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olivopontocerebellar atrophy
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Olivopontine cerebellar degeneration (Olivopontocerebellar atrophy (OPCA)) may occur sporadically or as an autosomal dominant trait. It is characterised by a general atrophy of the cerebellum spreading in time to involve the pons, medullary olives and other brain stem structures. It can occur at any age but onset in middle life is most common.

Presentation is initially with ataxia, dysarthria, and tremor. Parkinsonian features may develop, accompanied by mild dementia, ophthalmoplegia, pyramidal tract signs and autonomic disturbance.

Notes:

  • olivopontocerebellar atrophy (OPCA) is in fact a group of diseases pathologically characterized by neuronal degeneration of the inferior olivary nuclei, pontine nuclei, and cerebellum (1,2,3)
    • most common form of sporadic OPCA is multiple system atrophy (MSA). MSA is an adult-onset neurodegenerative disease clinically characterized by varying degrees of parkinsonism, cerebellar ataxia, and autonomic dysfunction and was first recognized as a clinicopathologic entity by Graham and Oppenheimer

    • MSA is a fatal adult-onset neurodegenerative disorder of uncertain aetiopathogenesis manifesting with autonomic failure, parkinsonism, and ataxia in any combination
      • mean age of disease onset is approximately 57 years and survival after disease onset lies between six and nine years (1)

      • underlying neuropathology affects central autonomic, striatonigral and olivopontocerebellar pathways and it is associated with distinctive glial cytoplasmic inclusions (GCIs, Papp-Lantos bodies) that contain aggregates of alph-synuclein

    • current treatment options are very limited and mainly focused on symptomatic relief

Reference:

  • N. Stefanova, P. Bucke, S. Duerr, G.K. Wenning Multiple system atrophy: an update Lancet Neurol.;8 (2009):1172-1178
  • Arima K, Ueda K, Sunohara N, et al. NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy. Acta Neuropathol. 1998;96:439-444
  • Jellinger KA, Lantos PL. Papp-Lantos inclusions and the pathogenesis of multiple system atrophy: an update. Acta Neuropathol. 2010;119:657-667

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