Beta-propeller protein-associated neurodegeneration

Beta-propeller protein-associated neurodegeneration (BPAN) is a rare subtype of neurodegeneration with brain iron accumulation disease (NBIA). It is the only X-linked subtype of NBIA.

Clinical presentation

Characteristic clinical features include: 1

  • global developmental delay during chilhood
  • sudden onset of neurocognitive changes during adolescence or young adulthood associated with levodopa-resistant parkinsonism, dystonia, and dementia

It was previously known as static encephalopathy with neurodegeneration in adulthood (SENDA) because of these clinical features.

Radiographic features

MRI

MRI findings are characterized by iron accumulation in the globus pallidus and substantia nigra.

T1: hyperintense signal in the globus pallidus (called the halo sign) 2 and substantia nigra, hypointense T1 central band within the hyperintense substantia nigra

T2: markedly hypointense signal in the globus pallidus and substantia nigra 

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Article information

rID: 53442
Synonyms or Alternate Spellings:
  • BPAN
  • static encephalopathy with neurodegeneration in adulthood
  • SENDA

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