SENDA syndrome refers to a static encephalopathy of childhood with neurodegeneration in adulthood . It is a subtype of neurodegeneration with brain iron accumulation.
Iron accumulation in the brain specifically in globus pallidus and substantia nigra . This disorder is thought be genetically determined with defective iron metabolism .
- spastic cerebral palsy
- mental retardation in childhood
- parkinsonism features
- intellectual decline
- spastic quadriplegia
Signal charcteristics include
- T1 - hyperintense area with central hypointense area in substantia nigra.
- T2 - hypointense signals in globus pallidus and subsatantia nigra.
- 1. Schneider SA, Bhatia KP. Syndromes of neurodegeneration with brain iron accumulation. Semin Pediatr Neurol. 2012;19 (2): 57-66. doi:10.1016/j.spen.2012.03.005 - Pubmed citation
- 2. Kimura Y, Sato N, Sugai K et-al. MRI, MR spectroscopy, and diffusion tensor imaging findings in patient with static encephalopathy of childhood with neurodegeneration in adulthood (SENDA). Brain Dev. 2013;35 (5): 458-61. doi:10.1016/j.braindev.2012.07.008 - Pubmed citation