Ataxia (Dyssynergia)

Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or PERIPHERAL NERVE DISEASES. Motor ataxia may be associated with CEREBELLAR DISEASES; CEREBRAL CORTEX diseases; THALAMIC DISEASES; BASAL GANGLIA DISEASES; injury to the RED NUCLEUS; and other conditions.

Also Known As:

Dyssynergia; Lack of Coordination; Ataxias; Incoordination; Ataxia, Appendicular; Ataxia, Limb; Ataxia, Motor; Ataxia, Sensory; Ataxia, Truncal; Ataxy; Dyscoordination; Tremor, Rubral; Appendicular Ataxia; Appendicular Ataxias; Ataxias, Appendicular; Ataxias, Limb; Ataxias, Motor; Ataxias, Sensory; Ataxias, Truncal; Coordination Impairments; Coordination Lack; Impairment, Coordination; Impairments, Coordination; Incoordinations; Limb Ataxia; Limb Ataxias; Motor Ataxia; Motor Ataxias; Rubral Tremor; Rubral Tremors; Sensory Ataxia; Sensory Ataxias; Tremors, Rubral; Truncal Ataxia; Truncal Ataxias; Coordination Impairment

Networked: 6487 relevant articles (308 outcomes, 420 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tremor (Tremors)
2.	Constipation
3.	Seizures (Absence Seizure)
4.	Dysarthria (Spastic Dysarthria)
5.	Spinocerebellar Ataxias (Spinocerebellar Ataxia)

