Jaw Tremor Research Articles

Cranial Tremors in Essential Tremor: Prevalence, Anatomic Distribution and Predictors of Occurrence.

There are few published data on the prevalence, pattern of anatomic distribution and predictors of various cranial tremors (eg, head, voice, jaw) in essential tremor (ET). Given the high levels of diagnostic misclassification in ET, data on observed clinical patterns would be valuable. To assess the prevalence, pattern of anatomic distribution and predictors of occurrence of cranial tremors in ET. ET cases who enrolled in two studies (respective n's = 301 and 175, total n = 476) underwent a detailed clinical assessment, which included a videotaped neurological examination. A senior movement disorders neurologist reviewed the examination and coded a range of cranial tremors as present or absent. The proportion of cases with any cranial tremor was 57.1% (Study 1) and 73.7% (Study 2). The following patterns emerged: (1) Head only and head + voice were the most commonly observed anatomical distribution of tremor. (2) Jaw tremors were rare, and face and tongue tremors, exceedingly rare. (3) There was a general layering-on of tremor from head to head + voice to head + voice + jaw in more advanced cases. (4) Women were more likely to have head tremors than men, although there was no sex predilection tremor in other locations. (5) The likelihood of cranial tremor increased with greater tremor severity and age but was unrelated to tremor duration. We observed and quantified several patterns in the expression of cranial tremors in ET. Data on such patterns may assist clinicians and researchers in assigning diagnoses.

DaT Scan Positive Parkinson’s Disease in a Patient with Anti-MuSK Positive Myasthenia Gravis (P5-11.007)

<h3>Objective:</h3> We present a unique clinical case of antibody positive Muscle Specific Kinase (MuSK) Myasthenia Gravis and DaT scan positive Parkinson’s Disease to highlight the overlapping nature of clinical symptoms and challenges seen in clinical diagnosis. <h3>Background:</h3> The concomitant diagnosis of Myasthenia Gravis (MG), an autoimmune condition that affects the neuromuscular junction, and Parkinson’s disease (PD), a neurodegenerative disease that affects the basal ganglia, is remarkably exceptional. Furthermore, there are very few case reports in the literature that have recognized muscle-specific kinase (MuSK), a subtype of MG with overlapping features of PD. We present a unique case of MuSK MG and DaT scan positive Parkinson’s Disease, which to our knowledge has not been previously reported. <h3>Design/Methods:</h3> NA <h3>Results:</h3> 49-year-old male initially presented to neurology clinic with six months of waxing and waning nasal quality dysarthria. After thorough workup with serologic and electrophysiologic studies, patient was diagnosed with seropositive bulbar predominant MuSK Myasthenia Gravis for which steroid sparing immunosuppressive treatment was initiated with mycophenolate mofetil. He subsequently developed clinical exam findings of hypomimia, bilateral bradykinesia, asymmetrical limb rigidity, right arm and jaw tremor that raised concern for the possibility of parkinsonism. Review of systems for premotor symptoms was positive for constipation and dream enactment behavior. There was no previous exposure to anti-dopaminergic therapies and MRI Brain was unrevealing. DaT scan was subsequently obtained, which demonstrated objective evidence of bilateral striatal dopaminergic deficiency, indicative of an inherent basal ganglia disorder. In the absence of any clinical findings of atypical parkinsonism, patient met clinical diagnostic criteria for Idiopathic Parkinson’s Disease. <h3>Conclusions:</h3> This case demonstrates the clinical challenges that present in this overlap of neuromuscular and neurodegenerative disease in a single individual. We highlight the importance of a thorough history and physical examination, supported with objective evidence by a positive DaT scan, to elucidate the appropriate etiology of symptoms. <b>Disclosure:</b> Dr. Andraos has nothing to disclose. Dr. Veerappan has nothing to disclose.

Isolated Jaw and Tongue Tremor in a Definite Case of Progressive Supranuclear Palsy.

