SENDA syndrome

SENDA syndrome refers to a static encephalopathy of childhood with neurodegeneration in adulthood . It is a subtype of neurodegeneration with brain iron accumulation. 

Pathology

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

Clinical presentation

  • spastic cerebral palsy
  • mental retardation in childhood
  • parkinsonism features
  • dystonia
  • intellectual decline
  • spastic quadriplegia

Radiographic features 

MRI 

Signal charcteristics include

  • T1 - hyperintense area with central hypointense area in substantia nigra.
  • T2 - hypointense signals in globus pallidus and subsatantia nigra.
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Article Information

rID: 25502
Synonyms or Alternate Spellings:

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