Cerebellar ataxia

Main article: Ataxia
Cerebellar ataxia
Classification and external resources
Specialty neurology
ICD-10 G11.1-G11.3
ICD-9-CM 334.3
DiseasesDB 2218
MedlinePlus 001397
MeSH D002524

Cerebellar ataxia is a form of ataxia originating in the cerebellum.[1]

Overview

Cerebellar ataxia can occur as a result of many diseases and presents 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]

Causes

There are many causes of cerebellar ataxia including, among others, autoimmunity to Purkinje cells or other neural cells in the cerebellum,[4][5][6] CNS vasculitis, multiple sclerosis, infection, bleeding, infarction, tumors, direct injury, toxins (e.g., alcohol), genetic disorders, and an association with statin use.[7]

Associated morbidity

Damage to the cerebellum, particularly to the cerebrocerebellum area and the cerebellar vermis, is almost always associated with clinical depression and often with alcoholism. Due to difficulties in mobility, self-care, everyday activities, and pain/discomfort, those with cerebellar ataxia are more likely to be diagnosed with anxiety and depression.[8] Almost a third of patients with isolated, late onset cerebellar ataxia go on to develop multiple system atrophy.[9]

In recent years the cerebellum's role has been observed as not purely motor. It is intimately combined with intellect, emotion and planning.[10]

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."[11]

One approach is that it can be ameliorated to varying degrees by means of Frenkel exercises.

Research using Transcranial direct-current stimulation (TCDCS) and Transcranial magnetic stimulation (TMS) shows promising results.[12]

Additionally, mild to moderate cerebellar ataxia is treated by buspirone.[13]

It is thought that the buspirone increases the serotonin levels in the cerebellum and so decreases ataxia.

Implications for Intervention

Individuals with cerebellar ataxia have full cognitive awareness: it's usually only the physical deterioration that prohibits them from participating in activities of daily living and any other relevant or desired interests. One of the most significant barriers in the lives of these individuals is dysarthria. Due to their cognitive stability, it is important that people who spend time with individuals with this disease are able to communicate as fully as possible with them. This is necessary in order to improve their day-to-day interactions.

Behavioral intervention is successful when it involves engaging knowledge of the interests and general backgrounds of individuals with cerebellar ataxia. Communication maximizing strategies are also useful, such as exaggeration of articulatory gestures, giving full attention to their responses, repeating where necessary, and slowing down speaking rate.[14] Another intervention technique for speech is to focus on optimizing respiratory and vocal resources as well as training compensatory strategies.[15]

These listed intervention techniques can improve quality of life in individuals with this disease and can be helpful for professionals/clinicians in the field as well as loved ones of those affected.

See also

References

  1. ↑ "Cerebellar ataxia". BBC News. November 30, 2004.
  2. 1 2 3 Ferrarin, 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.
  3. ↑ Diener, H.-C.; Dichgans, J. (1992). "Pathophysiology of cerebellar ataxia". Movement Disorders. 7 (2): 95–109. doi:10.1002/mds.870070202. PMID 1584245.
  4. ↑ S. Jarius, B. Wildemann: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 1: Anti-mGluR1, anti-Homer-3, anti-Sj/ITPR1 and anti-CARP VIII J Neuroinflammation 2015; 12, 166 (free)
  5. ↑ S. Jarius, B. Wildemann: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 2: Anti-PKC-gamma, anti-GluR-delta2, anti-Ca/ARHGAP26 and anti-VGCC J Neuroinflammation 2015; 12, 167 (free)
  6. ↑ S. Jarius, B. Wildemann: ‘Medusa head ataxia’: the expanding spectrum of Purkinje cell antibodies in autoimmune cerebellar ataxia. Part 3: Anti-Yo/CDR2, anti-Nb/AP3B2, PCA-2, anti-Tr/DNER, other antibodies, diagnostic pitfalls, summary and outlook J Neuroinflammation 2015; 12, 168 (free)
  7. ↑ "Statin-associated cerebellar ataxia. a brazilian case series". MDLinx. Retrieved 2016-02-22.
  8. ↑ López-Bastida, Julio; Perestelo-Pérez, Lilisbeth; Montón-álvarez, Fernando; Serrano-Aguilar, Pedro (2008). "Social economic costs and health-related quality of life in patients with degenerative cerebellar ataxia in Spain". Movement Disorders. 23 (2): 212–7. doi:10.1002/mds.21798. PMID 17999424.
  9. ↑ Multiple System Atrophy~differential at eMedicine
  10. ↑ Manto, Mario; Marien, Peter (2015). "Schmahmann's syndrome - identification of the third cornerstone of clinical ataxiology.". Cerebellum and Ataxias. 2 (1). doi:10.1186/s40673-015-0023-1. PMC 4552302Freely accessible. PMID 26331045.
  11. ↑ 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.
  12. ↑ 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). doi:10.3389/fnsys.2014.00009. PMC 3906576Freely accessible. PMID 24523678.
  13. ↑ 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.
  14. ↑ Mackenzie, Catherine; Lowit, Anja (2007). "Behavioural intervention effects in dysarthria following stroke: communication effectiveness, intelligibility and dysarthria impact". International Journal of Language & Communication Disorders. 42 (2): 131–53. doi:10.1080/13682820600861776. PMID 17365091.
  15. ↑ 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.
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