MovDisordV3, Main, Exploration, bibRecord, 004862

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Identifieur interne : 004862 ( Main/Exploration ); précédent : 004861; suivant : 004863

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Auteurs : Bala V. Manyam [États-Unis] ; Arthur S. Walters [États-Unis] ; Koteswara R. Narla [États-Unis]

Source :

Movement Disorders [ 0885-3185 ] ; 2001-03.

RBID : ISTEX:C98609A64894CECEF7DE5368B3E4A897CB967BBF

Descripteurs français

Pascal (Inist)
- Calcification, Calcinose, Corps strié, Diagnostic, Fahr maladie, Homme, Morphométrie, Mouvement involontaire, Noyau dentelé, Pallidum, Symptomatologie, Tomodensitométrie, Volumétrie.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Adult, Age Distribution, Atrophy (pathology), Brain Diseases (complications), Brain Diseases (diagnosis), Brain Diseases (epidemiology), Calcification, Calcinosis, Calcinosis (diagnosis), Calcinosis (epidemiology), Calcinosis (genetics), Computerized axial tomography, Corpus Striatum (pathology), Corpus Striatum (radiography), Corpus striatum, Dentate Gyrus (pathology), Dentate Gyrus (radiography), Dentate nucleus, Diagnosis, Fahr's disease, Female, Functional Laterality (physiology), Globus Pallidus (pathology), Globus Pallidus (radiography), Human, Humans, Involuntary movement, Male, Middle Aged, Morphometry, Movement Disorders (diagnosis), Movement Disorders (epidemiology), Movement Disorders (etiology), Pallidum, Registries, Severity of Illness Index, Symptomatology, Thalamus (pathology), Tomography, X-Ray Computed, Volumetric analysis, basal ganglia, bilateral striopallidodentate calcinosis (BSPDC), calcium, computed tomography, coordinate digitizer, electronic planimeter.
MESH :
- complications : Brain Diseases.
- diagnosis : Brain Diseases, Calcinosis, Movement Disorders.
- epidemiology : Brain Diseases, Calcinosis, Movement Disorders.
- etiology : Movement Disorders.
- genetics : Calcinosis.
- pathology : Atrophy, Corpus Striatum, Dentate Gyrus, Globus Pallidus, Thalamus.
- physiology : Functional Laterality.
- radiography : Corpus Striatum, Dentate Gyrus, Globus Pallidus.
- Adult, Age Distribution, Female, Humans, Male, Middle Aged, Registries, Severity of Illness Index, Tomography, X-Ray Computed.

Abstract

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. © 2001 Movement Disorder Society.

Url:

https://api.istex.fr/document/C98609A64894CECEF7DE5368B3E4A897CB967BBF/fulltext/pdf

DOI: 10.1002/mds.1049

Affiliations:

États-Unis
Illinois, New Jersey, Texas

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<term>Brain Diseases (diagnosis)</term>
<term>Brain Diseases (epidemiology)</term>
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<term>Calcinosis (epidemiology)</term>
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<front><div type="abstract" xml:lang="en">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. © 2001 Movement Disorder Society.</div>
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Movement Disorders (revue)

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

	Movement Disorders (revue)
	Attention, ce site est en cours de développement ! Attention, site généré par des moyens informatiques à partir de corpus bruts. Les informations ne sont donc pas validées.