Fahr’s disease: a rare degenerative disorder presenting with psychosis: a case report
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Date
2014-07
Journal Title
Journal ISSN
Volume Title
Publisher
Indian Psychiatric Society
Abstract
Background: Fahr’s syndrome (or Fahr’s disease) is a rare idiopathic degenerative disorder, characterized by bilateral symmetrical intracranial
calcification and manifested as movement disorders, dementia and other behavioural disturbances.
Case description: A 50-year-old male with long standing seizure disorder with intact motor and cognitive function presented with behavioural disturbances.
Brain CT showed symmetrical calcification in cerebral hemispheres involving caudate nucleus, internal capsule, adjoining area and cerebellum. The
history, clinical and radiological features and exclusion of other possibilities in our case point towards this uncommon disorder.
Discussion: A substantial number of patients with Fahr‘s disease present with behavioural disturbances. Usually late onset Fahr’s disease with behavioural
disturbances is accompanied by cognitive and motor abnormalities. However, in our case no such neurocognitive abnormalities were found along with the
behavioural disturbances in the form of psychosis inspite of widespread intracranial calcification.
Conclusion: Psychosis may be the sole presentation in a case of late onset Fahr‘s disease.
Key message: Psychiatrist and Neurologist should remain vigilant regarding this uncommon presentation of this degenerative disease.
Description
Keywords
Fahr’s Disease, Degenerative Disorder, Intracranial Calcification, Movement Disorder, Psychosis
Citation
Sengupta Soumik, Nath Subhashish, Chetia Dhrubajyoti, Dutta Hemanta. Fahr’s disease: a rare degenerative disorder presenting with psychosis: a case report. Andhra Pradesh Journal of Psychological Medicine. 2014 Jul-Dec; 15(2): 255-257