Does Head Tremor Predict Postural Instability After Bilateral Thalamic Stimulation in Essential Tremor?

Essential tremor (ET) may present with head tremor (HT), of presumed cerebellar nature. Deep brain stimulation (DBS) targeting the ventral intermediate (Vim) nucleus of the thalamus is a highly effective therapy for medication-refractory ET. However, stimulation-related side effects may include cerebellar abnormalities, such as postural instability. This retrospective cohort study evaluated the risk of post-Vim DBS postural instability (primary outcome measure) in patients with versus without head tremor (HT vs. nHT). The primary outcome measure, namely post-DBS postural instability, was assessed in both groups using a Wilcoxon rank sum t-test. The time to postural instability was determined using Cox proportional hazards regression analysis adjusted for age and sex. Out of 30 patients analyzed during the follow up period, there was similar postural instability detected in HT (9/14, 64%) and nHT patients (11/16, 69%) at 24 months post-Vim DBS (p=0.82), adjusted hazard ratio[aHR]=0.82, p=0.69). These data suggest that the presence or absence of HT does not have an impact on postural instability after bilateral Vim DBS in patients with ET.

French validation of the Quality of life in Essential Tremor Questionnaire (QUEST) and the Essential Tremor Embarrassment Assessment (ETEA).

Two scales have been developed and validated in English to evaluate the impact of tremor on daily life, namely Quality of life in Essential Tremor Questionnaire (QUEST) and Essential Tremor Embarrassment Assessment (ETEA). The psychometric properties of the French version of these two scales were assessed for 117 patients with head tremor. Both scales showed excellent acceptability, very good internal consistency (Cronbach's alpha coefficient>0.8) and reproducibility (Lin concordance coefficient>0.8), satisfactory external validity and satisfactory sensitivity to change. In conclusion, the French versions of QUEST and ETEA are comprehensive, valid and reliable instruments for assessing patients with head tremor.

Non-motor features of essential tremor with midline distribution.

BACKGROUND: Midline essential tremor (Mid-ET) is a distinctive group of essential tremor (ET) in which tremor affects the neck, jaw, tongue, and/or voice. For long, it has been considered as an ultimate stage of the disease and a marker of its severity. However, recent studies pointed its complexity in terms of non-motor presentation. Thus, we aimed to investigate the non-motor signs (NMS) in Mid-ET. DESIGN: We conducted a cross-sectional study in a tertiary neurology referral center including ET patients classified into two groups based on the presence or not of midline tremor (Mid-ET vs. No-Mid-ET). We assessed NMS using the non-motor severity scale (NMSS), a large battery of cognitive tests, clinical and electrophysiological study of the autonomic nervous system along with the evaluation of sleep disturbances. RESULTS: A total of 163 patients were included: Mid-ET (n = 79) and No-Mid-ET (n = 84) matched in gender and age of onset. Mid-ET patients had higher proportion of late-onset ET (> 60 years old, p = 0.002) and more extrapyramidal signs (p = 0.005). For NMS, Mid-ET was marked with cognitive dysfunction (p = 0.008). The hallmarks of the neuropsychiatric profile of Mid-ET were executive dysfunction (p = 0.004), attention problems (p < 0.000), episodic memory impairment (p = 0.003), and greater depression (p = 0.010). The presence of RBD was a trait of Mid-ET (p = 0.039). In both Mid-ET and No-Mid-ET phenotypes, clinical and neurophysiological dysautonomia correlated with cognitive dysfunction. CONCLUSION: Mid-ET patients had greater cognitive dysfunction, depression, RBD, higher proportion of late-onset ET, and more extrapyramidal signs. Taken all together, these findings could provide a redesigned insight into the underlying physiopathology of Mid-ET indicative of a greater cerebellar dysfunction.

Quantification of Head Tremors in Medical Conditions: A Comparison of Analyses Using a 2D Video Camera and a 3D Wireless Inertial Motion Unit.

