Progressive Idiopathic Strio-Pallido-Dentate Calcinosis (Fahr’s Disease) with Autosomal Recessive Inheritance

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Abstract
Siblings (3) with symmetrical calcifications in the strio-pallido-dentate system are described. Parathyroid function was normal and there were no signs of central or peripheral myelinopathy. This is the 9th family reported with autosomal recessive idiopathic striopallido-dentate calcinosis and the 1st to be investigated by computerized tomography (CT). CT scans appeared superior to plain skull radiograms for assessment of the localization and extent of calcifications in vivo. Calcifications were least extensive in the youngest and most extensive in the oldest. The calcifying process is probably a progressive disorder. It seems to start in the dentate nuclei and pons, and subsequently extends to the basal ganglia and to the radiation of the corpus callosum.