A novel dorsal root ganglionopathy: CANVAS syndrome

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) syndrome is  a novel cerebellar ataxia clinically characterized by a combination of cerebellar dysfunction, bilateral vestibular dysfunction, and peripheral sensory neuropathy/neuronopathy.  This novel syndrome may be confused with Machado-Joseph disease or Friedreich ataxia, although the genetics remain elusive.

In the April issue of neurology (22nd April), Professor Halmagyi’s group establish the pathology of this unusual disorder, documenting marked loss of Purkinje cells in the cerbellum, predominantly the vermis, along with degeneration of dorsal columns and dorsal root ganglia.

 

Read more at,  http://www.neurology.org/content/82/16/1410.abstract

 

Dorsal root ganglionopathy is responsible for the sensory impairment in CANVAS

  1. David J. Szmulewicz, FRACP,
  2. Catriona A. McLean, FRCPA,
  3. Michael L. Rodriguez, FRCPA,
  4. Andrew M. Chancellor, FRACP,
  5. Stuart Mossman, FRACP,
  6. Duncan Lamont, FRCP,
  7. Leslie Roberts, FRACP,
  8. Elsdon Storey, FRACP and
  9. G. Michael Halmagyi, FRACP

 

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