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Familial idiopathic basal ganglia calcification (Fahr’s disease)

Familial idiopathic basal ganglia calcification (Fahr’s disease) is a rare neurodegenerative disorder characterized by symmetrical and bilateral calcification of the basal ganglia. Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both famil...

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Detalles Bibliográficos
Autores principales: Mufaddel, Amir A., Al-Hassani, Ghanem A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Riyadh : Armed Forces Hospital 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4727649/
https://www.ncbi.nlm.nih.gov/pubmed/24983277
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author Mufaddel, Amir A.
Al-Hassani, Ghanem A.
author_facet Mufaddel, Amir A.
Al-Hassani, Ghanem A.
author_sort Mufaddel, Amir A.
collection PubMed
description Familial idiopathic basal ganglia calcification (Fahr’s disease) is a rare neurodegenerative disorder characterized by symmetrical and bilateral calcification of the basal ganglia. Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both familial and non-familial cases of Fahr’s disease have been reported, predominantly with autosomal-dominant fashion. The disease has a wide range of clinical presentations, predominantly with neuropsychiatric features and movement disorders. Psychiatric features reported in the literature include: cognitive impairment, depression, hallucinations, delusions, manic symptoms, anxiety, schizophrenia-like psychosis, and personality change. Other clinical features include: Parkinsonism, ataxia, headache, seizures, vertigo, stroke-like events, orthostatic hypotension, tremor, dysarthria, and paresis. Fahr’s disease should be considered in the differential diagnosis of psychiatric symptoms, particularly when associated with movement disorder. The disease should be differentiated from other conditions that can cause intracranial calcification. No specific treatment is currently available. Further research is needed to bridge the gap existing in our current knowledge of the prevalence, etiology, symptoms, and treatment of Fahr’s disease.
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spelling pubmed-47276492016-02-02 Familial idiopathic basal ganglia calcification (Fahr’s disease) Mufaddel, Amir A. Al-Hassani, Ghanem A. Neurosciences (Riyadh) Review Article Familial idiopathic basal ganglia calcification (Fahr’s disease) is a rare neurodegenerative disorder characterized by symmetrical and bilateral calcification of the basal ganglia. Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both familial and non-familial cases of Fahr’s disease have been reported, predominantly with autosomal-dominant fashion. The disease has a wide range of clinical presentations, predominantly with neuropsychiatric features and movement disorders. Psychiatric features reported in the literature include: cognitive impairment, depression, hallucinations, delusions, manic symptoms, anxiety, schizophrenia-like psychosis, and personality change. Other clinical features include: Parkinsonism, ataxia, headache, seizures, vertigo, stroke-like events, orthostatic hypotension, tremor, dysarthria, and paresis. Fahr’s disease should be considered in the differential diagnosis of psychiatric symptoms, particularly when associated with movement disorder. The disease should be differentiated from other conditions that can cause intracranial calcification. No specific treatment is currently available. Further research is needed to bridge the gap existing in our current knowledge of the prevalence, etiology, symptoms, and treatment of Fahr’s disease. Riyadh : Armed Forces Hospital 2014-07 /pmc/articles/PMC4727649/ /pubmed/24983277 Text en Copyright: © Neurosciences Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work.
spellingShingle Review Article
Mufaddel, Amir A.
Al-Hassani, Ghanem A.
Familial idiopathic basal ganglia calcification (Fahr’s disease)
title Familial idiopathic basal ganglia calcification (Fahr’s disease)
title_full Familial idiopathic basal ganglia calcification (Fahr’s disease)
title_fullStr Familial idiopathic basal ganglia calcification (Fahr’s disease)
title_full_unstemmed Familial idiopathic basal ganglia calcification (Fahr’s disease)
title_short Familial idiopathic basal ganglia calcification (Fahr’s disease)
title_sort familial idiopathic basal ganglia calcification (fahr’s disease)
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4727649/
https://www.ncbi.nlm.nih.gov/pubmed/24983277
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