Complete Resolution of Symptoms of Primary Orthostatic Tremor with Perampanel.
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ABSTRACT:
SUBMITTER: Ruiz-Julian M
PROVIDER: S-EPMC5910539 | biostudies-literature | 2018
REPOSITORIES: biostudies-literature
Publications
Complete Resolution of Symptoms of Primary Orthostatic Tremor with Perampanel.
Ruiz-Julián María M Orozco Jorge Luís JL Gironell Alexandre A
Tremor and other hyperkinetic movements (New York, N.Y.) 20180417
<h4>Background</h4>Primary orthostatic tremor (POT) is an infrequent disorder whose physiopathology is unknown. Current medication is largely ineffective or only offers mild benefits.<h4>Case report</h4>A 75-year-old female with refractory POT treated with 4 mg/day of perampanel achieved complete symptom resolution. Owing to adverse effects, the patient reduced intake to 2 mg/day, but even at this lower dose the benefit was maintained.<h4>Discussion</h4>We report the complete resolution of POT s ...[more]
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Project description:Background:Primary orthostatic tremor (POT) remains a therapeutic conundrum. Various medication classes have been tried, yielding modest results at best. Case Report:A 62-year-old female with a 13-year history of POT, refractory to clonazepam up to 20 mg/day, was treated with perampanel 1-2 mg/day. She reported 90% subjective symptomatic improvement. Discussion:This case highlights the potential for use of perampanel, a novel AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor antagonist for the treatment of POT. There has been one prior report citing its use for POT with complete resolution of symptoms. We encourage further studies to highlight its efficacy for POT.
| S-EPMC6691907 | biostudies-other
Project description:Background:Primary orthostatic tremor (POT) is a rare disorder for which current treatments are largely ineffective. Following up on our recent report of complete resolution of POT symptoms in a patient using low doses of perampanel, we describe our experience of perampanel in 20 patients. Methods:Twenty patients whose neurologists prescribed perampanel were recruited. Initial dose was 2 mg/day, which was increased to 4 mg/day after the first month. Treatment efficacy was self-scored from +3 to -3 at 1 and 3 months. Results:Eight patients withdrew due to adverse effects. Of the 12 patients who completed the study, 92% indicated that their POT symptoms had improved after 1 month, with 75% indicating moderate to marked improvement (mean score 1.9 ± 0.9). This improvement was not sustained by follow-up at 3 months (mean score 0.9 ± 1.3). A rebound of POT symptoms that lasted 2-6 weeks was observed in most patients who withdrew. Discussion:Our experience with this series of cases points to the potential of low-dose perampanel as a treatment for POT, although poor tolerance and the possibility of a non-persistent therapeutic benefit need to be considered. Controlled studies are needed to confirm these findings.
| S-EPMC6800298 | biostudies-literature
Project description:BackgroundOrthostatic tremor (OT) is a rare movement disorder characterized by high-frequency (>12 Hz) involuntary, rhythmic, sinusoidal movements affecting predominantly the limbs while standing.ObjectiveTo describe the signalment, presenting complaints, phenotype, diagnostic findings, treatment, and outcome of a large sample of dogs with OT.AnimalsSixty dogs diagnosed with OT based on conscious electromyography.MethodsMulticenter retrospective case series study. Dogs were included if they had a conscious electromyography consistent with muscle discharge frequency >12 Hz while standing.ResultsFifty-three cases were diagnosed with primary OT (POT). Giant breed dogs represented most cases (83%; 44/53). Most dogs (79%; 42/53) were younger than 2 years of age at onset of signs, except for Retrievers which were all older than 3.5 years of age. The most common presenting complaints were pelvic limb tremors while standing (85%; 45/53) and difficulty when rising or sitting down (45%; 24/53). Improvement of clinical signs occurred in most dogs (85%; 45/53) treated medically with phenobarbital, primidone, gabapentin, pregabalin or clonazepam, but it was mostly partial rather than complete. Orthostatic tremor-plus was seen in 7 dogs that had concurrent neurological diseases.Conclusions and clinical importancePrimary OT is a progressive disease of young, purebred, giant/large-breed dogs, which appears to begin later in life in Retrievers. Primary OT apparently responds partially to medications. Orthostatic tremor-plus exists in dogs and can be concomitant or associated with other neurological diseases.
