Thyroid disease in cervical dystonia.

There are many possible etiologies for cervical dystonia (CD), but a cause cannot be identified in most cases. Most recent attention has focused on genetic causes, although a few prior studies have highlighted autoimmune mechanisms instead. Because autoimmune disorders frequently co-exist, the current study evaluated the hypothesis that autoimmune disorders might be more common in CD than neurological controls. The frequency of 32 common autoimmune disorders was evaluated using a systematic survey comparing 300 subjects with CD with 391 neurological controls. The frequency of thyroid disease was significantly higher in CD (20%) compared with controls (6%). Regression analyses that accounted for age and sex revealed an odds ratio of 4.5 (95% CI 2.5-8.1, p < 0.001). All other autoimmune disorders occurred with similar frequencies in CD and controls. Although these studies do not establish a mechanistic link between CD and autoimmune disease, they suggest the need for further attention to a potential relationship, and more specifically with thyroid disease.

A model for the development of binocular congruence in primary visual cortex.

Neurons in primary visual cortex are selective for stimulus orientation, and a neuron's preferred orientation changes little when the stimulus is switched from one eye to the other. It has recently been shown that monocular orientation preferences are uncorrelated before eye opening; how, then, do they become aligned during visual experience? We aimed to provide a model for this acquired congruence. Our model, which simulates the cat's visual system, comprises multiple on-centre and off-centre channels from both eyes converging onto neurons in primary visual cortex; development proceeds in two phases via Hebbian plasticity in the geniculocortical synapse. First, cortical drive comes from waves of activity drifting across each retina. The result is orientation tuning that differs between the two eyes. The second phase begins with eye opening: at each visual field location, on-centre cortical inputs from one eye can cancel off-centre inputs from the other eye. Synaptic plasticity reduces the destructive interference by up-regulating inputs from one eye at the expense of its fellow, resulting in binocular congruence of orientation tuning. We also show that orthogonal orientation preferences at the end of the first phase result in ocular dominance, suggesting that ocular dominance is a by-product of binocular congruence.

A Multi-center Genome-wide Association Study of Cervical Dystonia.

BACKGROUND: Several monogenic causes for isolated dystonia have been identified, but they collectively account for only a small proportion of cases. Two genome-wide association studies have reported a few potential dystonia risk loci; but conclusions have been limited by small sample sizes, partial coverage of genetic variants, or poor reproducibility. OBJECTIVE: To identify robust genetic variants and loci in a large multicenter cervical dystonia cohort using a genome-wide approach. METHODS: We performed a genome-wide association study using cervical dystonia samples from the Dystonia Coalition. Logistic and linear regressions, including age, sex, and population structure as covariates, were employed to assess variant- and gene-based genetic associations with disease status and age at onset. We also performed a replication study for an identified genome-wide significant signal. RESULTS: After quality control, 919 cervical dystonia patients compared with 1491 controls of European ancestry were included in the analyses. We identified one genome-wide significant variant (rs2219975, chromosome 3, upstream of COL8A1, P-value 3.04 × 10-8 ). The association was not replicated in a newly genotyped sample of 473 cervical dystonia cases and 481 controls. Gene-based analysis identified DENND1A to be significantly associated with cervical dystonia (P-value 1.23 × 10-6 ). One low-frequency variant was associated with lower age-at-onset (16.4 ± 2.9 years, P-value = 3.07 × 10-8 , minor allele frequency = 0.01), located within the GABBR2 gene on chromosome 9 (rs147331823). CONCLUSION: The genetic underpinnings of cervical dystonia are complex and likely consist of multiple distinct variants of small effect sizes. Larger sample sizes may be needed to provide sufficient statistical power to address the presumably multi-genic etiology of cervical dystonia. © 2021 International Parkinson and Movement Disorder Society.

A Model for the Origin of Motion Direction Selectivity in Visual Cortex.

