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{{Ataxia}} | {{Ataxia}} | ||
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==Overview== | ==Overview== | ||
There is no specific treatment for ataxia as such, | There is no specific treatment for ataxia as such, although there may be for the underlying cause. The main treatment available to patients is rehabilitation training, which can include physical, occupational, and speech therapy. The disability of ataxia may be reduced by physical therapy, including exercises, along with leg braces or shoe splints. If foot alignment has been affected then a cane or walker is often used in the effort to prevent falls. | ||
==Medical Therapy== | ==Medical Therapy== | ||
* The movement disorders associated with ataxia can be managed by pharmacological treatments and through [[physical therapy]] and [[occupational therapy]] to reduce [[disability]].<ref name="pmid17000340">{{cite journal | author = Perlman SL | title = Ataxias | journal = Clin. Geriatr. Med. | volume = 22 | issue = 4 | pages = 859–77, vii | year = 2006 | month = November | pmid = 17000340 | doi = 10.1016/j.cger.2006.06.011 }}</ref> Some drug treatments that have been used to control ataxia include: 5-hydroxytryptophan (5-HTP), idebenone, [[amantadine]], [[physostigmine]], L-carnitine or derivatives, [[trimethoprim–sulfamethoxazole]], [[vigabatrin]], phosphatidylcholine, [[acetazolamide]], 4-aminopyridine, [[buspirone]], or a combination of [[coenzyme Q10]] and [[vitamin E]]. | *Recovery tends to be better in individuals with a single focal injury (such as [[stroke]] or a [[benign tumour]]), compared to those who have a neurological degenerative condition.<ref name="can rehabilitation help">{{cite journal | author = Morton SM, Bastian AJ | title = Can rehabilitation help ataxia? | journal = Neurology | volume = 73 | issue = 22 | pages = 1818–9 | year = 2009 | month = December | pmid = 19864635 | doi = 10.1212/WNL.0b013e3181c33b21 | url = }}</ref> | ||
*The movement disorders associated with ataxia can be managed by pharmacological treatments and through [[physical therapy]] and [[occupational therapy]] to reduce [[disability]].<ref name="pmid17000340">{{cite journal | author = Perlman SL | title = Ataxias | journal = Clin. Geriatr. Med. | volume = 22 | issue = 4 | pages = 859–77, vii | year = 2006 | month = November | pmid = 17000340 | doi = 10.1016/j.cger.2006.06.011 }}</ref> Some drug treatments that have been used to control ataxia include: 5-hydroxytryptophan (5-HTP), idebenone, [[amantadine]], [[physostigmine]], L-carnitine or derivatives, [[trimethoprim–sulfamethoxazole]], [[vigabatrin]], phosphatidylcholine, [[acetazolamide]], 4-aminopyridine, [[buspirone]], or a combination of [[coenzyme Q10]] and [[vitamin E]]. | |||
* [[Physical therapy]] requires a focus on adapting activity and facilitating [[motor learning]] for retraining specific functional motor patterns.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease">{{cite journal | author = Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L | title = Intensive coordinative training improves motor performance in degenerative cerebellar disease | journal = Neurology | volume = 73 | issue = 22 | pages = 1823–30 | year = 2009 | month = December | pmid = 19864636 | doi = 10.1212/WNL.0b013e3181c33adf }}</ref> A recent systematic review suggested that physical therapy is effective, but there is only moderate evidence to support this conclusion.<ref name="pmid19114434">{{cite journal | author = Martin CL, Tan D, Bragge P, Bialocerkowski A | title = Effectiveness of physiotherapy for adults with cerebellar dysfunction: a systematic review | journal = Clin Rehabil | volume = 23 | issue = 1 | pages = 15–26 | year = 2009 | month = January | pmid = 19114434 | doi = 10.1177/0269215508097853 }}</ref> The most commonly used physical therapy interventions for cerebellar ataxia are vestibular habituation, proprioceptive neurofaciliation, Frenkel exercises, and balance training; however, therapy is often highly individualized and gait and coordination training are large components of therapy. | *[[Physical therapy]] requires a focus on adapting activity and facilitating [[motor learning]] for retraining specific functional motor patterns.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease">{{cite journal | author = Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L | title = Intensive coordinative training improves motor performance in degenerative cerebellar disease | journal = Neurology | volume = 73 | issue = 22 | pages = 1823–30 | year = 2009 | month = December | pmid = 19864636 | doi = 10.1212/WNL.0b013e3181c33adf }}</ref> A recent systematic review suggested that physical therapy is effective, but there is only moderate evidence to support this conclusion.<ref name="pmid19114434">{{cite journal | author = Martin CL, Tan D, Bragge P, Bialocerkowski A | title = Effectiveness of physiotherapy for adults with cerebellar dysfunction: a systematic review | journal = Clin Rehabil | volume = 23 | issue = 1 | pages = 15–26 | year = 2009 | month = January | pmid = 19114434 | doi = 10.1177/0269215508097853 }}</ref> The most commonly used physical therapy interventions for cerebellar ataxia are vestibular habituation, proprioceptive neurofaciliation, Frenkel exercises, and balance training; however, therapy is often highly individualized and gait and coordination training are large components of therapy. | ||
