Jaw tremor: Prevalence and clinical correlates in three essential tremor case samples

Louis, Elan D.; Rios, Eileen; Applegate, La Keisha M.; Hernández, Nora; Andrews, Howard

doi:10.1002/mds.21069

Cited by 69 publications

(62 citation statements)

References 29 publications

(48 reference statements)

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“…On videotaped examination, jaw and voice tremors were coded as present or absent while cases were seated facing the camera. Jaw tremor was assessed while the mouth was stationary (closed), while the mouth was slightly open, during sustained phonation, and during speech [16]. Voice tremor was assessed during sustained phonation, while reading a prepared paragraph, and during speech.…”

Section: Clinical Assessmentmentioning

confidence: 99%

“…Hence, that there are changes in the neocerebellum (i.e., cerebellar hemispheres) is not surprising. Given the changes in gait, balance, and postural control, more recently described in ET [13,14], as well as the presence of midline tremors (head [i.e., neck], voice, jaw tremors) in large subsets of patients [14][15][16][17], we questioned whether the cerebellar vermis might also exhibit increased torpedo pathology. Using brain samples from the Essential Tremor Centralized Brain Repository (ETCBR), which holds the largest collection of ET brains worldwide, we addressed the following aims: (1) Do ET cases have greater torpedo pathology in the vermis than controls?…”

Section: Introductionmentioning

confidence: 99%

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Torpedoes in the Cerebellar Vermis in Essential Tremor Cases vs. Controls

et al. 2011

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The study of the postmortem changes in essential tremor (ET) is in its infancy, although recent evidence points to a central role of the cerebellum, where Purkinje cell axonal swellings ("torpedoes") are significantly more common in ET than control brains. Yet, all existing studies have been confined to the cerebellar hemispheres, and whether there is a more widely distributed cerebellar problem is presently unknown. Our aims were to address whether: (1) ET cases have greater numbers of torpedoes in the vermis than controls, (2) there a correlation between the extent of vermal torpedo pathology and hemispheric torpedo pathology, and (3) vermal torpedo pathology is correlated with clinical features of the disease. A parasagittal neocerebellar block and a vermal block were harvested from 24 ET and 10 control brains. Paraffin sections (7 μm) were stained with Luxol fast blue/hematoxylin and eosin, and torpedoes were quantified. All torpedo counts were corrected for Purkinje cell layer length. Vermal corrected torpedo count (VermTc) was higher in ET cases than controls (7.1±6.8 [median, 4.3] vs. 2.6±2.5 [median, 2]), p=0.002). The VermTc and the hemispheric corrected torpedo count (HemTc) were correlated with one another (Spearman's r=0.54, p=0.002). ET cases with neck, voice, and jaw tremors had the highest VermTc (p=0.046). The abundance of torpedoes in the ET brain is not confined to the ponto- or neocerebellum but is more broadly distributed, also involving the spino- or paleocerebellum. These data further confirm the central role of the cerebellum in the underlying pathophysiology of this common neurological disorder.

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Section: Clinical Assessmentmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Torpedoes in the Cerebellar Vermis in Essential Tremor Cases vs. Controls

et al. 2011

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“…Head tremor was noted as present or absent while seated comfortably and facing the camera as well as during other phases of the examination [1]. Jaw tremor was assessed while the mouth was closed, during sustained phonation, and during speech [9]. Voice tremor was evaluated during sustained phonation, conversational speech, and while reading a prepared passage [10].…”

Section: Methodsmentioning

confidence: 99%

“…Each videotaped examination was reviewed by E.D.L., who rated the severity of postural and kinetic arm tremors (range = 0-3) [1], assigning a total tremor score (range 0-36). Head, jaw, and voice tremors were marked as present if they were seen at any point during the videotaped examination [9]. Diagnoses of ET were reconfirmed by E.D.L.…”

Section: Methodsmentioning

confidence: 99%

Incidence Rates of Cranial Tremors in Essential Tremor: A Prospective, Longitudinal Study

