Epidemiology of Cerebellar Diseases and Therapeutic Approaches

Michael S. Salman

Diseases involving the cerebellum occur relatively commonly in children and adults around the globe. Many factors influence their epidemiology including geography, ethnicity, consanguinity, and the methodology used to ascertain patients. In addition, reliable epidemiological data rely heavily on accurate disease classification. Continuous advances in genetic research and neuroimaging modalities have resulted in improved understanding of cerebellar diseases and have led to several revisions in their classification. Recent global epidemiological studies on ataxia reported an estimated overall prevalence rate of 26/100,000 in children, a prevalence rate of dominant hereditary cerebellar ataxia of 2.7/100,000, and a prevalence rate of recessive hereditary cerebellar ataxia of 3.3/100,000. The management of cerebellar diseases is multidisciplinary and multimodal. General supportive and symptomatic therapies should be initiated. Genetic counseling should be offered, where appropriate. Few drugs, specific motor rehabilitation programs, and noninvasive cerebellar stimulation for the treatment of ataxia have been developed and seem to show early promise, but more studies are needed to replicate and fine-tune their benefits further. Some disease-specific treatments are available. For example, acetazolamide or 4-aminopyridine for patients with episodic ataxia type 2 and vitamin E for patients with ataxia caused by vitamin E deficiency.