MRI,MR spectroscopy,and diffusion tensor imaging findings in patient with static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) |
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Authors: | Yukio Kimura Noriko Sato Kenji Sugai Shinsuke Maruyama Miho Ota Kouhei Kamiya Kimiteru Ito Yasuhiro Nakata Masayuki Sasaki Hideharu Sugimoto |
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Affiliation: | 1. Department of Radiology, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan;2. Child Neurology, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan;3. Department of Radiology, Jichi Medical University, Shimotsuke, Tochigi, Japan |
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Abstract: | Static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) is a recently established disorder that is a subtype of neurodegeneration with brain iron accumulation (NBIA). We presented the first case report of SENDA of a 39-year-old female. She had psychomotor retardation from childhood and remained static for two decades. Then, at the age of 30, she developed severe dystonia and parkinsonism. Brain MRI revealed T2-weighted hypointensity signal in the globus pallidus and substantia nigra, and T1-weighted hyperintensity signal in the substantia nigra with a central hypointensity area. These clinical and imaging findings are characteristic of SENDA. Advanced MRI, including 1H-MR spectroscopy (MRS) and diffusion tensor imaging (DTI), demonstrated similar findings of pantothenate kinase-associated neurodegeneration (PKAN), which is a major syndrome of SENDA. MRI plays a crucial role in the diagnosis of NBIA, especially SENDA. |
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Keywords: | Static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) Neurodegeneration with brain iron accumulation (NBIA) DTI MRS PRESTO |
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