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 progressivedegeneration of basal ganglia, globuspallidus and reticular part of the substantianigra, produced byironaccumulation. The defect has been found in the pantothenate kinase 2 (PANK2) producing gene locatedinchromosome 20p13-p12.3. Clinical presentations include dystonia, dysarthria, dysphasia, dementia, severemental retardation and severe movement disability may develop at later stages. Rare clinical features includerigidity, choreoathetosis, seizures, optic atrophy and pigmentary retinopathy. The characteristic MRI brainpattern of HSD shows the “eye of the tiger ” pattern. Treatment is symptomatic. We present the case of apatient, 19 years old boy with Hallervorden-Spatz disease who came to our physiotherapy department withfeatures of spasticity, dystonia and gait difficulty. He was diagnosed on the basis of clinical findings and typicalMRI brain of “eye of the tiger” pattern. His detailed evaluation was carried out and physiotherapy treatmentwas started.