic supports (wrist/hand splints) to maintain function; sling for dangling arm",
    "RESPIRATORY SUPPORT: NIV (non-invasive ventilation) when FVC <50% or orthopnea develops; significantly improves quality of life and survival",
    "NUTRITIONAL SUPPORT: PEG (percutaneous endoscopic gastrostomy) if weight loss >10% or FVC >50%; dysphagia management with modified diet",
    "SPASTICITY / CRAMPS: Baclofen, tizanidine for spasticity; quinine sulfate for cramps; mexiletine for fasciculations/cramps",
    "PSYCHOLOGICAL SUPPORT: Depression and anxiety common; SSRIs, counseling, palliative care; advance care planning and ACP discussions",
  ],
  { fontSize: 13 }
);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 13 – PROGNOSIS
// ═══════════════════════════════════════════════════════════════
highlightSlide("Prognosis & Survival in Bibrachial MND", [
  { label: "Prevalence in ALS", value: "Up to 10% of all\nALS patients have\nFlail Arm Syndrome", color: C.teal },
  { label: "Sex Ratio", value: "M:F ~9:1\nStrongly male\npredominant variant", color: C.slideAccent },
  { label: "Median Survival", value: "Longer than classic\nALS — often ≥4 years\nfrom symptom onset", color: C.green },
  { label: "Classic ALS Survival", value: "Median ~2–4 years\n20% survive 5 yrs\n10% survive 10 yrs", color: C.red },
  { label: "Leg Involvement", value: "Lower limb spread\noccurs eventually\nbut delayed by years", color: C.teal },
  { label: "Cause of Death", value: "Respiratory failure\n(diaphragmatic +\nintercostal weakness)", color: C.slideAccent },
]);

// ═══════════════════════════════════════════════════════════════
//  SLIDE 14 – SUMMARY
// ═══════════════════════════════════════════════════════════════
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const takeaways = [
  { num: "01", text: "Bibrachial MND = Flail Arm Syndrome — a segmental ALS variant with bilateral arm LMN predominance, up to 10% of ALS cases" },
  { num: "02", text: "Caused by selective degeneration of C5–T1 anterior horn cells; TDP-43 aggregation is the pathological hallmark" },
  { num: "03", text: "Proximal pattern: deltoid/biceps wasting, dangling arm sign (C5–C6 myotomes); Distal pattern: thenar wasting, split-hand sign (C8–T1 myotomes)" },
  { num: "04", text: "No sensory signs; EMG shows widespread denervation + reinnervation; MRI cervical spine excludes spondylotic myelopathy" },
  { num: "05", text: "Treatment: Riluzole + MDT + NIV + physiotherapy + nutritional support; prognosis better than classic ALS" },
];

takeaways.forEach((t, i) => {
  const y = 1.25 + i * 1.05;
  sl14.addShape(pres.ShapeType.roundRect, { x: 0.4, y, w: 0.7, h: 0.7, fill: { color: C.amber }, rectRadius: 0.08 });
  sl14.addText(t.num, { x: 0.4, y, w: 0.7, h: 0.7, fontSize: 16, bold: true, color: C.deepNavy, fontFace: "Calibri", align: "center", valign: "middle" });
  sl14.addText(t.text, { x: 1.25, y: y + 0.05, w: 11.5, h: 0.65, fontSize: 14, color: C.white, fontFace: "Calibri", valign: "middle" });
});

// ═══════════════════════════════════════════════════════════════
//  WRITE FILE
// ═══════════════════════════════════════════════════════════════
const outPath = "/home/daytona/workspace/bibrachial-mnd-ppt/Bibrachial_MND.pptx";
pres.writeFile({ fileName: outPath }).then(() => {
  console.log("SUCCESS:", outPath);
}).catch(err => {
  console.error("ERROR:", err);
});

cd /home/daytona/workspace/bibrachial-mnd-ppt && node build.js

ls -lh /home/daytona/workspace/bibrachial-mnd-ppt/Bibrachial_MND.pptx