-
Tremor and Other Hyperkinetic Movements... 2023Episodic ataxia (EA), characterized by recurrent attacks of cerebellar dysfunction, is the manifestation of a group of rare autosomal dominant inherited disorders. EA1... (Review)
Review
BACKGROUND
Episodic ataxia (EA), characterized by recurrent attacks of cerebellar dysfunction, is the manifestation of a group of rare autosomal dominant inherited disorders. EA1 and EA2 are most frequently encountered, caused by mutations in and . EA3-8 are reported in rare families. Advances in genetic testing have broadened the and phenotypes, and detected EA as an unusual presentation of several other genetic disorders. Additionally, there are various secondary causes of EA and mimicking disorders. Together, these can pose diagnostic challenges for neurologists.
METHODS
A systematic literature review was performed in October 2022 for 'episodic ataxia' and 'paroxysmal ataxia', restricted to publications in the last 10 years to focus on recent clinical advances. Clinical, genetic, and treatment characteristics were summarized.
RESULTS
EA1 and EA2 phenotypes have further broadened. In particular, EA2 may be accompanied by other paroxysmal disorders of childhood with chronic neuropsychiatric features. New treatments for EA2 include dalfampridine and fampridine, in addition to 4-aminopyridine and acetazolamide. There are recent proposals for EA9-10. EA may also be caused by gene mutations associated with chronic ataxias (), epilepsy syndromes (), GLUT-1, mitochondrial disorders (), metabolic disorders (Maple syrup urine disease, Hartnup disease, type I citrullinemia, thiamine and biotin metabolism defects), and others. Secondary causes of EA are more commonly encountered than primary EA (vascular, inflammatory, toxic-metabolic). EA can be misdiagnosed as migraine, peripheral vestibular disorders, anxiety, and functional symptoms. Primary and secondary EA are frequently treatable which should prompt a search for the cause.
DISCUSSION
EA may be overlooked or misdiagnosed for a variety of reasons, including phenotype-genotype variability and clinical overlap between primary and secondary causes. EA is highly treatable, so it is important to consider in the differential diagnosis of paroxysmal disorders. Classical EA1 and EA2 phenotypes prompt single gene test and treatment pathways. For atypical phenotypes, next generation genetic testing can aid diagnosis and guide treatment. Updated classification systems for EA are discussed which may assist diagnosis and management.
Topics: Humans; Ataxia; Cerebellar Ataxia; Acetazolamide; Mutation
PubMed: 37008993
DOI: 10.5334/tohm.747 -
Brazilian Journal of Physical Therapy 2022Facial palsy (FP) is defined as an injury of the seventh cranial nerve pair, partial or total, which can be classified as central or peripheral. Proprioceptive... (Review)
Review
BACKGROUND
Facial palsy (FP) is defined as an injury of the seventh cranial nerve pair, partial or total, which can be classified as central or peripheral. Proprioceptive neuromuscular facilitation (PNF) is primarily used in the functional recovery of upper and lower limb conditions, however the technique has also been used for FP.
OBJECTIVE
To analyze the effect of PNF in the treatment of dysfunctions in FP.
METHODS
Ten databases including BVS, CENTRAL Cochrane, CINAHL, PEDro, PubMed, Scielo, ScienceDirect, SCOPUS, Web of Science, and Google Scholar were comprehensively searched for dates prior to April 2021. Randomized controlled trials of PNF in individuals with dysfunctions caused by facial paralysis were eligible. Outcomes measures were recovery rate and clinical recovery, both measured by using the House Brackmann Scale. Recovery time was measured in days and synkinesis assessed with the Synkinesis Assessment Questionnaire.
RESULTS
A total of 184 patients were included. In general, the included studies have low methodological quality. None of the five studies used PNF as the sole intervention. In all of the included studies PNF was used in combination with other interventions. Our findings show very low evidence that PNF is more effective than minimal intervention for treating FP.
CONCLUSION
We conclude that given the limited number of studies included and the low methodological quality presented, recommendations based on these studies should be interpreted with caution. The effects of PNF on facial paralysis are not clear.