| S-EPMC8783359 | biostudies-literature
Project description:Background:Orthostatic tremor (OT), a rare and complex movement disorder, is characterized by rapid tremor of both legs and the trunk while standing. These disappear while the patient is either lying down or walking. OT may be idiopathic/primary or it may coexist with several neurological conditions (secondary OT/OT plus). Primary OT remains an enigmatic movement disorder and its pathogenesis and neural correlates are not fully understood. Methods:A PubMed search was conducted in July 2017 to identify articles for this review. Results:Structural and functional neuroimaging studies of OT suggest possible alterations in the cerebello-thalamo-cortical network. As with essential tremor, the presence of a central oscillator has been postulated for OT; however, the location of the oscillator within the tremor network remains elusive. Studies have speculated a possible dopaminergic deficit in the pathogenesis of primary OT; however, the evidence in favor of this concept is not particularly robust. There is also limited evidence favoring the concept that primary OT is a neurodegenerative disorder, as a magnetic resonance spectroscopic imaging study revealed significant reduction in cerebral and cerebellar N-acetyl aspartate (NAA) levels, a marker of neuronal compromise or loss. Discussion:Based on the above, it is clear that the pathogenesis of primary OT still remains unclear. However, the available evidence most strongly favors the existence of a central oscillatory network, and involvement of the cerebellum and its connections.
| S-EPMC5712672 | biostudies-literature
Project description:SEE MUTHURAMAN ET AL DOI101093/AWW164 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Primary orthostatic tremor is characterized by high frequency tremor affecting the legs and trunk during the standing position. Cerebellar defects were suggested in orthostatic tremor without direct evidence. We aimed to characterize the anatomo-functional defects of the cerebellar motor pathways in orthostatic tremor. We used multimodal neuroimaging to compare 17 patients with orthostatic tremor and 17 age- and gender-matched healthy volunteers. Nine of the patients with orthostatic tremor underwent repetitive transcranial stimulation applied over the cerebellum during five consecutive days. We quantified the duration of standing position and tremor severity through electromyographic recordings. Compared to healthy volunteers, grey matter volume in patients with orthostatic tremor was (i) increased in the cerebellar vermis and correlated positively with the duration of the standing position; and (ii) increased in the supplementary motor area and decreased in the lateral cerebellum, which both correlated with the disease duration. Functional connectivity between the lateral cerebellum and the supplementary motor area was abnormally increased in patients with orthostatic tremor, and correlated positively with tremor severity. After repetitive transcranial stimulation, tremor severity and functional connectivity between the lateral cerebellum and the supplementary motor area were reduced. We provide an explanation for orthostatic tremor pathophysiology, and demonstrate the functional relevance of cerebello-thalamo-cortical connections in tremor related to cerebellar defects.
| S-EPMC4958903 | biostudies-literature
Project description:BACKGROUND:We aimed to characterize the clinical and electrophysiological features of patients with slow orthostatic tremor. CASE REPORT:The clinical and neurophysiological data of patients referred for lower limb tremor on standing were reviewed. Patients with symptomatic or primary orthostatic tremor were excluded. Eight patients were identified with idiopathic slow 4-8 Hz orthostatic tremor, which was associated with tremor and dystonia in cervical and upper limb musculature. Coherence analysis in two patients showed findings different to those seen in primary orthostatic tremor. DISCUSSION:Slow orthostatic tremor may be associated with dystonia and dystonic tremor.
| S-EPMC4749092 | biostudies-literature
Project description:BackgroundPrimary orthostatic tremor (OT) is a rare movement disorder characterized by a 13-18 Hz leg tremor, which arises when standing and is relieved by walking/sitting. Those affected generally do not fall, but experience fear of falling, lessened by ambulation. Because of its low amplitude, the tremor is not readily visible, and diagnosis requires confirmation with surface electromyography (sEMG). Recently, applications using the accelerometer feature of smartphones have been used to detect and quantify tremors, including OT, though the accuracy of smartphone accelerometry (SPA) in diagnosing OT is unknown.MethodsWe completed SPA in consecutive adults (18+ years), who presented to our neurology clinic with either subjective leg shakiness upon standing or unsteadiness when standing that lessened with ambulation, which comprised 59 of 2578 patients. We assessed tremor using the StudyMyTremor application on an iPhone 6 s adhered with tape to the patient's tibialis anterior. Surface electromyography was completed on the same muscle. The primary outcome of this study was to determine SPA's sensitivity and specificity in detecting OT compared with surface electromyography.ResultsFifty-nine patients with the following diagnoses were included: OT (6), Parkinson's disease, Hereditary Spastic Paraplegia, orthostatic hypotension, essential tremor, spinal cerebellar ataxia, sensory ataxia and functional movement disorder. Smartphone accelerometry detected a 13-18 Hz tremor in 5 of 6 patients diagnosed with OT by sEMG with no false positives in other conditions, yielding a sensitivity of 83%, specificity of 100% in the cohort we studied.ConclusionsThough a larger sample size is desirable, preliminary data suggest that smartphone accelerometry is an alternative to surface electromyography in diagnosing OT.
