Movement perception of the tonic vibration reflex is abnormal in functional limb weakness

Author(s):  
Michele Tinazzi ◽  
Angela Marotta ◽  
Mara Zenorini ◽  
Marianna Riello ◽  
Angelo Antonini ◽  
...  
Brain ◽  
2019 ◽  
Vol 142 (7) ◽  
pp. 2137-2148 ◽  
Author(s):  
Jeannette M Gelauff ◽  
Alan Carson ◽  
Lea Ludwig ◽  
Marina A J Tijssen ◽  
Jon Stone

Abstract 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.


2020 ◽  
Vol 32 (1) ◽  
pp. 50-57 ◽  
Author(s):  
Jon Stone ◽  
Charles Warlow ◽  
Ian Deary ◽  
Michael Sharpe

2015 ◽  
Vol 79 (3) ◽  
pp. 246-249 ◽  
Author(s):  
Lea Ludwig ◽  
Kimberley Whitehead ◽  
Michael Sharpe ◽  
Markus Reuber ◽  
Jon Stone

2018 ◽  
Vol 89 (7) ◽  
pp. 769-776 ◽  
Author(s):  
Ingrid Hoeritzauer ◽  
Alan J Carson ◽  
Jon Stone

ObjectiveIn their 1973 BMJ paper ‘Cryptogenic Drop Attacks’, Stevens and Matthews described 40, mostly middle-aged, female patients with drop attacks of unknown cause. Although clinically common, there has been little on this topic since. We aimed to determine clinical features, comorbidity and outcome of patients with drop attacks.MethodsWe carried out a retrospective review of patients with cryptogenic drop attacks seen consecutively by one clinician (JS) between 2006 and 2016. Demographics, phenomenology, duration and frequency of attacks, attack description and comorbid diagnoses were recorded. Patients were followed up with a notes review.Results83 patients with cryptogenic drop attacks were predominantly female (89%, n=79), mean age 44  years. The majority (93%, n=77) could not remember the fall itself and almost half (43%, n=36) experienced prodromal dissociative symptoms. Mechanical trips or syncope preceded drop attacks, historically, in 24% (n=20) of cases. Persistent fatigue (73%, n=61), chronic pain (40%, n=33), functional limb weakness (31%,n=26) and dissociative (non-epileptic) attacks 28% (n=23) were common, with the latter usually preceding or emerging from drop attacks. At follow-up (88%, mean 38 months), 28% (n=23) had resolution of their drop attacks. Predisposing (but non-causative) disease comorbidity was found at baseline (n=12) and follow-up (n=5).ConclusionsCryptogenic drop attacks are associated with high frequency of comorbid functional somatic and functional neurological disorders. Patients commonly have prodromal dissociative symptoms and in some there was a clear relationship with prior or subsequent dissociative (non-epileptic) attacks. Some cryptogenic drop attacks may be best understood as phenomena on the spectrum of dissociative attacks.


2017 ◽  
Vol 4 (4) ◽  
pp. 597-602 ◽  
Author(s):  
Dimitri Horn ◽  
Silvio Galli ◽  
Alexandre Berney ◽  
François Vingerhoets ◽  
Selma Aybek

2021 ◽  
pp. jnnp-2020-323953
Author(s):  
David L Perez ◽  
Mark J Edwards ◽  
Glenn Nielsen ◽  
Kasia Kozlowska ◽  
Mark Hallett ◽  
...  

Functional neurological disorder (FND) is a prevalent, disabling and costly condition at the neurology–psychiatry intersection. After being marginalised in the late 20th century, there has been renewed interest in this field. In this article, we review advances that have occurred over the past decade (2011–2020) across diagnosis, mechanisms, aetiologies, treatments and stigma in patients with motor FND (mFND, that is, functional movement disorder and functional limb weakness). In each content area, we also discuss the implications of recent advances and suggest future directions that will help continue the momentum of the past decade. In diagnosis, a major advance has been the emphasis on rule-in physical signs that are specific for hyperkinetic and hypokinetic functional motor symptoms. Mechanistically, greater importance has been given to determining ‘how’ functional neurological symptoms develop, highlighting roles for misdirected attention, expectation and self-agency, as well as abnormal influences of emotion/threat processing brain areas on motor control circuits. Aetiologically, while roles for adverse life experiences remain of interest in mFND, there is recognition of other aetiologic contributors, and efforts are needed to investigate links between aetiological factors and mechanisms. This decade has seen the first randomised controlled trials for physiotherapy, multidisciplinary rehabilitation and psychotherapy performed in the field, with consensus recommendations for physiotherapy, occupational therapy and outcome measures also published. Across patients, clinicians, healthcare systems and society, stigma remains a major concern. While challenges persist, a patient-centred integrated clinical neuroscience approach is primed to carry forward the momentum of the past decade into the future.


Author(s):  
Melinda L. Estes ◽  
Samuel M. Chou

Many muscle diseases show common pathological features although their etiology is different. In primary muscle diseases a characteristic finding is myofiber necrosis. The mechanism of myonecrosis is unknown. Polymyositis is a primary muscle disease characterized by acute and subacute degeneration as well as regeneration of muscle fibers coupled with an inflammatory infiltrate. We present a case of polymyositis with unusual ultrastructural features indicative of the basic pathogenetic process involved in myonecrosis.The patient is a 63-year-old white female with a one history of proximal limb weakness, weight loss and fatigue. Examination revealed mild proximal weakness and diminished deep tendon reflexes. Her creatine kinase was 1800 mU/ml (normal < 140 mU/ml) and electromyography was consistent with an inflammatory myopathy which was verified by light microscopy on biopsy muscle. Ultrastructural study of necrotizing myofiber, from the right vastus lateralis, showed: (1) degradation of the Z-lines with preservation of the adjacent Abands including M-lines and H-bands, (Fig. 1), (2) fracture of the sarcomeres at the I-bands with disappearance of the Z-lines, (Fig. 2), (3) fragmented sarcomeres without I-bands, engulfed by invading phagocytes, (Fig. 3, a & b ), and (4) mononuclear inflammatory cell infiltrate in the endomysium.


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