Experts

1.	Klockgether, Thomas: 47 articles (03/2024 - 05/2004)
2.	Schöls, Ludger: 46 articles (03/2024 - 02/2002)
3.	Hadjivassiliou, Marios: 45 articles (01/2024 - 03/2003)
4.	Synofzik, Matthis: 34 articles (04/2024 - 12/2012)
5.	Giunti, Paola: 27 articles (02/2024 - 03/2012)
6.	Durr, Alexandra: 25 articles (02/2024 - 01/2010)
7.	Manto, Mario: 21 articles (04/2024 - 08/2009)
8.	Timmann, Dagmar: 21 articles (11/2023 - 11/2007)
9.	Houlden, Henry: 21 articles (10/2023 - 05/2010)
10.	Bertini, Enrico: 20 articles (11/2023 - 10/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ataxia:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/2012 - "Her posture and gait disturbance scores on the International Cooperative Ataxia Rating Scale had improved from 20 to 9. Even 16 years after onset, intravenous immunoglobulins were effective. " 06/01/2005 - "Although mild cerebellar-like ataxia appeared on day 5, intravenous immunoglobulin (0.4 g/kg/day for four days) rapidly improved his neurological abnormalities. " 12/15/2015 - "All patients were treated with cycles of intravenous immunoglobulin and had mild/partial ataxia improvement and no improvement of DBN. " 12/01/2008 - "Discontinuation of IVIG in two patients resulted in worsened ataxia that reversed after resumption of IVIG. " 08/01/2006 - "Intravenous immunoglobulin treatment in painful sensory neuropathy without sensory ataxia associated with Sjögren's syndrome."
2.	Thiamine (Aneurin)FDA Link 05/01/2012 - "She was treated with parenteral thiamine and her visual loss reversed and her ataxia improved dramatically. " 09/01/2016 - "She was treated with thiamine repletion, and had marked improvement in her mental status and some improvement in her vision problems and ataxia, although some nystagmus and significant short-term memory impairment persisted. " 03/01/1994 - "Thiamine produced a clinically impressive and statistically significant improvement in clinical severity grading in patients with the seasonal ataxia compared with placebo (MANOVA, df = 1, F = 35.087, p < 0.0001), with a significant time-treatment interaction (MANOVA, df = 3, F = 32.36, p < 0.0001). " 04/01/2016 - "The patients were treated with intramuscular (IM) thiamine (1200 mg/d for 2 months and 900 mg/d for a month, respectively) with gradual cognitive and behavioral improvement and brain MRI normalization, while ataxia and oculomotion improved in following months. " 02/26/2024 - "Patients with vitamin B1 deficiency exhibited abnormal eye movements (n = 4/4), fluctuations in consciousness (n = 3/4), and ataxia (n = 3/4). "
3.	salicylhydroxamic acid (SHAM)IBA 01/01/2023 - "Anodal ctDCS led to a significant improvement in the Scale for the Assessment and Rating of Ataxia (-6.5%) and in the cerebellar cognitive affective syndrome scale (+11%) compared with sham ctDCS. " 02/01/2024 - "Comparing real and sham-rTMS interventions, the utilization of rTMS on cerebellum improved the scale for the assessment and rating of ataxia (SARA) (SMD - 0.87, 95% CI - 1.41 to - 0.34; P = 0.001; I2 = 62%), the International Cooperative Ataxia Rating Scale (ICARS) (SMD - 1.06, 95% CI - 1.47 to - 0.64; P < 0.00001; I2 = 0%) and Berg balance Scale (BBS) (SMD 0.76, 95% CI 0.33 to 1.19; P = 0.0005; I2 = 39%). " 11/01/2020 - "The International Cooperative Ataxia Rating Scale score also improved after active stimulation versus sham [median (interquartile range) of 29.0 (21.0, 43.5) versus 32.8 (22.0, 47.0); p = 0.005]. " 03/01/2024 - "Phase I of the trial involved the randomization of 43 patients with RRMS and truncal ataxia to either real (n = 20) or sham (n = 19) rTMS (2 participants from each treatment group were excluded from the study; one developed a relapse before treatment, 2 declined to participate, and one did not show up). " 01/01/2023 - "rTMS could significantly improve the Scale for the Assessment and Rating of Ataxia (SARA) (MD: -2.00; 95% CI: -3.97 to -0.02, p = 0.05), International Cooperative Ataxia Rating Scale (ICARS) (MD: -3.96; 95% CI: -5.51 to -2.40, p < 0.00001), Timed Up-and-Go test (TUG) (MD: -1.54; 95% CI: -2.24 to -0.84, p < 0.0001), 10-m walk test (10 MWT) (MD10-m steps: -2.44; 95% CI: -4.14 to -0.73, p = 0.005), and Berg Balance Scale (BBS) (MD: 2.59; 95% CI: 1.15-4.03, p = 0.0004) as compared to sham stimulation. "
4.	SteroidsIBA 01/01/2009 - "Ataxia improved but the patient still required low daily doses of steroids for one year." 01/01/2017 - "Steroids were restarted and his ataxia stabilized." 06/01/2015 - "The protocol included serial assessment of ataxia (by International Cooperative Ataxia Rating Scale) and adaptive behavior (by Vineland Adaptive Behavior Scales) and clinical and laboratory tests revealing treatment- and steroid-dependent adverse effects, if present. " 06/24/2003 - "Indeed, this steroid is able to prevent Purkinje cells from death in organotypic cultures of cerebellar slices from Purkinje cell degeneration (pcd) mutant mice, a murine model of hereditary neurodegenerative ataxia. " 09/15/2003 - "Her symptoms briefly improved after steroid therapy, but ataxia gradually progressed. "