| S-EPMC8607557 | biostudies-literature
Project description:Background:Orthostatic tremor (OT) is a weight-bearing hyperkinetic disorder characterized by unsteadiness while standing that is relieved when sitting or walking. Case report:A 66-year-old male presented with a 5 year-history of tremor in his abdomen, but only when he stood in a stationary position. The tremor disappeared when he stood or walked. On examination, he had palpable tremor in his rectus abdominis and gastrocnemius virtually instantaneously after standing. His electromyography findings confirmed the presence of a 12-Hz tremor in the tibialis anterior while standing, with subharmonics recorded in the external obliques and rectus abdominis. Discussion:Our case illustrates an unusual presentation of OT. The diagnosis is supported by its characteristic frequency and specific appearance only during upright stance.
| S-EPMC6315046 | biostudies-other
Project description:Background:Orthostatic tremor (OT) is defined as tremor in the legs and trunk evoked during standing. While the classical description is tremor of ?13 Hz, slower frequencies are recognized. There is disagreement as to whether the latter represents a slow variant of classical OT, or different tremor disorder(s) given frequent coexistent neurological disease. Methods:A systematic literature search of PubMed was performed in February 2019 for "slow orthostatic tremor" and related terms which generated 573 abstracts, of which 61 were included. Results:Between 1970 and 2019, there were 70 cases of electrophysiologically confirmed slow OT. Two-thirds were female, of mean age 60 years (range 26-86), and mean disease duration 6 years (range 0-32). One-third of cases were isolated, and two-thirds had a coexistent disorder(s), including parkinsonism (30%), ataxia (12%), and dystonia (10%). Postural arm tremor was present in 34%. Median tremor frequency was 6-7 Hz (range 3-12). Tremor bursts ranged from 50 to 150 ms duration, and were alternating or synchronous in antagonistic and/or analogous muscles. Low and high coherences were reported. Five cases (7%) had coexistent classical OT. Clonazepam was the most effective medication across all frequencies, and levodopa was effective for 4-7 Hz OT with coexistent parkinsonism. Two cases resolved with the treatment of Graves' disease. Electrophysiology and imaging predominantly support a central tremor generator. Discussion:While multiple lines of evidence separate slow OT from classical OT, clinical and electrophysiological overlap may occur. Primary and secondary causes are identified, similar to classical OT. Further exploration to clarify these slow OT subtypes, clinically and neurophysiologically, is proposed.
| S-EPMC6886496 | biostudies-literature
Project description:BackgroundPerampanel is a noncompetitive antagonist of alpha-amino-3-hydroxy-5-methylisoxazole propionic acid glutamate receptors suggested to modulate tremor.ObjectivesTo assess the efficacy and tolerability of perampanel for essential tremor.MethodsThis was a double-blind, placebo-controlled, randomized, cross-over trial involving 26 patients titrated to 8 mg/day or a lower maximally tolerated dose as monotherapy or adjunct to antitremor medication. Tremor was assessed at the beginning and end of each 14-week treatment arm. The primary endpoint was change in the videotaped performance subscale of The Essential Tremor Rating Assessment Scale, scored by a blinded rater. Secondary endpoints included change in The Essential Tremor Rating Assessment Scale Activity of Daily Living and Quality of Life in Essential Tremor and Subject Global Impression of Change subscales.ResultsData are available for 15 and 11 participants who completed placebo and perampanel arms, respectively. Perampanel was superior to placebo on the primary endpoint (P = 0.028), Activity of Daily Living (P = 0.009), and Subject Global Impression of Change (P = 0.016), but not Quality of Life (p = 0.48). Video scores were rated >50% improved in 3/11 on perampanel and 0/15 on placebo. Adverse events were more likely on perampanel (especially at >4 mg/day) than on placebo, leading to withdrawal (36% vs. 10%) and dose reduction (41% vs. 15%). Adverse events more common with perampanel included imbalance/falls (50% vs. 10%), dizziness (36% vs. 10%), and irritability (27% vs. 5%).ConclusionsThese findings suggest that perampanel exerts efficacy for some persons with essential tremor, but this population appears prone to adverse events.
| S-EPMC7197304 | biostudies-literature