Jeannette M. Gelauff

The prognosis of functional (psychogenic) motor symptoms: a systematic review

Background The prognosis of functional (or psychogenic) motor symptoms (weakness and movement disorder) has not been systematically reviewed. Methods We systematically reviewed PubMed for all studies of eight or more patients with functional motor symptoms reporting follow-up data longer than 6 months (excluding studies reporting specific treatments). We recorded symptom duration, physical and psychiatric comorbidity, disability, occupational functioning at follow-up and prognostic factors. Results 24 studies were included. There was heterogeneity regarding study size (number of patients (n)=10 491), follow-up duration clinical setting and data availability. Most studies (n=15) were retrospective. Reported symptom outcome was highly variable. Mean weighted follow-up duration was 7.4 years (in 13 studies where data was extractable). The mean percentage of patients same or worse at follow-up for all studies was 39%, range 10% to 90%, n=1134. Levels of physical disability and psychological comorbidity at follow-up were high. Short duration of symptoms, early diagnosis and high satisfaction with care predicted positive outcome in two studies. Gender had no effect. Delayed diagnosis and personality disorder were negatively correlated with outcome. Prognostic factors that varied between studies included age, comorbid anxiety and depression, IQ, educational status, marital status and pending litigation. Conclusions Existing follow-up studies of functional motor symptoms give us some insights regarding outcome and prognostic factors but are limited by their largely retrospective and selective nature. Overall, prognosis appears unfavourable. The severity and chronicity of functional motor symptoms argues for larger prospective studies including multiple prognostic factors at baseline in order to better understand their natural history.

Functional neurological disorders: mechanisms and treatment.

Functional neurological disorders are common problems in neurologic practice. In the past decade there has been an increasing interest in this group of disorders both from a clinical as well as research point of view. In this review, we highlight some of the most salient and exciting publications from recent years focusing especially on new findings illuminating mechanism and studies examining treatment.

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 < .001) and higher QoL (16 studies, n = 1504, g = 0.66, 95% confidence interval = 0.34–0.99, p < .001) directly posttreatment. Similar effect sizes were found at follow-up. A high risk of bias was established in most of the included studies. However, sensitivity analyses suggested that this did not significantly influence study results. Funnel plot asymmetry indicated potential publication bias. 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.

Electrophysiologic testing aids diagnosis and subtyping of myoclonus

Objective To determine the contribution of electrophysiologic testing in the diagnosis and anatomical classification of myoclonus. Methods Participants with a clinical diagnosis of myoclonus were prospectively recruited, each undergoing a videotaped clinical examination and battery of electrophysiologic tests. The diagnosis of myoclonus and its subtype was reviewed after 6 months in the context of the electrophysiologic findings and specialist review of the videotaped clinical examination. Results Seventy-two patients with myoclonus were recruited. Initial clinical anatomical classification included 25 patients with cortical myoclonus, 7 with subcortical myoclonus, 2 with spinal myoclonus, and 15 with functional myoclonic jerks. In 23 cases, clinical anatomical classification was not possible because of the complexity of the movement disorder. Electrophysiologic testing was completed in 66, with agreement of myoclonus in 60 (91%) and its subtype in 28 (47%) cases. Subsequent clinical review by a movement disorder specialist agreed with the electrophysiologic findings in 52 of 60; in the remaining 8, electrophysiologic testing was inconclusive. Conclusions Electrophysiologic testing is an important additional tool in the diagnosis and anatomical classification of myoclonus, also aiding in decision-making regarding therapeutic management. Further development of testing criteria is necessary to optimize its use in clinical practice.

The efficacy of the modified Atkins diet in North Sea Progressive Myoclonus Epilepsy : an observational prospective open-label study

BackgroundNorth Sea Progressive Myoclonus Epilepsy is a rare and severe disorder caused by mutations in the GOSR2 gene. It is clinically characterized by progressive myoclonus, seizures, early-onset ataxia and areflexia. As in other progressive myoclonus epilepsies, the efficacy of antiepileptic drugs is disappointingly limited in North Sea Progressive Myoclonus Epilepsy. The ketogenic diet and the less restrictive modified Atkins diet have been proven to be effective in other drug-resistant epilepsy syndromes, including those with myoclonic seizures. Our aim was to evaluate the efficacy of the modified Atkins diet in patients with North Sea Progressive Myoclonus Epilepsy.ResultsFour North Sea Progressive Myoclonus Epilepsy patients (aged 7–20 years) participated in an observational, prospective, open-label study on the efficacy of the modified Atkins diet. Several clinical parameters were assessed at baseline and again after participants had been on the diet for 3 months. The primary outcome measure was health-related quality of life, with seizure frequency and blinded rated myoclonus severity as secondary outcome measures.Ketosis was achieved within 2 weeks and all patients completed the 3 months on the modified Atkins diet. The diet was well tolerated by all four patients. Health-related quality of life improved considerably in one patient and showed sustained improvement during long-term follow-up, despite the progressive nature of the disorder. Health-related quality of life remained broadly unchanged in the other three patients and they did not continue the diet. Seizure frequency remained stable and blinded rating of their myoclonus showed improvement, albeit modest, in all patients.ConclusionsThis observational, prospective study shows that some North Sea Progressive Myoclonus Epilepsy patients may benefit from the modified Atkins diet with sustained health-related quality of life improvement. Not all our patients continued on the diet, but nonetheless we show that the modified Atkins diet might be considered as a possible treatment in this devastating disorder.