5.	Gabapentin (Neurontin)FDA LinkGeneric 03/15/2009 - "The total period of gabapentin treatment was 4 weeks, and outcome measures were determined with the International Cooperative Ataxia Rating Scales (ICARS) and postural sway studies. " 01/01/2023 - "Comparing the gabapentin with the placebo groups, proprioceptive ataxia was identified in 4/17 (23.5%) vs 0/18 cats (P = 0.0288); paw placement deficits were seen in 10/11 (90.9%) vs 1/4 (25%) cats; table tactile placement deficits were identified in 13/17 (76.5%) vs 0/18 cats (P <0.0001); hopping deficits were seen in 5/17 (29.4%) vs 0/16 cats (P = 0.0185); and abnormalities on wheelbarrowing and extensor postural thrust were reported in 5/17 (29.4%) vs 0/18 cats (P = 0.0129). " 01/01/2021 - "Gabapentin (GBP) is an established drug that has been used in the management of symptoms of neuropathy but it is associated with unwanted side effects such as sedation and motor incoordination. " 12/08/2009 - "Gabapentin treatment improves motor coordination in a mice model of progressive ataxia." 03/01/2007 - "Gabapentin, renally excreted, with a half-life of 6.5-10.5 h, may cause ataxia. "
6.	Acetazolamide (Diamox)FDA LinkGeneric 01/01/2012 - "Acetazolamide can be dramatic in controlling episodes of ataxia with EA2 but is typically less beneficial with EA1." 01/01/1998 - "Treatment is effective for many of these, and the treatment of the episodic ataxias with acetazolamide can sometimes have a dramatic effect. " 01/01/1996 - "EEG studies were performed in six family members affected by acetazolamide-responsive paroxysmal ataxia. " 05/01/1988 - "A magnetic resonance imaging study was performed in three symptomatic members of two families with a diagnosis of acetazolamide-responsive familial paroxysmal ataxia. " 11/01/1984 - "The acetazolamide trial was terminated at 7 to 11 weeks because of reported side effects or increased ataxia in 3 of the patients. "
7.	Valproic Acid (Depakote)FDA LinkGeneric 01/01/2018 - "For patients with SCA type 3, valproic acid 1,200 mg/d possibly improves ataxia at 12 weeks. " 01/01/2017 - "Repeated prenatal exposure to valproic acid results in cerebellar hypoplasia and ataxia." 01/01/2011 - "The first is an 8-year-old boy, who developed severe motor problems and ataxia during treatment with valproate. " 01/01/2010 - "[Ataxia secondary to valproate. " 06/01/1982 - "Impairment of rotarod performance and marked ataxia occurred at plasma valproic acid concentrations above 510 microgram ml-1 and loss of righting reflex became evident at 970 microgram ml-1. "
8.	Methylprednisolone FDA LinkGeneric 03/24/2021 - "The laboratory and clinical findings as well as the marked improvement after high-dose methylprednisolone therapy suggest a post-infectious, immune-mediated cause of ataxia. " 01/01/2016 - "She presented to an outside hospital with acute onset of ataxia on three occasions before presenting to our institution, initially misdiagnosed as acute disseminated encephalomyelitis and treated with intravenous methylprednisolone. " 09/21/2008 - "As ataxia developed after 1 year, she was treated with pulsed methylprednisolone for 3 d, then readmitted to our hospital when liver dysfunction occurred. " 05/02/2022 - "He responded well to pulse methylprednisolone therapy and was discharged with mild tremor and ataxia. " 01/01/2019 - "The Susac syndrome was diagnosed and treatment started with methylprednisolone pulses therapy, intravenously 1000 mg/day for 5 days, followed by maintenance with mycophenolate, which completely reversed the encephalopathy, with persistence of mild ataxia and hearing loss. "
9.	acetylleucineIBA 02/01/2023 - "Supplementation with N-acetyl-DL-leucine at a dose of 4 g/day for 16 weeks was well tolerated and significantly improved ataxia symptoms and quality of life measures in a young child with AT." 12/01/2021 - "In this large multicenter, double-blind, randomized, placebo-controlled clinical crossover trial, acetyl-DL-leucine in the investigated dosage and treatment duration was not superior to placebo for the symptomatic treatment of certain types of ataxia. " 01/10/2017 - "Based on three observational studies, the primary objective of the acetyl-DL-leucine on ataxia (ALCAT) trial is to examine the efficacy and tolerability of a symptomatic therapy with acetyl-DL-leucine compared to placebo on motor function measured by the Scale for the Assessment and Rating of Ataxia (SARA) in patients with CA. An investigator-initiated, multicenter, European, randomized, double-blind, placebo-controlled, 2-treatment 2-period crossover phase III trial will be carried out. " 01/01/2022 - "In an open-label study, N-acetyl-l-leucine administered for 6-weeks significantly improved clinical impression of change, ataxia, and quality of life in patients with Niemann-Pick disease type C1. " 01/01/2021 - "In observational clinical studies, acetyl-dl-leucine improved symptoms of ataxia, in particular in patients with the lysosomal storage disorder, Niemann-Pick disease type C1. "
10.	coenzyme Q10 (CoQ10)IBA 06/01/2019 - "COQ4 Mutation Leads to Childhood-Onset Ataxia Improved by CoQ10 Administration." 07/15/2010 - "However, the remarkable clinical response in 1 case without CoQ(10) deficiency highlights the importance of treatment trials for identification of patients with CoQ(10)-responsive ataxia." 04/19/2024 - "Among the so-termed mitochondrial ataxias, variants in genes encoding steps of the coenzyme Q10 biosynthetic pathway represent a common cause of autosomal recessive primary coenzyme Q10 deficiencies (PCoQD)s. " 06/01/2023 - "Supplementation of CoQ10 alleviated his symptoms, with the Scale for the Assessment and Rating of Ataxia (SARA) dropping from 16 to 14. " 01/01/2022 - "To address this question, we mapped individual responses in two patients with COQ8A-related ataxia following coenzyme Q10 supplementation using noninvasive imaging. "