This study compares two methods to quantify the amplitude and frequency of head movements in patients with head tremor: one based on video-based motion analysis, and the other using a miniature wireless inertial magnetic motion unit (IMMU). Concomitant with the clinical assessment of head tremor severity, head linear displacements in the frontal plane and head angular displacements in three dimensions were obtained simultaneously in forty-nine patients using one video camera and an IMMU in three experimental conditions while sitting (at rest, counting backward, and with arms extended). Head tremor amplitude was quantified along/around each axis, and head tremor frequency was analyzed in the frequency and time-frequency domains. Correlation analysis investigated the association between the clinical severity of head tremor and head linear and angular displacements. Our results showed better sensitivity of the IMMU compared to a 2D video camera to detect changes of tremor amplitude according to examination conditions, and better agreement with clinical measures. The frequency of head tremor calculated from video data in the frequency domain was higher than that obtained using time-frequency analysis and those calculated from the IMMU data. This study provides strong experimental evidence in favor of using an IMMU to quantify the amplitude and time-frequency oscillatory features of head tremor, especially in medical conditions.

Long-Term Successful Outcome of Dystonic Head Tremor after Bilateral Deep Brain Stimulation of the Ventral Intermediate and Ventro-Oral Internus Nuclei: A Case Report and Literature Review of Dystonic Head Tremor.

Head tremor in patients with dystonia is referred to as dystonic tremor. During surgical treatment, numerous targets may be selected, including the internal segment of the globus pallidus and the ventral intermediate (Vim) nucleus; however, there is no consensus concerning the most effective treatment target. We report herein a case of dystonic head tremor in which improvement persisted for 5 years after deep brain stimulation (DBS) of the bilateral thalamic Vim and ventro-oral internus (Voi) nuclei. The patient, a 67-year-old woman, has a horizontal head tremor associated with cervical dystonia that had been resistant to drug treatment over 3 years. Immediately following surgery, dystonia and tremor symptoms had completely improved. Voice volume declined and dysarthria occurred but improved upon adjusting the stimulation conditions. Over 5 years, both head tremor and cervical dystonia have been completely controlled, and no other obvious complications have been observed. As the Voi nucleus receives pallidothalamic projections involved in dystonia and the Vim nucleus receives cerebellothalamic projections involved in tremors, stimulating these 2 nuclei with the same electrode appears reasonable in the treatment of dystonic tremor. This case suggests that Vim-Voi DBS may be effective for treating dystonic head tremor.

Non-motor symptoms are associated with midline tremor in essential tremor.

OBJECTIVES: Essential tremor (ET) patients presenting tremor in the midline structures may be a distinct subtype of the syndrome. Therefore, we sought to explore the clinical manifestations, especially non-motor symptoms (NMS) of Chinese ET patients with midline tremor (MT). METHODS: In the cross-sectional study, we grouped 290 definite or probable ET patients based on their MT conditions. The NMS in ET patients were evaluated using the NMS scale (NMSS). NMS and other clinical correlates were then compared among subgroups with, and without MT. RESULTS: We revealed that 39.0%, 27.6%, and 6.9% of the patients respectively had neck, voice, and facial tremors. With the accumulation of tremor in midline structures, NMS became more severe and prevalent. Logistic regression analyses revealed that factors such as: female gender (OR = 2.164, 95% CI: 1.307-3.583), having least or highest action arm tremor (OR = 2.512, 95% CI: 1.520-4.151), having higher score of sleep/fatigue domain (OR = 1.692, 95% CI: 1.004-2.850) and mood/apathy (OR = 1.926, 95% CI: 1.143-3.246) domain, to be independently associated with MT manifestation. CONCLUSIONS: Our study demonstrates the heterogeneity of symptoms in ET patients with MT, especially in prominent NMS. In addition, the discrepancy of NMS between patients with, and without MT provides novel insight into the underlying pathophysiology and therapeutic of ET.

Distinct non-motor features of essential tremor with head tremor patients.

