How is olivopontocerebellar atrophy (OPCA) treated?

Updated: Dec 17, 2018
  • Author: Sombat Muengtaweepongsa, MD, MSc; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Care of olivopontocerebellar atrophy (OPCA) is directed to the treatment of symptoms.

  • Dopaminergic agents, such as levodopa, bromocriptine, or amantadine, have shown minimal benefit.

  • Propranolol has been used for tremor, but the clinical response is generally minimal.

  • Related to the fact that serotonin 5-hydroxytryptophan (5HT) 1-A receptor agonists modulate the serotonergic motor output from the cerebellum, a few small studies have focused on 5HT 1-A receptor agonists, such as tandospirone or buspirone, as a treatment for ataxia. Several such studies have indicated that such medications may produce modest improvement in ataxia caused by various neurodegenerative conditions, including OPCA. [120, 121]

  • Another small trial concluded that buspirone is ineffective. [122]

  • It had also been hypothesized that estrogen might potentiate the 5-HT1A effect because it is neuroprotective of the nigrostriatal system in some animal models. [123, 124, 125] It also appears to have a beneficial effect on the dopamine transporter. [126] Therefore, investigators undertook a trial of 18 patients to study the effect of combined estrogen and buspirone therapy for OPCA. Both groups showed significant improvements in finger-to-nose and rapid alternating movements at 1 month; however, at 12 months (which was the end of the study), there was no statistically significant improvement in any test of cerebellar function. Indeed, there was no evidence that the benefits lasted longer than 3 months. There were trends toward improvement in dysarthria, heel-to-shin testing, and gait speed. No benefit was seen by adding estrogen. [127]

  • A small, brief randomized, double-blind pilot study of patients with cerebellar ataxia of different etiologies assessed the responses of 20 patients given riluzole versus 20 patients given a placebo. Assessment at 4 and 8 weeks showed Class I evidence that riluzole reduced the International Cooperative Ataxia Rating Scale (ICARS) score by at least 5 points. [128] The authors stated their belief that riluzole should now be tested in larger and longer studies. Also needed are studies that use more homogenous patient groups.

  • Supportive care with gait-assisting devices is especially important to minimize falls.


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