Fahr’s Disease (FD) is a rare neurodegenerative disorder of uncertain etiology characterized by abnormal intracranial calcium deposition in the basal ganglia. It initially presents in the 4th to 6th decade of life with diverse neuropsychiatric manifestations. The correlation between vascular calcification and coronary artery disease had been studied extensively, but its correlation with cerebrovascular disease remains elusive. Vascular calcification is thus an important risk factor for coronary artery disease. It was also postulated that a similar risk for cerebrovascular disease may be attributable to intracranial vascular calcification. We present the case of a 46-year-old male with progressive affective and cognitive disturbances, parkinsonism and ataxia. Eight years after initial presentation, he developed an acute ischemic infarct in the background of diffuse intracranial calcification characteristic of FD, confirmed by neuroimaging (Figure 1). While a number of cases FD with associated stroke or stroke-like symptoms have been reported, we are unaware of such occurrence in a Filipino. A pathologic model linking intracranial vascular calcification in FD with cerebrovascular disease and ischemic stroke has been proposed, and warrants further investigation.
Keywords: Fahr’s disease, Fahr’s syndrome, intracranial calcification, stroke, cerebrovascular disease
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