Cerebellar ataxia can occur as a result of many diseases and may present with symptoms of an inability to coordinate balance, gait, extremity and eye movements.[2] Lesions to the cerebellum can cause dyssynergia, dysmetria, dysdiadochokinesia, dysarthria and ataxia of stance and gait.[3] Deficits are observed with movements on the same side of the body as the lesion (ipsilateral).[2] Clinicians often use visual observation of people performing motor tasks in order to look for signs of ataxia.[2]
Signs and symptoms
Damage to the cerebellum causes impairment in motor skills and can cause nystagmus. Almost a third of people with isolated, late onset cerebellar ataxia go on to develop multiple system atrophy.[4]
The cerebellum's role has been observed as not purely motor. It is combined with intellect, emotion and planning.[5] Cerebellar deficits can be estimated using clinical rating scales, such as SODA for ocular deficits. [6]
Primary auto-immune ataxias (PACA) lack diagnostic biomarkers.[10] Cerebellar ataxias can be classified as sporadic, autosomal recessive, X-linked, autosomal dominant and of mitochondrial origin. [11]
Treatment
"For many years, it was thought that postural and balance disorders in cerebellar ataxia were not treatable. However, the results of several recent studies suggest that rehabilitation can relieve postural disorders in patients with cerebellar ataxia...There is now moderate level evidence that rehabilitation is efficient to improve postural capacities of patients with cerebellar ataxia – particularly in patients with degenerative ataxia or multiple sclerosis. Intensive rehabilitation programs with balance and coordination exercises are necessary. Although techniques such as virtual reality, biofeedback, treadmill exercises with supported bodyweight and torso weighting appear to be of value, their specific efficacy has to be further investigated. Drugs have only been studied in degenerative ataxia, and the level of evidence is low."[12]
Some effects of cerebellar ataxia may be reduced to varying degrees by means of Frenkel exercises.
Additionally, mild to moderate cerebellar ataxia may be treatable with buspirone.[15]
It is thought that the buspirone increases the serotonin levels in the cerebellum and so decreases ataxia.
Behavioral intervention
Behavioral intervention is successful when it involves engaging knowledge of the interests and general backgrounds of individuals with cerebellar ataxia. An intervention technique for speech is to focus on optimizing respiratory and vocal resources as well as training compensatory strategies.[16]
^"Cerebellar ataxia". BBC News. November 30, 2004.
^ a b cFerrarin, M.; Gironi, M.; Mendozzi, L.; Nemni, R.; Mazzoleni, P.; Rabuffetti, M. (2005). "Procedure for the quantitative evaluation of motor disturbances in cerebellar ataxic patients". Medical & Biological Engineering & Computing. 43 (3): 349–56. doi:10.1007/BF02345812. PMID 16035223. S2CID 1307431.
^Diener, H.-C.; Dichgans, J. (1992). "Pathophysiology of cerebellar ataxia". Movement Disorders. 7 (2): 95–109. doi:10.1002/mds.870070202. PMID 1584245. S2CID 19100385.
^Multiple System Atrophy~differential at eMedicine
^Manto, Mario; Marien, Peter (2015). "Schmahmann's syndrome - identification of the third cornerstone of clinical ataxiology". Cerebellum & Ataxias. 2 (1): 2. doi:10.1186/s40673-015-0023-1. PMC 4552302. PMID 26331045.
^Shaikh, A. G.; Kim, J. S.; Froment, C.; Koo, Y. J.; Dupre, N.; Hadjivassiliou, M.; Honnorat, J.; Kothari, S.; Mitoma, H.; Rodrigue, X.; Soong, B. W.; Subramony, S. H.; Strupp, M.; Schmahmann, J.; Manto, M. (2022). "Scale for Ocular motor Disorders in Ataxia (SODA)". Journal of the Neurological Sciences. 443: 120472. doi:10.1016/j.jns.2022.120472. PMID 36403298. S2CID 253156325.
^Hadjivassiliou, Marios; Graus, Francesc; Honnorat, Jerome; Jarius, Sven; Titulaer, Maarten; Manto, Mario; Hoggard, Nigel; Sarrigiannis, Ptolemaios; Mitoma, Hiroshi (2020-04-23). "Diagnostic Criteria for Primary Autoimmune Cerebellar Ataxia-Guidelines from an International Task Force on Immune-Mediated Cerebellar Ataxias". Cerebellum (London, England). 19 (4): 605–610. doi:10.1007/s12311-020-01132-8. ISSN 1473-4230. PMC 7351847. PMID 32328884.
^Manto, Mario; Gruol, Donna L.; Schmahmann, Jeremy; Koibuchi, Noriyuki; Rossi, Ferdinando, eds. (2013). Handbook of the Cerebellum and Cerebellar Disorders. Springer Netherlands. ISBN 9789400713321.
^Marquer, A.; Barbieri, G.; Pérennou, D. (2014). "The assessment and treatment of postural disorders in cerebellar ataxia: A systematic review". Annals of Physical and Rehabilitation Medicine. 57 (2): 67–78. doi:10.1016/j.rehab.2014.01.002. PMID 24582474.
^Mitoma, H.; Manto, M. (2016-05-20). "The physiological basis of therapies for cerebellar ataxias". Therapeutic Advances in Neurological Disorders. 9 (5): 396–413. doi:10.1177/1756285616648940. PMC 4994778. PMID 27582895.
^Grimaldi, Giuliana; Oulad Ben Taib, Nordeyn; Manto, Mario; Bodranghien, Florian (2014). "Marked reduction of cerebellar deficits in upper limbs following transcranial cerebello-cerebral DC stimulation: tremor reduction and re-programming of the timing of antagonist commands." Front Syst Neurosci. 8 (9): 9. doi:10.3389/fnsys.2014.00009. PMC 3906576. PMID 24523678.
^Trouillas, Paul; Xie, Jing; Adeleine, Patrice (1996). "Treatment of cerebellar ataxia with buspirone: a double-blind study". The Lancet. 348 (9029): 759. doi:10.1016/S0140-6736(05)65674-7. PMID 8806320. S2CID 29423962.
^Schalling, Ellika; Hartelius, Lena (2013). "Speech in spinocerebellar ataxia". Brain and Language. 127 (3): 317–22. doi:10.1016/j.bandl.2013.10.002. PMID 24182841. S2CID 24639141.