Affiliations: Eko X Ray & Imaging Institute, Kolkata, India
Note: [] Correspondence: Dushyant R. Gondaliya, Eko X Ray &
Imaging Institute, 54, J L Nehru road, Kolkata, 700071, India. E-mail:
[email protected]
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
