BACKGROUND

Perampanel is an antagonist of the a-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor. It is currently FDA-approved to treat focal and generalized tonic-clonic seizures in epilepsy, but recent evidence suggests its potential in treating severe and refractory tremors.

To determine the safety and efficacy of perampanel in treating tremors via a systematic review of existing literature. 

We performed a literature search on five large databases (PubMed, Cochrane, Google Scholar, HERDIN, and Scopus) for clinical studies within the last 10 years and screened a total of 1,539 unique articles for full assessment. We filtered out papers on epilepsy as well as hypokinetic diseases and assessed nine articles for quality assessment and review.

A total of four case reports/series, four open-label trials, and one randomized controlled trial were assessed to be of fair to good quality. All trials showed that low-dose perampanel (2-4 mg/day) was safe and well-tolerated with minor adverse events reported by participants. A net benefit from baseline was observed in patients with essential and primary orthostatic tremors. However, current evidence is weak because the trials employed a non-randomized before-after study design with a small sample size and significant dropout rates.

Low-dose perampanel at 2-4 mg/day shows promising potential in treating refractory tremors and myoclonus in recent clinical studies, but current evidence is weak or anecdotal. Additional randomized controlled trials are needed to determine the conclusive benefit of perampanel for hyperkinesia.

Dentatorubral-pallidoluysian atrophy（DRPLA） is a rare autosomal dominant neurodegenerative disease caused by CAG triplet expansion in the atrophin 1 （ATN1） gene. Up to now， the pathogenesis of DRPLA remains unclear. The main clinical features of DRPLA include myoclonus， epilepsy， ataxia， choreoathetosis， and cognitive impairment. DRPLA has great clinical heterogeneity， and the number of CAG repeats is negatively correlated with age of onset and disease severity. Some patients with late-onset DRPLA may have atypical manifestations without typical imaging changes， which brings challenges to the diagnosis of the disease. This article reviews the pathogenesis， pathological features， clinical and imaging manifestations， diagnosis， and potential treatment of DRPLA， in order to deepen the understanding of this disease.
Ataxia ; Myoclonus

BACKGROUND

Perampanel is an antagonist of the a-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor. It is currently FDA-approved to treat focal and generalized tonic-clonic seizures in epilepsy, but recent evidence suggests its potential in treating severe and refractory tremors.

To determine the safety and efficacy of perampanel in treating tremors via a systematic review of existing literature. 

We performed a literature search on five large databases (PubMed, Cochrane, Google Scholar, HERDIN, and Scopus) for clinical studies within the last 10 years and screened a total of 1,539 unique articles for full assessment. We filtered out papers on epilepsy as well as hypokinetic diseases and assessed nine articles for quality assessment and review.

A total of four case reports/series, four open-label trials, and one randomized controlled trial were assessed to be of fair to good quality. All trials showed that low-dose perampanel (2-4 mg/day) was safe and well-tolerated with minor adverse events reported by participants. A net benefit from baseline was observed in patients with essential and primary orthostatic tremors. However, current evidence is weak because the trials employed a non-randomized before-after study design with a small sample size and significant dropout rates.

Low-dose perampanel at 2-4 mg/day shows promising potential in treating refractory tremors and myoclonus in recent clinical studies, but current evidence is weak or anecdotal. Additional randomized controlled trials are needed to determine the conclusive benefit of perampanel for hyperkinesia.

We describe an unusual case of hypoxic ischemic encephalopathy in a preterm female of 36 weeks who presented with status epilepticus and atypical abdominal myoclonus. The seizures were confirmed electrographically using video electroencephalography (EEG), while the abdominal myoclonus was demonstrated to be nonepileptic, as it had no EEG correlate. Other possible causes of neonatal seizures were excluded. The infant then responded to a gamut of antiseizure medications but the myoclonus persisted. To the best of our knowledge, this is the first report of atypical myoclonus in a preterm baby caused by hypoxic ischemic encephalopathy.

