Fahr's Disease (fahr + disease)

Distribution by Scientific Domains

Selected Abstracts

A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three-generation Fahr disease

M. Shirahama
Objective:, Fahr disease (FD) is a rare neurological and psychiatric disorder. The disease is classified by intracranial calcification of the basal ganglia with the globus pallidus region being particularly affected. We examined a young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible third-generation FD. Method:, Case report of third-generation FD. Results:, A 23-year-old woman was arrested for two arsons: i) The patient exhibited progressive psychotic symptoms, including visual hallucinations, delusion of injury, irritability, lability of mood, mental retardation and visual disorders and ii) Computed tomography (CT) imaging demonstrated bilateral calcifications of the basal ganglia (globus pallidus) in the patient, her mother and her grandmother. Conclusion:, We found a family with a three-generation history of FD who exhibited calcification in the brain and mental retardation. Compared to her mother, the patient described here displayed anticipation of disease onset. [source]

Fahr's disease , a model of neuropsychiatric illness with cognitive and psychotic symptoms

J. A. Bourgeois
No abstract is available for this article. [source]

Corticobasal degeneration syndrome with basal ganglia calcification: Fahr's disease as a corticobasal look-alike?

Anthony E. Lang MD, FRCPC

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

Bala V. Manyam MD
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. [source]