* Current research suggests that, if a person is able to walk with or without a [[mobility aid]], physical therapy should include an exercise program addressing five components: static balance, dynamic balance, trunk-limb coordination, stairs, and [[contracture]] prevention. Once the physical therapist determines that the individual is able to safely perform parts of the program independently, it is important that the individual be prescribed and regularly engage in a supplementary home exercise program that incorporates these components to further improve long term outcomes. These outcomes include balance tasks, gait, and individual activities of daily living. While the improvements are attributed primarily to changes in the brain and not just the hip and/or ankle joints, it is still unknown whether the improvements are due to adaptations in the cerebellum or compensation by other areas of the brain.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease"/> | *Current research suggests that, if a person is able to walk with or without a [[mobility aid]], physical therapy should include an exercise program addressing five components: static balance, dynamic balance, trunk-limb coordination, stairs, and [[contracture]] prevention. Once the physical therapist determines that the individual is able to safely perform parts of the program independently, it is important that the individual be prescribed and regularly engage in a supplementary home exercise program that incorporates these components to further improve long term outcomes. These outcomes include balance tasks, gait, and individual activities of daily living. While the improvements are attributed primarily to changes in the brain and not just the hip and/or ankle joints, it is still unknown whether the improvements are due to adaptations in the cerebellum or compensation by other areas of the brain.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease" /> | ||
* Decomposition, simplification, or slowing of multi-joint movement may also be an effective strategy that therapists may use to improve function in patients with ataxia.<ref name="pmid9184691">{{cite journal | author = Bastian AJ | title = Mechanisms of ataxia | journal = Phys Ther | volume = 77 | issue = 6 | pages = 672–5 | year = 1997 | month = June | pmid = 9184691 | doi = }}</ref> Training likely needs to be intense and focused (as indicated by one study performed with stroke patients experiencing limb ataxia who underwent intensive upper limb retraining).<ref name="stroke ataxia">{{cite journal | author = Richards L, Senesac C, McGuirk T, Woodbury M, Howland D, Davis S, Patterson T | title = Response to intensive upper extremity therapy by individuals with ataxia from stroke | journal = Top Stroke Rehabil | volume = 15 | issue = 3 | pages = 262–71 | year = 2008 | pmid = 18647730 | doi = 10.1310/tsr1503-262 }}</ref> Their therapy consisted of [[constraint-induced movement therapy]] which resulted in improvements of their arm function.<ref name ="stroke ataxia" /> Treatment should likely include strategies to manage difficulties with everyday activities such as walking. Gait aids (such as a cane or walker) can be provided to decrease the risk of falls associated with impairment of [[Balance (ability)|balance]] or poor [[Motor coordination|coordination]]. Severe ataxia may eventually lead to the need for a [[wheelchair]]. In order to obtain better results, possible coexisting motor deficits need to be addressed in addition to those induced by ataxia. For example, muscle weakness and decreased endurance could lead to increasing fatigue and worse movement patterns. | *Decomposition, simplification, or slowing of multi-joint movement may also be an effective strategy that therapists may use to improve function in patients with ataxia.<ref name="pmid9184691">{{cite journal | author = Bastian AJ | title = Mechanisms of ataxia | journal = Phys Ther | volume = 77 | issue = 6 | pages = 672–5 | year = 1997 | month = June | pmid = 9184691 | doi = }}</ref> Training likely needs to be intense and focused (as indicated by one study performed with stroke patients experiencing limb ataxia who underwent intensive upper limb retraining).<ref name="stroke ataxia">{{cite journal | author = Richards L, Senesac C, McGuirk T, Woodbury M, Howland D, Davis S, Patterson T | title = Response to intensive upper extremity therapy by individuals with ataxia from stroke | journal = Top Stroke Rehabil | volume = 15 | issue = 3 | pages = 262–71 | year = 2008 | pmid = 18647730 | doi = 10.1310/tsr1503-262 }}</ref> Their therapy consisted of [[constraint-induced movement therapy]] which resulted in improvements of their arm function.