Two Member of a Family with Hallervorden Spatz Disease

Hallervorden Spatz Disease is a rare familial neurodegenerative disorder, which primarily affects children but also, can occur in adults. Major clinical features are abnormal involuntary movements and cognitive impairment. Diagnosis is based on clinical and radiological features. The radiological features are hypointense signals in globus pallidus and substantia nigra on MRÏ of brain. Ôccasionally the pallidal hypointense signals surround hyperintense signals, this is known as “tiger-eye-sign” and is postulated to be specific for Hallervorden Spatz Disease. We report a brother and sister with such MRÏ findings.