Polyneuropathy

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  • i. Time course: acute onset is nearly always diagnostic of an inflammatory, immunologic, toxic or vascular etiology; a polyneuropathy evolving slowly over many years is indicative of a hereditary or metabolic disease; most toxic, nutritional, and systemic diseases of nerve develop subacutely over weeks and months
  • ii. Acute motor paralysis with variable disturbance of sensory and autonomic function
    • 1. Guillan Barre
    • 2. porphyric polyneuropathy
    • 3. toxic polyneuropathyies (thallium)
    • 4. tick paralysis
    • 5. critical illness polyneuropathy
  • iii. subacute sensorimotor paralysis
    • 1. symmetrical
      • a. alcoholism, vitamin B12, pellagra
      • b. heavy metals: arsenic, lead, mercury, thallium
        • i. thallium intoxication causes engorgement of the cortical blood vessels affecting the pyramidal tracts, globus pallidus, and substantia nigra and also results in a dying back of peripheral axons; symptoms: vomiting, cardiac dysfunction, joint pain, diarrhea, headache, and abdominal pain followed by confusion and convulstions
      • c. drug toxicity: isoniazid, vincristine, cisplatin, amitriptyline
      • d. uremic polyneuropathy
      • e. chronic inflammatory demyelinating polyneuropathy (CIDP) (often accompanied by proximal muscle weakness and elevated CSF protein)
    • 2. asymmetric
      • a. diabetes
      • b. polyarteritis nodosa
      • c. sarcoid
      • d. ischemic neuropathy with peripheral vascular disease
      • e. Lyme disease
  • iv. Chronic sensorimotor polyneuropathy
    • 1. paraneoplatic (carcinoma, lymphoma, myeloma)
    • 2. uremia
    • 3. beriberi
    • 4. diabetes
    • 5. amyloidosis
    • 6. hypothyroidism
  • v. inherited polyneuropathies of mixed sensorimotor type
    • 1. idiopathic
      • a. Charcot Marie Tooth (AD)
      • b. Dejerine-Sottas (AR)
      • c. Roussy Levy (=Charcot Marie Tooth + spinocerebellar degeneration)
    • 2. inherited
      • a. Refsum
      • b. Metachromatic leukodystrophy
      • c. Krabbe
      • d. Adrenoleukodystrophy
      • e. Amyloid
      • f. Porphyric
      • g. Fabry
      • h. Tangier (autosomoal recessive with deficiency of high density lipoprotein, low cholesterol and high triglyceride concentrations with orange tonsils; deposition of massive amounts of cholesterol occur in the organs and tissues; sensorimotor neuropathy affecting predominantly the distal upper extremities); Remember: Tang(ier) is a healthy orange breakfast drink that may keep arteries and tissues free from depositing lipid and cholesterol
      • i. Bassen-Kornzweig (abetalipoproteinemia) syndrome is an absence of very low density lipoproteins (VLDL) and low density lipoproteins (LDL) with extensive CNS and peripheral demyelination and causes retinitis pigmentosa, acanthocytosis, high arched palate, pes cavus, scoliosis, and spinocerebellar signs; patient will have trouble with fat absorption; it is due to a mutation of the microsomal triglyceride transfer protein (MTP); Remember: eating ‘Bass’ and ‘Korn’ may lower your lipid profile but may cause ‘arching’ problems with your bones (pes cavus, high arched palate, scoliosis)
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