Peripheral Nerve Disorders

Peripheral Nerve Disorders

Peripheral Nerve Disorders

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Peripheral Nerve Disorders: Injury Patterns - Shocking Revelations

  • Nerve Fiber Components: Axon, Myelin sheath (Schwann cells in PNS), Connective tissues (Endoneurium, Perineurium, Epineurium).
  • Key Injury Responses:
    • Wallerian Degeneration: Occurs distal to axonal transection or severe crush.
      • Axon & myelin breakdown; phagocytosis by macrophages.
      • Central chromatolysis (cell body swelling, Nissl dispersion).
      • Proximal stump attempts regeneration (axonal sprouting).
    • Segmental Demyelination: Myelin loss with axon preservation.
      • Caused by Schwann cell dysfunction/damage (e.g., Guillain-Barré Syndrome, diphtheria toxin).
      • Results in ↓ conduction velocity; remyelination is possible.
    • Axonal Degeneration (Dying-back Neuropathy): Distal parts of axon degenerate first, progressing proximally.
      • Often due to metabolic derangements or toxic exposures. Peripheral nerve degeneration types

Sunderland Classification (Grades I-V) is crucial for categorizing nerve injury severity, directly impacting prognosis and guiding management. Grade I (neurapraxia) involves temporary conduction block with full recovery.

Peripheral Nerve Disorders: Inflammatory - Immune System Mayhem

  • Guillain-Barré Syndrome (GBS)
    • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP).
    • Rapidly progressive, ascending symmetrical weakness/paralysis; often post-infectious (e.g., Campylobacter jejuni due to molecular mimicry).
    • CSF: ↑ Protein (albuminocytologic dissociation), normal cells after 1st week.
    • Variant: Miller Fisher Syndrome (ophthalmoplegia, ataxia, areflexia).
    • Treatment: IVIg, plasmapheresis. Steroids NOT effective.
  • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
    • Chronic (>8 weeks), progressive or relapsing-remitting sensorimotor neuropathy.
    • Symmetrical proximal & distal weakness, sensory loss.
    • Nerve biopsy:

Peripheral Nerve Disorders: Metabolic & Hereditary - Sweet & Inherited Woes

  • Diabetic Neuropathy: Most common metabolic neuropathy.

    • Pathogenesis: Advanced glycation end-products (AGEs), sorbitol accumulation, oxidative stress, microangiopathy.
    • Patterns:
      • Distal Symmetrical Polyneuropathy (DSPN): "Stocking-glove" sensory loss (most common).
      • Autonomic Neuropathy: Gastroparesis, orthostatic hypotension.
      • Mononeuropathies: Cranial nerves (CN III, VI), focal limb.
    • Morphology: Axonal degeneration, segmental demyelination, endoneurial arteriolar hyalinization.
  • Hereditary Sensory & Motor Neuropathies (HSMN) / Charcot-Marie-Tooth (CMT):

    • CMT1 (AD): Demyelinating. PMP22 gene duplication (1A) most common.
      • Features: ↓ Nerve Conduction Velocity (NCV), palpable nerves, pes cavus, "stork leg" deformity.
      • Morphology: "Onion bulb" formations (repetitive demyelination & remyelination).
    • CMT2 (AD): Axonal. Normal/mildly ↓ NCV. MFN2 mutations common.
    • CMTX (X-linked): Demyelinating. GJB1 (connexin-32) mutations.
    • Hereditary Neuropathy with liability to Pressure Palsies (HNPP): PMP22 deletion. Recurrent focal neuropathies. Tomacula ("sausage-like" myelin thickenings).

Onion bulb formation in Charcot-Marie-Tooth disease

CMT1A, due to PMP22 gene duplication, is the most common inherited neuropathy, characterized by hypertrophic "onion bulb" Schwann cell changes visible on biopsy.

Peripheral Nerve Disorders: Other Key Types - Traps, Toxins & Bugs

  • Entrapment Neuropathies (Traps):
    • Carpal Tunnel Syndrome (CTS): Median nerve at wrist; Phalen's, Tinel's signs.
    • Saturday Night Palsy: Radial nerve compression (spiral groove); wrist drop.
    • Meralgia Paresthetica: Lateral femoral cutaneous nerve under inguinal ligament.
    • Tarsal Tunnel Syndrome: Tibial nerve compression at ankle.
  • Toxic Neuropathies:
    • Lead: Predominantly motor neuropathy; wrist drop, foot drop.
    • Arsenic: Painful sensorimotor neuropathy; Mees' lines on nails.
    • Alcohol: Chronic use; axonal degeneration, painful sensory > motor.
    • Drugs: Isoniazid (give B6), Vincristine, Amiodarone.
  • Infectious Neuropathies (Bugs):
    • Leprosy (Hansen's Disease): M. leprae; affects cool areas, palpable thickened nerves, sensory loss.

      ⭐ Leprosy is a major global cause of peripheral neuropathy, with characteristic anaesthetic skin patches and thickened nerves.

    • Diphtheria: C. diphtheriae exotoxin; bulbar palsy, polyneuropathy.
    • Lyme Disease: B. burgdorferi; facial nerve palsy (often bilateral). Skin lesions and necrosis in leprosy neuropathy

High‑Yield Points - ⚡ Biggest Takeaways

  • Guillain-Barré syndrome: Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), ascending paralysis, albumino-cytological dissociation in CSF.
  • Charcot-Marie-Tooth disease (CMT1A): PMP22 duplication, hereditary demyelinating neuropathy, pes cavus, onion bulbs.
  • Diabetic neuropathy: Most common; distal symmetric polyneuropathy ("stocking-glove"), axonal degeneration and demyelination.
  • Leprosy: M. leprae infects Schwann cells; Tuberculoid (localized) vs. Lepromatous (widespread anesthesia).
  • Wallerian degeneration: Anterograde axonal degeneration distal to injury; forms myelin ovoids.
  • Lead neuropathy: Predominantly motor neuropathy, causing wrist drop and foot drop.

Practice Questions: Peripheral Nerve Disorders

Test your understanding with these related questions

Bilateral loss of ankle jerk and extensor plantar response is seen in:

1 of 5

Flashcards: Peripheral Nerve Disorders

1/10

The earliest _____ evidence for Diffuse Axonal Injury appears at? (Bonus Q: And what is it?)12 hours

TAP TO REVEAL ANSWER

The earliest _____ evidence for Diffuse Axonal Injury appears at? (Bonus Q: And what is it?)12 hours

histological

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