Peripheral Neuropathies

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PN Basics - Nerve Nuisance Intro

  • Peripheral Neuropathy (PN): Disorder of peripheral nerves (motor, sensory, autonomic).
  • Classification (Distribution):
    • Mononeuropathy: Single nerve (e.g., Carpal Tunnel).
    • Mononeuritis Multiplex: ≥2 separate nerves (e.g., Vasculitis).
    • Polyneuropathy: Symmetrical, distal (e.g., Diabetic).
  • Classification (Pathology):
    • Axonal:
      • Primary axon damage.
      • ↓CMAP/SNAP amplitude.
      • NCV normal/mildly ↓.
    • Demyelinating:
      • Primary myelin damage.
      • ↓NCV, ↑distal latencies.
      • Conduction block common.
  • Pathophysiology:
    • Wallerian Degeneration: Axon breakdown distal to injury.
    • Segmental Demyelination: Focal myelin loss.
    • Neuronopathy: Nerve cell body (soma) damage.

Healthy vs. demyelinating vs. axonal neuropathy

⭐ Demyelinating neuropathies primarily slow conduction velocity (CV) & prolong distal latencies. Axonal neuropathies reduce SNAP/CMAP amplitude.

Causes & Clues - The Why & How

  • Metabolic:
    • Diabetes Mellitus (DM): Most common; length-dependent.
    • Uremia (CKD).
  • Immune-Mediated:
    • Guillain-Barré Syndrome (GBS): Acute, ascending.
    • Vasculitis: Mononeuritis multiplex.
    • Paraneoplastic syndromes.
  • Infectious:
    • Leprosy: Affects cool areas.
    • HIV: Distal symmetric.
    • Lyme disease: Cranial nerves, radiculopathy.
    • Diphtheria: Exotoxin.
  • Hereditary:
    • Charcot-Marie-Tooth (CMT): Distal weakness/atrophy.
  • Toxic:
    • Alcohol.
    • Lead: Wrist/foot drop.
    • Drugs: Vincristine, Isoniazid (INH).
  • Nutritional:
    • Deficiency: Vit B12, B1, B6, E.
    • Excess: Vit B6 (sensory).
  • Other:
    • Amyloidosis: Protein infiltration.

⭐ Diabetes mellitus is the leading cause of peripheral neuropathy in developed countries, typically presenting as a length-dependent sensorimotor polyneuropathy.

Signs & Sleuthing - Spotting the Damage

  • Clinical Features:
    • Sensory:
      • Negative: Numbness, ↓proprioception, ataxia.
      • Positive: Paraesthesia, dysesthesia, allodynia, neuropathic pain.
    • Motor: Weakness (distal > proximal), atrophy, fasciculations, cramps, ↓/absent reflexes.
    • Autonomic: Orthostatic hypotension, gastroparesis, bowel/bladder/sweating issues, erectile dysfunction.
  • Patterns:
    • Stocking-glove, length-dependent.
    • Mononeuropathy, Mononeuritis multiplex, Radiculopathy. Stocking-glove sensory loss pattern
  • Diagnosis:
    • History & Neuro Exam: Sensory, motor, reflexes, gait.
    • Electrodiagnosis:
      • NCS: ↓Velocity, ↓amplitude, ↑latency, conduction block.
      • EMG: Fibrillations, positive sharp waves, MUP changes.
    • Nerve Biopsy Indications: Vasculitis, amyloidosis, CIDP, leprosy.

⭐ NCS differentiates axonal (↓amplitude) vs. demyelinating (↓velocity, ↑latency, conduction block) neuropathies, guiding investigation.

Key Syndromes - Neuropathy Notables

  • Guillain-Barré Syndrome (GBS): Acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Ascending paralysis, areflexia. 📌 GBS: Ground to Brain Syndrome. CSF: Albuminocytologic dissociation. Rx: IVIG/Plasmapheresis.
  • Diabetic Neuropathy: Distal Symmetric Polyneuropathy (DSPN) "stocking-glove"; autonomic (gastroparesis); focal (CN III palsy). Rx: Glycemic control, pain relief.
  • Charcot-Marie-Tooth (CMT): Hereditary Motor & Sensory Neuropathy (HMSN). Pes cavus, distal muscle wasting ("stork legs"). Biopsy (CMT1A): "Onion bulb".
  • Bell's Palsy: Idiopathic CN VII palsy. Acute unilateral facial weakness (upper/lower face). Rx: Corticosteroids +/- antivirals.
  • Carpal Tunnel Syndrome (CTS): Median nerve entrapment at wrist. Pain/paresthesia (thumb, index, middle fingers). Tinel's/Phalen's signs. Rx: Splinting, steroids, surgery.

⭐ Albuminocytologic dissociation (elevated CSF protein with normal or minimally increased white blood cell count) is a hallmark finding in Guillain-Barré Syndrome, typically seen after the first week.

High‑Yield Points - ⚡ Biggest Takeaways

  • Guillain-Barré Syndrome (GBS): Ascending paralysis, areflexia, albuminocytologic dissociation in CSF.
  • Charcot-Marie-Tooth (CMT): Most common hereditary neuropathy; pes cavus, distal muscle atrophy.
  • Diabetic Neuropathy: Most common cause; stocking-glove sensory loss, autonomic dysfunction.
  • Bell's Palsy: Unilateral LMN facial nerve (CN VII) palsy; sudden onset, good prognosis.
  • Carpal Tunnel Syndrome: Median nerve compression at wrist; Phalen's/Tinel's positive, nocturnal symptoms.
  • Vitamin B12 Deficiency: Subacute combined degeneration; dorsal columns, corticospinal tracts, peripheral neuropathy.
  • Leprosy (Hansen's Disease): Mononeuritis multiplex, anesthetic skin patches, thickened nerves.

Practice Questions: Peripheral Neuropathies

Test your understanding with these related questions

A 45-year-old woman presents with burning pain and numbness in the wrist and hand. Tinel's sign is positive. What is the most likely diagnosis?

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Flashcards: Peripheral Neuropathies

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The most common type of HIV-associated peripheral neuropathy is _____ polyneuropathy.

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The most common type of HIV-associated peripheral neuropathy is _____ polyneuropathy.

distal symmetric

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