Adult-Onset Neurodegeneration With Brain Iron Accumulation and Cortical α-Synuclein and Tau Pathology | Zendy

George K. Tofaris | Zendy; Tamás Révész | Zendy; Thomas S. Jacques | Zendy; Savvas Papacostas | Zendy; Jeremy Chataway | Zendy

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Adult-Onset Neurodegeneration With Brain Iron Accumulation and Cortical α-Synuclein and Tau Pathology

Author(s) -

George K. Tofaris,

Tamás Révész,

Thomas S. Jacques,

Savvas Papacostas,

Jeremy Chataway

Publication year - 2007

Publication title -

archives of neurology

Language(s) - English

Resource type - Journals

eISSN - 1538-3687

pISSN - 0003-9942

DOI - 10.1001/archneur.64.2.280

Subject(s) - neurodegeneration , pathology , substantia nigra , globus pallidus , neuroscience , lewy body , basal ganglia , medicine , parkinson's disease , biology , disease , central nervous system

Neurodegeneration with brain iron accumulation is a rare neurodegenerative disorder characterized by iron deposition in the basal ganglia and neuroaxonal dystrophy. Familial cases with mutations in the pantothenate kinase gene are associated with a specific phenotype. In contrast, sporadic cases are heterogeneous in their clinical presentation.

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