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Tremor and Other Hyperkinetic Movements... 2024Subacute Sclerosing Panencephalitis (SSPE) typically presents with periodic myoclonus; however, a spectrum of movement disorders including dystonia, chorea, tremor, and... (Review)
Review
BACKGROUND
Subacute Sclerosing Panencephalitis (SSPE) typically presents with periodic myoclonus; however, a spectrum of movement disorders including dystonia, chorea, tremor, and parkinsonism have also been described. This review aims to evaluate the array of movement disorders in SSPE, correlating them with neuroimaging findings, disease stages, and patient outcomes.
METHODS
A comprehensive review of published case reports and case series was conducted on patients with SSPE exhibiting movement disorders other than periodic myoclonus. PRISMA guidelines were followed, and the protocol was registered with PROSPERO (2023 CRD42023434650). A comprehensive search of multiple databases yielded 37 reports detailing 39 patients. Dyken's criteria were used for SSPE diagnosis, and the International Movement Disorders Society definitions were applied to categorize movement disorders.
RESULTS
The majority of patients were male, with an average age of 13.8 years. Approximately, 80% lacked a reliable vaccination history, and 39% had prior measles infections. Dystonia was the most common movement disorder (49%), followed by parkinsonism and choreoathetosis. Rapid disease progression was noted in 64% of cases, with a disease duration of ≤6 months in 72%. Neuroimaging showed T2/FLAIR MR hyperintensities, primarily periventricular, with 26% affecting the basal ganglia/thalamus. Brain biopsies revealed inflammatory and neurodegenerative changes. Over half of the patients (56%) reached an akinetic mute state or died.
CONCLUSION
SSPE is associated with diverse movement disorders, predominantly hyperkinetic. The prevalence of dystonia suggests basal ganglia dysfunction.
Topics: Humans; Chorea; Dystonia; Hyperkinesis; Hypokinesia; Movement Disorders; Parkinsonian Disorders; Subacute Sclerosing Panencephalitis; Case Reports as Topic; Male; Female; Adolescent
PubMed: 38765932
DOI: 10.5334/tohm.875 -
Tremor and Other Hyperkinetic Movements... 2024Opsoclonus is a rare disorder characterized by conjugate multidirectional, horizontal, vertical, and torsional saccadic oscillations, without intersaccadic interval,...
BACKGROUND
Opsoclonus is a rare disorder characterized by conjugate multidirectional, horizontal, vertical, and torsional saccadic oscillations, without intersaccadic interval, resulting from dysfunction within complex neuronal pathways in the brainstem and cerebellum. While most cases of opsoclonus are associated with autoimmune or paraneoplastic disorders, infectious agents, trauma, or remain idiopathic, opsoclonus can also be caused by medications affecting neurotransmission. This review was prompted by a case of opsoclonus occurring in a patient with Multiple System Atrophy, where amantadine, an NMDA-receptor antagonist, appeared to induce opsoclonus.
METHODS
Case report of a single patient and systematized review of toxic/drug-induced opsoclonus, selecting articles based on predefined criteria and assessing the quality of included studies.
RESULTS
The review included 30 articles encompassing 158 cases of toxic/drug-induced opsoclonus. 74% of cases were attributed to bark scorpion poisoning, followed by 9% of cases associated with chlordecone intoxication. The remaining cases were due to various toxics/drugs, highlighting the involvement of various neurotransmitters, including acetylcholine, glutamate, GABA, dopamine, glycine, and sodium channels, in the development of opsoclonus.
CONCLUSION
Toxic/drug-induced opsoclonus is very rare. The diversity of toxics/drugs impacting different neurotransmitter systems makes it challenging to define a unifying mechanism, given the intricate neuronal pathways underlying eye movement physiology and opsoclonus pathophysiology.
