Prominent Jaw Closure Dystonia, Awake Bruxism, and Stereotypies in a Patient with Hypoglycemic Encephalopathy
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ABSTRACT:
SUBMITTER: Vijayaraghavan A
PROVIDER: S-EPMC9540944 | biostudies-literature | 2022 Jan
REPOSITORIES: biostudies-literature
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Project description: Not available
| S-EPMC6159909 | biostudies-literature
Project description:BackgroundStudies have shown co-contraction of jaw and neck muscles in healthy subjects during (sub) maximum voluntary jaw clenching, indicating functional inter-relation between these muscles during awake bruxism. So far, coherence of jaw and neck muscles has not been evaluated during either awake or sleep bruxism.ObjectiveThe objective of this study was to evaluate the coherence between jaw and neck muscle activity during sleep bruxism.MethodsIn a cross-sectional observational design, the electromyographic activity of jaw (masseter, temporalis) and neck (sternocleidomastoid, trapezius) muscles in individuals with "definite" sleep bruxism was measured using ambulatory polysomnography (PSG). Coherence for masseter-temporalis, masseter-sternocleidomastoid and masseter-trapezius was measured during phasic and mixed rhythmic masticatory muscle activity episodes using coherence-analysing software. Outcome measures were as follows: presence or absence of significant coherence per episode (in percentages), frequency of peak coherence (FPC) per episode and sleep stage.ResultsA total of 632 episodes within 16 PSGs of eight individuals were analysed. Significant coherence was found between the jaw and neck muscles in 84.9% of the episodes. FPCs of masseter-temporalis were significantly positively correlated with those of masseter-sternocleidomastoid or masseter-trapezius (P < .001). Sleep stages did not significantly influence coherence of these muscular couples.ConclusionDuring sleep bruxism, jaw and neck muscle activation is significantly coherent. Coherence occurs independently of sleep stage. These results support the hypothesis of bruxism being a centrally regulated phenomenon.
| S-EPMC7079051 | biostudies-literature
Project description:The future of awake bruxism assessment will incorporate physiological data, possibly electromyography (EMG) of the temporal muscles. But up to now, temporal muscle contraction patterns in awake bruxism have not been characterized to demonstrate clinical utility. The present study aimed to perform surface EMG evaluations of people assessed for awake bruxism to identify possible different subtypes. A 2-year active search for people with awake bruxism in three regions of the country resulted in a total of 303 participants (223 women, 38 ± 13 years, mean and SD). Their inclusion was confirmed through non-instrumental approaches for awake bruxism: self-reported questionnaire and clinical exam, performed by three experienced and calibrated dentists (Kappa = 0.75). Also, 77 age- and sex-matched healthy controls were recruited (49 women, 36 ± 14 years). Temporalis surface EMG was performed with a portable device (Myobox; NeuroUp, Brazil). EMG signals were sent to a computer via Bluetooth 4.0 at a sampling rate of 1,000 Hz. Digital signal processing was performed using the commercial neuroUP software, transformed in RMS and then normalized for peak detection (EMG peaks/min), in a 10 min session. Cluster analysis revealed three distinct subtypes of awake bruxism: phasic, tonic, and intermediate. Individuals with a predominance of EMG peaks/min were classified as the "phasic" subtype (16.8%). Those with the highest EMG rest power were classified as the "tonic" subtype (32.3%). There was also an "intermediate" subtype (50.8%), when both variables remained low. Characterization of awake bruxism physiology is important for future establishment of instrumental assessment protocols and treatment strategies.
| S-EPMC7876308 | biostudies-literature
Project description:BACKGROUND: Bruxism is a disorder of jaw-muscle activity characterised by repetitive clenching or grinding of the teeth which results in discomfort and damage to dentition. The two clinical manifestations of the condition (sleep and awake bruxism) are thought to have unrelated aetiologies but are palliated using similar techniques. The lack of a definitive treatment has prompted renewed interest in biofeedback, a behaviour change method that uses electronic detection to provide a stimulus whenever bruxism occurs. This systematic review aims to provide a comprehensive overview of the state of research into biofeedback for bruxism; to assess the efficacy and acceptability of biofeedback therapy in management of awake bruxism and, separately, sleep bruxism in adults; and to compare findings between the two variants. METHODS: A systematic review of published literature examining biofeedback as an intervention directed at controlling primary bruxism in adults. We will search electronic databases and the grey literature using a predefined search strategy to identify randomised and non-randomised studies, technical reports and patents. Searches will not be restricted by language or date and will be expanded through contact with authors and experts, and by following up reference lists and citations. Two authors, working independently, will conduct screening of search results, study selection, data extraction and quality assessment and a third will resolve any disagreements. The primary outcomes of acceptability and effectiveness will be assessed using only randomised studies, segregated by bruxism subtype. A meta-analysis of these data will be conducted only if pre-defined conditions for quality and heterogeneity are met, otherwise the data will be summarized in narrative form. Data from non-randomised studies will be used to augment a narrative synthesis of the state of technical developments and any safety-related issues. PROSPERO registration number: CRD42013006880. DISCUSSION: Biofeedback is not new, but its place in the clinical management of bruxism remains unclear. New research, and the availability of miniaturized consumer-grade devices, makes a systematic review timely to guide treatment decisions and inform future research.
