BPAN 101: NBIA

NEURODEGENERATION WITH BRAIN IRON ACCUMULATION (NBIA)

Neurodegeneration with brain iron accumulation (NBIA) comprises a genetically heterogeneous group of progressive neurologic disorders. NBIA is clinically characterized by a motor disorder with intellectual or cognitive deterioration in the majority of cases. Increased iron accumulation is evident in the basal ganglia and other areas of the brain although, in certain NBIA subtypes, this is not always present at the onset of neurologic symptoms. Iron accumulation is a normal

phenomenon in the aging brain.

Excessive iron accumulation is also seen in other neurodegenerative disorders such as Parkinson, Alzheimer, and Huntington diseases.

Overview of NBIA Disorders

http://www.nbiadisorders.org/about-nbia/overview-of-nbia-disorders

https://www.e-jmd.org/upload/jmd-8-1-1.pdf

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