Movement Disorders Clinical PracticeVolume 10, Issue 2 p. 348-349 LETTERS: PUBLISHED ARTICLES Isolated Jaw and Tongue Tremor in a Definite Case of Progressive Supranuclear Palsy Nahid Olfati MD, Nahid Olfati MD orcid.org/0000-0003-4621-2490 Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorDennis W. Dickson MD, Dennis W. Dickson MD Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USASearch for more papers by this authorAli Shoeibi MD, Ali Shoeibi MD Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranSearch for more papers by this authorDietrich Haubenberger MD, Dietrich Haubenberger MD UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorIrene Litvan MD, Corresponding Author Irene Litvan MD [email protected] UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USA Correspondence to: Dr. Irene Litvan, UC San Diego Department of Neurosciences, Altman Clinical Translational Research Institute, Parkinson & Other Movement Disorders Center, 9452 Medical Center Drive, 2W114, La Jolla, California, USA; E-mail: [email protected]Search for more papers by this author Nahid Olfati MD, Nahid Olfati MD orcid.org/0000-0003-4621-2490 Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorDennis W. Dickson MD, Dennis W. Dickson MD Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USASearch for more papers by this authorAli Shoeibi MD, Ali Shoeibi MD Department of Neurology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranSearch for more papers by this authorDietrich Haubenberger MD, Dietrich Haubenberger MD UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USASearch for more papers by this authorIrene Litvan MD, Corresponding Author Irene Litvan MD [email protected] UC San Diego Department of Neurosciences, Parkinson and Other Movement Disorder Center, La Jolla, California, USA Correspondence to: Dr. Irene Litvan, UC San Diego Department of Neurosciences, Altman Clinical Translational Research Institute, Parkinson & Other Movement Disorders Center, 9452 Medical Center Drive, 2W114, La Jolla, California, USA; E-mail: [email protected]Search for more papers by this author First published: 11 December 2022 https://doi.org/10.1002/mdc3.13640Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume10, Issue2February 2023Pages 348-349 RelatedInformation

Clinicopathologic Correlations of Jaw Tremor in a Longitudinal Aging Study.

The aim of this study is to assess clinical and pathologic correlations of jaw tremor in 3 cohorts enrolled in a long-term aging study. Jaw/lip tremor has been described in various movement disorders but the impact of seeing a jaw tremor on clinician diagnosis and whether the presence of isolated jaw tremor is correlated with subsequent phenoconversion to a different movement disorder are unclear. Data from the Arizona Study of Aging and Neurodegenerative Disease, a longitudinal clinicopathologic study, were used. Control subjects (n = 708) did not have any tremor or parkinsonism. At initial evaluation, 276 subjects who had jaw tremor were categorized as isolated jaw tremor (jaw tremor without limb action tremor or parkinsonism), suspect/possible PD (1 or 2 cardinal features of PD without a history of dopaminergic treatment), parkinsonism (probable PD and other parkinsonian disorders), or nonparkinsonian tremor (e.g., essential tremor). Initial clinical diagnosis was compared with "final diagnosis" based on longitudinal assessments and with clinicopathologic diagnosis when available. In subjects with jaw tremor, we identified 45 isolated jaw tremor, 92 nonparkinsonian tremor, 56 suspect/possible PD, and 83 parkinsonism cases at baseline and followed longitudinally. Neuropathologic diagnosis was available for 137 cases. The mean time from initial to final assessment or autopsy was 6.8 years (SD 4.4). Of the subjects with follow-up data, only 15.4% of those with isolated jaw tremor (6/39) and 8.8% of those with nonparkinsonian tremor (6/68) evolved into a clinical parkinsonian disorder. Neither of these groups was associated with clinicopathologic PD: isolated jaw tremor (1/18) and nonparkinsonian tremor (1/43). Those with jaw tremor initially classified into a parkinsonian group were more highly associated with clinicopathologic PD: 27 of 51 subjects with parkinsonism other and 4 of 25 possible PD. The presence of either jaw tremor in isolation or associated with nonparkinsonian tremor does not portend a neurodegenerative diagnosis.

