Document Detail


Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry.
MedLine Citation:
PMID:  11295778     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Clinical features in bilateral striopallidodentate calcinosis (BSPDC), popularly referred to as Fahr's disease (five autosomal dominant families and eight sporadic cases, n = 38), recruited through a registry, are reported. Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (+/- S.D.) age of Registry patients was 43 +/- 21 and that of literature was 38 +/- 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others.
Authors:
B V Manyam; A S Walters; K R Narla
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  16     ISSN:  0885-3185     ISO Abbreviation:  Mov. Disord.     Publication Date:  2001 Mar 
Date Detail:
Created Date:  2001-04-11     Completed Date:  2001-08-30     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  258-64     Citation Subset:  IM    
Copyright Information:
Copyright 2001 Movement Disorder Society.
Affiliation:
Department of Neurology, Scott & White Clinic and Memorial Hospital and Texas A&M University System Health Science Center College of Medicine, Temple, Texas 76508, USA. bmanyam@swmail.sw.org
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MeSH Terms
Descriptor/Qualifier:
Adult
Age Distribution
Atrophy / pathology
Brain Diseases / complications,  diagnosis*,  epidemiology*
Calcinosis / diagnosis*,  epidemiology*,  genetics
Corpus Striatum / pathology*,  radiography*
Dentate Gyrus / pathology*,  radiography*
Female
Functional Laterality / physiology*
Globus Pallidus / pathology*,  radiography*
Humans
Male
Middle Aged
Movement Disorders / diagnosis,  epidemiology,  etiology
Registries*
Severity of Illness Index
Thalamus / pathology
Tomography, X-Ray Computed

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


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