Therapies and Procedures

1.	Therapeutics 04/01/1993 - "Currently, the most effective and practical way of controlling ataxia which cannot be effectively treated with drugs, surgery or therapy is to use viscous damping. " 01/01/2019 - "Mechanistic studies of high-dose irradiation are important to improve our understanding on how the efficacy of stereotactically delivered high-dose irradiation can be enhanced by therapeutics such as ataxia-telangiesctasia-mutated (ATM) inhibitors. " 01/01/1996 - "After these therapy, he noted marked improvement in his gait and ataxia. " 01/01/2010 - "Our study pointed out significant improvement in the group treated with combined therapy for detrusor sphincter dyssynergia and unstable contractions after 3 months while after 6 months from the beginning of the treatment there was significant improvement (p < 0.001) for all urodynamic parameters. " 09/01/1999 - "Whether prophylactic therapy in the high risk bladder may also lead to improved long-term bladder dynamics prompted us to study the effect of early versus late treatment of bladder hypertonicity and detrusor-sphincter dyssynergia on the ultimate need for bladder augmentation. "
2.	Psychology Biofeedback (Biofeedback) 04/01/2007 - "This investigation provides definitive support for the efficacy of biofeedback for pelvic floor dyssynergia and shows that instrumented biofeedback is essential to successful treatment." 07/01/2004 - "Good results-a response rate of up to 90%-demonstrates that biofeedback training is successful in the treatment of detrusor-sphincter dyscoordination. " 10/01/2001 - "The modified biofeedback program including appropriate explanations and psychological approaches appeared effective for treating 92% of children with detrusor-sphincter dyssynergia. " 06/01/2015 - "Efficacy of biofeedback plus transanal stimulation in the management of pelvic floor dyssynergia: a randomized trial." 03/11/2024 - "Patients in the biofeedback group (n = 19) learned to correct abdominophrenic dyssynergia triggered by the offending meal (intercostal activity decreased by a mean ± SE of 82% ± 10%, anterior wall activity increased by a mean ± SE of 97% ± 6%, and increase in girth was a mean ± SE of 108% ± 4% smaller) and experienced improved clinical symptoms (abdominal distension scores decreased by a mean ± SE of 66% ± 5%). "
3.	Transcranial Direct Current Stimulation 04/01/2024 - "Cerebellar tDCS and cerebellar tACS are effective in ameliorating symptoms of neurodegenerative ataxias, with the former being more beneficial than the latter. " 02/01/2019 - "The coupled approach (i.e., tDCS and Lokomat) demonstrated better improvement in functional motor outcomes on the Scale for the Assessment and Rating of Ataxia (SARA). " 01/01/2019 - "Results from this case report suggest that consecutively administered tDCS treatments paired with cognitive and physical exercise hold promise for improving balance, gait, and manual dexterity in patients with progressive ataxia. " 01/01/2023 - "To evaluate whether expectations were specifically related to the application of cerebellar transcranial direct current stimulation (tDCS) or more generally reflect an overly positive attitude of patients with ataxia toward trial participation and results, the last questions involved a hypothetical scenario in which an oral drug was tested against placebo with an aim identical to that of our tDCS study. " 01/01/2022 - "Taken together, our study does not provide evidence that a two-week treatment with daily cerebellar tDCS sessions reduces ataxia severity or restores cerebellar-M1 connectivity in early-to-middle-stage SCA3 patients at the group level. "
4.	Injections 07/01/2010 - "All patients showed significant improvement in bladder capacity (P <.01), maximal detrusor pressure (P <.01), and detrusor-sphincter dyssynergia (P <.01) after 3 and 6 months of receiving injections. " 06/01/2006 - "Sphincter injections are effective in the short-term treatment of neurogenic detrusor-sphincter dyssynergia (level 1c). " 07/01/2003 - "Both transurethral and transperineal injections monitored by EMG are equally effective in improving detrusor-sphincter dyssynergia. " 11/01/2023 - "The most common complications were hindlimb weakness/ataxia after all types of injections (diagnostic: 44/110, 40%, 95% confidence interval [CI]: 30.8-49.8; therapeutic: 15/187, 8.0%, CI: 4.6-12.9; combined: 2/49, 4.1%, CI: 0.5-14.0). " 01/01/2023 - "For the first time, we report ataxia and lethality after intravitreal or intrastromal rAAV-PHP.B virus injections. "
5.	Physical Therapy Modalities (Physical Therapy Technique) 02/01/2013 - "Currently, as there is no cure for most ataxias, treatment predominantly involves physical therapy. " 11/01/2023 - "Dual-task program is more effective than traditional physical therapy alone in improving balance in children with ataxia after MB resection." 10/01/2019 - "The aim of this systematic review was to critically evaluate the range, scope and methodological quality of studies investigating the effectiveness of exercise and physical therapy interventions for children with ataxia. " 02/01/2014 - "This study reinforces the need for long-term, multidimensional physical therapy for individuals with ataxia." 12/01/2013 - "An exploratory study to examine specialist neurological physiotherapy service provision and utilisation for people with progressive ataxia. "