Motion perception is a vital part of our sensory repertoire in that it contributes to navigation, awareness of moving objects, and communication. Motion sense in carnivores and primates originates with primary visual cortical neurons selective for motion direction. More than 60 years after the discovery of these neurons, there is still no consensus on the mechanism underlying direction selectivity. This paper describes a model of the cat's visual system in which direction selectivity results from the well-documented orientation selectivity of inhibitory neurons: inhomogeneities in the orientation preference map for inhibitory neurons leads to spatially asymmetric inhibition, and thus to direction selectivity. Stimulation of the model with a drifting grating shows that direction selectivity results from the relative timing of excitatory and inhibitory inputs to a neuron. Using a stationary contrast-reversing grating reveals that the inhibitory input is spatially displaced in the preferred direction relative to the excitatory input, and that this asymmetry leads to the timing difference. More generally, the model yields physiologically realistic estimates of the direction selectivity index, and it reproduces the critical finding with contrast-reversing gratings that response phase advances with grating spatial phase. It is concluded that a model based on intracortical inhibition can account well for the known properties of direction selectivity in carnivores and primates.SIGNIFICANCE STATEMENT Motion perception is vital for navigation, communication, and the awareness of moving objects. Motion sense depends on cortical neurons that are selective for motion direction, and this paper describes a model for the physiological mechanism underlying cortical direction selectivity. The essence of the model is that intracortical inhibition of a direction-selective cell is spatially inhomogeneous and therefore depends on whether a stimulus generates inhibition before or after reaching the cell's receptive field: the response is weaker in the former than in the latter case. If the model is correct, it will contribute to the understanding of motion processing in carnivores and primates.

A metabolomic study of cervical dystonia.

INTRODUCTION: Cervical dystonia is the most common of the adult-onset focal dystonias. Most cases are idiopathic. The current view is that cervical dystonia may be caused by some combination of genetic and environmental factors. Genetic contributions have been studied extensively, but there are few studies of other factors. We conducted an exploratory metabolomics analysis of cervical dystonia to identify potentially abnormal metabolites or altered biological pathways. METHODS: Plasma samples from 100 cases with idiopathic cervical dystonia and 100 controls were compared using liquid chromatography coupled with mass spectrometry-based metabolomics. RESULTS: A total of 7346 metabolic features remained after quality control, and up to 289 demonstrated significant differences between cases and controls, depending on statistical criteria chosen. Pathway analysis revealed 9 biological processes to be significantly associated at p < 0.05, 5 pathways were related to carbohydrate metabolism, 3 pathways were related to lipid metabolism. CONCLUSION: This is the first large scale metabolomics study for any type of dystonia. The results may provide potential novel insights into the biology of cervical dystonia.

Dystonia and Tremor: A Cross-Sectional Study of the Dystonia Coalition Cohort.

OBJECTIVE: To assess the clinical manifestations and predictors of different types of tremors in individuals with different types of isolated dystonia. METHODS: Clinical manifestations of tremor were assessed in a multicenter, international cross-sectional, cohort study of 2,362 individuals with all types of isolated dystonia (focal, segmental, multifocal, and generalized) recruited through the Dystonia Coalition. RESULTS: Methodical and standardized assessments of all participants in this cohort revealed the overall prevalence of any type of tremor was 53.3%. The prevalence of dystonic tremor varied from 36.9% to 48.4%, depending on criteria used to define it. To identify the factors associated with tremors in dystonia, the data were analyzed by generalized linear modeling and cluster analyses. Generalized linear modeling indicated 2 of the strongest factors associated with tremor included body region affected by dystonia and recruitment center. Tremor was also associated with severity of dystonia and duration of dystonia, but not with sex or race. The cluster analysis distinguished 8 subgroups within the whole cohort; defined largely by body region with dystonia, and secondarily by other clinical characteristics. CONCLUSION: The large number of cases evaluated by an international team of movement disorder experts facilitated the dissection of several important factors that influence the apparent prevalence and phenomenology of tremor in dystonia. These results are valuable for understanding the many differences reported in prior studies, and for guiding future studies of the nosology of tremor and dystonia.

A model for the origin and development of visual orientation selectivity.

Orientation selectivity is a key property of primary visual cortex that contributes, downstream, to object recognition. The origin of orientation selectivity, however, has been debated for decades. It is known that on- and off-centre subcortical pathways converge onto single neurons in primary visual cortex, and that the spatial offset between these pathways gives rise to orientation selectivity. On- and off-centre pathways are intermingled, however, so it is unclear how their inputs to cortex come to be spatially segregated. We here describe a model in which the segregation occurs through Hebbian strengthening and weakening of geniculocortical synapses during the development of the visual system. Our findings include the following. 1. Neighbouring on- and off-inputs to cortex largely cancelled each other at the start of development. At each receptive field location, the Hebbian process increased the strength of one input sign at the expense of the other sign, producing a spatial segregation of on- and off-inputs. 2. The resulting orientation selectivity was precise in that the bandwidths of the orientation tuning functions fell within empirical estimates. 3. The model produced maps of preferred orientation-complete with iso-orientation domains and pinwheels-similar to those found in real cortex. 4. These maps did not originate in cortical processes, but from clustering of off-centre subcortical pathways and the relative location of neighbouring on-centre clusters. We conclude that a model with intermingled on- and off-pathways shaped by Hebbian synaptic plasticity can explain both the origin and development of orientation selectivity.

