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The Neurology of Cobalamin

Published online by Cambridge University Press:  18 September 2015

Michael I. Shevell
Affiliation:
Departments of Neurology/Neurosurgery & Pediatrics, McGill University and the Division of Pediatric Neurology, Montreal Children’s Hospital, Montreal
David S. Rosenblatt*
Affiliation:
Medical Research Council of Canada Genetics Group, Centre for Human Genetics; Departments of Medicine, Pediatrics & Biology, McGill University, Montreal
*
Division of Medical Genetics, Royal Victoria Hospital, 687 Pine Ave. West, Montreal, Quebec, Canada H3A 1A1
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Abstract:

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The following review indicates that the impact of cobalamin on neurologic disease extends far beyond the traditional myelopathy of classical pernicious anemia. The delineation of a broad spectrum of inherited disorders of cobalamin processing has served to illustrate and precisely define each step in the normal absorption, transport and intracellular metabolism of this essential vitamin. Recent clinical work has extended the boundaries of acquired cobalamin deficiency to encompass a variety of neuropsychiatric disturbances without identifiable concomitant hematologic derangements and emphasized the utility and sensitivity of new laboratory tests. These findings will demand increased vigilance from clinicians so that atypical and subtle cobalamin deficiency states will be readily diagnosed. The wide range of neurologic dysfunction observed in both inherited and acquired disorders of cobalamin metabolism challenges basic scientists to delineate cobalamin’s presumed important role in the normal development and homeostasis of the nervous system.

Type
Review Article
Copyright
Copyright © Canadian Neurological Sciences Federation 1992

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