Topics: Humans; Facial Paralysis; Muscle Stretching Exercises; Synkinesis; Recovery of Function
PubMed: 36279766
DOI: 10.1016/j.bjpt.2022.100454 -
The Cochrane Database of Systematic... May 2017Dementia is a clinical syndrome with a number of different causes which is characterised by deterioration in cognitive, behavioural, social and emotional functions.... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Dementia is a clinical syndrome with a number of different causes which is characterised by deterioration in cognitive, behavioural, social and emotional functions. Pharmacological interventions are available but have limited effect to treat many of the syndrome's features. Less research has been directed towards non-pharmacological treatments. In this review, we examined the evidence for effects of music-based interventions as a treatment.
OBJECTIVES
To assess the effects of music-based therapeutic interventions for people with dementia on emotional well-being including quality of life, mood disturbance or negative affect, behavioural problems, social behaviour, and cognition at the end of therapy and four or more weeks after the end of treatment.
SEARCH METHODS
We searched ALOIS, the Specialized Register of the Cochrane Dementia and Cognitive Improvement Group (CDCIG) on 14 April 2010 using the terms: music therapy, music, singing, sing, auditory stimulation. Additional searches were also carried out on 3 July 2015 in the major healthcare databases MEDLINE, Embase, psycINFO, CINAHL and LILACS; and in trial registers and grey literature sources. On 12 April 2016, we searched the major databases for new studies for future evaluation.
SELECTION CRITERIA
We included randomized controlled trials of music-based therapeutic interventions (at least five sessions) for people with dementia that measured any of our outcomes of interest. Control groups either received usual care or other activities.
DATA COLLECTION AND ANALYSIS
Two reviewers worked independently to screen the retrieved studies against the inclusion criteria and then to extract data and assess methodological quality of the included studies. If necessary, we contacted trial authors to ask for additional data, including relevant subscales, or for other missing information. We pooled data using random-effects models.
MAIN RESULTS
We included 17 studies. Sixteen studies with a total of 620 participants contributed data to meta-analyses. Participants in the studies had dementia of varying degrees of severity, but all were resident in institutions. Five studies delivered an individual music intervention; in the others, the intervention was delivered to groups of participants. Most interventions involved both active and receptive musical elements. The methodological quality of the studies varied. All were at high risk of performance bias and some were at high risk of detection or other bias. At the end of treatment, we found low-quality evidence that music-based therapeutic interventions may have little or no effect on emotional well-being and quality of life (standardized mean difference, SMD 0.32, 95% CI -0.08 to 0.71; 6 studies, 181 participants), overall behaviour problems (SMD -0.20, 95% CI -0.56 to 0.17; 6 studies, 209 participants) and cognition (SMD 0.21, 95% CI -0.04 to 0.45; 6 studies, 257 participants). We found moderate-quality evidence that they reduce depressive symptoms (SMD -0.28, 95% CI -0.48 to -0.07; 9 studies, 376 participants), but do not decrease agitation or aggression (SMD -0.08, 95% CI -0.29 to 0.14; 12 studies, 515 participants). The quality of the evidence on anxiety and social behaviour was very low, so effects were very uncertain. The evidence for all long-term outcomes was also of very low quality.
AUTHORS' CONCLUSIONS
Providing people with dementia with at least five sessions of a music-based therapeutic intervention probably reduces depressive symptoms but has little or no effect on agitation or aggression. There may also be little or no effect on emotional well-being or quality of life, overall behavioural problems and cognition. We are uncertain about effects on anxiety or social behaviour, and about any long-term effects. Future studies should employ larger sample sizes, and include all important outcomes, in particular 'positive' outcomes such as emotional well-being and social outcomes. Future studies should also examine the duration of effects in relation to the overall duration of treatment and the number of sessions.
Topics: Aged; Aggression; Dementia; Depression; Humans; Mental Disorders; Music Therapy; Psychomotor Agitation; Quality of Life; Randomized Controlled Trials as Topic
PubMed: 28462986
DOI: 10.1002/14651858.CD003477.pub3 -
Developmental Medicine and Child... Sep 2021To update a systematic review of evidence published up to December 2015 for pharmacological/neurosurgical interventions among individuals with cerebral palsy (CP) and... (Meta-Analysis)
Meta-Analysis
AIM
To update a systematic review of evidence published up to December 2015 for pharmacological/neurosurgical interventions among individuals with cerebral palsy (CP) and dystonia.
