Document Detail

Nonprogressive type II hereditary sensory autonomic neuropathy: a homogeneous clinicopathologic entity.
MedLine Citation:
PMID:  1469243     Owner:  NLM     Status:  MEDLINE    
Two different clinical subtypes were previously identified within hereditary sensory autonomic neuropathy (HSAN) type II: a stable congenital form and a progressive one. This paper discusses two clinicopathologic cases of nonprogressive HSAN type II with morphometric correlations. In addition, a retrospective literature search was carried out to locate other cases where an accurate histologic examination, including ultrastructural features, was available in order to relate clinical and pathologic aspects of the disease. The combined data support the individualization of this neuropathic form as a homogeneous disease, as has been suggested during the last century and underline the clinical importance of this concept for the prognosis and investigation of sensory, auditory, autonomic, and motor functions in children with sensory neuropathies.
G Ferrière; F Guzzetta; S Kulakowski; P Evrard
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of child neurology     Volume:  7     ISSN:  0883-0738     ISO Abbreviation:  J. Child Neurol.     Publication Date:  1992 Oct 
Date Detail:
Created Date:  1993-01-26     Completed Date:  1993-01-26     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  364-70     Citation Subset:  IM    
Unité de Neurologie du Developpement, Université Catholique de Louvain, Medical School, Brussels, Belgium.
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MeSH Terms
Child, Preschool
Hearing Disorders / etiology
Hereditary Sensory and Autonomic Neuropathies / complications,  diagnosis*,  physiopathology
Motor Skills
Muscles / pathology*
Myelin Sheath / ultrastructure

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