Case of neurological manifestations of Fahr's syndrome

The article contains a description of the clinical observation of a patient with Fahr’s syndrome. A review of the literature on this rare neurodegenerative disease, Fahr’s disease, and Fahr’s syndrome is presented. An observation of a 54-year-old patient Sh. with Fahr’s syndrome is presented. The authors of the article set themselves the goal of drawing attention to Fahr’s syndrome, which is the sphere of interests of doctors of various specialties. Raising the awareness of doctors about this disease will contribute to its timely diagnosis and treatment.

Dumbness revealing Fahr syndrome

Fahr’s syndrome is an anatomoclinical entity, defined by Theodor Fahr in 1930. It is determined by the presence of intracerebral, bilateral and symmetrical calcifications, non-arteriosclerotic, located in the basal ganglia. One of its main aetiologies is Pseudohypoparathyroidism (PHP), which shows resistance to the action of Parathyroid hormone (PTH), with mainly hypocalcemia and normal to high levels of PTH. The clinical manifestations of the disease do not correspond to any specific picture, but they can be dominated by various neuropsychiatric symptoms. We report the case of a 28-year-old young man who had a first psychotic attack a year ago, treated but without therapeutic observance, and who presented for mutism revealing Fahr syndrome. The evolution was marked by an improvement under conventional neuroleptics. Keywords: Fahr syndrome; chronic psychotic disorder; neuroleptic.