نتایج جستجو برای: pantothenate kinase

تعداد نتایج: 227686  

2016
Daniel Nassif João Santos Pereira Mariana Spitz Cláudia Capitão Alessandra Faria

Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder caused by mutation in the PANK2 gene. It is characterized by abnormal brain iron accumulation, mainly in the globus pallidus. PKAN is included in a group of disorders known as neurodegeneration with brain iron accumulation (NBIA). We report a case of atypical PKAN with its most characteristic presentation...

Journal: :Neuro endocrinology letters 2005
Alena Zumrová Anna Krepelová Martin Kyncl Tatána Maríková Miluse Prosková Renáta Cíbochová Vera Sebronová Vladimír Komárek

Hallervorden-Spatz disease (HSD) was and is known as a rare disorder primarily characterized by progressive extrapyramidal dysfunction and dementia alongside optic nerve atrophy or retinal degeneration and pyramidal signs. The rate of occurence of HSD is thus far unknown. Progress in DNA diagnostics stirred up a nomenclature and from HSD, or, perhaps better put, the Hallervorden-Spatz syndrome,...

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