Topics: Humans; Male; Amantadine; Multiple System Atrophy; Ocular Motility Disorders; Aged
PubMed: 38737300
DOI: 10.5334/tohm.832 -
PloS One 2024Juvenile Myoclonic Epilepsy (JME) is a prevalent form of epileptic disorder, specifically categorized within the realm of Genetic Generalized Epilepsy (GGE). Its... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Juvenile Myoclonic Epilepsy (JME) is a prevalent form of epileptic disorder, specifically categorized within the realm of Genetic Generalized Epilepsy (GGE). Its hallmark features encompass unprovoked bilateral myoclonus and tonic-clonic seizures that manifest during adolescence. While most JME patients respond favorably to anti-seizure medication (ASM), a subset experiences refractory JME, a condition where seizures persist despite rigorous ASM treatment, often termed "Drug-Resistant Epilepsy" (DRE). This systematic review and meta-analysis aims to determine the prevalence of refractory JME, and further to identify socio-demographic, electrophysiological and clinical risk factors associated with its occurrence. Pinpointing these factors is crucial as it offers the potential to predict ASM responsiveness, enabling early interventions and tailored care strategies for patients.
MATERIAL AND METHODS
The systematic review and meta-analysis followed the Cochrane Handbook and adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The study evaluated outcomes post ASM treatment in JME cohorts by searching papers published up to September 2023 in PubMed/MEDLINE, Scopus, and Google Scholar databases. Predefined inclusion criteria were met by 25 eligible studies, forming the basis for analysis.
RESULTS
A total of 22 potential risk factors for refractory JME were documented. Notably, robust risk factors for treatment resistance included Psychiatric Disorder (Odds Ratio (OR), 3.42 [2.54, 4.61] (95% Confidence Inverval (Cl)), Febrile Seizures (OR, 1.83 [1.14, 2.96] (95% Cl)), Alcohol Consumption (OR, 16.86 [1.94, 146.88] (95%Cl)), Aura (OR, 2.15 [1.04, 4.47] (95%Cl)), childhood absence epilepsy (CAE) evolving into JME (OR, 4.54 [1.61, 12.78] (95%CI)), occurrence of three seizure types (OR, 2.96 [1.96, 4.46] (95%CI)), and Focal EEG abnormalities (OR, 1.85 [1.13, 3.01] (95%Cl)). In addition, there were some non-significant risk factors for DRE because of noticeable heterogeneity.
CONCLUSION
In aggregate, over 36% of JME patients demonstrated drug resistance, with seven significant risk factors closely linked to this refractoriness. The interplay between these factors and whether they denote treatment non-response or heightened disease burden remains an open question and more studies would be required to fully examine their influence.
Topics: Adolescent; Humans; Child; Myoclonic Epilepsy, Juvenile; Epilepsy, Absence; Seizures; Drug Resistant Epilepsy; Risk Factors; Electroencephalography; Anticonvulsants
PubMed: 38593118
DOI: 10.1371/journal.pone.0300930 -
Journal of Movement Disorders Mar 2024Since the release of vaccines against COVID-19, there have been reports of vaccine-related neurologic complications. This study aimed to create a descriptive systematic...
OBJECTIVES
Since the release of vaccines against COVID-19, there have been reports of vaccine-related neurologic complications. This study aimed to create a descriptive systematic review of movement disorders associated with COVID-19 vaccines.
METHODS
We described the demographics, clinical presentation, management, outcomes, and proposed patho-mechanism. A systematic review was performed according to the PRISMA guidelines. A standardized tool was used to assess the quality of the cases.
RESULTS
We have identified 8 articles that met our inclusion criteria consisting of 10 patients who developed movement disorders after vaccination. The majority were males (n = 8), with a median age of 64.5 years. The most common movement disorder was hemichorea. The rest presented with generalized chorea with myoclonus, cervical dystonia, and akathisia. Most cases responded with immunotherapy. The standardized tool used showed that most studies have a low risk of bias.
CONCLUSION
The reported incidence of vaccine-related movement disorders was low in occurrence based on available published cases that were found.
PubMed: 38500249
DOI: 10.14802/jmd.24001 -
Annals of Indian Academy of Neurology 2023COVID-19 infection is associated with neurological manifestations, including various types of movement disorders (MD). A thorough review of individual patients with...
BACKGROUND
COVID-19 infection is associated with neurological manifestations, including various types of movement disorders (MD). A thorough review of individual patients with COVID-19-induced MD would help in better understanding the clinical profile and outcome of these patients and in prognostication.
OBJECTIVE
We conducted an individual patient-systematic review to study the clinical and imaging profile and outcomes of patients with COVID-19-associated MD.
