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Fahr's disease associated with anaplastic ependymoma: a case report and review of the literature.
Br J Neurosurg
June 2023
Faculty of Medicine, Department of Radiology, Selcuk University, Konya, Turkey.
Fahr's disease, also known as familial idiopathic basal ganglia calcification or bilateral strio-pallido-dentate calcinosis, is a rare entity characterized by abnormal vascular calcium depositionin the thalamus, basal ganglia, cerebral cortex and the dentate nuclei of the cerebellum. Intracranial ependymomas comprise approximately 2% to 9% of all neuroepithelial tumors. It is reported that supratentorial ependymoma constitute 30% to 50% of all intracranial ependymal tumors.
View Article and Find Full Text PDFBilateral strio-pallido-dentate calcinosis (Fahr's disease): report of seven cases and revision of literature.
BMC Neurol
September 2016
Department of Medical Sciences, Section of Internal and Cardiopulmonary Medicine, University of Ferrara, Via Savonarola n°9, 44100, Ferrara, Italy.
Background: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed.
View Article and Find Full Text PDFPDGFB partial deletion: a new, rare mechanism causing brain calcification with leukoencephalopathy.
J Mol Neurosci
June 2014
Inserm U1079, Rouen, France.
Idiopathic basal ganglia calcification (IBGC) is a progressive cerebral disorder with diverse motor, cognitive, and psychiatric expression. It is inherited as an autosomal dominant trait. Three IBGC-causing genes have been identified in the past 2 years: SLC20A2, PDGFRB, and PDGFB.
View Article and Find Full Text PDFA case of strio-pallido-dentate calcinosis associated with micromolecular myeloma.
Neurol Sci
April 2014
Department of Anesthesiological, Cardiovascular, Respiratory, Nephrological, and Geriatric Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161, Rome, Italy.
Idiopathic bilateral strio-pallido-dentate calcinosis (Fahr's disease): a case report and review of the literature.
Ann Afr Med
January 2013
Department of Medicine, Ahmadu Bello University Teaching Hospital Zaria, Nigeria.
Bilateral calcification involving the basal ganglia (Fahr's disease) is a rare disease. A high index of suspicion is required to make clinical diagnosis; more so that the calcinosis may remain asymptomatic in a vast majority of cases. Movement disorder is by far the most common manifestation in symptomatic individuals.
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