Motion changes response balance between ON and OFF visual pathways.

Humans are faster at detecting dark than light stationary stimuli, a temporal difference that originates early in the visual pathway. Here we show that this difference reverses when stimuli move, making detection faster for moving lights than darks. Human subjects judged the direction of moving edges and bars, and made faster and more accurate responses for light than for dark stimuli. This light/dark asymmetry is greatest at low speeds and disappears at high speeds. In parallel experiments, we recorded responses in the cat visual cortex for moving bars and again find that responses are faster for light bars than for dark bars moving at low speeds. We show that differences in the luminance-response function between ON and OFF pathways can reproduce these findings, and may explain why ON pathways are used for slow-motion image stabilization in many species.

Pilot Single-Blind Trial of AbobotulinumtoxinA in Oromandibular Dystonia.

Oromandibular dystonia (OMD) causes involuntary movements of masticatory and lingual muscles impairing eating, speaking, and swallowing. Treatment options are limited. The objective of this study was to determine the safety and efficacy of abobotulinumtoxinA (aboBoNTA) in OMD. A dose-finding study (phase 1) followed by a single session, prospective, single-blind trial (phase 2) was carried out. OMD subjects were evaluated at baseline, 6 and 12 weeks. Muscles injected were tailored to individual symptoms using EMG guidance, but the aboBoNTA dose for each muscle was pre-specified based on phase 1 results. Evaluations were Global Dystonia Rating Scale (GDS), Unified Dystonia Rating Scale (UDRS), Clinical Global Impression (CGI) improvement and severity, and quality of life (OMDQ-25). Adverse events were monitored. The lowest dosage in phase 1 resulted in adverse effects in two of three patients and thus was used in phase 2. In phase 2, adverse effects were observed in 50% of subjects including dysphagia, voice change, and soft palate weakness. Most were mild. Significant improvement was seen in quality of life (OMDQ-25), speech (BFMq21), and change in GDS, UDRS, CGI severity assessed by the unblinded investigator, but not in blinded video ratings. We conclude that aboBoNTA therapy in this study was associated with improved quality of life and was generally well tolerated in OMD, but occurrence of dysphagia dictated the importance of using low genioglossus dosing. Face to face assessment appears to be more sensitive than video assessment for change in OMD severity. Consideration of the disability in OMD places constraints on traditional placebo-control trial design. Development of novel trial designs is warranted.

Affective Correlates of Psychosis in Parkinson's Disease.

OBJECTIVE: To examine the nature of the association between affective disorders and psychosis in Parkinson's disease (PD). BACKGROUND: In PD, psychosis and affective disorders are common and independently impact quality of life and mortality. Both depression and psychosis are correlated with the occurrence of cognitive dysfunction, suggesting that they may share neurobiological substrates. Anxiety has not been examined as a correlate of psychosis. METHODS: 144 PD subjects were evaluated with the Schedule for Assessment of Positive Symptoms to assess psychotic features, while depression and anxiety were examined by the Structured Clinical Interview for DSM-IV-TR (SCID) and self-assessment scales Beck Depression Inventory II (BDI-II) and Beck Anxiety Inventory (BAI). Correlational analyses assessed associations between hallucinations and delusions with depression and anxiety. RESULTS: A diagnosis of anxiety (SCID) was significantly (p=.015) associated with hallucinations (OR=4.81, CI=1.36-16.99). Severity of anxiety (BAI) significantly predicted (p=.03) the presence of hallucinations (OR=1.08, CI=1.01-1.15) and delusions (OR=1.09, CR=1.01-1.17). Current depression (SCID) was significantly (p=.001) associated with the presence of hallucinations (OR=6.12, CI=2.04-18.39) and delusions (OR=7.14, CI=2.23-22.93). Multiple linear regressions revealed that severity of anxiety remained an independent predictor (p<.05) of both the number of types of hallucinations (t=3.06, p=.003) and delusions (t=2.87, p=.005). Severity of depression was a significant predictor of the total number of delusions (t=2.28, p=.024). CONCLUSIONS: This study demonstrates an association between depression and psychosis and, for the first time, an association between anxiety and psychosis. These associations may have implications on pathophysiology and treatment of psychosis in PD.

