Isolated vitamin E deficiency with demyelinating neuropathy.
A 22-year-old man, with a past history of generalized tonic-clonic seizures treated with phenobarbital, presented with spinocerebellar ataxia. The electrophysiological studies revealed a demyelinating motor-sensory neuropathy. The serum vitamin E level was low. Sural nerve biopsy revealed loss of large myelinated fibers with evidence of remyelination. Vitamin E supplementation led to clinical and electrophysiological recovery of sensory conduction and evoked potentials. Motor nerve conduction, however, showed only partial recovery. Vitamin E deficiency leading to a demyelinating neuropathy, as in the present case, suggests that the full spectrum of the disease entity is not fully defined.
Puri
Vinod
V
Department of Neurology, G. B. Pant Hospital, New Delhi 110002, India.
Chaudhry
Neera
N
Tatke
Medha
M
Prakash
Ved
V
D000328:Adult; D001706:Biopsy; D003711:Demyelinating Diseases / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D004568:Electrodiagnosis; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D009133:Muscular Atrophy / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009431:Neural Conduction / Q000502:physiology; D010525:Peripheral Nerves / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D012640:Seizures / Q000209:etiology; D020886:Somatosensory Disorders / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D020754:Spinocerebellar Ataxias / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D013497:Sural Nerve / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D014810:Vitamin E / Q000627:therapeutic use; D014811:Vitamin E Deficiency / Q000150:complications*
D014810:Vitamin E