| S-EPMC4028105 | biostudies-literature
Project description:BackgroundBruxism is a repetitive masticatory muscle activity characterized by clenching or grinding of the teeth, or by bracing or thrusting of the mandible, or both.ObjectivesTo investigate whether bruxism in awake dogs could be associated with brain lesions.AnimalsFour dogs with episodic bruxism in the awake state.MethodsObservational retrospective single-center case series. Inclusion criteria were dogs examined between 2010 and 2021 with episodic bruxism as a presenting complaint or observed during the examination or hospitalization, complete medical records and magnetic resonance imaging or computed tomography of the brain. Bruxism during epileptic seizures as oroalimentary automatism was an exclusion criterion.ResultsFour dogs met the inclusion criteria. Two dogs had bruxism while awake as a presenting complaint, whereas in the remaining 2 it was a clinical finding. All dogs had neuroanatomical localization consistent with a forebrain lesion, with diencephalic involvement in 3/4. The diagnostic evaluation was consistent with neoplasia (n = 2) and meningoencephalitis of unknown origin (n = 2), in 1 case accompanied by corpus callosum abnormality affecting the forebrain, in 3 dogs advanced imaging findings were suggestive of increased intracranial pressure. All dogs were euthanized.Conclusions and clinical importanceOur results suggest that the presence of bruxism in the awake state associated with other neurological deficits might indicate a forebrain lesion.
| S-EPMC9708440 | biostudies-literature
Project description:We report on a large family with myoclonus-dystonia resulting from an epsilon-sarcoglycan mutation, with prominent early and late lower-limb involvement. The proband's condition has evolved to include marked lower-limb dystonia and dystonic gait impairment in the fourth decade. Other family members had evidence of prominent lower-limb involvement at presentation or a more typical phenotype of axial and upper-limb myoclonus and dystonia. Prominent lower-limb involvement developing late in the disease course is an atypical feature and exemplifies the wide phenotypic heterogeneity observed in people with myoclonus-dystonia.
| S-EPMC6183303 | biostudies-literature
Project description: Not available
| S-EPMC6183243 | biostudies-literature
Project description:BackgroundChronic stress is one of the leading predisposing factors in bruxism aetiology, but the influence of genetic factors is also suggested. We aimed to study whether sequence variants in genes involved in stress regulation pathways: NTRK2 and BDNF, may be associated with awake bruxism susceptibility, clinical presentation, and patients' perceived stress level.MethodsThe study group included 104 patients with probable awake bruxism and 191 population controls. Patients underwent dental examination concerning the symptoms of bruxism and psychological testing. Genotyping was performed using HRMA and sequencing. Statistical analyses were conducted, and P values below 0.05 were considered statistically significant.ResultsWe observed a positive correlation of measured stress level and pathological teeth attrition in the anterior segment (r = 0.45, P < 0.001), enamel attritions (r = 0.44, P < 0.001), tongue impressions (r = 0.50, P < 0.001) and posterior teeth attrition (r = 0.27, P = 0.005). Moreover, the c.196A variant (p.66Met) of the BDNF gene and c.1397-31392G allele of the NTRK2 gene were present with elevated frequency, comparing to controls.ConclusionsThis study hence the thesis that perceived stress level is a substantial contributing factor to awake bruxism occurrence and its clinical manifestations. Moreover, sequence variants in genes related to stress coping may be correlated with awake bruxism's susceptibility via elevated perceived stress level.
| S-EPMC8491599 | biostudies-literature
Project description:De novo mutations of the sodium channel gene SCN8A result in an epileptic encephalopathy with refractory seizures, developmental delay, and elevated risk of sudden death. p.Arg1872Trp is a recurrent de novo SCN8A mutation reported in 14 unrelated individuals with epileptic encephalopathy that included seizure onset in the prenatal or infantile period and severe verbal and ambulatory comorbidities. The major biophysical effect of the mutation was previously shown to be impaired channel inactivation accompanied by increased current density. We have generated a conditional mouse mutation in which expression of this severe gain-of-function mutation is dependent upon Cre recombinase. Global activation of p.Arg1872Trp by EIIa-Cre resulted in convulsive seizures and lethality at 2 weeks of age. Neural activation of the p.Arg1872Trp mutation by Nestin-Cre also resulted in early onset seizures and death. Restriction of p.Arg1872Trp expression to excitatory neurons using Emx1-Cre recapitulated seizures and juvenile lethality between 1 and 2 months of age. In contrast, activation of p.Arg1872Trp in inhibitory neurons by Gad2-Cre or Dlx5/6-Cre did not induce seizures or overt neurological dysfunction. The sodium channel modulator GS967/Prax330 prolonged survival of mice with global expression of R1872W and also modulated the activity of the mutant channel in transfected cells. Activation of the p.Arg1872Trp mutation in adult mice was sufficient to generate seizures and death, indicating that successful therapy will require lifelong treatment. These findings provide insight into the pathogenic mechanism of this gain-of-function mutation of SCN8A and identify excitatory neurons as critical targets for therapeutic intervention.
| S-EPMC6351781 | biostudies-literature
Project description: Not available
| S-EPMC6476598 | biostudies-literature