Orientation discrimination requires coactivation of on- and off-dominated visual channels.

Orientation sensitivity depends on the cortical convergence of on- and off-center subcortical neurons. Off-center inputs are faster and stronger than their on-center counterparts: How does this asymmetry affect orientation discrimination? We tackled this question psychophysically with grating stimuli that either increased or decreased luminance. The gratings were of low contrast in order to avoid the complicating influences of nonlinearities such as response saturation, masking, and aftereffects. Gratings were presented in either of two locations, and subjects indicated the perceived location. Stimuli were randomly timed, and response correctness and reaction time were recorded. We found the following: (a) Contrast sensitivity was insignificant for a range of contrasts around zero. (b) Outside this range, contrast sensitivity for contrast decrements exceeded that for increments by an average of 15%. (c) Reaction times for contrast decrements were up to 45 ms less than for increments. (d) These findings are reproduced by a signal-detection model which incorporates recent physiological findings: Neurons in primary visual cortex are hyperpolarized at rest; these neurons respond more to darks than to lights; and off-dominated cortical neurons have shorter latencies than their on-dominated neighbors. (e) We tested orientation discrimination by splitting a grating into two components, one containing the light bars and the other the dark, and presenting the two components asynchronously. Discrimination was optimal when light bars preceded dark bars, consistent with coactivation of on- and off-center cortical inputs. We conclude that the ability to discriminate between orientations is intimately connected with the properties of subcortical channels.

A Functional Magnetic Resonance Imaging Study of Head Movements in Cervical Dystonia.

Cervical dystonia (CD) is a neurological disorder characterized by abnormal movements and postures of the head. The brain regions responsible for these abnormal movements are not well understood, because most imaging techniques for assessing regional brain activity cannot be used when the head is moving. Recently, we mapped brain activation in healthy individuals using functional magnetic resonance imaging during isometric head rotation, when muscle contractions occur without actual head movements. In the current study, we used the same methods to explore the neural substrates for head movements in subjects with CD who had predominantly rotational abnormalities (torticollis). Isometric wrist extension was examined for comparison. Electromyography of neck and hand muscles ensured compliance with tasks during scanning, and any head motion was measured and corrected. Data were analyzed in three steps. First, we conducted within-group analyses to examine task-related activation patterns separately in subjects with CD and in healthy controls. Next, we directly compared task-related activation patterns between participants with CD and controls. Finally, considering that the abnormal head movements in CD occur in a consistently patterned direction for each individual, we conducted exploratory analyses that involved normalizing data according to the direction of rotational CD. The between-group comparisons failed to reveal any significant differences, but the normalization procedure in subjects with CD revealed that isometric head rotation in the direction of dystonic head rotation was associated with more activation in the ipsilateral anterior cerebellum, whereas isometric head rotation in the opposite direction was associated with more activity in sensorimotor cortex. These findings suggest that the cerebellum contributes to abnormal head rotation in CD, whereas regions in the cerebral cortex are involved in opposing the involuntary movements.

Botulinum toxin treatment failures in cervical dystonia: causes, management, and outcomes.

Botulinum toxin (BoNT) is highly effective in the treatment of cervical dystonia (CD), yet a significant proportion of patients report low levels of satisfaction following treatment and fail to follow up for repeated treatments. The goal of this study was to determine the reasons that some patients have unsatisfactory responses. A total of 35 subjects who came to our center requesting alternative treatments due to unsatisfactory responses following BoNT treatment for CD were evaluated. Included were 26 women and 9 men with an average age of 57.1 years (range 25-82 years), and an average duration of illness of 12.5 years (range 1-55 years). Details of unsatisfactory BoNT treatments were methodically collected by a movement specialist using a standardized intake form, including provider subspecialty, product used, the number of satisfactory or unsatisfactory trials, doses given, specific muscles treated, the use of electromyographic guidance, side effects, and tests of resistance. The specialist then provided repeat treatments if indicated, and followed each case until the reasons for unsatisfactory outcomes could be determined. Multiple reasons for unsatisfactory outcomes were found. They included suboptimal BoNT doses, suboptimal muscle targeting, intolerable side effects, complex movement patterns, discordant perceptions, and incorrect diagnoses. Only one patient was functionally resistant to BoNT. Of 32 subjects who received repeat BoNT treatments, 25 (78 %) achieved satisfactory responses after revision of the original treatment plan. These results indicate that the majority of unsatisfactory responses to BoNT treatment of CD were caused by correctible factors and imply a need for improved education regarding optimal treatment methods.