Functional correlation between cerebellum and basal ganglia: A parkinsonism model

IntroductionThe cerebellar response has been studied for years with different models of alteration of other brain structures to understand its complex functioning and its relationship with the rest of the body. Studies in patients with Parkinson's disease (PD) showed that the cerebellar function is modified by deficit of the basal ganglia; which supports the hypothesis that both structures are related anatomically and functionally. MethodsIn our study, the ventrolateral striatum (VLS) of the basal ganglia was altered by an electrolytic lesion, in order to produce a similar jaw frequency of jaw tremor movements presented in parkinsonism, thereafter we analyzed the effect of the lesion on the expression of multiunit activity (MUA) of the cerebellum. ResultsWe found cerebellar activation during mandibular movements and increment during oral jaw tremor movements. In addition, the amplitude of baseline MUA registered in animals with alteration of the VLS decreased with respect to the intact group. ConclusionsAccordingly, we conclude that cerebellar changes in MUA may be due to a decrease in the cerebellar inflectional or as a possible compensatory function between cerebellum and basal ganglia.

Teaching Video NeuroImage: Tongue Tremor as a Presenting Feature of Progressive Supranuclear Palsy.

A 76-year-old man was referred to neurology by speech and language therapy after video fluoroscopy showed oropharyngeal dysphagia, aspiration, and tongue tremor (Video 1). He had presented with 6 months of progressive dysphagia, dysarthria, weight loss, and falls. Examination revealed akinetic rigidity, vertical supranuclear gaze palsy, slowed saccades, dysarthrophonia, and tongue tremor without palatal, jaw, or limb tremor. We diagnosed probable progressive supranuclear palsy (PSP). He did not respond to levodopa (400 mg/d). On a follow-up assessment 27 months later, the tremor persisted (Video 1). Tremor, usually upper limb, occurs in 42% of PSP cases.1 Isolated tongue tremor is a rare feature of PSP.1,2

Benchmark Device To Mitigate Jaw Tremor Using Gyroscopic Effect

A tremor is a mildly rhythmic muscle contraction and relaxation that predominantly involves involuntary oscillations or twitching movements of one’s body parts. Jaw tremor looks more like shivering although with different amplitude and frequency which can vary. Jaw tremors hinder patients’ day to day activities and hamper confidence, causing social handicaps. There are only drug-based solutions for jaw tremors to date. Currently, there are some surgical treatments like Deep Brain Stimulation (DBS), but, they have side effects. Our solution - the palliator a wearable, ergonomic device that dampens jaw tremors without producing any side effects on patients. The proposed solution uses two accelerometers to detect and distinguish involuntary tremors from the voluntary jaw motion and then uses the gyroscopic principle to control the involuntary motion, thus resisting the tremors.

Facial tremor in patients with Parkinson\u2019s disease: prevalence, determinants and impacts on disease progression

BackgroundFacial (lip and jaw) tremor (FT) is associated with Parkinson’s disease (PD) but few studies have been conducted to explore its clinical profile. We performed this study to investigate the prevalence and clinical correlates of FT in PD, and further to evaluate its effect on disease progression.MethodsA retrospective, cross-sectional (n = 2224) and longitudinal (n = 674) study was conducted. The presence of FT was based on a ≥ 1 score in the United PD Rating Scale (UPDRS) item 20A. Group comparisons were conducted, followed by a forward binary logistic regression analysis. Inverse probability of treatment weighting (IPTW) based on the propensity score and weighted or unweighted Cox regression models were used to explore the impact of FT on five clinical milestones including death, UPDRS III 11-point increase, Hoehn and Yahr (H&Y) stage reaching 3, dyskinesia development, and Montreal Cognitive Assessment 3-point decrease.ResultsFT was presented in 403 patients (18.1%), which showed increasing trends with disease duration and H&Y score. Age (P < 0.001), female (P < 0.001), disease duration (P = 0.001), speech (P = 0.011), rigidity (P = 0.026), rest tremor on limbs (P < 0.001), kinetic tremor on hands (P < 0.001), and axial symptoms (P = 0.013) were independent factors associated with FT. Both unweighted and weighted Cox regression models indicated that baseline FT and FT as the initial symptom were not associated with the five outcomes.ConclusionsOur study suggested that FT was not uncommon and provided a deeper insight into the characteristics of FT in PD. The predict value of FT on long-term progronis of PD may need future longer follwe-up study.