2014

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Background: Cranial (head, jaw) tremors develop in a sizable number of essential tremor (ET) patients. They are particularly important because they are a significant source of embarrassment and are especially resistant to treatment. There are no data on the rate at which ET patients develop these cranial tremors. Our aim was to estimate the incidence rate of head and jaw tremors in ET. Methods: Cases, enrolled in a prospective, longitudinal study, were evaluated at baseline and one follow-up visit. The in-person evaluation included questionnaires and a videotaped neurological examination, which was reviewed by a senior movement disorder neurologist for the presence/absence of head and jaw tremors. The mean time between baseline and follow-up assessments was 6.3 ± 2.4 (median = 6.0; range = 1.5-12.4 years). Results: Five (6.3%) of 79 cases developed head tremor by follow-up (incidence rate = 1,012 per 100,000 person years; 95% confidence interval [CI] = 0-3,219). Twelve (10.6%) of 113 cases developed jaw tremor by follow-up (incidence rate = 1,692 per 100,000 person years; 95% CI = 0-1,692). Conclusion: These epidemiological data were designed to assist physicians in counseling ET patients when they ask about their risks of developing cranial tremors.

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“…It is characterized by 8-12 Hz action tremor of the arms; head tremor and other cranial tremors may also occur, as well as limb and gait ataxia and subtle eye motion abnormalities [1][2][3][4][5]. The prevalence and incidence increase with age, so that more than 20% of individuals over 90 years have ET [1,6].…”

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confidence: 99%

Recent Advances in Essential Tremor

Goksemin¹

2016

J Mult Scler

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Essential tremor (ET) is the most prevalent tremor disorder. It is characterized by 8-12 Hz action tremor of the arms; head tremor and other cranial tremors may also occur, as well as limb and gait ataxia and subtle eye motion abnormalities [1][2][3][4][5]. The prevalence and incidence increase with age, so that more than 20% of individuals over 90 years have ET [1,6].In recent year's neuroepidemiological, clinical and pathological studies have substantially contributed to the changing of view of ET. The traditional view of ET as a monosymptomatic condition is being replaced by an appreciation of the spectrum of clinical features and clinical associations, with both motor and non-motor features [7][8][9][10][11]. It is even possible that ET might be a family of diseases, unified by the presence of kinetic tremor, but further characterized by etiological, clinical and pathological heterogeneity [10].Both genetic and environmental factors play a role in ET, but the underlying causes are far from fully elaborated. Recent studies have reported a genome-wide significant association between leucinerich repeat and Ig domain containing 1 gene (LINGO1) and risk for essential tremor [12][13][14][15]. Further studies also confirmed this finding in populations of European and Asian descent, showing LINGO1 is likely a risk factor for essential tremor with widespread distribution [14,15]. Parkinson's disease (PD) and ET are regarded as distinct entities, but clinical evidence suggest that there is a considerable overlap. For instance, in some cases action tremor can precede the onset of PD symptoms and there is a fourfold increased risk of developing PD in patients with ET [16]. Moreover, in a number of ET cases Lewy bodies have been identified in the brain stem and imaging studies have found signs of dopaminergic deficits in some patients with ET [17]. Given these overlapping features and the association of LINGO 1 with ET, VillarinoGuell et al. [14] investigated the role of LINGO1 in PD and their results revealed an association between LINGO1 and risk of PD. Although these results are not confirmed by other reports, further studies of larger populations are required to investigate the role of LINGO1 in PD [18]. From the pathophysiological perspective, traditionally ET is considered as a primary electrical/elctrophysiological entitiy. It is the result of pacemaking neurons in the inferior olivary nucleus that fire in a coupled manner, and produce tremor through an abnormal olivocerebellar output. But this olivary model of ET suffers from a number of critical problems, such as lack of evidence that this process is occurring in human ET as well. Another major problem of this model is that there are numerous pacemakers in the central nervous system which are also connected with the cerebellum [19][20][21]. Therefore, olivary pacemakers are not unique. Moreover, the main empiric support of olivary model comes from the harmaline model which is not a model of human disease [22]. Over the past decade an alternative model, cerebella...

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Jaw tremor: Prevalence and clinical correlates in three essential tremor case samples

Cited by 69 publications

References 29 publications

Torpedoes in the Cerebellar Vermis in Essential Tremor Cases vs. Controls

Torpedoes in the Cerebellar Vermis in Essential Tremor Cases vs. Controls

Incidence Rates of Cranial Tremors in Essential Tremor: A Prospective, Longitudinal Study

Recent Advances in Essential Tremor

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