HALLERVORDEN-SPATZ DISEASE - A RARE CASE REPORT - “Eye of th e Tiger” Sign

Background: Hallervorden-Spatz disease (HSD) is a rare neurological disease characterized by progressive degeneration of basal ganglia, globuspallidus and reticular part of the substantianigra, produced by iron accumulation. The defect has been found in the pantothenate kinase 2 (PANK2) producing gene located in chromosome 20p13-p12.3. Clinical presentations include dystonia, dysarthria, dysphasia, dementia, se vere mental retardation and severe movement disability may develop at later stages. Rare clinical feature s include rigidity, choreoathetosis, seizures, optic atrophy and pigmentary retinopathy. The characteristic MR I brain pattern of HSD shows the “eye of the tiger ” pattern. Treatment is symptomatic. We present the case o f a patient, 19 years old boy with Hallervorden-Spatz disease who came to our physiotherapy department w ith features of spasticity, dystonia and gait difficulty. He was diagnosed on the basis of clinical find ings and typical MRI brain of “eye of the tiger” pattern. His detailed evaluation was carried out and physiotherapy t reatment was started.