Functional Jerky Movements

Functional jerky movements (or functional myoclonus) are commonly seen in patients with functional movement disorder. Positive features both from history and clinical examination are important for the diagnosis. However, due to their heterogeneous and paroxysmal nature a home-made video-recording of the jerky movements can be essential to make the diagnose by a movement disorder specialist. Supportive clinical clues include abrupt symptom onset often triggered by a physical event, whereas entrainment and distractibility are supportive signs during physical examination. Localization of the jerky movements, especially proximal localization, is supportive, and can also be helpful in distinguishing functional jerks from tics and myoclonus, which are the most important differential diagnostic considerations. Additional neurophysiological tests include polymyography and electroencephalography-electromyography (EEG-EMG) co-registration in order to demonstrate a readiness potential (RP). Management includes disease education and specialized physical therapy

The prognosis of functional limb weakness, a 14-year case-control study

Reliable data on the prognosis of functional motor disorder are scarce, as existing studies of the prognosis of functional motor disorder are nearly all retrospective, small and uncontrolled. In this study we used a prospectively recruited, controlled cohort design to assess misdiagnosis, mortality and symptomatic and health outcome in patients with functional limb weakness compared to neurological disease and healthy control subjects. We also carried out an exploratory analysis for baseline factors predicting outcome. One hundred and seven patients with functional limb weakness, 46 neurological and 38 healthy control subjects from our previously studied prospective cohort were traced for follow-up after an average of 14 years. Misdiagnosis was determined in a consensus meeting using information from records, patients and their GPs. Numbers and causes of death were collected via death certificates. Outcome of limb weakness, physical and psychiatric symptoms, disability/quality of life and illness perception were recorded with self-rated questionnaires. Outcome measures were compared within and between groups. Seventy-six patients (71%) with functional limb weakness, 31 (67%) neurological and 23 (61%) healthy controls were included in follow-up. Misdiagnosis was found in one patient in the functional limb weakness group (1%) and in one neurological control (2%). Eleven patients with functional limb weakness, eight neurological control subjects and one healthy control subject had died. Weakness had completely remitted in 20% of patients in the functional limb weakness group and in 18% of the neurological controls (P = 0.785) and improved in a larger proportion of functional limb weakness patients (P = 0.011). Outcomes were comparable between patient groups, and worse than the healthy control group. No baseline factors were independent predictors of outcome, although somatization disorder, general health, pain and total symptoms at baseline were univariably correlated to outcome. This study is the largest and longest follow-up study of functional limb weakness. Misdiagnosis in functional limb weakness is rare after long-term follow-up. The disorder is associated with a higher mortality rate than expected, and symptoms are persistent and disabling. It appears difficult to predict outcome based on common baseline variables. These data should help inform clinicians to provide a more realistic outlook of the outcome and emphasize the importance of active and targeted therapy

Altered Posterior Midline Activity in Patients with Jerky and Tremulous Functional Movement Disorders

Objective: To explore changes in resting-state networks in patients with jerky and tremulous functional movement disorders (JT-FMD). Methods: Resting-state functional magnetic resonance imaging data from seventeen patients with JT-FMD and seventeen age-, sex-, and education-matched healthy controls (HC) were investigated. Independent component analysis was used to examine the central executive network (CEN), salience network, and default mode network (DMN). Frequency distribution of network signal fluctuations and intra- and internetwork functional connectivity were investigated. Symptom severity was measured using the Clinical Global Impression-Severity scale. Beck Depression Inventory and Beck Anxiety Inventory scores were collected tomeasure depression and anxiety in FMD, respectively. Results: Compared with HC, patients with JT-FMD had significantly decreased power of lower range (0.01-0.10 Hz) frequency fluctuations in a precuneus and posterior cingulate cortex component of the DMN and in the dorsal attention network (DAN) component of the CEN (false discovery rate-corrected p < 0.05). No significant group differences were found for intra- and internetwork functional connectivity. In patients with JT-FMD, symptom severity was not significantly correlated with network measures. Depression scores were weakly correlated with intranetwork functional connectivity in the medial prefrontal cortex, while anxiety was not found to be related to network connectivity. Conclusions: Given the changes in the posterodorsal components of the DMN and DAN, we postulate that the JT-FMD-related functional alterations found in these regions could provide support for the concept that particularly attentional dysregulation is a fundamental disturbance in these patients

