[Clinical early symptoms and CT findings in Fahr syndrome]

Abstract

We present the results of an investigation on initial symptomatology of patients with bilateral, symmetrical intracerebral calcification of the basal ganglia (Fahr's syndrome). 62 patients, who because of various neurological or psychiatric symptoms or other reasons were referred to cranial computer-tomography, revealed clear manifestation of Fahr's syndrome. In these cases estimations of the volume of the opaque bodies were made, based on computer-integrated programmes (SO-MATOM D2). In 9 cases there was a history of thyroidectomy, on average 25 years previously. As a rule patients were referred in the age range between 40 to 60 years, those who had undergone thyroidectomy being on average somewhat older than the others. As presenting symptoms extrapyramidal syndromes, apoplexias, cephalea, affective organic alterations, alcoholism and dementia were noted. Cerebral localisation of bilateral, symmetrical calcification was most frequent in the pallidum, though this localisation may be responsible for numerous different neuropsychiatric symptoms. The dimensions of the opaque structures can be very greatly. Symptomatology seems to be practically independent of either cerebral localisation or volume, except that greater volumes of calcification seem to cause more pronounced neurological (extrapyramidal) symptomatology and dementia. The time-span between manifestation of the initial symptoms and diagnosis was shorter in neurological syndromes than in psychiatric cases. Like earlier workers, we found a high incidence (21%) of organic affective syndromes as an initial manifestation of Fahr's syndrome.