Abstract
A 62-year-old man and his maternal uncle had a selective vitamin E deficiency without generalized fat malabsorption. A progressive neurological disorder comprising ataxia, areflexia, and loss of proprioception developed in their sixth and seventh decades. The vitamin E deficiency is thought to be due to abnormally accelerated utilization, excretion, or degradation of the vitamin. This adult-onset spinocerebellar syndrome is due to vitamin E deficiency not caused by malabsorption.
MeSH terms
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Age Factors
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Humans
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Male
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Middle Aged
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Muscles / pathology
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Neurons / pathology
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Pedigree
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Spinocerebellar Degenerations / etiology
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Spinocerebellar Degenerations / genetics*
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Spinocerebellar Degenerations / pathology
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Tocopherols
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Vitamin E / analogs & derivatives
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Vitamin E / therapeutic use
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Vitamin E Deficiency / complications
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Vitamin E Deficiency / drug therapy
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Vitamin E Deficiency / genetics*
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alpha-Tocopherol* / analogs & derivatives*
Substances
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Vitamin E
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alpha-Tocopherol
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Tocopherols