title: Cerebellar disorders: clinical/radiologic findings and modern imaging tools.
abstract: Cerebellar disorders, also called cerebellar ataxias, comprise a large group of sporadic and genetic diseases. Their core clinical features include impaired control of coordination and gait, as well as cognitive/behavioral deficits usually not detectable by a standard neurologic examination and therefore often overlooked. Two forms of cognitive/behavioral syndromes are now well identified: (1) the cerebellar cognitive affective syndrome, which combines an impairment of executive functions, including planning and working memory, deficits in visuospatial skills, linguistic deficiencies such as agrammatism, and inappropriate behavior; and (2) the posterior fossa syndrome, a very acute form of cerebellar cognitive affective syndrome occurring essentially in children. Sporadic ataxias include stroke, toxic causes, immune ataxias, infectious/parainfectious ataxias, traumatic causes, neoplasias and paraneoplastic syndromes, endocrine disorders affecting the cerebellum, and the so-called "degenerative ataxias" (multiple system atrophy, and sporadic adult-onset ataxias). Genetic ataxias include mainly four groups of disorders: autosomal-recessive cerebellar ataxias, autosomal-dominant ataxias (spinocerebellar ataxias and episodic ataxias), mitochondrial disorders, and X-linked ataxias. In addition to biochemical studies and genetic tests, brain imaging techniques are a cornerstone for the diagnosis, clinicoanatomic correlations, and follow-up of cerebellar ataxias. Modern radiologic tools to assess cerebellar ataxias include: functional imaging studies, magnetic resonance spectroscopy, volumetric studies, and tractography. These complementary methods provide a multimodal appreciation of the whole long-range cerebellar network functioning, and allow the extraction of potential biomarkers for prognosis and rating level of recovery after treatment.
authors.affiliation: Department of Neurology, Université Libre de Bruxelles Erasme, Brussels, Belgium. Electronic address: mmanto@ulb.ac.be.
authors.forename: Mario
authors.initials: M
authors.lastname: Manto
authors.affiliation: Neuroimaging Service, Centre National d'Ophtalmologie des Quinze-Vingts, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Habas
mesh_terms: D002526:Cerebellar Diseases / Q000000981:diagnostic imaging*; D002531:Cerebellum / Q000000981:diagnostic imaging*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D059906:Neuroimaging; D009460:Neurologic Examination
keywords: MRI; ataxias; cerebellum; genetic; imaging; sporadic; tractography; volumetry