Radiologic features of Hallervorden Spatz Disease

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

Moyamoya disease: an elusive diagnosis

Moyamoya disease is an idiopathic vasculopathy, affecting vessels of Circle of Willis.1 It usually manifests as stroke, but can also cause seizures and cognitive impairment.2 Ischemic strokes are common in children and hemorrhagic strokes in adults.1 We describe our experience with moyamoya disease in four patients who presented with ischemic strokes, at an academic tertiary care center and emphasize that this diagnosis should be considered in young patients, especially children, who present with stroke