BACKGROUND: There have been few comprehensive scale studies on the non-motor symptoms (NMS) of patients with essential tremor (ET) with head tremor (ETh) and those with ET without head tremor (ETol). We aimed to explore the motor symptoms and NMS of these two subgroups. METHODS: We enrolled 199 patients with ET (125, ETol; 74 ETh) and 132 healthy controls. We evaluated motor symptoms using the Fahn-Tolosa-Marin Tremor Rating Scale (TRS) and NMS using the Non-Motor Symptom Scale (NMSS). We compared NMSS scores and the prevalence of each NMS between the patient subgroups. Finally, we conducted a logistic regression analysis of the correlation between head tremor and NMS severity, as well as other determinants. RESULTS: There were no significant between-subgroup differences in demographic characteristics. Further, they presented similar tremor clinical manifestation; however, the ETh subgroup showed a higher prevalence of rest tremor, feeling of sadness, forgetting things or events, and swallowing difficulty, as well as TRS scores, compared with the ETol subgroup. Both patient subgroups showed high scores and prevalence (>50%) in difficulty falling asleep. Logistic regression analysis indicated age as a tremor severity determinant; further, head tremor and tremor severity were NMS determinants. CONCLUSION: Both patient subgroups presented various NMS including sleep disturbances, cognitive deficits, and affective disorders. The ETh subgroup showed a high prevalence of certain NMS aspects including memory and affective disorder; further, they had aggravated NMS. ET with both upper limb tremor and head tremor may be regarded as a more severe clinical subtype.

The role of pallidum in the neural integrator model of cervical dystonia.

Dystonia is the third most common movement disorder affecting three million people worldwide. Cervical dystonia is the most common form of dystonia. Despite common prevalence the pathophysiology of cervical dystonia is unclear. Traditional view is that basal ganglia is involved in pathophysiology of cervical dystonia, while contemporary theories suggested the role of cerebellum and proprioception in the pathophysiology of cervical dystonia. It was recently proposed that the cervical dystonia is due to malfunctioning of the head neural integrator - the neuron network that normally converts head velocity to position. Most importantly the neural integrator model was inclusive of traditional proposal emphasizing the role of basal ganglia while also accommodating the contemporary view suggesting the involvement of cerebellum and proprioception. It was hypothesized that the head neural integrator malfunction is the result of impairment in cerebellar, basal ganglia, or proprioceptive feedback that converge onto the integrator. The concept of converging input from the basal ganglia, cerebellum, and proprioception to the network participating in head neural integrator explains that abnormality originating anywhere in the network can lead to the identical motor deficits - drifts followed by rapid corrective movements - a signature of neural integrator dysfunction. We tested this hypothesis in an experiment examining simultaneously recorded globus pallidal single-unit activity, synchronized neural activity (local field potential), and electromyography (EMG) measured from the neck muscles during the standard-of-care deep brain stimulation surgery in 12 cervical dystonia patients (24 hemispheres). Physiological data were collected spontaneously or during voluntary shoulder shrug activating the contralateral trapezius muscle. The activity of pallidal neurons during shoulder shrug exponentially decayed with time constants that were comparable to one measured from the pretectal neural integrator and the trapezius electromyography. These results show that evidence of abnormal neural integration is also seen in globus pallidum, and that latter is connected with the neural integrator. Pretectal single neuron responses consistently preceded the muscle activity; while the globus pallidum internus response always lagged behind the muscle activity. Globus pallidum externa had equal proportion of lag and lead neurons. These results suggest globus pallidum receive feedback from the muscles or the efference copy from the integrator or the other source of the feedback. There was bi-hemispheric asymmetry in the pallidal single-unit activity and local field potentials. The asymmetry correlated with degree of lateral head turning in cervical dystonia patients. These results suggest that bihemispheric asymmetry in the feedback leads to asymmetric dysfunction in the neural integrator causing head turning.

Tremor Distribution and the Variable Clinical Presentation of Essential Tremor.

