Hallervorden Spatz disease: A case report

Abstract

Hallervorden-Spatz disease (HSD) is a rare neurodegenerative disorder of autosomal recessive inheritance with age of onset between 1 year and 10 years and a life span of about 10 years after onset. The main clinical picture is characterized by extra pyramidal dysfunction-dystonia, rigidity and choreoathetosis. Other corroborative findings include corticospinal tract involvement, intellectual impairment, visual problems and seizures. Typical findings in magnetic resonance imaging (MRI) of brain help in early diagnosis of HSD. Here, we are presenting a case of HSD with positive familial history, characteristic clinical features and MRI findings. In this report, we will discuss the clinical features of the patient with special emphasis on MRI findings and compare it with available literature.

Keywords

Hallervorden-Spatz disease - basal ganglia - iron deposition