METHOD
Searches were updated (January 2016 to May 2020) for oral baclofen, trihexyphenidyl, benzodiazepines, clonidine, gabapentin, levodopa, botulinum neurotoxin (BoNT), intrathecal baclofen (ITB), and deep brain stimulation (DBS), and from database inception for medical cannabis. Eligible studies included at least five individuals with CP and dystonia and reported on dystonia, goal achievement, motor function, pain/comfort, ease of caregiving, quality of life (QoL), or adverse events. Evidence certainty was evaluated using GRADE.
RESULTS
Nineteen new studies met inclusion criteria (two trihexyphenidyl, one clonidine, two BoNT, nine ITB, six DBS), giving a total of 46 studies (four randomized, 42 non-randomized) comprising 915 participants when combined with those from the original systematic review. Very low certainty evidence supported improved dystonia (clonidine, ITB, DBS) and goal achievement (clonidine, BoNT, ITB, DBS). Low to very low certainty evidence supported improved motor function (DBS), pain/comfort (clonidine, BoNT, ITB, DBS), ease of caregiving (clonidine, BoNT, ITB), and QoL (ITB, DBS). Trihexyphenidyl, clonidine, BoNT, ITB, and DBS may increase adverse events. No studies were identified for benzodiazepines, gabapentin, oral baclofen, and medical cannabis.
INTERPRETATION
Evidence evaluating the use of pharmacological and neurosurgical management options for individuals with CP and dystonia is limited to between low and very low certainty. What this paper adds Meta-analysis suggests that intrathecal baclofen (ITB) and deep brain stimulation (DBS) may improve dystonia and pain. Meta-analysis suggests that DBS may improve motor function. Clonidine, botulinum neurotoxin, ITB, and DBS may improve achievement of individualized goals. ITB and DBS may improve quality of life. No direct evidence is available for oral baclofen, benzodiazepines, gabapentin, or medical cannabis.
Topics: Baclofen; Botulinum Toxins; Cerebral Palsy; Clonidine; Deep Brain Stimulation; Dystonia; Humans; Injections, Spinal; Levodopa; Neurosurgical Procedures; Trihexyphenidyl
PubMed: 33772789
DOI: 10.1111/dmcn.14874 -
Cerebellum (London, England) Oct 2019The effectiveness of exercise and physical therapy for children with ataxia is poorly understood. The aim of this systematic review was to critically evaluate the range,...
The effectiveness of exercise and physical therapy for children with ataxia is poorly understood. 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. The following databases were searched: AMED, CENTRAL, CDSR, CINAHL, ClinicalTrials.gov, EMBASE, Ovid MEDLINE, PEDro and Web of Science. No limits were placed on language, type of study or year of publication. Two reviewers independently determined whether the studies met the inclusion criteria, extracted all relevant outcomes, and conducted methodological quality assessments. A total of 1988 studies were identified, and 124 full texts were screened. Twenty studies were included in the review. A total of 40 children (aged 5-18 years) with ataxia as a primary impairment participated in the included studies. Data were able to be extracted from eleven studies with a total of 21 children (aged 5-18 years), with a range of cerebellar pathology. The studies reported promising results but were of low methodological quality (no RCTs), used small sample sizes and were heterogeneous in terms of interventions, participants and outcomes. No firm conclusions can be made about the effectiveness of exercise and physical therapy for children with ataxia. There is a need for further high-quality child-centred research.
Topics: Ataxia; Child; Exercise Therapy; Humans; Physical Therapy Modalities; Prospective Studies; Retrospective Studies
PubMed: 31392562
DOI: 10.1007/s12311-019-01063-z -
The Lancet. Child & Adolescent Health Feb 2023In clinical practice guidelines there is no consensus about the medications that should be initially offered to children and young people with Tourette's syndrome. To... (Meta-Analysis)
Meta-Analysis
Comparative efficacy, tolerability, and acceptability of pharmacological interventions for the treatment of children, adolescents, and young adults with Tourette's syndrome: a systematic review and network meta-analysis.
