Hallervorden-Spatz syndrome is a rare neurodegenerative disease of autosomal recessive inheritance which presents in childhood or early adulthood with dystonia, dysarthria, rigidity and choreoathetosis. Here we present an unusual case of atypical Hallervorden-Spatz syndrome with onset during adolescence and rapid progression in a young female patient who showed the characteristic "eye of the ti...

“Hallervorden-Spatz disease” represents a distinctive and readily recognizable eponym to neurologists and pediatricians; it denotes a rare, inherited, autosomal recessive disorder tha t is perhaps a neuraxonal dystrophy, characterized by the childhood onset of unrelenting progressive gait disturbance, spasticity, and dementia associated with prominent extrapyramidal signs such as dystonia, chor...

BACKGROUND Hallervorden-Spatz syndrome is an autosomal recessive disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. Many patients with this disease have mutations in the gene encoding pantothenate kinase 2 (PANK2); these patients are said to have pantothenate kinase-associated neurodegeneration. In this study, we compared the clinical and radiographic features...

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 niagra on MR! of brain. Occasiona...

Hallervorden-Spatz disease is a rare neurological disorder characterized by pyramidal and extrapyramidal manifestations, dysarthria and dementia. Its onset is usually in childhood and most patients have a fatal outcome in few years. A high percentage of cases are hereditary with a recessive autosomal pattern. In the majority of the patients reported, a mutation of the gene that encodes the pant...

Senile chorea is a well recognised but poorly understood clinical entity characterised by a slowly progressive, generalised chorea in elderly people without mental deterioration or a clear underlying cause. The Hallervorden-Spatz syndrome is typically thought of as a paediatric condition with extrapyramidal features and dementia. However, it has been described in adults usually presenting with ...