Chronic and acute biases in perceptual stabilization.

When perceptually ambiguous stimuli are presented intermittently, the percept on one presentation tends to be the same as that on the previous presentation. The role of short-term, acute biases in the production of this perceptual stability is relatively well understood. In addition, however, long-lasting, chronic bias may also contribute to stability. In this paper we develop indices for both biases and for stability, and show that stability can be expressed as a sum of contributions from the two types of bias. We then apply this analytical procedure to binocular rivalry, showing that adjustment of the monocular contrasts can alter the relative contributions of the two biases. Stability is mainly determined by chronic bias when the contrasts are equal, but acute bias dominates stability when right-eye contrast is set lower than left-eye contrast. Finally, we show that the right-eye bias persists in continuous binocular rivalry. Our findings reveal a previously unappreciated contribution of chronic bias to stable perception.

Ocular palatal tremor plus dystonia - new syndromic association.

OBJECTIVE: Ocular palatal tremor typically develops after a breach in the Guillian-Mollaret triangle. We herein describe a variant of this syndrome in which dystonia is also present, hence called, here, ocular palatal tremor plus dystonia. METHODS: We assessed eye-head movements and dystonia in six patients with ocular palatal plus dystonia. RESULTS: Among six patients with ocular palatal tremor two had focal dystonia, three had multifocal dystonia, and one had generalized dystonia. The dystonia affected the upper extremities and neck in four patients, the lower extremities in three and the face in two. Three out of four cervical dystonia patients had head tremor. Two patients also had speech involvement. Lack of correlation between eye and head oscillations suggested that head oscillations were not compensatory or secondary to the eye oscillations and vice versa. CONCLUSIONS: We describe a novel variant of ocular palatal tremor with dystonia. We speculate that in such variant the dystonia is possibly could be a result of abnormal cerebellar outflow in patients with a breach in Guillain-Mollaret triangle.

Temporal profile of improvement of tardive dystonia after globus pallidus deep brain stimulation.

BACKGROUND: Several case reports and small series have indicated that tardive dystonia is responsive to globus pallidus deep brain stimulation. Whether different subtypes or distributions of tardive dystonia are associated with different outcomes remains unknown. METHODS: We assessed the outcomes and temporal profile of improvement of eight tardive dystonia patients who underwent globus pallidus deep brain stimulation over the past six years through record review. Due to the retrospective nature of this study, it was not blinded or placebo controlled. RESULTS: Consistent with previous studies, deep brain stimulation improved the overall the Burke-Fahn-Marsden motor scores by 85.1 ± 13.5%. The distributions with best responses in descending order were upper face, lower face, larynx/pharynx, limbs, trunk, and neck. Patients with prominent cervical dystonia demonstrated improvement in the Toronto Western Spasmodic Torticollis Rating Scale but improvements took several months. In four patients the effects of deep brain stimulation on improvement in Burke Fahn Marsden score was rapid, while in four cases there was partial rapid response of neck and trunk dystonia followed by was gradual resolution of residual symptoms over 48 months. CONCLUSION: Our retrospective analysis shows excellent resolution of tardive dystonia after globus pallidus deep brain stimulation. We found instantaneous response, except with neck and trunk dystonia where partial recovery was followed by further resolution at slower rate. Such outcome is encouraging for using deep brain stimulation in treatment of tardive dystonia.

Cognitive correlates of hallucinations and delusions in Parkinson's disease.

BACKGROUND: Hallucinations and delusions that complicate Parkinson's disease (PD) could lead to nursing home placement and are linked to increased mortality. Cognitive impairments are typically associated with the presence of hallucinations but there are no data regarding whether such a relationship exists with delusions. OBJECTIVE: We hypothesized that hallucinations would be associated with executive and visuospatial disturbance. An exploratory examination of cognitive correlates of delusions was also completed to address the question of whether they differ from hallucinations. METHODS: 144 PD subjects completed a neuropsychological battery to assess cognition and the SAPS to examine psychosis. Correlational analyses assessed associations between hallucinations and delusions with cognitive domains. RESULTS: 48 subjects (33%) reported psychotic symptoms: 25 (17%) experienced hallucinations without delusions, 23 (16%) had symptoms dominated by delusions. Severity and/or number of hallucination subtypes were significantly correlated with lower scores in language, memory, attention, executive functioning, and visuospatial ability. Correlations with delusions were non-significant. Tests of differences in the size of the correlations between groups revealed a significant relationship between language and visuospatial performance with hallucinations. CONCLUSIONS: Cognitive correlates of hallucinations and delusions appear to be different in PD, suggesting distinct pathogenic mechanisms and possibly anatomical substrates. Hence, delusions may not share the same associations with dementia as hallucinations. Since this is a new finding, further studies will be needed to confirm our results.