Orthostatic Hypotension in Elderly Patients with Essential Tremor

PurposeEssential tremor (ET) is the most common movement disorder in which dysautonomia symptoms can be present. We aimed to evaluate the presence of orthostatic hypotension (OH) and its relationship with the clinical features.Patients and MethodsForty-four elderly patients with ET and 118 healthy elderly controls were included. OH was assessed via the head-up tilt table test and defined, according to the change in position, as a drop of at least 20 mmHg in systolic blood pressure and/or 10 mmHg in diastolic blood pressure. Tremor severity was evaluated using the Fahn-Tolosa-Marin Tremor Rating (FTMTR) Scale. All patients underwent geriatric comprehensive assessment.ResultsThere were no differences between the controls and patients with ET regarding age and gender. The mean age was 72.8±6.1, the mean disease duration 19.1±13.5 years and the mean FTMTR score was 30.9±17.1 in patients with ET. The frequency of OH at the 1st minute in patients with ET was higher than in controls (31.8% vs 17.8%, p=0.046). Furthermore, the frequency of jaw tremor in patients with OH was higher than in those without OH (35.7% vs 6.7%, p=0.025). About 28.6% of ET patients with OH had orthostatic symptoms.ConclusionWe demonstrated that ET patients, particularly those with jaw tremors, had OH and that most of them were asymptomatic. Therefore, in order to protect patients from complications related to OH, it would be appropriate to evaluate OH in the follow-up and treatment of elderly patients with ET.

A Subset of Purposeless Oral Movements Triggered by Dopaminergic Agonists Is Modulated by 5-HT2C Receptors in Rats: Implication of the Subthalamic Nucleus

Dopaminergic medication for Parkinson’s disease is associated with troubling dystonia and dyskinesia and, in rodents, dopaminergic agonists likewise induce a variety of orofacial motor responses, certain of which are mimicked by serotonin2C (5-HT2C) receptor agonists. However, the neural substrates underlying these communalities and their interrelationship remain unclear. In Sprague-Dawley rats, the dopaminergic agonist, apomorphine (0.03–0.3 mg/kg) and the preferential D2/3 receptor agonist quinpirole (0.2–0.5 mg/kg), induced purposeless oral movements (chewing, jaw tremor, tongue darting). The 5-HT2C receptor antagonist 5-methyl-1-[[2-[(2-methyl-3-pyridyl)oxyl]-5-pyridyl]carbamoyl]-6-trifluoromethylindone (SB 243213) (1 mg/kg) reduced the oral responses elicited by specific doses of both agonists (0.1 mg/kg apomorphine; 0.5 mg/kg quinpirole). After having confirmed that the oral bouts induced by quinpirole 0.5 mg/kg were blocked by another 5-HT2C antagonist (6-chloro-5-methyl-1-[6-(2-methylpiridin-3-yloxy)pyridine-3-yl carbamoyl] indoline (SB 242084), 1 mg/kg), we mapped the changes in neuronal activity in numerous sub-territories of the basal ganglia using c-Fos expression. We found a marked increase of c-Fos expression in the subthalamic nucleus (STN) in combining quinpirole (0.5 mg/kg) with either SB 243213 or SB 242084. In a parallel set of electrophysiological experiments, the same combination of SB 243213/quinpirole produced an irregular pattern of discharge and an increase in the firing rate of STN neurons. Finally, it was shown that upon the electrical stimulation of the anterior cingulate cortex, quinpirole (0.5 mg/kg) increased the response of substantia nigra pars reticulata neurons corresponding to activation of the “hyperdirect” (cortico-subthalamonigral) pathway. This effect of quinpirole was abolished by the two 5-HT2C antagonists. Collectively, these results suggest that induction of orofacial motor responses by D2/3 receptor stimulation involves 5-HT2C receptor-mediated activation of the STN by recruitment of the hyperdirect (cortico-subthalamonigral) pathway.

