Document Detail

Benign hereditary cerebellar ataxia with extensive thermoanalgesia.
MedLine Citation:
PMID:  2168779     Owner:  NLM     Status:  MEDLINE    
A New Zealand family with 3 members affected by late onset hereditary cerebellar ataxia is reported, distinguished by the development of near global thermoanalgesia. Because proprioception and autonomic function were preserved, and ataxia only slowly worsened, this proved to be a benign syndrome. The pattern of sensory loss indicated a 'length-dependent' neuropathy. Evaluation of sural nerve biopsy revealed a marked loss of axons, especially of those with diameters 1-7 microns and 0.2-1.5 microns. It therefore seemed likely that impairment of pain and temperature in the syndrome resulted from a loss of primary sensory afferents. It is concluded that this family represents a previously unrecognized variant and that the spectrum of hereditary ataxias needs to be widened to include an associated severe but selective loss of pain and temperature sensation.
M Pollock; B Kies
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Brain : a journal of neurology     Volume:  113 ( Pt 4)     ISSN:  0006-8950     ISO Abbreviation:  Brain     Publication Date:  1990 Aug 
Date Detail:
Created Date:  1990-10-17     Completed Date:  1990-10-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372537     Medline TA:  Brain     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  857-65     Citation Subset:  AIM; IM    
Department of Medicine, University of Otago Medical School, Dunedin, New Zealand.
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MeSH Terms
Cerebellar Ataxia / complications*,  pathology
Genetic Diseases, Inborn / pathology
Peripheral Nervous System Diseases / complications*,  pathology

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