[Striato-pallido-dentate calcifications].
Striato-pallido-dentate calcifications (SPDC) is a well defined entity, characterized by calcium deposits in the basal ganglia, dentate nuclei and the centrum semiovale. Several metabolic derangements have been associated with this entity, particularly parathyroid disorders. The traditional designation of Fahr's syndrome should be restricted to the idiopathic cases. The authors report a study of seven patients with SPDC. Hypocalcemia was found in three cases, two with pseudohypoparathyroidism and one with hypoparathyroidism. Fahr's syndrome was diagnosed in four patients. Clinical and laboratory features are presented. Neurological manifestations included epilepsy, dementia and parkinsonism. Discussion focuses on the distinction of this entity from the small pallidal calcifications and on the pathophysiology of basal ganglia mineralisation, in view of recent reports.
Baptista
M V
MV
Serviço de Neurologia, Hospital Egas Moniz, Lisboa.
Vale
J
J
Leitão
O
O
D000293:Adolescent; D000328:Adult; D000368:Aged; D001480:Basal Ganglia Diseases / Q000175:diagnosis*; D002114:Calcinosis / Q000175:diagnosis*; D002526:Cerebellar Diseases / Q000175:diagnosis*; D002529:Cerebellar Nuclei / Q000000981:diagnostic imaging / Q000473:pathology*; D004569:Electroencephalography; D005260:Female; D005917:Globus Pallidus / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D014057:Tomography, X-Ray Computed