Antipsychotic Behavioral Phenotypes in the Mouse Collaborative Cross Recombinant Inbred Inter-Crosses (RIX).

Schizophrenia is an idiopathic disorder that affects approximately 1% of the human population, and presents with persistent delusions, hallucinations, and disorganized behaviors. Antipsychotics are the standard pharmacological treatment for schizophrenia, but are frequently discontinued by patients due to inefficacy and/or side effects. Chronic treatment with the typical antipsychotic haloperidol causes tardive dyskinesia (TD), which manifests as involuntary and often irreversible orofacial movements in around 30% of patients. Mice treated with haloperidol develop many of the features of TD, including jaw tremors, tongue protrusions, and vacuous chewing movements (VCMs). In this study, we used genetically diverse Collaborative Cross (CC) recombinant inbred inter-cross (RIX) mice to elucidate the genetic basis of antipsychotic-induced adverse drug reactions (ADRs). We performed a battery of behavioral tests in 840 mice from 73 RIX lines (derived from 62 CC strains) treated with haloperidol or placebo in order to monitor the development of ADRs. We used linear mixed models to test for strain and treatment effects. We observed highly significant strain effects for almost all behavioral measurements investigated (P < 0.001). Further, we observed strong strain-by-treatment interactions for most phenotypes, particularly for changes in distance traveled, vertical activity, and extrapyramidal symptoms (EPS). Estimates of overall heritability ranged from 0.21 (change in body weight) to 0.4 (VCMs and change in distance traveled) while the portion attributable to the interactions of treatment and strain ranged from 0.01 (for change in body weight) to 0.15 (for change in EPS). Interestingly, close to 30% of RIX mice exhibited VCMs, a sensitivity to haloperidol exposure, approximately similar to the rate of TD in humans chronically exposed to haloperidol. Understanding the genetic basis for the susceptibility to antipsychotic ADRs may be possible in mouse, and extrapolation to humans could lead to safer therapeutic approaches for schizophrenia.

The Clinical Role of Jaw Tremor in Movement Disorders (2143)

The Clinical Role of Jaw Tremor in Movement Disorders (2143)

DOUBLE JEOPARDY: UNTANGLING DEPRESSION, PARKINSON'S DISEASE & FRONTOTEMPORAL DEMENTIA IN CLINICAL PRACTICE WITH BEDSIDE COGNITIVE TESTING

