Fahr's disease: a rare degenerative disorder presenting with psychosis: a case report

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.

Keywords

• key message: psychiatrist and neurologist should remain vigilant regarding this uncommon presentation of this degenerative disease. fahr's disease; degenerative disorder; intracranial calcification; movement disorder; psychosis