The chronnectome as a model for Charcot's 'dynamic lesion' in functional movement disorders

This exploratory study set out to investigate dynamic functional connectivity (dFC) in patients with jerky and tremulous functional movement disorders (JT-FMD). The focus in this work is on dynamic brain states, which represent distinct dFC patterns that reoccur in time and across subjects. Resting-state fMRI data were collected from 17 patients with JT-FMD and 17 healthy controls (HC). Symptom severity was measured using the Clinical Global Impression-Severity scale. Depression and anxiety were measured using the Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI), respectively. Independent component analysis was used to extract functional brain components. After computing dFC, dynamic brain states were determined for every subject using k-means clustering. Compared to HC, patients with JT-FMD spent more time in a state that was characterized predominantly by increasing medial prefrontal, and decreasing posterior midline connectivity over time. They also tended to visit this state more frequently. In addition, patients with JT-FMD transitioned significantly more often between different states compared to HC, and incorporated a state with decreasing medial prefrontal, and increasing posterior midline connectivity in their attractor, i.e., the cyclic patterns of state transitions. Altogether, this is the first study that demonstrates altered functional brain network dynamics in JT-FMD that may support concepts of increased self-reflective processes and impaired sense of agency as driving factors in FMD

Treatment of functional motor disorders

OPINION STATEMENT: For the treatment of functional motor disorder, we recommend a three-stage approach. Firstly, patients must be assessed and given an unambiguous diagnosis, with an explanation that helps them understand that they have a genuine disorder, with the potential for reversibility. A key ingredient is allowing the patients to describe all of their symptoms as well as their ideas about what may be wrong. The patient should clearly understand that the positive diagnosis is based on the presence of typical signs (e.g., Hoover's sign for paralysis, entrainment test for tremor) that, in and of themselves, indicate the potential for reversibility. We suggest an approach that avoids the assumption that psychological stressors in the patient's life are causing the symptoms. The symptoms themselves are often the main stressor. Insisting that there must be others often leads to a frustrated doctor and an angry patient. Rather, at this initial stage, we encourage exploration of mechanisms - e.g., triggering of symptoms by pain, injury, or dissociation - and a discussion of how symptoms manifest as "abnormal motor programs" in the nervous system.Secondly, further time spent exploring the diagnosis, treating comorbidity, and, in the context of a multidisciplinary team, experimenting with altered movements and behaviors may benefit some patients, without the need for more complex intervention.Thirdly, some patients do require more complex treatment, often with a combination of physical rehabilitation and psychological treatments. Hypnosis, sedation, and transcranial magnetic stimulation may have a role in select patients.Finally, although they have confidence in the diagnosis, many patients do not respond to treatment. Ultimately, however, patients with functional motor disorder may have much greater potential for recovery than health professionals often consider

Self-help for medically unexplained symptoms: a systematic review and meta-analysis

Objective: Medically unexplained symptoms (MUS), which are highly prevalent in all fields of medicine, are considered difficult to treat. The primary objective of this systematic review and meta-analysis was to assess the efficacy of self-help for adults with MUS. Methods: Four electronic databases were searched for relevant studies. Randomized controlled trials comparing self-help to usual care or waiting list in adults with MUS were selected. Studies were critically appraised using the Cochrane "risk of bias assessment tool." Standardized mean differences (Hedges g) were pooled using a random-effects model. Outcomes were symptom severity and quality of life (QoL) directly posttreatment and at follow-up. Results: Of 582 studies identified, 18 studies met all inclusion criteria. Studies were heterogeneous with regard to patient populations, intervention characteristics, and outcome measures. Compared with usual care or waiting list, self-help was associated with lower symptom severity (17 studies, n = 1894, g = 0.58, 95% confidence interval = 0.32-0.84, p Conclusions: Self-help is associated with a significant reduction in symptom severity and improvement of QoL. The methodological quality of included studies was suboptimal, and further research is needed to confirm the findings of this meta-analysis