Introduction Progressive supranuclear palsy is a taupathy disorder often mistaken on diagnosis by other neurocognitive disorders such as Parkinson's (PD) and Alzheimer's (AD) disease amongst other. PSP tends to be diagnosed in the later course of the disease and by time of diagnosis, individuals have a median survival of 7?years. PSP is a disease in which a diagnosis and treatment is done in a multidisiplinary approach involving collaboration of Psychiatry and Neurology. As it was seen in this clinical case which describes a patient with undetected progressive supranuclear palsy who were referred to a university hospital psychiatric emergency services facility for treatment of late onset symptoms and signs attributed to primary psychopathology related to behavioral variant of frontotemporal lobar degeneration (FTLDbv). Methods The patient described in the clinical vignette underwent a comprehensive neuropsychiatric assessment, given that the accuracy of the diagnosis of FTLDbv was in doubt. The clinical interview identified no significant prior developmental, medical, neurological, psychiatric, or substance use disorders; no family history of neuropsychiatric disturbance; and no significant psychosocial stressor before the onset of his neuropsychiatric condition at age 59. Bedside cognitive testing showed a Geriatric Depression Scale (GDS) score suggestive of mild symptoms of depression, and would be unlikely to explain his new onset psychosis. His subclinical Executive Interview 25 (EXIT 25) score and normal Mini Mental State Examination (MMSE) were not indicative of a dementia syndrome. Given the strong temporal relationship between the onset of his behavioral changes and perceptual disturbances and the start of levodopa/carbidopa, this medication was discontinued. He was admitted to the inpatient psychiatric service for observation. He was started initially on an atypical antipsychotic, but had this medication discontinued, as he no longer exhibited symptoms of psychosis. His citalopram was titrated to 40mg daily to optimize treatment of his major depressive disorder. Patient was discharge with complete resolution of psychotic symptoms and with diagnosis of Depressive disorder, unspecified and medication-induced psychotic disorder with delusions and hallucinations. A dopamine transporter (DaT) protein scan, a marker for presynaptic terminals in dopaminergic nigrostriatal neuros (marker for PD) and or iodine-123-metaiodobenzylguanadine (MIBG) myocardial scintigraphy (marker for DLB), was not performed. Results CASE REPORT: A 59-year-old, left handed, previously healthy gentleman was brought by police to a university hospital psychiatry emergency services for evaluation of acute safety concerns, regarding behavioral and personality changes. Patient was admitted to the hospital displaying motor symptoms with appearance of jaw tremor, ataxia, and urinary incontinence. After a negative brain MRI and head CT scan, the neurology team concluded that he had Parkinson`s disease and patient was discharged on levodopa/carbidopa (25?mg/?100 mg) QID and citalopram 20?mg daily for associated depressive symptoms with no benefit. After discharge from the hospital, he had become increasingly impulsive, hypersexual and engaging in risky behaviors with firearms. Persistent and progressive change in personality and behavior were the earliest displayed signs and they remained the most prominent features of his condition. All of these disturbances worsened after patient was started on levodopa/carbidopa. After evaluation by the psychiatry consultation and liaison service, a Mental status examination revealed cognitive and neurovegetative symptoms of depression as well as auditory illusions and concerns that a stranger had invaded his home. He denied manic symptoms. No suicidal and homicidal thoughts. Despite extensive neurological and neuropsychological evaluation 10-months later, surprisingly, it was established the diagnosis of progressive supranuclear palsy (PSP), as at that visit the patient presented with predominantly delay axial Parkinsonism, supranuclear gaze palsy, and increased falls due to postural instability. A neuropsychological assessment provided evidence of an executive syndrome of subcortical frontal type. Conclusions Progressive supranuclear palsy is a debilitating taupathy disorder that often is mistaken on diagnosis by other neurocognitive disorders such as Parkinson's dementia, Alzheimer's dementia and Frontotemporal lobar degeneration amongst others. Thus, making PSP diagnosis difficult at initial presentation and often presenting later in life. PSP diagnosis requires a multidisciplinary approach, a collaboration between Psychiatry and Neurology to piece together the complex problems that often manifest in challenging neuropsychiatric syndromes. The possibility of PSP should be considered in the differential diagnosis of late onset psychiatric syndromes. Early PSP diagnosis is imperative for both patients and physicians, as proper treatment will be started without delay to address PSP symptomatology. Thus, reducing the burden of patient and family, as well as reducing unnecessary diagnostic tests, hospitalizations and sub-optimal psychiatric treatment. It is imperative that providers from all specialties be aware of PSP early presentation and how it can be mistaken, specially in early stages, with other neurocognitive disorders in order to make proper referrals for the most effective treatment available. This research was funded by: N/A

Oromandibular dystonia: a diagnosis not to miss.

Oromandibular dystonia (OMD) is characterised by sustained or repetitive involuntary movements of the jaw, face, and tongue. People with the condition may present to their dentist, general practitioner, or a secondary care specialist with non-specific symptoms including jaw or facial pain, bruxism, subluxations or dislocations of the jaw; fractured teeth or dental restorations, or both; or jaw tremor. Many clinicians are not aware of the disorder and this can lead to delayed diagnoses, unnecessary complications, and inappropriate treatment. OMD is an important diagnosis not to miss because referral for specialist management can provide good long-term results. To aid early, accurate diagnosis, this paper focuses on the key clinical features of the disorder and its dental and medical mimics.