Objective: To analyze the clinical characteristics and treatment of middle ear myoclonus. Methods: Fifty-six cases of middle ear myoclonus were enrolled in Shandong Provincial ENT Hospital, Shandong University from September 2019 to August 2021, including 23 males and 33 females. The age ranged from 6 to 75 years, with a median age of 35 years; Forty-seven cases were unilateral tinnitus, nine cases were bilateral tinnitus. The time of tinnitus ranged from 20 days to 8 years. The voice characteristics, inducing factors, nature (frequency) of tinnitus, tympanic membrane conditions during tinnitus, audiological related tests, including long-term acoustic tympanogram, stapedius acoustic reflex, pure tone auditory threshold, short increment sensitivity test, alternate binaural loudness balance test, loudness discomfort threshold, vestibular function examination, facial electromyography, and imaging examination were recorded. Oral carbamazepine and/or surgical treatment were used. The patients were followed up for 6-24 months and the tinnitus changes were observed. Results: Tinnitus was diverse, including stepping on snow liking sound, rhythmic drumming, white noise, and so on. The inducing factors included external sound, body position change, touching the skin around the face and ears, speaking, chewing and blinking, etc. Forty-four cases were induced by single factor and 9 cases were induced by two or more factors. There was no definite inducing factor in 1 case. One patient had tinnitus with epilepsy. One case of traumatic facial paralysis after facial nerve decompression could induce tinnitus on the affected side when the auricle moved. Tympanic membrane flutter with the same frequency as tinnitus was found in 12 cases by otoscopy, and the waveform with the same frequency as tinnitus was found by long-term tympanogram examination. There were 7 patients with no tympanic membrane activity by otoscopy, the 7 cases also with the same frequency of tinnitus by long-term tympanogram examination, but the change rate of the waveform was faster than that of the patients with tympanic membrane flutter. All patients with tinnitus had no change in hearing. One case of tinnitus complicated with epilepsy (a 6-year-old child) was treated with antiepileptic drug (topiramate) and tinnitus subsided. One case suffered from tinnitus after facial nerve decompression for traumatic facial paralysis was not given special treatment. Fifty-four cases were treated with oral drug (carbamazepine), of which 10 cases were completely controlled and 23 cases were relieved; 21 cases were invalid. Among the 21 patients with no effect of carbamazepine treatment, 8 patients were treated by surgery, 7 patients had no tinnitus after surgery, 1 patient received three times of operation, and the third operation was followed up for 6 months, no tinnitus occurred again. The other 13 cases refused the surgical treatment due to personal reasons. Conclusions: Middle ear myoclonus tinnitus and the inducing factors manifestate diversity. Oral carbamazepine and other sedative drugs are effective for some patients, and surgical treatment is feasible for those who are ineffective for medication.
Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Young Adult ; Ear, Middle/surgery* ; Hearing Tests ; Myoclonus/complications* ; Tinnitus/etiology* ; Tympanic Membrane

Acoustic Impedance Tests ; Ear Diseases/diagnosis* ; Ear, Middle ; Eustachian Tube ; Humans ; Myoclonus ; Otitis Media

Anticonvulsants ; therapeutic use ; Clonazepam ; therapeutic use ; Female ; Humans ; Levetiracetam ; therapeutic use ; MERRF Syndrome ; drug therapy ; Male ; Myoclonus ; drug therapy

Opioids are important drugs for the management of severe cancer pain without a ceiling effect. However, opioid administration leads to dose-limiting complications including drowsiness, hallucinations, delirium, respiratory depression, cognitive impairment, seizure, myoclonus, and hyperalgesia. Opioid-induced hyperalgesia (OIH) is a paradoxical phenomenon as opioid exposure increases pain sensitivity. Reducing or stopping opioids, opioid rotation, or co-administration of N-methyl-D-aspartate (NMDA) antagonists have been suggested for the management of OIH. In this study, we report two clinical cases of successful management of OIH in cancer pain patients that were treated with opioids.
Analgesics, Opioid ; Cognition Disorders ; Delirium ; Hallucinations ; Humans ; Hyperalgesia ; Myoclonus ; N-Methylaspartate ; Pain Management ; Respiratory Insufficiency ; Seizures ; Sleep Stages

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.
Brain ; Dizziness ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Paraneoplastic Syndromes, Nervous System ; Prevalence

BACKGROUND/AIMS: Recent studies have demonstrated that etomidate is a safe sedative drug with noninferior sedative effects. In our recent study, we revealed that etomidate/midazolam was more hemodynamically stable than propofol/midazolam in elderly patients undergoing colonoscopies. We aimed to investigate whether compared with propofol/midazolam, etomidate/midazolam causes fewer cardiopulmonary adverse events with noninferior efficacy for screening colonoscopies in patients of all ages. METHODS: In this single-center, randomized, double-blind study, we prospectively enrolled 200 patients. The patients were divided into etomidate and propofol groups. The primary outcome was the occurrence of cardiopulmonary adverse events. The secondary outcomes were the proportion of patients with fluctuations in vital signs (oxygen desaturation and transient hypotension), adverse events interrupting the procedure, and sedation-related outcomes. RESULTS: Adverse cardiopulmonary events were more common in the propofol group than the etomidate group (65.0% vs 51.0%, respectively; p=0.045). Forty-six patients (46.0%) in the propofol group and 29 (29.0%) in the etomidate group experienced fluctuations in their vital signs (p=0.013). The proportions of patients experiencing adverse events that interrupted the procedure, including myoclonus, were not significantly different between the two groups (etomidate: 20.0% vs propofol: 11.0%; p=0.079). Both groups had similar sedation-related outcomes. Multivariate analysis revealed that compared with the propofol groups, the etomidate group had a significantly lower risk of fluctuations in vital signs (odds ratio, 0.427; 95% confidence interval, 0.230 to 0.792; p=0.007). CONCLUSIONS: Compared with using propofol/midazolam, using etomidate/midazolam for screening colonoscopies results in more stable hemodynamic responses in patients of all ages; therefore, we recommend using etomidate/midazolam for colonoscopies in patients with cardiovascular risk factors.
Aged ; Colonoscopy ; Double-Blind Method ; Etomidate ; Hemodynamics ; Humans ; Hypnotics and Sedatives ; Mass Screening ; Midazolam ; Multivariate Analysis ; Myoclonus ; Propofol ; Prospective Studies ; Risk Factors ; Vital Signs