BACKGROUND
In clinical practice guidelines there is no consensus about the medications that should be initially offered to children and young people with Tourette's syndrome. To provide a rigorous evidence base that could help guide decision making and guideline development, we aimed to compare the efficacy, tolerability, and acceptability of pharmacological interventions for Tourette's syndrome.
METHODS
For this systematic review and network meta-analysis, we searched the Cochrane Central Register of Controlled Trials, Embase, PsycINFO, PubMed, Web of Science, the WHO International Clinical Trials Registry Platform, and ClinicalTrials.gov, for published and unpublished studies from database inception to Nov 19, 2021. We included double-blind randomised controlled trials of any medication administered as a monotherapy for at least 1 week against another medication or placebo in children and adolescents (aged ≥4 years and ≤18 years), adults (>18 years), or both, diagnosed with Tourette's syndrome according to standardised criteria. We excluded studies that exclusively recruited participants with comorbid attention-deficit hyperactivity disorder or obsessive-compulsive disorder. The primary outcome was change in severity of tic symptoms (efficacy). Secondary outcomes were treatment discontinuations due to adverse events (tolerability) and for any reason (acceptability). Pharmacological interventions were examined considering medication categories and medications individually in separate analyses. Summary data were extracted and pooled with a random-effects network meta-analysis to calculate standardised mean differences for efficacy and odds ratios for tolerability and acceptability, with 95% CIs. The Confidence in Network Meta-Analysis (CINeMA) framework was used to assess the certainty of evidence. The protocol was pre-registered in PROSPERO (CRD42022296975).
FINDINGS
Of the 12 088 records identified through the database search, 88 records representing 39 randomised controlled trials were included in the network meta-analysis; these 39 randomised controlled trials comprised 4578 participants (mean age 11·8 [SD 4·5] years; 3676 [80·8%] male participants) and evaluated 23 individual medications distributed across six medication categories. When considering medication categories, first-generation (standardised mean difference [SMD] -0·65 [95% CI -0·79 to -0·51]; low certainty of evidence) and second-generation (-0·71 [-0·88 to -0·54]; moderate certainty of evidence) antipsychotic drugs, as well as α-2 agonists (-0·21 [-0·39 to -0·03]; moderate certainty of evidence), were more efficacious than placebo. First-generation and second-generation antipsychotic drugs did not differ from each other (SMD 0·06 [95% CI -0·14 to 0·25]; low certainty of evidence). However, both first-generation (SMD 0·44 [95% CI 0·21 to 0·66]) and second-generation (0·49 [0·25 to 0·74]) antipsychotic drugs outperformed α-2 agonists, with moderate certainty of evidence. Similar findings were observed when individual medications were considered: aripiprazole (SMD -0·60 [95% CI -0·83 to -0·38]), haloperidol (-0·51 [-0·88 to -0·14]), olanzapine (-0·83 [-1·49 to -0·18]), pimozide (-0·48 [-0·84 to -0·12]), risperidone (-0·66 [-0·98 to -0·34]), and clonidine (-0·20 [-0·37 to -0·02]) all outperformed placebo, with moderate certainty of evidence. Antipsychotic medications did not differ from each other, but there was low to very low certainty of evidence for these comparisons. However, aripiprazole (SMD -0·40 [95% CI -0·69 to -0·12]) and risperidone (-0·46 [-0·82 to -0·11]) outperformed clonidine, with moderate certainty of evidence. Heterogeneity or inconsistency only emerged for a few comparisons. In terms of tolerability and acceptability, there were no relevant findings for any of the efficacious medication categories or individual medications against each other or placebo, but there was low to very low certainty of evidence associated with these comparisons.
INTERPRETATION
Our analyses show that antipsychotic drugs are the most efficacious intervention for Tourette's syndrome, while α-2 agonists are also more efficacious than placebo and could be chosen by those who elect not to take antipsychotic drugs. Shared decision making about the degree of tic-related severity and distress or impairment, the trade-offs of efficacy and safety between antipsychotic drugs and α-2 agonists, and other highly relevant individual factors that could not be addressed in the present analysis, should guide the choice of medication for children and young people with Tourette's syndrome.