In addition to having postural and kinetic tremor of the upper limbs, some patients with essential tremor (ET) may have head tremor as well as cognitive and psychiatric disorders. We aimed to investigate whether the variable clinical presentation in ET patients, including motor and non-motor symptoms, differs in patients with and without head tremor. We consecutively enrolled 70 patients with a diagnosis of ET. Tremor severity was assessed by means of clinical rating scales. Patients also underwent kinematic recordings of postural and kinetic tremor of the upper limbs based on an optoelectronic system. Several neuropsychological tests were also administered. Finally, we adopted the structured interviews for DSM-IV, SCID-I, and SCID-II to investigate psychiatric and personality disorders. ET patients with upper limb tremor plus head tremor exhibited more severe kinetic tremor of the upper limbs and a higher occurrence of axis I psychiatric disorders than ET patients with upper limb tremor only. Cognitive and other motor and psychiatric features did not differ significantly with respect to tremor distribution. The study findings support the hypothesis that body tremor distribution, i.e., the presence of head tremor, influences the variable clinical presentation of ET. The study results support the notion that cases with head tremor may represent a distinct ET subtype, characterized by a prominent cerebellar involvement, and that psychiatric disorders should be considered as a specific manifestation of ET.

Physiology of midbrain head movement neurons in cervical dystonia.

BACKGROUND: Early theories for cervical dystonia, as promoted by Hassler, emphasized the role of the midbrain interstitial nucleus of Cajal. Focus then shifted to the basal ganglia, and it was further supported with the success of deep brain stimulation. Contemporary theories suggested the role of the cerebellum, but even more recent hypotheses renewed interest in the midbrain. Although the pretectum was visited on several occasions, we still do not know about the physiology of midbrain neurons in cervical dystonia. METHODS: We analyzed the unique database of pretectal neurons collected in the 1970s and 1980s during historic stereotactic surgeries aimed to treat cervical dystonia. This database is valuable because such recordings could otherwise never be obtained from humans. RESULTS: We found the following 3 types of eye or neck movement sensitivity: eye-only neurons responded to pure vertical eye movements, neck-only neurons were sensitive to pure neck movements, and the combined eye-neck neurons responded to eye and neck movements. There were the 2 neuronal subtypes: burst-tonic and tonic. The eye-neck or eye-only neurons sustained their activity during eccentric gaze holding. In contrast, the response of neck-only and eye-neck neurons exponentially decayed during neck movements. CONCLUSIONS: Modern quantitative analysis of a historic database of midbrain single units from patients with cervical dystonia might support novel hypotheses for normal and abnormal head movements. This data, collected almost 4 decades ago, must be carefully viewed, especially because it was acquired using a less sophisticated technology available at that time and the aim was not to address specific hypothesis, but to make an accurate lesion providing optimal relief from dystonia. © 2017 International Parkinson and Movement Disorder Society.

Evaluation of Quantitative Measurement Techniques for Head Tremor With Thalamic Deep Brain Stimulation.

BACKGROUND: Ventralis intermedius thalamic deep brain stimulation (VIM DBS) has shown to be safe and effective for medically refractory essential tremor (ET). We evaluate the use of quantitative tremor measurement methods for head tremor in ET using a "smart" hat and a smartphone application. METHODS: We enrolled 13 ET patients who previously underwent VIM DBS. Head and arm tremor was measured ON and OFF stimulation using the clinical gold standard Fahn-Tolosa-Marin Tremor Rating Scale (TRS). Results were then compared to two quantitative measurement techniques: Lift Pulse (smartphone application) and modified Nizet (adapted laser point measurement from Nizet et al.). Spearman's rank correlation was used to compare tremor severity and improvement on stimulation using TRS and quantitative methods to measure tremor. RESULTS: Lift Pulse tremor severity measurement significantly correlated with TRS for head (ρ = 0.53, p < 0.01) and arm tremor (ρ = 0.49, p < 0.01). Modified Nizet tremor severity measurement significantly correlated with TRS for head (ρ = 0.83, p < 0.001) and arm tremor (ρ = 0.50, p < 0.01). Inter-method correlation for head tremor severity was significant (ρ = 0.45, p < 0.05). Lift Pulse tremor improvement measurement significantly correlated with TRS for arm tremor (ρ = 0.56, p < 0.05). Modified Nizet tremor improvement measurement significantly correlated with TRS for head tremor (ρ = 0.53, p < 0.01). DISCUSSION: Our results show that Lift Pulse and modified Nizet are both effective techniques to quantitatively measure head and arm tremor severity. We also show the utility of a "smart" hat to measure head tremor. Modified Nizet technique is more effective for measuring head tremor, while Lift Pulse is an effective measure of tremor severity, especially arm tremor improvement.