METHODS
A systematic literature search of PubMed, EMBASE, and Cochrane databases was conducted by two independent reviewers. Individual patient data COVID from case reports and case series on COVID-19-associated MD, published between December 2019 and December 2022, were extracted and analyzed.
RESULTS
Data of 133 patients with COVID-19-associated MD from 82 studies were analyzed. Mean age was 55 ± 18 years and 77% were males. A mixed movement disorder was most commonly seen (41%); myoclonus-ataxia was the most frequent (44.4%). Myoclonus significantly correlated with age (odds ratio (OR) 1.02 = 0.03, CI 1-1.04). Tremor had the longest latency to develop after SARS-CoV-2 infection [median (IQR) 21 (10-40) days, = 0.009, CI 1.01-1.05]. At short-term follow-up, myoclonus improved (OR 14.35, value = 0.01, CI 1.71-120.65), whereas parkinsonism (OR 0.09, value = 0.002, CI 0.19-0.41) and tremor (OR 0.16, value = 0.016, CI 0.04-0.71) persisted.
CONCLUSION
Myoclonus-ataxia was the most common movement disorder after COVID-19 infection. Myoclonus was seen in older individuals and usually improved. Tremor and parkinsonism developed after a long latency and did not improve in the short-term.
PubMed: 38022478
DOI: 10.4103/aian.aian_572_23 -
Parkinsonism & Related Disorders Sep 2023The human immunodeficiency virus (HIV) causes movement disorders in persons living with HIV (PLH). (Review)
Review
BACKGROUND
The human immunodeficiency virus (HIV) causes movement disorders in persons living with HIV (PLH).
OBJECTIVES AND METHODS
We conducted a systematic review on the spectrum of movement disorders in PLH using standard terms for each of the phenomenologies and HIV.
RESULTS
Movement disorders in PLH were commonly attributed to opportunistic infections (OI), dopamine receptor blockade reactions, HIV-associated dementia (HAD), presented during seroconversion, developed due to drug reactions or antiretroviral therapy (ART) itself and lastly, movement disorders occurred as a consequence of the HIV-virus. Parkinsonism in ART naïve PLH was associated with shorter survival, however when Parkinsonism presented in PLH on ART, the syndrome was indistinguishable from Idiopathic Parkinson's disease and responded to therapy. Tremor was often postural due to HAD, drugs or OI. Generalized chorea was most frequent in HIV encephalopathy and toxoplasmosis gondii caused most cases of hemichorea. Ataxia was strongly associated with JCV infection, ART efavirenz toxicity or due to HIV itself. Dystonia was reported in HAD, secondary to drugs and atypical facial dystonias. Both cortical/subcortical and segmental/spinal origin myoclonus were noted mainly associated with HAD. In patients with HIV related opsoclonus-myoclonus-ataxia-syndrome, seroconversion illness was the commonest cause of followed by IRIS and CSF HIV viral escape phenomenon.
CONCLUSIONS
Aetiology of movement disorders in PLH depend on the treatment state. Untreated, PLH are prone to develop OI and HAD and movement disorders. However, as the number of PLH on ART increase and survive longer, the frequency of ART and non-AIDS related complications are likely to increase.
Topics: Humans; HIV; Myoclonus; Movement Disorders; HIV Infections; Parkinson Disease; Parkinsonian Disorders; Ataxia
PubMed: 37532621
DOI: 10.1016/j.parkreldis.2023.105774 -
Seizure Jul 2023The late onset myoclonic epilepsy in Down Syndrome (LOMEDS) is a peculiar epilepsy type characterized by cortical myoclonus and generalized tonic-clonic seizures (GTCS),... (Review)
Review
INTRODUCTION
The late onset myoclonic epilepsy in Down Syndrome (LOMEDS) is a peculiar epilepsy type characterized by cortical myoclonus and generalized tonic-clonic seizures (GTCS), in people suffering from cognitive decline in Down syndrome (DS). In this review, we analyzed available data on the diagnostic and therapeutic management of individuals with LOMEDS.
METHODS
We performed a systematic search of the literature to identify the diagnostic and therapeutic management of patients with LOMEDS. The following databases were used: PubMed, Google Scholar, EMBASE, CrossRef. The protocol was registered on PROSPERO (registration code: CRD42023390748).
