BPAN 101: BASAL GANGLIA

WHAT IS THE BASAL GANGLIA?

The basal ganglia is an important cluster of nerve cells in the inner brain that are responsible for initiating and integrating movement and for cognitive coordination. It has a significant cognitive role in procedural memory, habit and skill learning, attention, perception and language. Neurodegeneration with brain iron accumulation (NBIA) is a group of inherited neurologic disorders characterized by abnormal accumulation of iron in the base of the basal ganglia.

WHAT IS THE SIGNIFICANCE OF THE BASAL GANGLIA IN BPAN?

The exact location of the abnormal iron accumulation in the basal ganglia (which has five different regions) and the specific cause of that accumulation is different for each of the known NBIA Disorders. This means that finding a cure for each type of NBIA will need different funding and research. Current research and funding has been focused on PKAN because it accounts for 35-50% of the cases of NBIA. There currently are several clinical trials for PKAN. 

 

Parents have been key in raising funds for the other NBIA disorders.

 

The next round of NBIA research grants to focus on BPAN and FAHN

http://www.nbiadisorders.org/news-events/286-bpan-fahn-grants

 

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