Document Detail


Bilateral striopallidodentate calcinosis: cerebrospinal fluid, imaging, and electrophysiological studies.
MedLine Citation:
PMID:  1586138     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report the genetic, clinical, electrophysiological, and imaging studies in a family with bilateral striopallidodentate calcinosis (Fahr's disease). The intracerebral calcium deposits occurred before onset of the symptoms in the third decade of life. Progressive neurological deterioration occurred in the fifth decade of life in the proband. Cerebrospinal fluid homocarnosine, a central nervous system-specific peptide, was increased twofold in patients with autosomal dominant bilateral stripallidodentate calcinosis; in sporadic cases, there was no detectable homocarnosine and a decreased level of histidine. With advancing age, the amount of calcification increases, but it has not been determined if a critical amount must be reached before symptoms occur. Computerized tomography is superior to magnetic resonance imaging for radiological diagnosis. Despite diffuse striatal calcification, striatal 6-[18F]fluoro-L-dopa uptake did not reveal any difference between patients and control subjects, from which we infer persisting integrity of the nigrostriatal dopaminergic pathway.
Authors:
B V Manyam; M H Bhatt; W D Moore; A B Devleschoward; D R Anderson; D B Calne
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  31     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1992 Apr 
Date Detail:
Created Date:  1992-06-17     Completed Date:  1992-06-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  379-84     Citation Subset:  IM    
Affiliation:
Division of Neurology, Southern Illinois University School of Medicine, Springfield 62794-9230.
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MeSH Terms
Descriptor/Qualifier:
Adult
Amino Acids / cerebrospinal fluid
Brain Diseases / cerebrospinal fluid,  diagnosis,  physiopathology
Calcinosis / cerebrospinal fluid*,  diagnosis,  physiopathology
Corpus Striatum*
Electrophysiology
Evoked Potentials
Globus Pallidus*
Hippocampus*
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Pedigree
Tomography, X-Ray Computed
Chemical
Reg. No./Substance:
0/Amino Acids

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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