RESULTS
Data from 46 patients were included. DS was diagnosed according to the patient's clinical and genetic characteristics. Diagnosis of Alzheimer's dementia (AD) preceded the onset of epilepsy in all cases. Both myoclonic seizures (MS) and generalized tonic-clonic seizures (GTCS) were reported, the latter preceding the onset of MS in 28 cases. EEG was performed in 45 patients, showing diffuse theta/delta slowing with superimposed generalized spike-and-wave or polyspike-and-wave. A diffuse cortical atrophy was detected in 34 patients on neuroimaging. Twenty-seven patients were treated with antiseizure medication (ASM) monotherapy, with reduced seizure frequency in 17 patients. Levetiracetam and valproic acid were the most used ASMs. Up to 41% of patients were unresponsive to first-line treatment and needed adjunctive therapy for seizure control.
CONCLUSIONS
AD-related pathological changes in the brain may play a role in LOMEDS onset, although the mechanism underlying this phenomenon is still unknown. EEG remains the most relevant investigation to be performed. A significant percentage of patients developed a first-line ASM refractory epilepsy. ASMs which modulate the glutamatergic system may represent a good therapeutic option.
Topics: Humans; Down Syndrome; Epilepsy; Epilepsies, Myoclonic; Levetiracetam; Seizures; Alzheimer Disease; Electroencephalography; Anticonvulsants; Epilepsy, Generalized
PubMed: 37267668
DOI: 10.1016/j.seizure.2023.05.017 -
Movement Disorders Clinical Practice Mar 2023There is overlap between movement disorders and neuroendocrine abnormalities. (Review)
Review
BACKGROUND
There is overlap between movement disorders and neuroendocrine abnormalities.
OBJECTIVES AND METHODS
To provide a systematic review on the association of thyroid dysfunction and movement disorders. Thyroid physiological function and classical thyroid disorders highlighting typical and atypical manifestations including movement disorders, as well as diagnostic procedures, and treatments are discussed.
RESULTS
Hypothyroidism may be associated with hypokinetic and hyperkinetic disorders. There is debate whether their concomitance reflects a causal link, is coincidence, or the result of one unmasking the other. Hypothyroidism-associated parkinsonism may resemble idiopathic Parkinson's disease. Hypothyroidism-associated hyperkinetic disorders mainly occur in the context of steroid-responsive encephalopathy with autoimmune thyroiditis, that is, Hashimoto disease, mostly manifesting with tremor, myoclonus, and ataxia present in 28-80%, 42-65% and 33-65% in larger series. Congenital hypothyroidism manifesting with movement disorders, mostly chorea and dystonia, due to Mendelian genetic disease are rare.Hyperthyroidism on the other hand mostly manifests with hyperkinetic movement disorders, typically tremor (present in three quarters of patients). Chorea (present in about 2% of hyperthyroid patients), dystonia, myoclonus, ataxia and paroxysmal movement disorders, as well as parkinsonism have also been reported, with correlation between movement intensity and thyroid hormone levels.On a group level, studies on the role of thyroid dysfunction as a risk factor for the development of PD remain non-conclusive.
CONCLUSIONS
In view of the treatability of movement disorders associated with thyroid disease, accurate diagnosis is important. The pathophysiology remains poorly understood. More detailed case documentation and systematic studies, along with experimental studies are needed.
PubMed: 36949803
DOI: 10.1002/mdc3.13656 -
European Review For Medical and... Feb 2023Myoclonus is one of the main complications of etomidate anesthesia, which would develop into serious consequences during surgery. The present analysis was performed to... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Myoclonus is one of the main complications of etomidate anesthesia, which would develop into serious consequences during surgery. The present analysis was performed to evaluate systematically the effect of propofol on preventing etomidate-induced myoclonus in adult patients.
MATERIALS AND METHODS
Systematic electronic literature search was performed in the databases PubMed, Cochrane Library, OVID, Wanfang and China National Knowledge Infrastructure (CNKI) from inception to May 20, 2021, without any language restrictions. All randomized controlled trials evaluating the efficacy of propofol on preventing etomidate-induced myoclonus were enrolled. The primary outcome included the incidence and degree of etomidate-induced myoclonus.
