نتایج جستجو برای: hallervorden

تعداد نتایج: 273  

Journal: :Indian journal of pediatrics 2002
Seema Kapoor Konstanze Hortnagel Siddhartha Gogia Ritu Paul Vishal Malhotra Anjali Prakash

Hallervorden-Spatz syndrome is a rare autosomal recessive hereditary condition characterized by early onset of progressive movement alteration that include dystonia, rigidity and choreoathetosis usually associated with pyramidal signs and mental deterioration. We report two sisters where diagnosis was missed till MRI showed classic imaging findings. Mutation analysis in one, revealed homozygous...

Journal: :Journal of Neurology, Neurosurgery & Psychiatry 1987

Journal: :Journal of Neurology, Neurosurgery & Psychiatry 1989

Journal: :Hong Kong medical journal = Xianggang yi xue za zhi 2009
Binila Chacko Pratibha Seshadri Thambu D Sudarsanam

Neurodegeneration with brain iron accumulation type 1 (previously known as Hallervorden-Spatz syndrome) is a rare neurodegenerative disorder characterised by its typical clinical and radiological features. We present a case of an adolescent girl with rapidly progressive dystonia in whom the diagnosis of the above disorder was made prior to radiological investigation. This report has been made t...

Journal: :Brain : a journal of neurology 2011
Michael C Kruer Mark Hiken Allison Gregory Alessandro Malandrini David Clark Penny Hogarth Marjorie Grafe Susan J Hayflick Randall L Woltjer

Pantothenate kinase-associated neurodegeneration is a form of neurodegeneration with brain iron accumulation, characterized by a progressive movement disorder and prominent iron deposition in the globus pallidus. Formerly referred to as Hallervorden-Spatz syndrome, the disorder was renamed pantothenate kinase-associated neurodegeneration after discovery of the causative gene, PANK2. Although th...

Journal: :AJNR. American journal of neuroradiology 1993
M Savoiardo W C Halliday N Nardocci L Strada L D'Incerti L Angelini V Rumi J D Tesoro-Tess

PURPOSE To compare the MR findings of eight cases with clinical diagnosis of Hallervorden-Spatz disease (HSD) with the pathologic findings of two other cases of HSD. MATERIALS AND METHODS The eight imaged cases were studied with 0.5-T (seven cases) and/or 1.5-T (five cases) units. Six patients also had CT scans. The two other cases with proven HSD had detailed histologic evaluation. RESULTS...

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