FUNDING
None.
Topics: Male; Adolescent; Child; Young Adult; Humans; Female; Tourette Syndrome; Antipsychotic Agents; Clonidine; Aripiprazole; Risperidone; Network Meta-Analysis; Tics; Adrenergic alpha-2 Receptor Agonists; Randomized Controlled Trials as Topic
PubMed: 36528030
DOI: 10.1016/S2352-4642(22)00316-9 -
Pediatric Physical Therapy : the... Apr 2023To systematically review current evidence on the physical therapy assessment, intervention, and prognosis of congenital muscular torticollis (CMT) to inform the update...
PURPOSE
To systematically review current evidence on the physical therapy assessment, intervention, and prognosis of congenital muscular torticollis (CMT) to inform the update to the 2018 CMT Clinical Practice Guideline (CPG).
METHODS
Six databases were searched for studies that informed assessment, intervention, and prognosis for physical therapy management of infants with CMT.
RESULTS
Fifteen studies were included. Four studies investigated the psychometric properties of new and established assessments. Six studies informed the feasibility and efficacy of first-choice and supplemental interventions including traditional Chinese medicine and neural and visceral manipulation. One qualitative study found that parents of infants with mild and severe CMT had different concerns. Five studies informed prognosis, including factors associated with treatment duration, clinical outcomes, and use of supplemental interventions.
CONCLUSION
Newer evidence reaffirms 5 of 17 recommendations of the 2018 CMT CPG and could increase the recommendation strength to strong for neck passive range of motion.
Topics: Infant; Humans; Torticollis; Muscular Diseases; Neck; Physical Therapy Modalities
PubMed: 36637442
DOI: 10.1097/PEP.0000000000000993 -
Annals of Internal Medicine Nov 2019Both pharmacologic and nonpharmacologic interventions are used to treat neuropsychiatric symptoms in persons with dementia. (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Both pharmacologic and nonpharmacologic interventions are used to treat neuropsychiatric symptoms in persons with dementia.
PURPOSE
To summarize the comparative efficacy of pharmacologic and nonpharmacologic interventions for treating aggression and agitation in adults with dementia.
DATA SOURCES
MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, CINAHL, and PsycINFO between inception and 28 May 2019 without language restrictions; gray literature; and reference lists scanned from selected studies and systematic reviews.
STUDY SELECTION
Randomized controlled trials comparing interventions for treating aggression and agitation in adults with dementia.
DATA EXTRACTION
Pairs of reviewers independently screened studies, abstracted data, and appraised risk of bias.
DATA SYNTHESIS
After screening of 19 684 citations, 163 studies (23 143 patients) were included in network meta-analyses. Analysis of interventions targeting aggression and agitation (148 studies [21 686 patients]) showed that multidisciplinary care (standardized mean difference [SMD], -0.5 [95% credible interval {CrI}, -0.99 to -0.01]), massage and touch therapy (SMD, -0.75 [CrI, -1.12 to -0.38]), and music combined with massage and touch therapy (SMD, -0.91 [CrI, -1.75 to -0.07]) were clinically more efficacious than usual care. Recreation therapy (SMD, -0.29 [CrI, -0.57 to -0.01]) was statistically but not clinically more efficacious than usual care.
LIMITATIONS
Forty-six percent of studies were at high risk of bias because of missing outcome data. Harms and costs of therapies were not evaluated.
CONCLUSION
Nonpharmacologic interventions seemed to be more efficacious than pharmacologic interventions for reducing aggression and agitation in adults with dementia.
PRIMARY FUNDING SOURCE
Alberta Health Services Critical Care Strategic Clinical Network. (PROSPERO: CRD42017050130).
