Olivopontocerebellar Atrophy Treatment & Management

Updated: Nov 11, 2014
  • Author: Christina J Azevedo, MD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Medical Care

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. [113, 114]
  • Another small trial concluded that buspirone is ineffective. [115]
  • 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. [116, 117, 118] It also appears to have a beneficial effect on the dopamine transporter. [119] 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. [120]
  • 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. [121] 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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Surgical Care

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  • At times, patients may require enteral feeding to decrease the risk of aspiration.
  • Percutaneous endoscopic gastrostomy and jejunostomy tube (J-tube) placement may be necessary.
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Consultations

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  • Consultations with physical and occupational therapists are helpful to increase mobility; the use of assistive devices can significantly increase functional ability. A recent case study using progressively more challenging static and dynamic balance tasks over a 12-week period produced significant improvement in balance in a patient with proven olivopontocerebellar atrophy. [122] A swallowing evaluation can be a very important part of the early consultation.
  • Now that genetic testing is available, it can be performed to confirm the diagnosis of autosomal dominant OPCAs. These patients may not develop symptoms until after the onset of their reproductive years; therefore, family members must be evaluated early if a diagnosis of autosomal dominant OPCA is made. Referral for genetic counseling is advisable in these individuals. Not all patients wish to learn of their risks in the absence of an available treatment, while some individuals may use the information for family planning and other types of planning for the future.
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Diet

As dysphagia progresses with the disease, a pureed diet or enteral feeding may be required.

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Activity

Activity should be allowed ad libitum; however, appropriate measures should be used to minimize falls.

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