Idiopathic head tremor in English bulldogs.

Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder.

Effect of Thalamic versus Pallidal Deep Brain Stimulation on Head Tremor in Dystonic and Essential Tremor Patients-A Retrospective Video-Blinded Study.

BACKGROUND: Head tremor is common in dystonia syndromes and difficult to treat. Deep brain stimulation (DBS) is a therapeutic option in medically-refractory cases. In most DBS-centers, the globus pallidus internus (GPi) is targeted in patients with predominant dystonia and the ventrointermediate nucleus of the thalamus (Vim) in predominant tremor. The aim of the study was to evaluate the effect of GPi- versus Vim-DBS in dystonic or essential head tremor. METHODS: All patients with dystonia or essential tremor (ET) (n = 381) who underwent DBS surgery at our institution between 1999 and 2020 were screened for head tremor in our database according to predefined selection criteria. Of the 33 patients meeting inclusion criteria tremor and dystonia severity were assessed at baseline, short- (mean 10 months) and long-term follow-up (41 months) by two blinded video-raters. RESULTS: Twenty-two patients with dystonic head tremor received either GPi- (n = 12) or Vim-stimulation (n = 10), according to the prevailing clinical phenotype. These two groups were compared with 11 patients with ET, treated with Vim-stimulation. The reduction in head tremor from baseline to short- and long-term follow-up was 60-70% and did not differ significantly between the three groups. CONCLUSIONS: GPi-DBS effectively and sustainably reduced head tremor in idiopathic dystonia. The effect was comparable to the effect of Vim-DBS on head tremor in dystonia patients with predominant limb tremor and to the effect of Vim-DBS on head tremor in ET.

Idiopathic and structural episodic nonintentional head tremor in dogs: 100 cases (2004-2022).

BACKGROUND: Although idiopathic episodic head tremor (IEHT) in dogs is well-known, little is known about structural brain lesions causing structural episodic head tremor (SEHT). HYPOTHESIS/OBJECTIVES: Describe semiology, magnetic resonance imaging (MRI) findings and outcome of dogs with IEHT or SEHT. We hypothesized that structural lesions affecting the middle cranial fossa or mesencephalic aqueduct could lead to SEHT. ANIMALS: One hundred dogs with IEHT (n = 71) or SEHT (n = 29). METHODS: Retrospective, multicenter, study of dogs with episodic (nonintentional) head tremor and brain MRI between 2004 and 2022. RESULTS: Lesions on MRI in SEHT dogs were localized to the middle cranial fossa (15/29), cerebrocortex (3/29), brainstem (2/29), fourth ventricle (1/29) or multifocal (8/29) with thalamus involvement (6/8). Secondary compression of the mesencephalic aqueduct (19/29), third ventricle or interthalamic adhesion or both (14/29) was common. The most common underlying condition in dogs with SEHT was a pituitary mass. Dogs with SEHT were older, had additional neurological signs and were more likely to be euthanized after diagnosis (P < .001 for all) compared to IEHT dogs. Two SEHT dogs had only tremor. In IEHT dogs, 8/10 owners reported that the tremor decreased or abated over time (range, 106-2315 days) without treatment. Tremor remission occurred in SEHT dogs treated for underlying meningoencephalitis. CONCLUSIONS AND CLINICAL IMPORTANCE: Presence of additional neurological signs and older age may indicate an underlying structural cause for episodic (nonintentional) head tremor involving the mesencephalic aqueduct, third ventricle, interthalamic adhesion or some combination of these. An intracranial structural abnormality cannot be excluded in dogs with normal neurological examination.