RESULTS
1,420 patients (with 602 received etomidate anesthesia and 818 received propofol plus etomidate anesthesia) from 13 studies were eventually included. Whatever the intravenous propofol dose for anesthesia induction 0.8-2 mg/kg (RR:4.04, 95% CI [2.42,6.74] p<0.0001, I2=56.5%), or the dose of propofol for anesthesia induction 0.5-0.8 mg/kg (RR:3.26, 95% CI [2.03,5.22] p<0.0001, I2=0%), or the dose of propofol for anesthesia induction 0.25-0.5mg/kg (RR:1.68, 95% CI [1.1,2.56] p=0.0160, I2=0%), combination of propofol and etomidate could significantly decrease the occurrence of etomidate-related myoclonus (RR=2.99, 95% CI [2.40, 3.71] p<0.0001, I2=43.4%), compared with etomidate alone. In addition, propofol plus etomidate attenuated the incidence of mild (RR:3.40, 95% CI [1.7,6.82] p=0.0010, I2=54.3%), moderate (RR:5.4, 95% CI [3.01, 9.67] p<0.0001, I2=12.6%), severe (RR:4.15, 95% CI [2.11, 8.13] p<0.0001, I2=0%) of etomidate-induced myoclonus without adverse effects except for the increased incidence of pain on injection (RR:0.47, 95% CI [0.26, 0.83] p=0.0100, I2=41.5%) compared with etomidate alone.
CONCLUSIONS
The meta-analysis currently generates the evidence of combination of propofol with the dosage of 0.25-2 mg/kg and etomidate can alleviate the occurrence and severity of etomidate-induced myoclonus, with decreased incidence of postoperative nausea and vomiting (PONV) and comparative side effects of hemodynamic and respiratory depression of patients in comparison with etomidate alone.
Topics: Adult; Humans; Anesthesia, General; Drug-Related Side Effects and Adverse Reactions; Etomidate; Myoclonus; Pain; Propofol
PubMed: 36876671
DOI: 10.26355/eurrev_202302_31366 -
Medicine Feb 2023Propofol is increasingly being used for sedation in gastrointestinal endoscopy; however, owing to its side effects, an alternative drug is needed. We aimed to compare... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Propofol is increasingly being used for sedation in gastrointestinal endoscopy; however, owing to its side effects, an alternative drug is needed. We aimed to compare the safety, satisfaction, and efficacy outcomes of etomidate versus propofol in patients undergoing gastrointestinal endoscopy, including advanced endoscopic procedures.
METHODS
We systematically searched Embase, PubMed, Cochrane Central Register of Controlled Trials, CINAHL (via EBSCO), China National Knowledge Infrastructure, and Web of Science (1946-April 2020) databases for randomized controlled trials of gastrointestinal endoscopy (upper gastrointestinal endoscopy, colonoscopy, and advanced endoscopy) using etomidate or propofol as sedatives. We pooled odds ratios (ORs) for the safety profile and patient and anesthesiologist satisfaction using mixed-effects conditional logistic models and standardized mean differences for efficiency outcomes using random-effects models.
RESULTS
Twenty-four studies involving 3875 patients were included. Compared with propofol, etomidate resulted in significantly reduced apnea (OR: 0.22; 95% confidence interval [CI]: 0.13-0.37; P < .001), hypoxemia (OR: 0.43; 95% CI: 0.35-0.54; P < .001), hypotension (OR: 0.20; 95% CI: 0.11-0.36; P < .001), and bradycardia (OR: 0.52; 95% CI: 0.30-0.91; P = .02) but led to increased myoclonus (OR: 8.54; 95% CI: 5.20-14.01; P < .001) and lowered anesthesiologist satisfaction (OR: 0.60; 95% CI: 0.39-0.91; P = .02).
CONCLUSION
Etomidate may be a good alternative to propofol for gastrointestinal endoscopy, especially advanced endoscopy. Etomidate appears to be safe as an inducer for hemodynamically unstable patients or older adult patients undergoing gastrointestinal endoscopy.
Topics: Humans; Aged; Propofol; Etomidate; Endoscopy, Gastrointestinal; Hypnotics and Sedatives; Colonoscopy
PubMed: 36820568
DOI: 10.1097/MD.0000000000032876