title: Cyanide in human disease: a review of clinical and laboratory evidence.
abstract: Experimental cyanide exposure in animals causes demyelination and circumstantial clinical and laboratory evidence suggest that there are human parallels. In Leber's hereditary optic atrophy there appears to be a defect in the conversion of cyanide to thiocyanate because of deficient rhodanese activity. For transmitters of the disease smoking carries the risk of blindness and in the most severely affected patients, there is diffuse neurological disease. It is possible that other hereditary optic atrophies (dominant and recessive) may also reflect inborn errors of cyanide metabolism. In the retrobulbar neuritis and optic atrophy of vitamin B12 deficiency there may be a conditional abnormality of cyanide metabolism in smokers, and likewise in so-called tobacco-alcohol amblyopia in which there are more complex nutritional deficiencies. Epidemiological evidence (differing sex ratios, excess of smokers) indicates that defective cyanide metabolism may contribute to the development of sub-acute combined degeneration of the cord in vitamin B12 deficiency. In protein-malnourished populations consuming large amounts of cyanide or cyanogens, viz. in tropical Africa where the staple diet includes cassava containing large amounts of linamarin, similar maladies occur as acquired disorders. There may be a similar explanation for lathyrism. The known pathways of human cyanide metabolism are reviewed and evidence supporting the clinical data is presented.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000550:Amblyopia / Q000209:etiology; D001259:Ataxia / Q000209:etiology; D002648:Child; D003486:Cyanides / Q000378:metabolism* / Q000633:toxicity; D005260:Female; D005333:Fetus / Q000187:drug effects; D006801:Humans; D008658:Inactivation, Metabolic; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008875:Middle Aged; D009896:Optic Atrophy / Q000209:etiology; D011247:Pregnancy; D013118:Spinal Cord Diseases / Q000209:etiology; D014029:Tobacco Use Disorder / Q000150:complications
chemical_list: D003486:Cyanides