Globus pallidus deep brain stimulation for adult-onset axial dystonia.

INTRODUCTION: Generalized dystonia, both primary and secondary forms, and axial dystonias such as tardive dystonia, and idiopathic cervical dystonia are responsive to globus pallidus interna (GPi) DBS. There is a paucity of investigations probing the impact of DBS on adult-onset axial dystonia. We assessed the efficacy of GPi DBS in four patients with rare adult-onset axial dystonia. METHODS: Primary outcome measure was improvement in the motor component of the Burke-Fahn-Marsden (BFM) rating scale. Secondary outcome measures were quality of life as determined by the SF-36 questionnaire, time to achieve best possible benefit and DBS parameters that accounted for the best response. In patients with prominent concomitant cervical dystonia we also used the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). RESULTS: GPi DBS improved BFM scores by 87.63 ± 11.46%. Improvement in total severity scale of TWSTRS was 71.5 ± 12.7%. Quality of life also remarkably improved as evidenced by 109.38 ± 82.97 and 7.05 ± 21.48% percent change in psychometrically-based physical component summary (PCS), and a mental component summary (MCS) score respectively. CONCLUSIONS: GPi DBS is a very effective treatment for adult-onset axial dystonia. Considering its refractoriness to medical therapy and significant impact on quality of life DBS should be considered for this disorder.

Measuring perception without introspection.

Binocular rivalry, the perceptual alternation between incompatible monocular stimuli, is conventionally measured by asking the subject which percept is currently visible. This is problematic because the response is unverifiable, open to response bias, and falsely assumes that the perceptual experience is binary. We overcame these limitations in a new approach that does not require subjective reporting of perceptual state. A brief test stimulus was added to one eye's inducing stimulus at random times and contrasts. The test was presented at one of two spatial locations, the subject indicated which alternative had been shown, and the correctness of the response was recorded as a function of test contrast. Given the random timing of the test stimulus, it was sometimes delivered when the tested eye was dominant and, at other times, suppressed. Accordingly, the psychometric function recorded during rivalry should be a mixture of the dominance and suppression forms of the function. This was indeed the case: The probability of a correct response during rivalry was significantly less than that obtained with a binocularly congruent stimulus. The psychometric function during rivalry was well modeled as a weighted sum of the congruence curve with an assumed suppression curve. Optimal fitting provided estimates of both suppression depth and percept predominance that corresponded closely with estimates obtained with the conventional method. We have therefore characterized rivalry without the uncertainties introduced by the subject's perceptual report. This provides a model that may be applicable to the broader field of perceptual ambiguity.

An ¹8F-FDG PET study of cervical muscle in parkinsonian anterocollis.

The underlying etiology of parkinsonian anterocollis has been the subject of recent debate. The purpose of this study is to test the hypothesis that anterocollis in parkinsonian syndromes is associated with dystonia of the deep cervical flexors (longus colli and capitis). Eight patients with anterocollis, six in the setting of parkinsonism and two primary cervical dystonia control subjects with anterocollis underwent prospective structured clinical evaluations (interview, examination and rating scales), systematic electromyography of the cervical extensor musculature and (18)F-FDG PET/CT studies of cervical muscles to examine evidence of hypermetabolism or overactivity of deep cervical flexors. Subjects with parkinsonian anterocollis were found to have hypermetabolism of the extensor and sub-occipital muscles but not in the cervical flexors (superficial or deep). EMG abnormalities were observed in all evaluated patients, but only one patient was definitely myopathic. Meanwhile, both dystonia controls exhibited hypermetabolism of cervical flexors (including the longus colli). In conclusion, we were able to demonstrate hypermetabolism of superficial and deep cervical flexors with muscle (18)F-FDG PET/CT in dystonic anterocollis patients, but not in parkinsonian anterocollis patients. The hypermetabolic changes seen in parkinsonian anterocollis patients in posterior muscles may be compensatory. Alternative explanations for anterocollis include myopathy of the cervical extensors, or unbalanced rigidity of the cervical flexors, but this remains to be proven.