Topics: Aggression; Dementia; Humans; Network Meta-Analysis; Psychomotor Agitation
PubMed: 31610547
DOI: 10.7326/M19-0993 -
Anaesthesia Mar 2019Delirium is common in intensive care patients. Dexmedetomidine is increasingly used for sedation in this setting, but its effect on delirium remains unclear. The primary... (Meta-Analysis)
Meta-Analysis
Delirium is common in intensive care patients. Dexmedetomidine is increasingly used for sedation in this setting, but its effect on delirium remains unclear. The primary aim of this review was to examine whether dexmedetomidine reduces the incidence of delirium and agitation in intensive care patients. We sought randomised clinical trials in MEDLINE, EMBASE, PubMed and CENTRAL from their inception until June 2018. Observational studies, case reports, case series and non-systematic reviews were excluded. Twenty-five trials including 3240 patients were eligible for inclusion in the data synthesis. In the patients who received dexmedetomidine (eight trials, 1425 patients), delirium was reduced, odds ratio (95%CI) 0.36 (0.26-0.51), p < 0.001 and high quality of evidence. The use of dexmedetomidine was associated with a reduced incidence of agitation, OR (95%CI) 0.34 (0.20-0.59), p < 0.001, moderate quality of evidence. Patients who were randomly assigned to dexmedetomidine had a significantly higher incidence of bradycardia, OR (95%CI) 2.18 (1.46-3.24), p < 0.001, moderate quality of evidence; and hypotension, OR (95%CI) 1.89 (1.48-2.41), p < 0.001, high quality of evidence. We found no evidence of an effect on mortality, OR (95%CI) 0.86 (0.66-1.10), p = 0.23, moderate quality of evidence. The trial sequential analyses for the incidence of delirium, bradycardia and hypotension was conclusive but not for the incidence of agitation and mortality. In summary, this meta-analysis suggests that dexmedetomidine reduces the incidence of delirium and agitation in intensive care patients. The general quality of evidence ranged from moderate to high.
Topics: Delirium; Dexmedetomidine; Humans; Intensive Care Units; Psychomotor Agitation
PubMed: 30367689
DOI: 10.1111/anae.14472 -
BMJ Open Jul 2019The aim of this systematic review was to assess the efficacy and safety of pharmacological agents in the management of agitated behaviours following traumatic brain...
OBJECTIVE
The aim of this systematic review was to assess the efficacy and safety of pharmacological agents in the management of agitated behaviours following traumatic brain injury (TBI).
METHODS
We performed a search strategy in PubMed, OvidMEDLINE, Embase, CINAHL, PsycINFO, Cochrane Library, Google Scholar, Directory of Open Access Journals, LILACS, Web of Science and Prospero (up to 10 December 2018) for published and unpublished evidence on the risks and benefits of 9 prespecified medications classes used to control agitated behaviours following TBI. We included all randomised controlled trials, quasi-experimental and observational studies examining the effects of medications administered to control agitated behaviours in TBI patients. Included studies were classified into three mutually exclusive categories: (1) agitated behaviour was the presenting symptom; (2) agitated behaviour was not the presenting symptom, but was measured as an outcome variable; and (3) safety of pharmacological interventions administered to control agitated behaviours was measured.
RESULTS
Among the 181 articles assessed for eligibility, 21 studies were included. Of the studies suggesting possible benefits, propranolol reduced maximum intensities of agitation per week and physical restraint use, methylphenidate improved anger measures following 6 weeks of treatment, valproic acid reduced weekly agitated behaviour scale ratings and olanzapine reduced irritability, aggressiveness and insomnia between weeks 1 and 3 of treatment. Amantadine showed variable effects and may increase the risk of agitation in the critically ill. In three studies evaluating safety outcomes, antipsychotics were associated with an increased duration of post-traumatic amnesia (PTA) in unadjusted analyses. Small sample sizes, heterogeneity and an unclear risk of bias were limits.
CONCLUSIONS
Propranolol, methylphenidate, valproic acid and olanzapine may offer some benefit; however, they need to be further studied. Antipsychotics may increase the length of PTA. More studies on tailored interventions and continuous evaluation of safety and efficacy throughout acute, rehabilitation and outpatient settings are needed.
PROSPERO REGISTRATION NUMBER
CRD42016033140.
Topics: Antipsychotic Agents; Brain Injuries, Traumatic; Humans; Psychomotor Agitation; Psychoses, Substance-Induced; Randomized Controlled Trials as Topic
PubMed: 31289093
DOI: 10.1136/bmjopen-2019-029604