A rare case of Fahr's disease presenting as epileptic seizure in the emergency department

Fahr's disease (striato-pallido-dentate calcifications) is a rare neurodegenerative disorder characterised by bihemispherical and symmetrical calcium deposits in certain areas of the brain, particularly in the basal ganglia. We report an unusual case of Fahr's disease in a 36-year-old man who presented with generalised seizure in our emergency department. Basing on clinical, radiological, and endocrinological aspects, the patient was diagnosed as Fahr's disease associated with hypoparathyroidism. Administration of parenteral calcium and calcitriol supplementation were started in the emergency department. The clinical outcome was favourable after the treatment. The case illustrates that Fahr's disease, though rarely seen, has to be considered in a patient with convulsive state associated with calcifications of the basal ganglia. Systematically searching for dysparathyroidism in such patients is extremely important. (Hong Kong j.emerg.med. 2010;17:262-264)