Fahr's Disease With Late Onset: A Case Report

Abstract

Fahr's disease is a rare genetically dominant disease. It is characterized by the idiopathic deposition of calcium in the basal ganglia and cerebral cortex. The condition may cause motor impairment, impaired muscle tone, dementia, seizures, impairment of eye movements, speech, abnormal hand movements, cognitive impairment, and ataxia. The thalamus, white matter, and basal ganglia can be involved. A 77-year-old man with multiple comorbidities presented with a complaint of increasing confusion, altered mental status, dystonia, tremor, and hallucinations. The patient's daughter reported that he sounded confused and inappropriate in his speech. A computerized tomography (CT) scan of the head without contrast revealed a "dense calcification of the dentate nuclei and the basal ganglia" and "subcortical calcification of the frontal and occipital lobes." The patient was diagnosed with late-onset Fahr's disease. Fahr's disease is caused by idiopathic calcification of the bilateral basal ganglia. A wide variety of symptoms are associated with this condition. Fahr's disease should be considered in the differential diagnosis in geriatric patients suffering from cognitive impairment and movement disorders.

Keywords: basal ganglia disease; fahr’s disease or fahr’s syndrome; geriatric patient; idiopathic basal ganglia calcification (ibgc); rare cause of altered mental status; rare genetic diseases.

Figure 1. A CT scan of the head without contrast showed dense calcification of the dentate nuclei and the basal ganglia and subcortical calcification of the frontal and occipital lobes