<ref name="stroke ataxia" /> Treatment should likely include strategies to manage difficulties with everyday activities such as walking. Gait aids (such as a cane or walker) can be provided to decrease the risk of falls associated with impairment of [[Balance (ability)|balance]] or poor [[Motor coordination|coordination]]. Severe ataxia may eventually lead to the need for a [[wheelchair]]. In order to obtain better results, possible coexisting motor deficits need to be addressed in addition to those induced by ataxia. For example, muscle weakness and decreased endurance could lead to increasing fatigue and worse movement patterns. | ||
* There are several assessment tools available to therapists and health care professionals working with patients with ataxia. The [[International Cooperative Ataxia Rating Scale]] (ICARS) is one of the most widely used and has been proven to have very high reliability and validity.<ref>{{cite journal | author = Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B, Mariotti C, Melegh B, Rakowicz M, Rola R, Romano S, Schöls L, Szymanski S, van de Warrenburg BP, Zdzienicka E, Dürr A, Klockgether T | title = Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients | journal = Mov. Disord. | volume = 21 | issue = 5 | pages = 699–704 | year = 2006 | month = May | pmid = 16450347 | doi = 10.1002/mds.20781 }}</ref> Other tools that assess motor function, balance and coordination are also highly valuable to help the therapist track the progress of their patient, as well as to quantify the patient's functionality. These tests include, but are not limited to: | *There are several assessment tools available to therapists and health care professionals working with patients with ataxia. The [[International Cooperative Ataxia Rating Scale]] (ICARS) is one of the most widely used and has been proven to have very high reliability and validity.<ref>{{cite journal | author = Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B, Mariotti C, Melegh B, Rakowicz M, Rola R, Romano S, Schöls L, Szymanski S, van de Warrenburg BP, Zdzienicka E, Dürr A, Klockgether T | title = Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients | journal = Mov. Disord. | volume = 21 | issue = 5 | pages = 699–704 | year = 2006 | month = May | pmid = 16450347 | doi = 10.1002/mds.20781 }}</ref> Other tools that assess motor function, balance and coordination are also highly valuable to help the therapist track the progress of their patient, as well as to quantify the patient's functionality. These tests include, but are not limited to: | ||
** The [[Berg Balance Scale]] | **The [[Berg Balance Scale]] | ||
** Tandem Walking (to test for [[Tandem gait]]ability) | **Tandem Walking (to test for [[Tandem gait]]ability) | ||
** Scale for the Assessment and Rating of Ataxia<ref name="pmid16769946">{{cite journal | author = Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schöls L, Szymanski S, van de Warrenburg BP, Dürr A, Klockgether T, Fancellu R | title = Scale for the assessment and rating of ataxia: development of a new clinical scale | journal = Neurology | volume = 66 | issue = 11 | pages = 1717–20 | year = 2006 | month = June | pmid = 16769946 | doi = 10.1212/01.wnl.0000219042.60538.92 }}</ref> | **Scale for the Assessment and Rating of Ataxia<ref name="pmid16769946">{{cite journal | author = Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schöls L, Szymanski S, van de Warrenburg BP, Dürr A, Klockgether T, Fancellu R | title = Scale for the assessment and rating of ataxia: development of a new clinical scale | journal = Neurology | volume = 66 | issue = 11 | pages = 1717–20 | year = 2006 | month = June | pmid = 16769946 | doi = 10.1212/01.wnl.0000219042.60538.92 }}</ref> | ||
** Tapping tests - The person must quickly and repeatedly tap their arm or leg while the therapist monitors the amount of [[dysdiadochokinesia]].<ref name="Notermans_1994">{{cite journal | author = Notermans NC, van Dijk GW, van der Graaf Y, van Gijn J, Wokke JH | title = Measuring ataxia: quantification based on the standard neurological examination | journal = J. Neurol. Neurosurg. Psychiatr. | volume = 57 | issue = 1 | pages = 22–6 | year = 1994 | month = January | pmid = 8301300 | pmc = 485035 | doi = 10.1136/jnnp.57.1.22 }}</ref> | **Tapping tests - The person must quickly and repeatedly tap their arm or leg while the therapist monitors the amount of [[dysdiadochokinesia]].<ref name="Notermans_1994">{{cite journal | author = Notermans NC, van Dijk GW, van der Graaf Y, van Gijn J, Wokke JH | title = Measuring ataxia: quantification based on the standard neurological examination | journal = J. Neurol. Neurosurg. Psychiatr. | volume = 57 | issue = 1 | pages = 22–6 | year = 1994 | month = January | pmid = 8301300 | pmc = 485035 | doi = 10.1136/jnnp.57.1.22 }}</ref> | ||
** [[Finger-nose testing]]<ref name="Notermans_1994"/> - This test has several variations including finger-to-therapist's finger, finger-to-finger, and alternate nose-to-finger.