Impaired visual processing may play a role in the pathophysiology of visual hallucinations in Parkinson's disease. In order to study involved neuronal circuitry, we assessed cerebral activation patterns both before and during recognition of gradually revealed images in Parkinson's disease patients with visual hallucinations (PDwithVHs), Parkinson's disease patients without visual hallucinations (PDnonVHs) and healthy controls. We hypothesized that, before image recognition, PDwithVHs would show reduced bottom-up visual activation in occipital-temporal areas and increased (pre)frontal activation, reflecting increased top-down demand. Overshoot of the latter has been proposed to play a role in generating visual hallucinations. Nine non-demented PDwithVHs, 14 PDnonVHs and 13 healthy controls were scanned on a 3 Tesla magnetic resonance imaging scanner. Static images of animals and objects gradually appearing out of random visual noise were used in an event-related design paradigm. Analyses were time-locked on the moment of image recognition, indicated by the subjects' button-press. Subjects were asked to press an additional button on a colour-changing fixation dot, to keep attention and motor action constant and to assess reaction times. Data pre-processing and statistical analysis were performed with statistical parametric mapping-5 software. Bilateral activation of the fusiform and lingual gyri was seen during image recognition in all groups (P < 0.001). Several seconds before image recognition, PDwithVHs showed reduced activation of the lateral occipital cortex, compared with both PDnonVHs and healthy controls. In addition, reduced activation of extrastriate temporal visual cortices was seen just before image recognition in PDwithVHs. The association between increased vulnerability for visual hallucinations in Parkinson's disease and impaired visual object processing in occipital and temporal extrastriate visual cortices supported the hypothesis of impaired bottom-up visual processing in PDwithVHs. Support for the hypothesized increased top-down frontal activation was not obtained. The finding of activation reductions in ventral/lateral visual association cortices in PDwithVHs before image recognition further helps to explain functional mechanisms underlying visual hallucinations in Parkinson's disease.

Background The Psychogenic Movement Disorders Rating Scale (PMDRS) has potential as a useful objective assessment in clinical research, but the current scale has limitations. We developed a simplified version (S‐FMDRS) and assessed inter‐rater reliability, concurrent validity, and sensitivity. Methods Fifty‐two videos of subjects with functional (psychogenic) movement disorders (FMD) were rated according to the PMDRS and S‐FMDRS by three neurologists. Inter‐rater reliability was assessed using intraclass correlation coefficient (ICC). Agreement of symptomatic body regions and movement disorder classification was assessed using Light's kappa. Spearman's correlation coefficient was used to assess concurrent validity. A physiotherapist also rated videos on the S‐FMDRS. The simplified scale was piloted in a feasibility study of physiotherapy for FMD to assess sensitivity. Results ICC of total scores was 0.84 for the original scale and 0.85 for the simplified scale. Light's kappa for agreement of symptomatic body regions and movement disorder classification was moderate to low. Concurrent validity was demonstrated by Spearman's correlation between the two scales ranging from 0.84 to 0.95. The simplified scale was sensitive to change, with an effect size in the feasibility study of 0.79. Inter‐rater reliability between physiotherapist and neurologist was high (ICC 0.85). Discussion Both versions of the scale had good inter‐rater reliability for the total score. Low agreement on movement disorder classification and identification of symptomatic body regions support our argument for a simplified scale. Conclusions The S‐FMDRS has high inter‐rater reliability and good sensitivity to change. Further psychometric evaluation is warranted.

Visual hallucinations (VH) are common in Parkinson's disease (PD) and are hypothesized to be due to impaired visual perception and attention deficits. We investigated whether PD patients with VH showed attention deficits, a more specific impairment of higher order visual perception, or both. Forty-two volunteers participated in this study, including 14 PD patients with VH, 14 PD patients without VH and 14 healthy controls (HC), matched for age, gender, education level and for level of executive function. We created movies with images of animals, people, and objects dynamically appearing out of random noise. Time until recognition of the image was recorded. Sustained attention was tested using the Test of Attentional Performance. PD patients with VH recognized all images but were significantly slower in image recognition than both PD patients without VH and HC. PD patients with VH showed decreased sustained attention compared to PD patients without VH who again performed worse than HC. In conclusion, the recognition of objects is intact in PD patients with VH; however, these patients where significantly slower in image recognition than patients without VH and HC, which was not explained by executive dysfunction. Both image recognition speed and sustained attention decline in PD, in a more progressive way if VH start to occur.

