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Medicina 2023Juvenile myoclonic epilepsy (JME) is an epileptic syndrome with onset in childhood and adolescence with myoclonus, absences, and generalized tonic-clonic seizures....
INTRODUCTION
Juvenile myoclonic epilepsy (JME) is an epileptic syndrome with onset in childhood and adolescence with myoclonus, absences, and generalized tonic-clonic seizures. Reflex stimuli such as sensitivity to light or photosensitivity, eyelid opening and closing, and praxis induction produce epileptiform discharges and seizures. These reflex triggers are not all systematically studied.
OBJECTIVE
Examine reflex features in patients with JME.
METHODS
One hundred adolescents and adults with JME who received different anti-seizure treatments were evaluated consecutively. A standard electroencephalogram was performed with an intermittent light stimulation (SLI) protocol and another for the evaluation of praxias through neurocognitive activity (CNA). The statistical analysis was descriptive and of correlation with a p > 0.05.
RESULTS
Current age was 28±11 (14-67). The seizure began at 15 years ±3 (Range 8-25 years). They presented myoclonus and generalized tonic-clonic seizures in 58%. 50% received valproic acid and 31% continued with seizures. Epileptiform discharges at rest 20%; hyperventilation 30%; eyelid opening and closing 12%; photoparoxysmal response in SLI 40%; CNA 23%. Higher percentage of discharges and delay in performing CNA in those who presented seizures. Valproic acid compared to other drugs did not demonstrate superiority in seizure control.
CONCLUSIONS
These findings confirm the importance of studying reflex traits for diagnosis, follow-up, and therapeutic control.
Topics: Adult; Adolescent; Humans; Myoclonic Epilepsy, Juvenile; Valproic Acid; Myoclonus; Electroencephalography; Reflex; Seizures; Epilepsies, Myoclonic
PubMed: 38117708
DOI: No ID Found -
Journal of Clinical Neurophysiology :... Feb 2023Diagnosing and characterizing myoclonus can be challenging. Many authors agree on the need to complement the clinical findings with an electrophysiological study to...
Diagnosing and characterizing myoclonus can be challenging. Many authors agree on the need to complement the clinical findings with an electrophysiological study to characterize the movements. Besides helping to rule out other movements that may look like myoclonus, electrophysiology can help localize the source of the movement. This article aims to serve as a practical manual on how to do a myoclonus study. For this purpose, the authors combine their experience with available evidence. The authors provide detailed descriptions of recording poly-electromyography, combining electroencephalography and electromyography, Bereitschaftspotentials, somatosensory evoked potentials, and startle techniques. The authors discuss analysis considerations for these data and provide a simplified algorithm for their interpretation. Finally, the authors discuss some factors that they believe have hindered the broader use of these useful techniques.
Topics: Humans; Myoclonus; Electroencephalography; Electromyography; Movement; Evoked Potentials, Somatosensory
PubMed: 36735457
DOI: 10.1097/WNP.0000000000000885 -
Neurology Nov 2014Propriospinal myoclonus (PSM) is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. It has a... (Review)
Review
OBJECTIVE
Propriospinal myoclonus (PSM) is a rare disorder with repetitive, usually flexor arrhythmic brief jerks of the trunk, hips, and knees in a fixed pattern. It has a presumed generation in the spinal cord and diagnosis depends on characteristic features at polymyography. Recently, a historical paradigm shift took place as PSM has been reported to be a functional (or psychogenic) movement disorder (FMD) in most patients. This review aims to characterize the clinical features, etiology, electrophysiologic features, and treatment outcomes of PSM.
METHODS
Re-evaluation of all published PSM cases and systematic scoring of clinical and electrophysiologic characteristics in all published cases since 1991.
RESULTS
Of the 179 identified patients with PSM (55% male), the mean age at onset was 43 years (range 6-88 years). FMD was diagnosed in 104 (58%) cases. In 12 cases (26% of reported secondary cases, 7% of total cases), a structural spinal cord lesion was found. Clonazepam and botulinum toxin may be effective in reducing jerks.
