نتایج جستجو برای: neurodegeneration with brain iron accumulation

تعداد نتایج: 9480785  

Journal: :Brain : a journal of neurology 2013
Susan J Hayflick Michael C Kruer Allison Gregory Tobias B Haack Manju A Kurian Henry H Houlden James Anderson Nathalie Boddaert Lynn Sanford Sami I Harik Vasuki H Dandu Nardo Nardocci Giovanna Zorzi Todd Dunaway Mark Tarnopolsky Steven Skinner Kenton R Holden Steven Frucht Era Hanspal Connie Schrander-Stumpel Cyril Mignot Delphine Héron Dawn E Saunders Margaret Kaminska Jean-Pierre Lin Karine Lascelles Stephan M Cuno Esther Meyer Barbara Garavaglia Kailash Bhatia Rajith de Silva Sarah Crisp Peter Lunt Martyn Carey John Hardy Thomas Meitinger Holger Prokisch Penelope Hogarth

Neurodegenerative disorders with high iron in the basal ganglia encompass an expanding collection of single gene disorders collectively known as neurodegeneration with brain iron accumulation. These disorders can largely be distinguished from one another by their associated clinical and neuroimaging features. The aim of this study was to define the phenotype that is associated with mutations in...

Journal: :basic and clinical neuroscience 0
sunil gothwal department of pediatrics, all india institute of medical science, new delhi, india. swati nayan sms mc

hallervorden-spatz syndrome is a disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. the disease is caused by mutations in gene encoding pantothenate kinase 2 (pank2) and patients have pantothenate kinase-associated neurodegeneration. we present an 8-year-old boy with progressive muscle dystonia, neuroregression, frequent fall and multiple injury marks of diffe...

Journal: :Pediatrics 2015
Michelle Long Nishard Abdeen Michael T Geraghty Penelope Hogarth Susan Hayflick Sunita Venkateswaran

β-propeller protein-associated neurodegeneration (BPAN) is a recently identified X-linked dominant form of neurodegeneration with brain iron accumulation caused by mutations in the WDR45 gene. BPAN commonly presents as global developmental delay in childhood with rapid onset of parkinsonism and dementia in early adulthood and associated pathognomonic changes seen on brain MRI. In this case repo...

Hallervorden-Spatz syndrome is a disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. The disease is caused by mutations in gene encoding pantothenate kinase 2 (PANK2) and patients have pantothenate kinase-associated neurodegeneration. We present an 8-year-old boy with progressive muscle dystonia, neuroregression, frequent fall and multiple injur...

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: :American journal of clinical pathology 2004
S M Hossein Sadrzadeh Yasi Saffari

Iron is the most important element in the body, essential for almost all types of cells, including brain cells. The role of iron in the brain has been known for years. Iron deficiency and iron excess have been associated with pathophysiology of different brain disorders. Iron deficiency has been reported to have a role in brain development and the pathophysiology of restless legs syndrome. Iron...

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