Multiple System Atrophy – Cerebellar type (a.k.a MSA-C)

Male / 65 , Chief complaint : bradykinesia, gait disturbance (onset : 10MA)

Multiple System Atrophy - Cerebellar type
1) no remarkable finding at mid brain level (no definite atrophic change at mid brain, no definite par compacta narrowing, no definite atrophic change or flattening of both putamina)

Multiple System Atrophy - Cerebellar type
2) diffuse atrophic change in cerebellum and pons. note that subtle T2 hyperintense line at pons, hot cross bun sign.

Multiple system atrophy (MSA)

  • Adult onset fatal neurodegenerative disease, unknown etiology
  • Various degrees of cerebellar ataxia, autonomic dysfunction, parkinsonism, and corticospinal dysfunction (Parkinson-like feature in 85-90%)
  • No effective treatment (nonresponsive to L-dopa)

Three clinical subtypes of MSA

  1. Extrapyramidal (MSA-P) (60%) : Striatonigral degeneration (SND) – parkinsonian symptoms are main
  2. Cerebellar (MSA-C) (32%) : Sporadic olivopontocerebellar atrophy (sOPCA) – cerebellar symptoms are main
  3. Autonomic (MSA-A) (less than 5%) : Shy-Drager syndrome (SDS) – autonomic failure

Most paitents expire in 10 years after diagnosis

Reference)

J Mov Disord. 2018 Sep; 11(3): 107–120. 2018 Aug 9. doi: 10.14802/jmd.18020

Ann Indian Acad Neurol. 2008 Oct-Dec; 11(4): 257–258. doi: 10.4103/0972-2327.44565

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