Fahr's Disease: An Uncommon Cause of Intracranial Calcification in Children

A Banerjee,K Nayek,S Bhattacharjya,S Pati

doi:10.3126/jnps.v32i1.5335

Abstract

ABSTRACT Idiopathic bilateral striopallidodentate calcification or Fahr’s disease is a degenerative disease normally found in the 5-6th decade of life. Here we report one of the youngest cases of sporadic Fahr’s disease presenting at five years of age with minimal clinical features but extensive intracranial calcification, a rarity. DOI: http://dx.doi.org/10.3126/jnps.v32i1.5335 J. Nepal Paediatr. Soc. Vol.32(1) 2012 76-78

Highlights

Idiopathic bilateral striopallidodentate calcification or Fahr’s disease is a degenerative disease normally found in the 5-6th decade of life with autosomal dominant inheritance[1,2,3,4]
Extensive intracranial calcification is rare in children and needs proper investigation to find out the cause to guide management
Dystrophic calcification seen in following encephalitis,TORCH infection parasitosis, ischaemic or haemorrhagic stroke, A-Vmalformations, tumours, phakomatoses, MELAS and Fahr's disease[5,6]

Summary

Introduction

Idiopathic bilateral striopallidodentate calcification or Fahr’s disease is a degenerative disease normally found in the 5-6th decade of life with autosomal dominant inheritance[1,2,3,4]. We report one of the youngest cases of sporadic Fahr’s disease presenting at five years of age with minimal clinical features but extensive intracranial calcification, a rarity. Fahr’s disease was the diagnosis of exclusion[1,4,5] based on clinical, biochemical, and radiological features, ruling out the more common causes of intracranial calcification in children. Symptomatic management was done and the child is currently on follow up. Extensive intracranial calcification is rare in children and needs proper investigation to find out the cause to guide management

The Case

Discussion