Very-Low-Frequency Spike-Wave Complex Partial Motor Seizure Mimicking Canine Idiopathic Head Tremor Syndrome in a Dog.

Canine idiopathic head tremor syndrome (CIHTS) represents a benign condition characterized by episodic, uncontrolled movement of the head. Even though the condition might be an expression of a partial motor epilepsy, to date, there is a limited number of studies describing the electroencephalographic features. In this report, we describe the case of a dog diagnosed with partial motor epilepsy resembling CIHTS symptomatology, a new slow spike-wave complex pattern similar to that of Lennox-Gastaut syndrome in humans identified on electroencephalographic examination. We also studied the efficacy of phenobarbitone therapy over a period of two years.

Essential and dystonic head tremor: More similarities than differences.

INTRODUCTION: Head tremor is a common symptom of essential tremor (ET) and cervical dystonia (CD). In clinical practice, it is often difficult to distinguish between these two conditions, especially in cases where head tremor predominates. OBJECTIVES: To investigate which clinical and instrumental methods best differentiate ET and CD in patients with head tremor. METHODS: 65 patients were included, of which 23 were diagnosed with ET and head tremor (HT+), 21 with ET without head tremor (HT-) and 21 with CD and dystonic head tremor. 22 healthy volunteers served as controls. All patients were examined using the rating scales for ET (TETRAS), cervical dystonia (TWSTRS), and ataxia (SARA). The Somatosensory Temporal Discrimination Threshold (STDT) was defined as the shortest interval in which an individual recognizes two tactile stimuli as temporally separated. RESULTS: TETRAS and SARA scores were higher in the HT+ group compared with HT- and CD, with no significant difference between mild head tremor subscores in HT+ and CD. In most HT+ and CD patients, head tremor disappeared supine. The STDT values were significantly higher in the HT+ group compared with controls. CONCLUSION: While TWSTRS contributed to assess dystonia severity, the scales of tremor and ataxia were not helpful in differentiating head tremor syndromes. The cessation of head tremor in the supine position could be related to the overall mild head tremor scores in both groups. Increased SARA scores and STDT values in HT+ patients suggest a possible role of cerebellar involvement and altered somatosensory timing that merit further verification.

Exploring the heterogeneous morphometric data in essential tremor with probabilistic modelling.

Essential tremor (ET) is a prevalent movement disorder characterized by marked clinical heterogeneity. Here, we explored the morphometric underpinnings of this cross-subject variability on a cohort of 34 patients with right-dominant drug-resistant ET and 29 matched healthy controls (HCs). For each brain region, group-wise morphometric data was modelled by a multivariate Gaussian to account for morphometric features' (co)variance. No group differences were found in terms of mean values, highlighting the limits of more basic group comparison approaches. Variance in surface area was higher in ET in the left lingual and caudal anterior cingulate cortices, while variance in mean curvature was lower in the right superior temporal cortex and pars triangularis, left supramarginal gyrus and bilateral paracentral gyrus. Heterogeneity further extended to the right putamen, for which a mixture of two Gaussians fitted the ET data better than a single one. Partial Least Squares analysis revealed the rich clinical relevance of the ET population's heterogeneity: first, increased head tremor and longer symptoms' duration were accompanied by broadly lower cortical gyrification. Second, more severe upper limb tremor and impairments in daily life activities characterized the patients whose morphometric profiles were more atypical compared to the average ET population, irrespective of the exact nature of the alterations. Our results provide candidate morphometric substrates for two different types of clinical variability in ET. They also demonstrate the importance of relying on analytical approaches that can efficiently handle multivariate data and enable to test more sophisticated hypotheses regarding its organization.

From null to midline: changes in head posture do not predictably change head tremor in cervical dystonia.