<ref name="urlOPETA: Neurologic Examination">{{cite web | url = http://medinfo.ufl.edu/other/opeta/neuro/NE_ch3.html | title = OPETA: Neurologic Examination | author = | date = | format = | work = Online physical exam teaching assistant | publisher = The UF College of Medicine Harrell Center | accessdate = 2012-05-07 }}</ref> | **[[Finger-nose testing]]<ref name="Notermans_1994" /> - This test has several variations including finger-to-therapist's finger, finger-to-finger, and alternate nose-to-finger.<ref name="urlOPETA: Neurologic Examination">{{cite web | url = http://medinfo.ufl.edu/other/opeta/neuro/NE_ch3.html | title = OPETA: Neurologic Examination | author = | date = | format = | work = Online physical exam teaching assistant | publisher = The UF College of Medicine Harrell Center | accessdate = 2012-05-07 }}</ref> | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Neurology]] | [[Category:Neurology]] | ||
Latest revision as of 17:04, 24 August 2020
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Overview
There is no specific treatment for ataxia as such, although there may be for the underlying cause. The main treatment available to patients is rehabilitation training, which can include physical, occupational, and speech therapy. The disability of ataxia may be reduced by physical therapy, including exercises, along with leg braces or shoe splints. If foot alignment has been affected then a cane or walker is often used in the effort to prevent falls.
Medical Therapy
- Recovery tends to be better in individuals with a single focal injury (such as stroke or a benign tumour), compared to those who have a neurological degenerative condition.[1]
- The movement disorders associated with ataxia can be managed by pharmacological treatments and through physical therapy and occupational therapy to reduce disability.[2] Some drug treatments that have been used to control ataxia include: 5-hydroxytryptophan (5-HTP), idebenone, amantadine, physostigmine, L-carnitine or derivatives, trimethoprim–sulfamethoxazole, vigabatrin, phosphatidylcholine, acetazolamide, 4-aminopyridine, buspirone, or a combination of coenzyme Q10 and vitamin E.
- Physical therapy requires a focus on adapting activity and facilitating motor learning for retraining specific functional motor patterns.[3] A recent systematic review suggested that physical therapy is effective, but there is only moderate evidence to support this conclusion.[4] The most commonly used physical therapy interventions for cerebellar ataxia are vestibular habituation, proprioceptive neurofaciliation, Frenkel exercises, and balance training; however, therapy is often highly individualized and gait and coordination training are large components of therapy.
- Current research suggests that, if a person is able to walk with or without a mobility aid, physical therapy should include an exercise program addressing five components: static balance, dynamic balance, trunk-limb coordination, stairs, and contracture prevention. Once the physical therapist determines that the individual is able to safely perform parts of the program independently, it is important that the individual be prescribed and regularly engage in a supplementary home exercise program that incorporates these components to further improve long term outcomes. These outcomes include balance tasks, gait, and individual activities of daily living. While the improvements are attributed primarily to changes in the brain and not just the hip and/or ankle joints, it is still unknown whether the improvements are due to adaptations in the cerebellum or compensation by other areas of the brain.[3]
- Decomposition, simplification, or slowing of multi-joint movement may also be an effective strategy that therapists may use to improve function in patients with ataxia.[5] Training likely needs to be intense and focused (as indicated by one study performed with stroke patients experiencing limb ataxia who underwent intensive upper limb retraining).[6] Their therapy consisted of constraint-induced movement therapy which resulted in improvements of their arm function.[6] Treatment should likely include strategies to manage difficulties with everyday activities such as walking. Gait aids (such as a cane or walker) can be provided to decrease the risk of falls associated with impairment of balance or poor coordination. Severe ataxia may eventually lead to the need for a wheelchair. In order to obtain better results, possible coexisting motor deficits need to be addressed in addition to those induced by ataxia. For example, muscle weakness and decreased endurance could lead to increasing fatigue and worse movement patterns.
