Hostname: page-component-586b7cd67f-tf8b9 Total loading time: 0 Render date: 2024-11-25T07:13:16.414Z Has data issue: false hasContentIssue false

Pyridoxine Dependent Epilepsy with Iatrogenic Sensory Neuronopathy

Published online by Cambridge University Press:  18 September 2015

Richard S. McLachlan*
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, University Hospital, London
William F. Brown
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, University Hospital, London
*
University Hospital, 339 Windermere Road. London, Ontario N6A 5A5
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

An 18-year-old man was treated from birth with chronic high dose pyridoxine (vitamin B6) up to 2000 mg per day for pyridoxine-dependent seizures. Within two years of onset of treatment, he developed a sensory neuropathy which did not progress over the following 16 years. Electrophysiological studies were consistent with a pure sensory neuronopathy expressed as centripetal degeneration of processes of the dorsal root ganglion cells.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1995

References

1. Hunt, AD Jr, Stokes, J Jr, McCrory, WW, Stroud, HH.Pyridoxine dependency: report of a case of intractable convulsions in an infant controlled by pyridoxine. Pediatrics 1954; 13: 140145.CrossRefGoogle Scholar
2. Mikati, MA, Trevathan, E, Krishnamoorthy, KS, Lombroso, CT.Pyridoxine-dependent epilepsy: EEG investigation and long-term follow-up. Electroencephalogr Clin Neurophysiol 1991; 78: 215221.CrossRefGoogle ScholarPubMed
3. Schaumburg, H, Kaplan, J, Windebank, A, et al. Sensory neuropathy from pyridoxine abuse. N Engl J Med 1983; 309: 445448.CrossRefGoogle ScholarPubMed
4. Parry, GJ, Bredesen, DE.Sensory neuropathy with low dose pyridoxine. Neurology 1985; 35: 14661468.CrossRefGoogle ScholarPubMed
5. Dalton, K, Dalton, JT.Characteristics of pyridoxine overdose neuropathy syndrome. Acta Neurol Scand 1987; 76: 811.CrossRefGoogle ScholarPubMed
6. Xu, Y, Sladky, JT, Brown, MJ.Dose-dependent expression of neuronopathy after experimental pyridoxine intoxication. Neurology 1989; 39: 10771083.CrossRefGoogle ScholarPubMed
7. Berger, AR, Schaumburg, HH, Schroeder, C, et al. Dose response, coasting, and differential fibre vulnerability in human toxic neuropathy: a prospective study of pyridoxine neurotoxicity. Neurology 1992; 42: 13671370.CrossRefGoogle ScholarPubMed
8. Albin, RL, Alpers, JW, Greenberg, HS, et al. Acute sensory neuropathy-neuronopathy from pyridoxine overdose. Neurology 1987; 37: 17291732.CrossRefGoogle ScholarPubMed