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Hereditary Sensory Neuropathy: A Case With Pain and Temperature Dissociation

Published online by Cambridge University Press:  18 September 2015

Beth O'brien
Affiliation:
Faculty of Medicine of University of Ottawa, Ottawa Civic Hospital, Ottawa, Ontario
R. Jackson
Affiliation:
Faculty of Medicine of University of Ottawa, Ottawa Civic Hospital, Ottawa, Ontario
R. Tang-wai
Affiliation:
Faculty of Medicine of University of Ottawa, Ottawa Civic Hospital, Ottawa, Ontario
A.J. Lewis
Affiliation:
Faculty of Medicine of University of Ottawa, Ottawa Civic Hospital, Ottawa, Ontario
E.A. Atack*
Affiliation:
Faculty of Medicine of University of Ottawa, Ottawa Civic Hospital, Ottawa, Ontario
*
Division of Neurology, Ottawa Civic Hospital, 1053 Carling Avenue, Ottawa, Ontario K1Y 4E9 Canada
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Summary:

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A case of hereditary sensory neuropathy is described resembling Dyck's Type I hereditary sensory neuropathy. Sensory testing revealed marked pain impairment in feet and hands shading at mid calf to normal at mid thigh and shading above the wrist to normal at the elbow. Other sensory modalities including temperature were intact except painful heat and painful cold and they produced very little if any discomfort. Stimuli at 0° C or 45° to 70° C elicited a temperature response but not pain. Sural nerve biopsy findings (including elecironmicroscopy) were consistent with a diagnosis of Type I hereditary sensory neuropathy, but also showed evidence of abortive axonal regeneration and profound Schwann cell vacuolation.

Type
Research Article
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

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