IDIOPATHIC FAMILIAL CEREBROVASCULAR FERROCALCINOSIS (FAHR'S DISEASE) AND REVIEW OF DIFFERENTIAL DIAGNOSIS OF INTRACRANIAL CALCIFICATION IN CHILDREN

¹ Director, Department of Radiology, Milwaukee Children's Hospital; Associate Professor of Radiology, Marquette University School of Medicine
² Assistant Professor of Pathology, Marquette University School of Medicine
³ Assistant Professor of Pediatrics, Marquette University School of Medicine; Head of Special Development Clinic, Milwaukee Children's Hospital
⁴ Assistant Clinical Professor of Pediatrics, Marquette University School of Medicine

Three siblings with idiopathic familial cerebrovascular ferrocalcinosis (Fahr's disease) are reported. This is an uncommon cause of intracerebral calcification in children.

The differential diagnosis is discussed and a Table, listing the diseases associated with intracranial calcification, is presented.

Idiopathic familial cerebrovascular ferrocalcinosis apparently is not the result of an infectious process, endocrine disorder, or due to any known specific etiology. The disease is probably, as suggested by Fritzsche⁷ and supported by Melchior et al.,¹¹ a genetically determined (autosomal recessive) metabolic disorder and/or vascular anomaly of a nature as yet unknown. It represents another cause of intracranial calcification in childhood.

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