Orthostatic Jaw Tremor, a Diagnostic Challenge for Neurologists

Jaw tremor is usually associated with tremor or other abnormal involuntary movements affecting additional body parts, but isolated jaw tremor is a rare clinical presentation. A 40-year-old man presented with history of paroxysmal jaw tremor for 3 years with no other neurological symptoms, psychiatric conditions, or a family history of neurological disorders. The tremor occurred only in the resting jaw position, with 10 episodes per day on an average, and lasted from 10 minutes to hours. It disappeared with any voluntary activation of the masseter muscle and did not change (in amplitude or frequency) with distraction maneuvers. The remainder of neurologic examination was unremarkable. The patient underwent extensive testing, including contrast-enhanced brain magnetic resonance imaging, blood tests and neuropsychiatric evaluation by two different neuropsychiatrists which failed to reveal the underlying cause. The needle electromyography examination revealed involuntary, intermittent, and synchronized muscular contractions in both masseter muscles, at a frequency of 13 to 14 Hz. No changes were discovered in the other muscles. He was treated with propranolol, primidone, valproic acid, diazepam, and amitriptyline, but the response was not significant. Injection of 25 units of botulinum toxin type A in the masseter muscle on both sides of the fac was effective five days after the injection. No adverse effects or masseter weakness were observed.

Periodontal mechanoreceptors and bruxism at low bite forces

ObjectiveIn this study, we examined if 6–9 Hz jaw tremor, an indirect indicator of Periodontal Mechanoreceptor (PMR) activity, is different in bruxists compared to healthy participants during production of a low-level constant bite force. MethodsBite force and surface EMG from the masseter muscle were recorded simultaneously as participants (13 patients, 15 controls) held a force transducer between the upper and lower incisors very gently. ResultsTremor in 6–9 Hz band for bruxists was greater on average compared to controls, but the difference was not significant, both for force recordings and EMG activity. ConclusionsThe low effect sizes measured with the current protocol contrast highly with those of our previous study, where larger, dynamic bite forces were used, and where jaw tremor was markedly different in bruxists compared with controls. SignificanceWe have now gained important insight into the conditions under which abnormal jaw tremor can be elicited in bruxism. From a scientific standpoint, this is critical for understanding the ‘abnormality’ of PMR feedback in bruxism. From a clinical perspective, our results represent progress towards the development of an optimal protocol in which jaw tremor can serve as a biological marker of bruxism.

Jaw tremor: a manifestation of vascular parkinsonism? - a case report

BackgroundVascular Parkinsonism (VP) is a heterogeneous group of conditions that manifest clinically in parkinsonian features, but are presumably of vascular cause. It is usually bilateral, non-tremulous, and frequently associated with pyramidal signs. Classically VP is described as lower body parkinsonism affecting predominantly the legs.Case presentationA 67 years old lady presented with a history of acute onset jaw tremor, with tremor predominantly in both upper limbs. Neurological examination revealed hypomimia of the face with cogwheel rigidity and bradykinesia bilaterally, predominantly in the upper limbs without pyramidal signs. She had a marked tremor of the jaw at rest. When she was asked to open her mouth the tremor was re-emergent. Non contrast CT scan of her brain revealed an infarction in the region of putamen on the left with no evidence of diffuse subcortical white matter ischemia or extension to the caudate nucleus. She was treated with levodopa and responded well to medication.ConclusionsThis case describes atypical clinical features which could be associated with VP including jaw tremor. This case also stresses the importance of initiating a trial of levodopa as certain patients may respond well to medication.

