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ACUTE NEUROMUSCULAR DISORDERS: Evolution over Days to Weeks

ACUTE NEUROPATHIES

• Guillain-Barré & variants
  • Demyelinating
    • Motor + Sensory: "Classic" Guillain-Barré
    • Motor
    • Sensory
  • Axonal
    • Motor + Sensory
    • Motor: Associated with
      • Infections: Campylobacter jejuni; Haemophilus influenzae
      • Serum antibodies: IgG vs GM1 or GalNAc-GD1a gangliosides
    • Sensory
    • Ataxic
    • Autonomic
  • Cranial Nerve Syndromes
    • Associated with serum IgG vs GQ1b ganglioside antibodies
      • Miller-Fisher: Ataxia; Areflexia; Ophthalmoplegia
      • Bickerstaff Brainstem Encephalitis
    • Associated with IgG vs GT1a antibodies
      • Pharyngo-cervico-brachial
    • Facial diplegia
• Paracarcinomatous Sensory (anti-Hu)
• Vasculitis
• Chronic neuropathies with occasional acute onset or relapse
  • CIDP
  • MMN

• Biological
  • Botulism
  • Buckthorn
  • Ciguatoxin
  • Diphtheria
  • Saxitoxin
  • Snake
  • Tetrodotoxin
  • Tick
• Chemical
  • Arsenic
  • Hexacarbons
  • Lead;
  • Organophosphates
  • Pyridoxine
  • Thallium
  • Vacor
• Drugs
  • Captopril (few case reports)
  • Gangliosides: GBS-like
  • Gold: Motor
  • Ifosfamide: Painful; Sensory
  • Lithium: Sensory-Motor
  • Nitrofurantoin: Motor
  • Suramin: Paresthesias & Weakness
  • Zimeldine: Paresthesias & Weakness

• Brucellosis
• Central European encephalitis: Spinal cord infection
• HIV (DILS)
• Leprosy: with Erythema nodosum leprosum
• Leptospirosis
• Lyme disease
• Poliomyelitis
• Rabies
• West Nile Virus
  

• Diabetes mellitus
• Maple syrup urine disease
  
• Nutritional deficiency (ANAN)
  • Thiamine
  • ? Pyridoxine
• Porphyria
• Tyrosinemia
  

ACUTE MYOPATHY & NMJ DISORDERS

• Myasthenia Gravis
• Carnitine Deficiency
• Myoglobinuria/ Rhabdomyolysis
• Myositis variants: anti-MAS; anti-SRP
• Periodic Paralysis
• Myosin deficiency myopathies
• Electrolyte disorders
  • Potassium: High or Low
  • Magnesium: High
  • PO₄: Low
  • Barium: Hypokalemic; External link

ACUTE MOTOR DYSFUNCTION: DIFFERENTIAL DIAGNOSIS (BELOW FORAMEN MAGNUM)

• Myopathy
  • Myosin deficiency (Critical illness myopathy)
  • Electrolyte Disorders
  • Carnitine Deficiency
  • Myoglobinuria/ Rhabdomyolysis
  • Periodic Paralysis
• Neuromuscular Blockade
  • Myasthenia Gravis
  • Pharmacologic blocking agents: Especially with long half-life in renal failure
• Neuropathy
  • Immune
    • Guillain-Barré Syndromes
      
    • Vasculitis
  • Porphyria
  • Toxins
  • Trauma
  • Infections
  • Intensive care (Critical illness) neuropathy
    • Not usually a cause of severe weakness
    • Patients may have painful sensory neuropathy without weakness
    • See: Myosin deficiency myopathy
• Spinal cord lesions
• Skeletal disorders
  • Aseptic necrosis of hips
  • Vertebral fractures

• Mild sensory-motor neuropathies often occur in severely ill patients in the intensive care unit.
• However, there is no strong evidence that the "Critical illness neuropathy" is ever severe enough to cause disabling weakness.
• Guillain-Barré is also unusual in this setting.
• Most ICU patients with respiratory failure due to muscle weakness, or severe generalized weakness with rapid onset have myopathies with myosin loss.
  • A muscle biopsy is usually necessary to make this diagnosis.