Document Detail

Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study.
MedLine Citation:
PMID:  10210919     Owner:  NLM     Status:  MEDLINE    
We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. Magnetic resonance imaging showed a diffuse brain atrophy. A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients.
K Hojo; T Imamura; M Takanashi; K Ishii; M Sasaki; S Imura; R Ozono; Y Takatsuki; S Takauchi; E Mori
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of neurology : the official journal of the European Federation of Neurological Societies     Volume:  6     ISSN:  1351-5101     ISO Abbreviation:  Eur. J. Neurol.     Publication Date:  1999 May 
Date Detail:
Created Date:  2000-06-26     Completed Date:  2000-06-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9506311     Medline TA:  Eur J Neurol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  357-61     Citation Subset:  IM    
Copyright Information:
Copyright 1999 Lippincott Williams & Wilkins
Division of Clinical Neurosciences, Hyogo Institute for Aging Brain and Cognitive Disorders, Himeji, Japan.
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MeSH Terms
Deafness / complications*,  genetics,  pathology*
Dementia / complications*,  genetics,  pathology*
Frontal Lobe / pathology,  radionuclide imaging
Hereditary Sensory and Autonomic Neuropathies / complications*,  genetics,  pathology*
Magnetic Resonance Imaging
Middle Aged
Thalamus / pathology,  radionuclide imaging
Tomography, Emission-Computed

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