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More recently, an outbreak of optic and peripheral neuropathy closely resembling Strachan's syndrome occurred in Cuba from 1992 to 1993 following a loss of food and fuel imports from the former Soviet Union (Roman 1994). Fifty thousand people developed either isolated or combined optic neuropathy, painful sensory neuropathy, dorsal lateral myelopathy, sensory neural deafness, spastic paraparesis, dysphonia, and dysautonomia. Forty- five percent developed centrocecal scotoma and optic neuropathy only, often following a period of weight loss. A number of possibilities were proposed, including vitamin B-complex and thiamin deficiency, cyanide intoxication, viral infections, and mitochondrial deletions. Heavy alcohol and tobacco use
were found most frequently in those with optic neuropathy (tobacco-alcohol amblyopia). Clinical evidence of neuropathy was often lacking despite the severe symptoms (Thomas et al 1995).Sural nerve biopsy showed axonal degeneration of large myelinated fibers. Most patients responded to supplementation of B-complex vitamins
Vitamin E deficiency
Vitamin E is fat soluble and found in abundance in vegetable oils and wheat germ. It is carried in portal blood to the liver, and alpha- tocopherol transfer protein binds it and recycles vitamin E in the liver for incorporation into low density and very low density lipoproteins. The patients at risk for development of vitamin E deficiency include those with hypo or abetalipoproteinemia, other disorders of the pancreas and liver, such as cystic fibrosis, protein-calorie malnutrition, familial vitamin E deficiency, and other malabsorption states (Jackson et al 1996). Symptoms include areflexia, cerebellar ataxia, cutaneous sensory impairment, position and vibratory sense abnormalities and less commonly, ophthalmoplegia, muscle weakness, nystagmus, extensor plantar
responses, ptosis, and dysarthria. The peripheral neuropathy is usually limited to the legs and is mild, axonal, and sensorimotor in nature (Brin et al 1986).
continued to Diagnostic Evaluation
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