SENDA disease

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

It was previously known as static encephalopathy with neurodegeneration in childhood (SENDA), but it was renamed after the underlying genetic abnormality was identified, and named in accordance to the other NBIA.

Iron accumulation in the brain specifically in globus pallidus and substantia nigra. This disorder is thought to be genetically determined with defective iron metabolism.

It is the only X-linked subtype of NBIA.

Characteristic clinical features include: 3

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

Other clinical features include:

  • spastic cerebral palsy
  • spastic quadriplegia
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) and substantia nigra, hypointense T1 central band within the hyperintense substantia nigra 3,4

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

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Article Information

rID: 25502
Synonyms or Alternate Spellings:
  • Static encephalopathy with neurodegeneration in childhood
  • SENDA disease
  • Static encephalopathy with neurodegeneration in childhood (SENDA)

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