Radiologic features of Hallervorden Spatz Disease

Abstract

Hallervorden Spatz Disease is a rare familial neurodegenerative disorder, which primarily affects children but also can occur in adults1 . Major clinical features are abnormal involuntary movements and cognitive impairment2 . Diagnosis is based on clinical and radiological features. The radiological features are hypointense signals in globus pallidus and substantia niagra on MR! of brain3 . Occasionally the pallidal hypointense signals surround hyperintense signals, this is known as “tigereye-sign”4 and is postulated to be specific for Hallervorden Spatz Disease. We report two brothers with such MRI findings

    Similar works