Hallervorden-Spatz syndrome in two siblings diagnosed by clinical features and magnetic resonance imaging.

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dc.contributor.authorTiamkao, Sen_US
dc.contributor.authorNitinavakarn, Ben_US
dc.contributor.authorJitpimolmard, Sen_US
dc.date.accessioned2009-05-27T19:17:31Z
dc.date.available2009-05-27T19:17:31Z
dc.date.issued2000-12-20en_US
dc.descriptionChotmaihet Thangphaet.en_US
dc.description.abstractThe Hallervorden-Spatz syndrome (HSS) is a rare condition characterized by extrapyramidal and pyramidal signs, dystonia, dysarthria, retinal degeneration, dementia and a progressive course. The development of magnetic resonance imaging (MRI) has increased the number of clinical and pathological reports of HSS. MRI pallidal abnormalities are called "eye of the tiger" signs. The combination of clinical features and MRI findings can be considered as highly suggestive of a diagnosis of HSS. Patient 1 was a 28 year old man who had been well until the age of 25 years. He developed dysarthria, difficulty with his gait and dystonia in his arms at the age of 28 years. Patient 2 was a 33 year old man who was the older brother of the first patient. He developed gait difficulty, tongue dystonia and dystonia of both arms at the age of 25 years. Each patient had spastic gait, dysarthria, dystonic posturing of both arms and generalized hyperreflexia, but no Kayser-Fleischer rings or retinitis pigmentosa. Blood chemistry, urine copper, serum copper and serum ceruloplasmin were all normal. MRI of the brain showed the "eye of the tiger" sign in the globus pallidus on T2 weighted images. These siblings had clinical features and MRI findings consistent with HSS. They are the first to be reported in Thailand.en_US
dc.description.affiliationDepartment of Medicine, Faculty of Medicine, Khon Kaen University, Thailand.en_US
dc.identifier.citationTiamkao S, Nitinavakarn B, Jitpimolmard S. Hallervorden-Spatz syndrome in two siblings diagnosed by clinical features and magnetic resonance imaging. Journal of the Medical Association of Thailand. 2000 Dec; 83(12): 1535-40en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/40841
dc.language.isoengen_US
dc.source.urihttps://www.mat.or.th/journal/all.phpen_US
dc.subject.meshAdulten_US
dc.subject.meshBrain --pathologyen_US
dc.subject.meshDystonia --etiologyen_US
dc.subject.meshGaiten_US
dc.subject.meshHumansen_US
dc.subject.meshMagnetic Resonance Imagingen_US
dc.subject.meshMaleen_US
dc.subject.meshPantothenate Kinase-Associated Neurodegeneration --diagnosisen_US
dc.titleHallervorden-Spatz syndrome in two siblings diagnosed by clinical features and magnetic resonance imaging.en_US
dc.typeCase Reportsen_US
dc.typeJournal Articleen_US
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