Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on 7 patients suffering from chronic renal failure (2 females, 5 males; aged 35-75 (phi 53.5) years) who showed severe neuromyeloencephalopathy (NME) after high doses of a new Henle's loop diuretic, Muzolimine. The temporal and phenomenological development of these systems was strikingly parallel. The neurological deficit was revealed on neurophysiological, neuroradiological and in 2 cases on neuropathological tests (gross demyelinisation of the posterior column, mainly of the fasciculus gracilis, less in the lateral corticospinal tract and in some spinal roots). The critical drug dose for first neurological impairment was 52 g on average; at this point the patients had been treated for 78 days. The maximal daily dose was 1.440 mg. Dominant clinical features were pallhypaesthesia, ataxia, signs of peripheral neuropathy in combination with hyperreflexia and progressive para- to tetraspastic paresis. Constellation of symptoms, course of disease and findings of additional investigations, especially those of neuropathology, very much resemble Vitamin B12 deficiency and SMON-(Subacute Myelo Optic Neuropathy) syndrome. The rare entity of Muzolimine-NME is discussed in respect to other endogenous and exotoxic neuromyelopathies. We present the hypothesis of a toxic, partially dialysable metabolite of Muzolimine.
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PMID:[Neuromyeloencephalopathy caused by high-dose muzolimine medication in patients with renal failure]. 215 46

Clioquinol is still consumed in India in considerable amounts but no new case reports have appeared since 1977. A review is made for a regional neurotoxicology group of an enquiry that we conducted in Bombay to gather information regarding SMON, spanning the period of 1967 to 1976. Nine patients were diagnosed with a variable degree of confidence as suffering from SMON, two from a retrospective search and seven after a prospective watch for the disease. Myelopathy with predominant more distal dysesthesia was seen more often than the full-blown picture of SMON. The peripheral neuropathy component (N) diagnosed clinically or electrophysiologically was seen only once. Pyramidal tract disturbances and resulting spasticity was as striking as posterior column disorder and sensory ataxia. Subacute myelopathy was seen in six patients, optico-myelopathy in two, and myeloneuropathy only once. It was clear that clioquinol has potential neurotoxicity, but no definitive explanation was forthcoming about the vast difference in the prevalence of SMON as reported from Japan and seen by us in Bombay.
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PMID:SMON as seen from Bombay. 609 94