Hallervorden-Spatz disease.
نویسندگان
چکیده
We describe fluid attenuated inversion recovery sequence MRI and proton MR spectroscopy appearances of the brain in a 7-year old Saudi boy who presented with the clinical features of Hallervorden-Spatz disease to King Fahad National Guard Hospital, Riyadh, Kingdom of Saudi Arabia.
منابع مشابه
Hallervorden-Spatz syndrome: a rare cause of extrapyramidal manifestations
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...
متن کاملRadiologic features of 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 niagra on MR! of brain. Occasiona...
متن کاملLate adult onset chorea with typical pathology of Hallervorden-Spatz syndrome.
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 ...
متن کاملHallervorden spatz disease: MR and pathological findings of a rare case.
We describe a child with pathologically proven Hallervorden Spatz disease. He presented with extrapyramidal symptoms and characteristic "eye-of-the-tiger" sign on magnetic resonance imaging. He was given the possible benefit if any of deep brain stimulation with no much improvement. Pathological examination of the brain showed iron deposition in bilateral globus pallidi, spongiform change and n...
متن کاملAdult onset Hallervorden-Spatz disease with psychotic symptoms.
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...
متن کاملGenetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome.
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...
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ورودعنوان ژورنال:
- Neurosciences
دوره 11 4 شماره
صفحات -
تاریخ انتشار 2006