CONCLUSIONS
FMD is more frequent than previously assumed. Structural lesions reported to underlie PSM are scarce. Based on our clinical experience and the reviewed literature, we recommend polymyography to assess recruitment variability combined with a Bereitschaftspotential recording in all cases.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Catenins; Child; Electrophysiology; Female; Follow-Up Studies; Humans; Male; Middle Aged; Myoclonus; Psychophysiologic Disorders; PubMed; Spinal Cord; Young Adult; Delta Catenin
PubMed: 25305154
DOI: 10.1212/WNL.0000000000000982 -
Medicine Jun 2017Myoclonus, a common complication during intravenous induction with etomidate, is bothersome to both anesthesiologists and patients. This study explored the preventive... (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVE
Myoclonus, a common complication during intravenous induction with etomidate, is bothersome to both anesthesiologists and patients. This study explored the preventive effect of pretreatment with propofol on etomidate-related myoclonus.
METHODS
This was a prospective, double-blind, clinical, randomized controlled study. Totally, 363 patients who were scheduled for a short-duration, painless gastrointestinal endoscopy were divided into 5 groups. Four groups received 0 mg/kg (E group), 0.25 mg/kg (LPE group), 0.50 mg/kg (MPE group), or 0.75 mg/kg (HPE group) propofol pretreatment before etomidate anesthesia. Another group only received 1 to 2 mg/kg of propofol (P group) as anesthesia. The incidence and severity of myoclonus, patient circulation and respiratory status, and intraoperative and postoperative complications were recorded.
RESULTS
The incidence of myoclonus in the LPE group (26.8%), MPE group (16.4%), HPE group (14.9%), and P group (0) was lower than the E group (48.6%, P < .05). The incidence of grade 1, 2, and 3 of myoclonus in the LPE group, MPE group, HPE group, and P group was significantly lower than the E group, and that in the P group was lower than the LPE group (P < .05). The incidence of hypoxemia in the P group was higher than the E group, and the incidence of adverse events in the HPE group and P group was lower than the E group (P < .05).
DISCUSSION
Pretreatment with propofol was feasible for preventing etomidate-related myoclonus. Furthermore, as propofol dosage increased, its effect on reducing the incidence and severity of myoclonic movements induced by etomidate increased.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anesthetics, Intravenous; Dose-Response Relationship, Drug; Double-Blind Method; Etomidate; Female; Gastroscopy; Hemodynamics; Humans; Incidence; Male; Middle Aged; Myoclonus; Postoperative Complications; Propofol; Respiration; Severity of Illness Index; Time Factors; Young Adult
PubMed: 28658112
DOI: 10.1097/MD.0000000000007212 -
Journal of Ayub Medical College,... 2019Neurology still remains one of the most underserved specialties of medicine in Pakistan with roughly one neurologist per million people. Movement disorders (MD) are... (Review)
Review
Neurology still remains one of the most underserved specialties of medicine in Pakistan with roughly one neurologist per million people. Movement disorders (MD) are neurological problems that interfere with patient's motor abilities and diagnosis is typically clinical. In this review, we describe a practical approach to common MD emergencies that may be encountered by a non-neurologist physician, emphasizing on formulating a working diagnosis and their immediate management. Movement disorder emergencies can be classified based on MD phenomenology and we will provide a brief overview of dystonia including acute dystonic reaction, PAID syndrome and dystonic storm; chorea, myoclonus including serotonin syndrome and startle disease; and rigidity including neuroleptic malignant syndrome and malignant hyperthermia.
Topics: Chorea; Delirium; Dystonia; Emergencies; Humans; Malignant Hyperthermia; Movement Disorders; Myoclonus; Neuroleptic Malignant Syndrome; Pakistan
PubMed: 31535526
DOI: No ID Found -
Clinical Neurophysiology : Official... Oct 2021To develop and test wearable monitoring of surface electromyography and motion for detection and quantification of positive and negative myoclonus in patients with...
OBJECTIVE
To develop and test wearable monitoring of surface electromyography and motion for detection and quantification of positive and negative myoclonus in patients with progressive myoclonic epilepsy type 1 (EPM1).
METHODS
Surface electromyography and three-dimensional acceleration were measured from 23 EPM1 patients from the biceps brachii (BB) of the dominant and the extensor digitorum communis (EDC) of the non-dominant arm for 48 hours. The patients self-reported the degree of myoclonus in a diary once an hour. Severity of myoclonus with action was evaluated by using video-recorded Unified Myoclonus Rating Scale (UMRS). Correlations of monitored parameters were quantified with the UMRS scores and the self-reported degrees of myoclonus.