- There are several assessment tools available to therapists and health care professionals working with patients with ataxia. The International Cooperative Ataxia Rating Scale (ICARS) is one of the most widely used and has been proven to have very high reliability and validity.[7] Other tools that assess motor function, balance and coordination are also highly valuable to help the therapist track the progress of their patient, as well as to quantify the patient's functionality. These tests include, but are not limited to:
- The Berg Balance Scale
- Tandem Walking (to test for Tandem gaitability)
- Scale for the Assessment and Rating of Ataxia[8]
- Tapping tests - The person must quickly and repeatedly tap their arm or leg while the therapist monitors the amount of dysdiadochokinesia.[9]
- Finger-nose testing[9] - This test has several variations including finger-to-therapist's finger, finger-to-finger, and alternate nose-to-finger.[10]
References
- ↑ Morton SM, Bastian AJ (2009). "Can rehabilitation help ataxia?". Neurology. 73 (22): 1818–9. doi:10.1212/WNL.0b013e3181c33b21. PMID 19864635. Unknown parameter
|month=ignored (help) - ↑ Perlman SL (2006). "Ataxias". Clin. Geriatr. Med. 22 (4): 859–77, vii. doi:10.1016/j.cger.2006.06.011. PMID 17000340. Unknown parameter
|month=ignored (help) - ↑ 3.0 3.1 Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L (2009). "Intensive coordinative training improves motor performance in degenerative cerebellar disease". Neurology. 73 (22): 1823–30. doi:10.1212/WNL.0b013e3181c33adf. PMID 19864636. Unknown parameter
|month=ignored (help) - ↑ Martin CL, Tan D, Bragge P, Bialocerkowski A (2009). "Effectiveness of physiotherapy for adults with cerebellar dysfunction: a systematic review". Clin Rehabil. 23 (1): 15–26. doi:10.1177/0269215508097853. PMID 19114434. Unknown parameter
|month=ignored (help) - ↑ Bastian AJ (1997). "Mechanisms of ataxia". Phys Ther. 77 (6): 672–5. PMID 9184691. Unknown parameter
|month=ignored (help) - ↑ 6.0 6.1 Richards L, Senesac C, McGuirk T, Woodbury M, Howland D, Davis S, Patterson T (2008). "Response to intensive upper extremity therapy by individuals with ataxia from stroke". Top Stroke Rehabil. 15 (3): 262–71. doi:10.1310/tsr1503-262. PMID 18647730.
- ↑ Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B, Mariotti C, Melegh B, Rakowicz M, Rola R, Romano S, Schöls L, Szymanski S, van de Warrenburg BP, Zdzienicka E, Dürr A, Klockgether T (2006). "Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients". Mov. Disord. 21 (5): 699–704. doi:10.1002/mds.20781. PMID 16450347. Unknown parameter
|month=ignored (help) - ↑ Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schöls L, Szymanski S, van de Warrenburg BP, Dürr A, Klockgether T, Fancellu R (2006). "Scale for the assessment and rating of ataxia: development of a new clinical scale". Neurology. 66 (11): 1717–20. doi:10.1212/01.wnl.0000219042.60538.92. PMID 16769946. Unknown parameter
|month=ignored (help) - ↑ 9.0 9.1 Notermans NC, van Dijk GW, van der Graaf Y, van Gijn J, Wokke JH (1994). "Measuring ataxia: quantification based on the standard neurological examination". J. Neurol. Neurosurg. Psychiatr. 57 (1): 22–6. doi:10.1136/jnnp.57.1.22. PMC 485035. PMID 8301300. Unknown parameter
|month=ignored (help) - ↑ "OPETA: Neurologic Examination". Online physical exam teaching assistant. The UF College of Medicine Harrell Center. Retrieved 2012-05-07.