Functional Disorders in Neurology: Case Studies

Functional, often called psychogenic, disorders are common in neurological practice. We illustrate clinical issues and highlight some recent research findings using six case studies of functional neurological disorders. We discuss dizziness as a functional disorder, describing the relatively new consensus term Persistent Posturo-Perceptual Dizziness (PPPD), axial jerking/myoclonus as a functional movement disorder, functional speech symptoms, post-concussion disorder with functional cognitive symptoms and finally advances in treatment of dissociative seizures and functional motor disorders.

Fatigue, not self-rated motor symptom severity, affects quality of life in functional motor disorders

While fatigue is found to be an impairing symptom in functional motor disorders (FMD) in clinical practice, scientific evidence is lacking. We investigated fatigue severity and subtypes in FMD compared to organic neurological disease. Furthermore, the role of fatigue within FMD and its impact on quality of life and self-rated health were investigated. Data from 181 patients participating in the self-help on the internet for functional motor disorders, randomised Trial were included. Data from 217 neurological controls with neuromuscular disorders (NMD) originated from a historical cohort. Fatigue was measured using the checklist individual strength (CIS). Motor symptom severity, depression and anxiety were correlated to fatigue. For multivariable regression analyses, physical functioning and pain were additionally taken into account. Severe fatigue was, respectively, present in 78 and 53% of FMD and NMD patients (p < 0.001). FMD patients scored higher than NMD patients on all fatigue subdomains (p < 0.001). In the FMD group, fatigue subdomains were correlated to depression, anxiety and partly to motor symptom severity. Quality of life was negatively associated with fatigue [OR 0.93 (0.90–0.96), p < 0.001] and depression [OR 0.87 (0.81–0.93), p < 0.001], but not self-rated motor symptom severity. Self-rated health was negatively associated with fatigue [OR 0.92 (0.88–0.96), p < 0.001] and pain [OR 0.98 (0.97–0.99), p < 0.001]. Fatigue was found to be a prevalent problem in FMD, more so than in organic neurological disease. It significantly affected quality of life and self-rated health, while other factors such as motor symptom severity did not. Fatigue should be taken into account in clinical practice and treatment trials.

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 difficult to treat. Psychological treatments have shown modest effects, but these are costly, time consuming, and not easily accessible. Self-help interventions might overcome these barriers. The primary aim of this systematic review and meta-analysis was to assess the efficacy of self-help interventions for adults with MUS. Method Relevant studies were identified by searching electronic databases (PubMed, Embase, PsycINFO, and CINAHL from inception to May 2014) and scanning reference lists of included articles and related reviews. Two researchers independently selected randomised controlled trials comparing self-help to usual care or waiting list in adults with MUS. Data were extracted independently by two researchers using standardised forms. Standardised mean differences (Hedges’ g) were pooled using a random-effects model. Studies were critically appraised using the Cochrane ‘risk of bias assessment tool’. Outcomes were symptom severity and quality of life directly post-treatment and at follow-up. Results Out of 582 studies identified, 18 studies met all inclusion criteria. Studies were heterogeneous with regard to patient populations, intervention characteristics, and outcome measures. Compared to usual care or waiting list, self-help was associated with lower symptom severity (17 studies, n=1894, g=0.58, 95% CI 0.32–0.84, p<0.001) and higher QoL (16 studies, n=1504, g=0.66, 95% CI 0.34–0.99, p<0.001) directly post-treatment. Similar effect sizes were found at follow-up. A high risk of bias was established in the majority of included studies. However, sensitivity analyses suggested that this did not significantly influence study results. Funnel plot asymmetry indicated potential publication bias. Conclusion Self-help is associated with a significant reduction in symptom severity and improvement of QoL. Due to the suboptimal methodological quality of the majority of included studies, further research is needed to confirm the findings of this study. For future studies we recommend the use of uniform and validated measurement instruments, inclusion of follow-up assessments, and performance of intention-to-treat analyses.