Fahr's syndrome and clinical correlation: a case series and literature review

Abstract

Introduction: Fahr's disease is characterized by bilateral calcium deposition within the basal ganglia, cerebellar dentate nucleus and subcortical brain white matter. The main clinical manifestations are rigid or hyperkinetic syndrome, mood disorders and cognitive impairment. The correlation between neurological impairment and symmetrical basal ganglia calcification is not so frequent. Aim of the study was to report the results of neurological assessment of three sporadic cases of Fahr's disease highlighting a correlation between the clinical syndrome and neuroimaging.

Case reports: Three adults of aged 32, 55 and 70, were studied. They all showed a heterogeneous clinical spectrum. One case developed neuropsychiatric symptoms, whereas the others complained of the tremorigen syndrome. Brain computed tomography scans revealed several calcifications in basal ganglia, cerebellar white matter and dentate nuclei.

Conclusions: The pathogenesis of Fahr's disease is probably secondary to the dysfunction of cortico-basal connections and their interhemispheric relations. No significant correlation between calcifications and neurological symptoms is proved.