RESULTS
The monitoring-based myoclonus index correlated significantly (p < 0.001) with the UMRS scores (ρ = 0.883 for BB and ρ = 0.823 for EDC) and with the self-reported myoclonus degrees (ρ = 0.483 for BB and ρ = 0.443 for EDC). Ten patients were assessed as probably having negative myoclonus in UMRS, while our algorithm detected that in twelve patients.
CONCLUSIONS
Wearable monitoring was able to detect both positive and negative myoclonus in EPM1 patients.
SIGNIFICANCE
Our method is suitable for quantifying objective, real-life treatment effects at home and progression of myoclonus.
Topics: Accelerometry; Adolescent; Adult; Electromyography; Female; Humans; Male; Middle Aged; Myoclonus; Unverricht-Lundborg Syndrome; Wearable Electronic Devices; Young Adult
PubMed: 34454274
DOI: 10.1016/j.clinph.2021.06.026 -
Brain : a Journal of Neurology Apr 2024Cortical myoclonus is produced by abnormal neuronal discharges within the sensorimotor cortex, as demonstrated by electrophysiology. Our hypothesis is that the loss of...
Cortical myoclonus is produced by abnormal neuronal discharges within the sensorimotor cortex, as demonstrated by electrophysiology. Our hypothesis is that the loss of cerebellar inhibitory control over the motor cortex, via cerebello-thalamo-cortical connections, could induce the increased sensorimotor cortical excitability that eventually causes cortical myoclonus. To explore this hypothesis, in the present study we applied anodal transcranial direct current stimulation over the cerebellum of patients affected by cortical myoclonus and healthy controls and assessed its effect on sensorimotor cortex excitability. We expected that anodal cerebellar transcranial direct current stimulation would increase the inhibitory cerebellar drive to the motor cortex and therefore reduce the sensorimotor cortex hyperexcitability observed in cortical myoclonus. Ten patients affected by cortical myoclonus of various aetiology and 10 aged-matched healthy control subjects were included in the study. All participants underwent somatosensory evoked potentials, long-latency reflexes and short-interval intracortical inhibition recording at baseline and immediately after 20 min session of cerebellar anodal transcranial direct current stimulation. In patients, myoclonus was recorded by the means of surface EMG before and after the cerebellar stimulation. Anodal cerebellar transcranial direct current stimulation did not change the above variables in healthy controls, while it significantly increased the amplitude of somatosensory evoked potential cortical components, long-latency reflexes and decreased short-interval intracortical inhibition in patients; alongside, a trend towards worsening of the myoclonus after the cerebellar stimulation was observed. Interestingly, when dividing patients in those with and without giant somatosensory evoked potentials, the increment of the somatosensory evoked potential cortical components was observed mainly in those with giant potentials. Our data showed that anodal cerebellar transcranial direct current stimulation facilitates-and does not inhibit-sensorimotor cortex excitability in cortical myoclonus syndromes. This paradoxical response might be due to an abnormal homeostatic plasticity within the sensorimotor cortex, driven by dysfunctional cerebello-thalamo-cortical input to the motor cortex. We suggest that the cerebellum is implicated in the pathophysiology of cortical myoclonus and that these results could open the way to new forms of treatment or treatment targets.
Topics: Humans; Aged; Transcranial Direct Current Stimulation; Transcranial Magnetic Stimulation; Myoclonus; Evoked Potentials, Motor; Cerebellum
PubMed: 37956080
DOI: 10.1093/brain/awad384 -
Journal of Neurology Oct 2021Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic in December 2019, neurological manifestations have been recognized as potential complications.... (Review)
Review
BACKGROUND
Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic in December 2019, neurological manifestations have been recognized as potential complications. Relatively rare movement disorders associated with COVID-19 are increasingly reported in case reports or case series. Here, we present a case and systematic review of myoclonus and cerebellar ataxia associated with COVID-19.
METHODS
A systematic review was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guideline using the PubMed and Ovid MEDLINE databases, from November 1, 2019 to December 6, 2020.
