Progressive myoclonic ataxia is a movement disorder characterized by defective muscular coordination of a muscle group with its origin in the brain.
The pathology is in the cerebellum.1
Myoclonus in a context of progressive ataxia suggests one clinical form of the Ramsay-Hunt syndrome (progressive myoclonic ataxia, PMA), whose most frequent causes are: celiac disease, mitochondriopathies, some spino-cerebellar degenerations, and some late metabolic disorders.2
What Is Progressive Myoclonic Ataxia In Celiac Disease and/or Gluten Sensitivity?
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- Bhatia KP, Brown P, Gregory R, et al. Progressive myoclonic ataxia associated with coeliac disease. The myoclonus is of cortical origin, but the pathology is in the cerebellum. Brain. Oct 1995;118(Pt 5):1087-93.
- Borg M. Symptomatic myoclonus. Neurophysiol Clin. 2006 Sep-Dec;36(5-6):309-18.