Síndrome de Fahr

Miss Merry, 17 years old girl hailing from Barobila., Patkelghata, Satkhira was admitted in Khulna Medical College Hospital on 27-06-2010 with the complaints of fever, convulsion and unconsciousness for 7 days. She has also some hearing impairment, behavioral abnormalities and stunted growth since her childhood. On examination she was deeply unconscious, anaemic and febrile. CT scan of brain reveals multiple bilateral symmetrical calcification seen in the brain parenchyma involving basal ganglia, thalamus, para ventricular region, and cerebellar nucleus. Multiple ill defined hypodense areas are seen in the both parieto-occipital region, suggestive of Fahr's syndrome with meningoencephalitis. Bang Med J (Khulna) 2012; 45 : 33-35 Introduction Idiopathic Basal Ganglia Calcification, also known as Fahr's disease or Fahr's Syndrome or Bilateral StriatoPallidoDentate Calcinosis (BSPDC) is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. The disease was first noted by German neurologist Karl Theodor Fahr in 1930.1 According to reports in medical literature, Fahr Disease is often familial. It is believed to have autosomal dominant inheritance but a few cases have been reported to have autosomal recessive inheritance and even some sporadic cases have been reported in literature. Idiopathic calcification of the basal ganglia, also known as Fahr's disease, is a rare neurologic disorder of unknown etiology characterized by neuropsychiatric abnormalities.2 Parkinsonian or choreoathetotic-type movement disturbance, and extensive symmetrical calcification of the basal ganglia and dentate nuclei in the cerebellum. These symptoms cannot be explained by any other particular disorder of the calcium phosphorus metabolism or any other disease. Dementia is a well-recognized neuropsychiatric, manifestation of Fahr's disease. In addition, a schizophrenia-like psychosis characterized by paranoia, hallucinations, and delusions has been reported.3 There is no cure for Fahr's syndrome, which worsens over time, nor is there a standard course of treatment.