Transient swelling in the globus pallidus and substantia nigra in childhood suggests SENDA/BPAN
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Static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) or β-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA), causing abnormal iron accumulation in the globus pallidus and substantia nigra through autophagic dysfunction due to WDR45 mutations.1,2 SENDA/BPAN is often difficult to diagnose because brain MRI does not detect the characteristic findings in childhood. We found transient hyperintensity and swelling in the globus pallidus and substantia nigra due to infections during childhood as characteristic findings.
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- Received November 27, 2017.
- Accepted in final form February 16, 2018.
- © 2018 American Academy of Neurology
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