A Case Study of Burning Mouth Syndrome with Oro-facial Dyskinesia Treated with Korean Medicine using Se-sim Pharmacopuncture

Objectives This clinical report describes one patient with the clinical characteristics of burning mouth syndrome accompanied by oro-facial dyskinesia. Methods The patient with burning mouth syndrome and jaw tremor was treated using the following Korean medicine treatments: the herbal medicines Yukgunja-tang-gami (å ­å›å­æ¹¯åŠ å‘³) and Sibak-tang-gami (æŸ´æœ´æ¹¯åŠ å‘³) and acupuncture for 16 days, together with 10 Se-sim (Uncaria Ramulus et Uncus) pharmacopuncture procedures. Patient symptoms were evaluated daily using a Visual Analog Scale questionnaire and twice using the STAI (State-Trait Anxiety Inventory) questionnaire. Results After the treatments, the oro-facial dyskinesia and burning sensation on the tongue were improved by 62.5%. The mental state of anxiety, regarded as the reason for these complaints, was also improved, based on the State-Trait Anxiety Inventory results (67/61 versus 57/52). We found that the improvement was better when combining Se-sim (Uncaria Ramulus et Uncus) pharmacopuncture with the conventional herbal medicine and acupuncture used in Korean medicine. Conclusion Korean medicine treatments may be valuable for patients with burning mouth syndrome and oro-facial dyskinesia. Keywords: burning mouth syndrome, oro-facial dyskinesia, Korean medical treatment, Yukgunja-tang-gami, Sibak-tang-gami

Gray matter density loss in essential tremor: a lobule by lobule analysis of the cerebellum

BackgroundThe pathophysiological basis for essential tremor (ET) remains unclear, although evidence increasingly links it to a disordered and perhaps degenerative cerebellum. Prior imaging studies have treated the cerebellum en bloc. Our hypothesis was that regional differences in cerebellar gray matter (GM) density may better distinguish ET cases from controls.Forty-seven ET cases and 36 control subjects were imaged using magnetic resonance imaging (MRI). The cerebellum was segmented into 34 lobes using a Spatially Unbiased Infra-Tentorial Template (SUIT) atlas within the Statistical Parametric Mapping (SPM) analysis package. Age, gender and Montreal Cognitive Assessment (MoCA) scores were regressed out from the statistical models to isolate group effects. ET cases were further stratified into phenotypically-defined subgroups. The Benjamini-Hochberg False Discovery Rate procedure (BH FDR) (α = 0.1) was used to correct for multiple comparisons.ResultsWhen all ET cases and controls were compared, none of the regions met the BH FDR criteria for significance. When compared with controls, ET cases with head or jaw tremor (n = 27) had significant changes in GM density in nine cerebellar lobules, with a majority in the left cerebellar region, and each meeting the BH FDR criteria. Likewise, ET cases with voice tremor (n = 22) exhibited significant changes in 11 lobules in both left and right regions and the vermis. These analyses, in sum, indicated decreases in GM density in lobules I-IV, V, VI, VII and VIII as well as the vermis. ET cases with severe tremor (n = 20) did not show regions of change that survived the BH FDR procedure when compared to controls.ConclusionsWe showed that ET cases with various forms of cranial tremor differed from controls with respect to cerebellar GM density, with evidence of GM reduction across multiple cerebellar regions. Additional work, using a lobule-by-lobule approach, is needed to confirm these results and precisely map the regional differences in ET cases, subgroups of ET cases, and controls.

Facial tremors in patients with and without parkinsonism.

Facial (lip and jaw) tremors can be an early sign of Parkinson's disease (PD), essential tremor and other parkinsonisms. Its response to acute dopaminergic therapy and further predictive clinical diagnosis has not been previously addressed. The aim of this study was to evaluate facial tremors response to acute dopaminergic therapy and further predictive value for clinical diagnosis. A retrospective review of medical records from patients with recent onset of facial tremor, with or without parkinsonism, submitted to acute levodopa challenge for clinical prediction of sustained long-term dopaminergic response was conducted. Twenty-eight out of 559 patients (5%) had facial tremors, which responded to levodopa in 46% of patients. Facial tremors response to acute levodopa challenge showed 92% sensitivity and 93% specificity to predict a final PD diagnosis. In PD patients, facial tremor magnitude of response to levodopa was not different from that of hand rest tremor (p=0.8). Facial tremors, although infrequent, can be an early sign of PD. Positive response to acute levodopa challenge predicts long-term PD diagnosis.