In our previous functional magnetic resonance imaging study, Parkinson's disease (PD) patients with visual hallucinations (VH) showed reduced activations in ventral/lateral visual association cortices preceding image recognition, compared with both PD patients without VH and healthy controls. The primary aim of the current study was to investigate whether functional deficits are associated with grey matter volume changes. In addition, possible grey matter differences between all PD patients and healthy controls were assessed. By using 3-Tesla magnetic resonance imaging (MRI) and voxel-based morphometry (VBM), we found no differences between PD patients with (n = 11) and without VH (n = 13). However, grey matter decreases of the bilateral prefrontal and parietal cortex, left anterior superior temporal, and left middle occipital gyrus were found in the total group of PD patients, compared with controls (n = 14). This indicates that previously demonstrated functional deficits in PD patients with VH are not associated with grey matter loss. The strong left parietal reduction in both nondemented patient groups was hemisphere specific and independent of the side of PD symptoms.

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Task-specific dystonia is a form of isolated focal dystonia with the peculiarity of being displayed only during performance of a specific skilled motor task. This distinctive feature makes task-specific dystonia a particularly mysterious and fascinating neurological condition. In this review, we cover phenomenology and its increasingly broad-spectrum risk factors for the disease, critically review pathophysiological theories and evaluate current therapeutic options. We conclude by highlighting the unique features of task-specific dystonia within the wider concept of dystonia. We emphasise the central contribution of environmental risk factors, and propose a model by which these triggers may impact on the motor control of skilled movement. By viewing task-specific dystonia through this new lens which considers the disorder a modifiable disorder of motor control, we are optimistic that research will yield novel therapeutic avenues for this highly motivated group of patients.

ObjectiveTo determine whether sensorimotor beta-frequency oscillatory power is raised during motor preparation in patients with functional movement disorders (FMD) and could therefore be a marker of abnormal “body-focused” attention.MethodsWe analyzed motor performance and beta-frequency cortical oscillations during a precued choice reaction time (RT) task with varying cue validity (50% or 95% congruence between preparation and go cues). We compared 21 patients with FMD with 13 healthy controls (HCs).ResultsIn HCs, highly predictive cues were associated with faster RT and beta desynchronization in the contralateral hemisphere (contralateral slope −0.045 [95% confidence interval (CI) −0.057 to −0.033] vs ipsilateral −0.033 [95% CI −0.046 to −0.021], p < 0.001) and with a tendency for reaching lower contralateral end-of-preparation beta power (contralateral −0.482 [95% CI −0.827 to −0.137] vs ipsilateral −0.328 [95% CI −0.673 to 0.016], p = 0.069). In contrast, patients with FMD had no improvement in RTs with highly predictive cues and showed an impairment of beta desynchronization and lateralization before movement.ConclusionsPersistent beta synchronization during motor preparation could reflect abnormal explicit control of movement in FMD. Excessive attention to movement itself rather than the goal might maintain beta synchronization and impair performance.

IntroductionDifferentiating between functional jerks (FJ) and organic myoclonus can be challenging. At present, the only advanced diagnostic biomarker to support FJ is the Bereitschaftspotential (BP). However, its sensitivity is limited and its evaluation subjective. Recently, event related desynchronisation in the broad beta range (13–45 Hz) prior to functional generalised axial (propriospinal) myoclonus was reported as a possible complementary diagnostic marker for FJ. Here we study the value of ERD together with a quantified BP in clinical practice.MethodsTwenty-nine patients with FJ and 16 patients with cortical myoclonus (CM) were included. Jerk-locked back-averaging for determination of the ‘classical’ and quantified BP, and time-frequency decomposition for the event related desynchronisation (ERD) were performed. Diagnostic gain, sensitivity and specificity were obtained for individual and combined techniques.ResultsWe detected a classical BP in 14/29, a quantitative BP in 15/29 and an ERD in 18/29 patients. At group level we demonstrate that ERD in the broad beta band preceding a jerk has significantly higher amplitude in FJ compared to CM (respectively −0.14 ± 0.13 and +0.04 ± 0.09 (p < 0.001)). Adding ERD to the classical BP achieved an additional diagnostic gain of 53%. Furthermore, when combining ERD with quantified and classical BP, an additional diagnostic gain of 71% was achieved without loss of specificity.ConclusionBased on the current findings we propose to the use of combined beta ERD assessment and quantitative BP analyses in patients with a clinical suspicion for all types of FJ with a negative classical BP.