RESULTS
51 cases of myoclonus or ataxia associated with COVID-19, including our case, were identified from 32 publications. The mean age was 59.6 years, ranging from 26 to 88 years, and 21.6% were female. Myoclonus was multifocal or generalized and had an acute onset, usually within 1 month of COVID-19 symptoms. Myoclonus occurred in isolation (46.7%), or with ataxia (40.0%) or cognitive changes (30.0%). Most cases improved within 2 months, and treatment included anti-epileptic medications or immunotherapy. Ataxia had an acute onset, usually within 1 month of COVID-19 symptoms, but could be an initial symptom. Concurrent neurological symptoms included cognitive changes (45.5%), myoclonus (36.4%), or a Miller Fisher syndrome variant (21.2%). Most cases improved within 2 months, either spontaneously or with immunotherapy.
CONCLUSIONS
This systematic review highlights myoclonus and ataxia as rare and treatable post-infectious or para-infectious, immune-mediated phenomena associated with COVID-19. The natural history is unknown and future investigation is needed to further characterize these movement disorders and COVID-19.
Topics: Ataxia; COVID-19; Cerebellar Ataxia; Female; Humans; Middle Aged; Myoclonus; SARS-CoV-2
PubMed: 33616739
DOI: 10.1007/s00415-021-10458-0 -
Drug Design, Development and Therapy 2023Remimazolam tosilate (RT) is a new ultrashort-acting γ-aminobutyric acid subtype A (GABA) agonist, with the characteristics of rapid onset and offset, minimal... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Remimazolam tosilate (RT) is a new ultrashort-acting γ-aminobutyric acid subtype A (GABA) agonist, with the characteristics of rapid onset and offset, minimal cardiorespiratory depression. Currently, few studies have compared the effect of RT and etomidate on hemodynamics during anesthesia induction. Here, we aimed to compare the hemodynamic effects of different doses of RT and etomidate for anesthesia induction in patients undergoing cardiac surgeries.
METHODS
Patients were recruited from January to September 2022 in this single-center, prospective, randomized, double-blind trial. A total of 117 patients undergoing selective valve replacement surgery were randomly divided into low-dose RT (0.2 mg/kg) group (group LR), high-dose RT (0.3 mg/kg) group (group HR), or etomidate (1.5 mg/kg) group (group E), respectively. The primary outcome was hemodynamic fluctuations (mean arterial pressure fluctuation value [∆MAP]; heart rate fluctuation value [∆HR]) during anesthesia induction. Secondary outcomes included the incidence of adverse drug reactions (injection pain and myoclonus) and adverse cardiovascular events, vital signs at different time points and the cumulative doses of vasoactive drugs.
RESULTS
The hemodynamic fluctuations (∆MAP) in group LR and group E were significantly lower than that in group HR. In addition, the incidence of hypotension and the cumulative norepinephrine doses in group E and group LR were also significantly lower than that in group HR. Furthermore, the incidence of injection pain and myoclonus in group LR and group HR were less frequently recorded compared with group E. There were no significant differences in terms of ∆HR, tachycardia, hypertension, severe bradycardia, vital signs at different time points, lactic acid and blood glucose between both groups.
CONCLUSION
Compared with etomidate, low-dose RT (0.2mg/kg) can not only provide stable hemodynamic parameters but also cause fewer adverse reactions when used for anesthesia induction in patients with cardiac disease.
Topics: Humans; Etomidate; Anesthetics, Intravenous; Myoclonus; Prospective Studies; Hemodynamics; Cardiac Surgical Procedures; Pain; Propofol
PubMed: 36789096
DOI: 10.2147/DDDT.S401969 -
Toxicology International 2014Second-generation antipsychotics (SGA), mainly clozapine have been reported to induce myoclonus. Although olanzapine-induced myoclonus is reported, dose-dependent...
Second-generation antipsychotics (SGA), mainly clozapine have been reported to induce myoclonus. Although olanzapine-induced myoclonus is reported, dose-dependent response has not been described. We report dose-related olanzapine-induced myoclonus in an early onset schizophrenia patient. We also suggest certain management strategies for such adverse side effects.
PubMed: 25948979
DOI: 10.4103/0971-6580.155393