WordNet

nervous disorder of late childhood and early adulthood; characterized by ataxic gait and hesitating or explosive speech and nystagmus
inability to coordinate voluntary muscle movements; unsteady movements and staggering gait (同)ataxy, dyssynergia, motor_ataxia
relating to or associated with the cerebellum; "cerebellar artery"

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/06/21 10:08:23」(JST)

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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 direct injury, alcohol use, and genetic disease.

Misfunction of the sodium-potassium pump may be a factor in some cerebellar ataxias. The Na+
-K+
pump has been shown to control and set the intrinsic activity mode of cerebellar Purkinje neurons.^[4] This suggests that the pump might not simply be a homeostatic, "housekeeping" molecule for ionic gradients; but could be a computational element in the cerebellum and the brain. Indeed, an ouabain block of Na+
-K+
pumps in the cerebellum of a live mouse results in it displaying ataxia and dystonia.^[5] Ataxia is observed for lower ouabain concentrations, dystonia is observed at higher ouabain concentrations.

Considering nicotine's apparent protective effect on other parts of the brain, surprisingly nicotine is toxic to the cerebellum.

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. ^[6]

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

Treatment

It is widely thought that cerebellar ataxia is untreatable.

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. ^[7]

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. ^[8] Another intervention technique for speech is to focus on optimizing respiratory and vocal resources as well as training compensatory strategies. ^[9]

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.

References

^ "Cerebellar ataxia". BBC News. 2004-11-30. Retrieved 2007-07-29.
^ ^a ^b ^c Ferrarin M, Gironi M, Mendozzi L, Nemni R, Mazzoleni P, Rabuffetti M (May 2005). "Procedure for the quantitative evaluation of motor disturbances in cerebellar ataxic patients". Med Biol Eng Comput 43 (3): 349–56. doi:10.1007/BF02345812. PMID 16035223.
^ Diener HC, Dichgans J (1992). "Pathophysiology of cerebellar ataxia". Mov. Disord. 7 (2): 95–109. doi:10.1002/mds.870070202. PMID 1584245.
^ Forrest MD, Wall MJ, Press DA, Feng J (December 2012). "The Sodium-Potassium Pump Controls the Intrinsic Firing of the Cerebellar Purkinje Neuron". PLoS ONE 7 (12): e51169. doi:10.1371/journal.pone.0051169. PMC 3527461. PMID 23284664.
^ Calderon DP, Fremont R, Kraenzlin F, Khodakhah K (March 2011). "The neural substrates of rapid-onset Dystonia-Parkinsonism". Nature Neuroscience 14 (3): 357–65. doi:10.1038/nn.2753. PMC 3430603. PMID 21297628.
^ Lopez-Bastida, J., Perestelo-Perez, L., Monton-alvarez, F., & Serrano-Aguilar, P. (2007). Social economic costs and health-related quality of life in patients with degenerative cerebellar ataxia in Spain. Movement Disorders, 23, 212-217. doi:10.1002/mds.21798
^ Source?
^ Mackenzie, C., & Lowit, A. (2007). Behavioural intervention effects in dysarthria following stroke: Communication effectiveness, intelligibility and dysarthria impact. International Journal of Language & Communication Disorders, 42(2), 131-153.
^ Schalling, E., & Hartelius, L. (2013). Speech in spinocerebellar ataxia. Brain & Language, 127, 317-322.

UpToDate Contents

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1. 成人における小脳性運動失調の概要 overview of cerebellar ataxia in adults
2. 小児における急性小脳性運動失調 acute cerebellar ataxia in children
3. 脊髄小脳変性症 the spinocerebellar ataxias
4. 急性運動失調を呈する小児に対するアプローチ approach to the child with acute ataxia
5. 遺伝性運動失調の概要 overview of the hereditary ataxias

English Journal

Subsurface cistern (SSC) proliferation in Purkinje cells of the rat cerebellum in response to acute and chronic exposure to paint thinner: A light and electron microscopy study.

Martínez-Alfaro M1, Cárabez-Trejo A2, Sandoval-Zapata F3, Morales-Tlalpan V4, Palma-Tirado L3.
Experimental and toxicologic pathology : official journal of the Gesellschaft für Toxikologische Pathologie.Exp Toxicol Pathol.2014 Sep;66(7):323-32. doi: 10.1016/j.etp.2014.04.003. Epub 2014 May 10.
Intentional inhalation and occupational exposure are two ways humans are exposed to thinner, a widely employed solvent in industry. Inhalation of thinner induces toxic effects in various organs, with the cerebellum being one of the most affected structures of the CNS. The aim of this work was to des
PMID 24820124

Age and CGG-repeat length are associated with neuromotor impairments in at-risk females with the FMR1 premutation.

Kraan CM1, Hocking DR2, Georgiou-Karistianis N1, Metcalfe SA3, Archibald AD4, Fielding J1, Trollor J5, Bradshaw JL1, Cohen J6, Cornish KM7.
Neurobiology of aging.Neurobiol Aging.2014 Sep;35(9):2179.e7-2179.e13. doi: 10.1016/j.neurobiolaging.2014.03.018. Epub 2014 Mar 20.
Recent studies report a higher risk of dementia and motor symptoms in females with the fragile X mental retardation 1 premutation (PM-carriers) than has hitherto been appreciated. Here, we use dual-task gait paradigms to identify potential markers of cognitive and motor decline in female PM-carriers
PMID 24814676

RNAi silencing of P/Q-type calcium channels in Purkinje neurons of adult mouse leads to episodic ataxia type 2.

Salvi J1, Bertaso F2, Mausset-Bonnefont AL3, Metz A1, Lemmers C4, Ango F2, Fagni L2, Lory P5, Mezghrani A6.
Neurobiology of disease.Neurobiol Dis.2014 Aug;68:47-56. doi: 10.1016/j.nbd.2014.04.005. Epub 2014 Apr 21.
Episodic ataxia type-2 (EA2) is a dominantly inherited human neurological disorder caused by loss of function mutations in the CACNA1A gene, which encodes the CaV2.1 subunit of P/Q-type voltage-gated calcium channels. It remains however unknown whether the deficit of cerebellar CaV2.1 in adult is in
PMID 24768804

Japanese Journal

多系統萎縮症の摂食・嚥下障害とその対策 (特集神経変性疾患の摂食・嚥下障害とコミュニケーション障害へのアプローチ)

山本敏之
コミュニケーション障害学 30(2), 89-94, 2013-09
NAID 40019813039

伝染性単核球症に続発し脳脊髄液に抗グルタミン酸受容体δ2抗体をみとめた急性小脳失調症

村上秀友,飯島昭二,河村満 [他]
臨床神経学 53(7), 555-558, 2013-07
NAID 40019732449

Related Pictures

★リンクテーブル★

リンク元	「小脳性運動失調症」「dysmetria」「小脳失調」「小脳失調症」「小脳性失調」
拡張検索	「spinocerebellar ataxia type 1」「spinocerebellar ataxia type 2」「spinocerebellar ataxia type 4」「spinocerebellar ataxia」
関連記事	「cerebellar」