Introduction: A common view is that head tremor (HT) in cervical dystonia (CD) decreases when the head assumes an unopposed dystonic posture and increases when the head is held at midline. However, this has not been examined with objective measures in a large, multicenter cohort. Methods: For 80 participants with CD and HT, we analyzed videos from examination segments in which participants were instructed to 1) let their head drift to its most comfortable position (null point) and then 2) hold their head straight at midline. We used our previously developed Computational Motor Objective Rater (CMOR) to quantify changes in severity, amplitude, and frequency between the two postures. Results: Although up to 9% of participants had exacerbated HT in midline, across the whole cohort, paired t-tests reveal no significant changes in overall severity (t = -0.23, p = 0.81), amplitude (t = -0.80, p = 0.43), and frequency (t = 1.48, p = 0.14) between the two postures. Conclusions: When instructed to first let their head drift to its null point and then to hold their head straight at midline, most patient's changes in HT were below the thresholds one would expect from the sensitivity of clinical rating scales. Counter to common clinical impression, CMOR objectively showed that HT does not consistently increase at midline posture in comparison to the null posture.

Head tremor in cervical dystonia: Quantifying severity with computer vision.

BACKGROUND: Head tremor (HT) is a common feature of cervical dystonia (CD), usually quantified by subjective observation. Technological developments offer alternatives for measuring HT severity that are objective and amenable to automation. OBJECTIVES: Our objectives were to develop CMOR (Computational Motor Objective Rater; a computer vision-based software system) to quantify oscillatory and directional aspects of HT from video recordings during a clinical examination and to test its convergent validity with clinical rating scales. METHODS: For 93 participants with isolated CD and HT enrolled by the Dystonia Coalition, we analyzed video recordings from an examination segment in which participants were instructed to let their head drift to its most comfortable dystonic position. We evaluated peak power, frequency, and directional dominance, and used Spearman's correlation to measure the agreement between CMOR and clinical ratings. RESULTS: Power averaged 0.90 (SD 1.80) deg2/Hz, and peak frequency 1.95 (SD 0.94) Hz. The dominant HT axis was pitch (antero/retrocollis) for 50%, roll (laterocollis) for 6%, and yaw (torticollis) for 44% of participants. One-sided t-tests showed substantial contributions from the secondary (t = 18.17, p < 0.0001) and tertiary (t = 12.89, p < 0.0001) HT axes. CMOR's HT severity measure positively correlated with the HT item on the Toronto Western Spasmodic Torticollis Rating Scale-2 (Spearman's rho = 0.54, p < 0.001). CONCLUSIONS: We demonstrate a new objective method to measure HT severity that requires only conventional video recordings, quantifies the complexities of HT in CD, and exhibits convergent validity with clinical severity ratings.

Longitudinal evaluation of patients with isolated head tremor.

INTRODUCTION: Isolated head tremor, a pathological condition characterized by head tremor without dystonic postures or tremor in other body parts, has recently been suggested to be a form of dystonia. It is however still unclear whether isolated head tremor precedes dystonia or remains unmodified overtime. METHODS: We enrolled 20 patients with isolated head tremor. For each patient, we assessed videos recorded at enrollment and after 5 years. The videotapes were reviewed by two independent experienced movement disorder specialists who evaluated and scored tremor and CD severity using the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor and the revised Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), respectively. RESULTS: Upon enrollment, all 20 patients showed isolated head tremor. Mean tremor severity was 2.7 ± 0.9 as measured using the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor total score. At the 5-year follow-up examination, 15 (75%) of the 20 patients with isolated head tremor showed dystonic postures in the neck, while the remaining 5 patients (25%) had only isolated head tremor. Mean severity of dystonia as measured using the TWSTRS-2 total score was 11.8 ± 3.6. Head tremor severity was unchanged between baseline and the 5-year follow-up examination (p > 0.05). At the follow-up examination, no patients had tremor or dystonia in a body part other than the neck, nor did they develop bradykinesia or other parkinsonian signs. CONCLUSIONS: Our longitudinal study demonstrated that patients with isolated head tremor may develop cervical dystonia over time.