‘Cryptogenic Drop Attacks’ revisited: evidence of overlap with functional neurological disorder

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.

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

Brain ◽

10.1093/brain/awz138 ◽

2019 ◽

Vol 142 (7) ◽

pp. 2137-2148 ◽

Cited By ~ 16

Author(s):

Jeannette M Gelauff ◽

Alan Carson ◽

Lea Ludwig ◽

Marina A J Tijssen ◽

Jon Stone

Keyword(s):

Psychiatric Symptoms ◽

Illness Perception ◽

Control Group ◽

Motor Disorder ◽

Limb Weakness ◽

Control Subjects ◽

Healthy Control ◽

Baseline Factors ◽

Functional Limb

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.

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Functional neurological disorders in Parkinson disease

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2017-317378 ◽

2018 ◽

Vol 89 (6) ◽

pp. 566-571 ◽

Cited By ~ 24

Author(s):

Benjamin D Wissel ◽

Alok K Dwivedi ◽

Aristide Merola ◽

Danielle Chin ◽

Cara Jacob ◽

...

Keyword(s):

Parkinson Disease ◽

Neurological Disorders ◽

Resource Utilisation ◽

Motor Disability ◽

Healthcare Resource Utilisation ◽

The Usa ◽

Neurological Features ◽

Functional Features ◽

Predominantly Female ◽

Functional Neurological Disorders

ObjectiveTo ascertain demographic and clinical features of Parkinson disease (PD) associated with functional neurological features.MethodsA standardised form was used to extract data from electronic records of 53 PD patients with associated functional neurological disorders (PD-FND) across eight movement disorders centres in the USA, Canada and Europe. These subjects were matched for age, gender and disease duration to PD patients without functional features (PD-only). Logistic regression analysis was used to compare both groups after adjusting for clustering effect.ResultsFunctional symptoms preceded or co-occurred with PD onset in 34% of cases, nearly always in the most affected body side. Compared with PD-only subjects, PD-FND were predominantly female (68%), had longer delay to PD diagnosis, greater prevalence of dyskinesia (42% vs 18%; P=0.023), worse depression and anxiety (P=0.033 and 0.025, respectively), higher levodopa-equivalent daily dose (972±701 vs 741±559 mg; P=0.029) and lower motor severity (P=0.019). These patients also exhibited greater healthcare resource utilisation, higher use of [(123)I]FP-CIT SPECT and were more likely to have had a pre-existing psychiatric disorder (P=0.008) and family history of PD (P=0.036).ConclusionsA subtype of PD with functional neurological features is familial in one-fourth of cases and associated with more psychiatric than motor disability and greater use of diagnostic and healthcare resources than those without functional features. Functional manifestations may be prodromal to PD in one-third of patients.

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Functional neurological disorders in patients with brain tumours

Neuro-Oncology ◽

10.1093/neuonc/noab195.026 ◽

2021 ◽

Vol 23 (Supplement_4) ◽

pp. iv12-iv12

Author(s):

Charmaine Toh ◽

Dorothy Joe ◽

Katia Cikurel ◽

Julia Johnson ◽

Francesco Vergani ◽

...

Keyword(s):

Brain Tumour ◽

Neurological Disorders ◽

Neurological Disorder ◽

Brain Tumours ◽

Neurological Symptoms ◽

Adult Brain ◽

Case Review ◽

Functional Neurological Symptoms ◽

Functional Neurological Disorders ◽

Functional Neurological Disorder

Abstract Aims Signs and symptoms that develop in people with brain tumours are often attributed to their tumour. The prevalence and management of functional neurological symptoms in brain tumour patients have received little attention. This is surprising because functional neurological symptoms complicate management greatly and misdiagnosis can lead to inappropriate treatment and iatrogenic side-effects. Therefore, we investigated the presentation, diagnosis and management of functional neurological disorders (FND) in patients who had a brain or meningeal tumour. Method A retrospective case review was performed from 2017 - 2021 to identify adult brain tumour patients who developed a functional neurological disorder that caused significant disability necessitating expedited investigations. All patients attended a regional neuro-oncology centre. We recorded type of brain tumour and diagnostic investigations. The onset of functional symptoms was divided into three time windows: before tumour diagnosis, after diagnosis and before treatment or after tumour treatment. A neuropsychological review looked for evidence of previous adverse life events. Therapeutic interventions for functional neurological disorder and their outcomes were documented. The case review was combined with a systematic review of the literature to identify the published presentations of functional neurological disorder in the adult brain tumour population. MEDLINE, EMBASE and PsycINFO databases were searched for studies published between January 1980 and February 2021. Results Six patients (5 female, 1 male) were identified from the case review with a median age of 41 (range 29 - 56) years old. Four patients had non-epileptic attack disorder, which was diagnosed with videotelemetry of habitual attacks. One patient had a functional hemiparesis with normal central motor conduction time. One patient had a functional speech disorder with normal EEG. Half of these patients had functional neurological symptoms prior to surgery/oncological treatment. Five patients (83%) were referred for further neuropsychiatric or psychological evaluation. A history of significant psychological trauma prior to the brain tumour diagnosis was elicited in four (66%) patients. Conclusion Patients with either a brain or meningeal tumour may develop functional neurological symptoms. Our findings suggest the possibility that diagnosis of a brain tumour may precipitate a debilitating functional neurological disorder. The neurobiological basis for functional neurological disorders is being actively investigated. There are suggestions in the literature that some brain diseases increase the risk of developing a functional neurological disorder. Further work is needed to determine whether this is true for patients with brain tumours. Increased awareness of functional neurological disorders will improve management. Withdrawal of unnecessary treatment, such as anticonvulsant drugs, reduces the risk of iatrogenic side effects. Initiation of multi-disciplinary care pathways, e.g. physiotherapy, speech and language therapy and psychological treatments, promotes recovery. Collectively, these interventions improve our patients’ quality of life.

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The vicious cycle of functional neurological disorders: a synthesis of healthcare professionals’ views on working with patients with functional neurological disorder

Disability and Rehabilitation ◽

10.1080/09638288.2020.1822935 ◽

2020 ◽

pp. 1-10

Author(s):

Caroline Barnett ◽

Rebecca Davis ◽

Claire Mitchell ◽

Sarah Tyson

Keyword(s):

Neurological Disorders ◽

Neurological Disorder ◽

Healthcare Professionals ◽

Vicious Cycle ◽

Functional Neurological Disorders ◽

Functional Neurological Disorder

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Functional Neurological Disorders as Seen by a Cohort of General Practitioners in Northern Italy: Evidence From an Online Survey

Frontiers in Neurology ◽

10.3389/fneur.2021.583672 ◽

2021 ◽

Vol 12 ◽

Author(s):

Angela Marotta ◽

Mirta Fiorio ◽

Isabella Fracasso ◽

Carlo Andrea Franchini ◽

Giovanni Defazio ◽

...

Keyword(s):

General Practitioners ◽

Professional Education ◽

Neurological Disorders ◽

Online Survey ◽

Northern Italy ◽

Web Based ◽

Novel Approaches ◽

Psychological View ◽

Functional Neurological Disorders ◽

Functional Neurological Disorder

General practitioners (GPs) provide primary care and advise their patients on which diagnostic and therapeutic pathways they judge most appropriate. For patients with functional neurological disorders (FND), receiving a proper explanation of diagnosis by their GP from the very beginning may drastically improve prognosis. Novel approaches to the diagnosis and treatment of FND have important implications for effective management. The aim of this study was to investigate Italian GP opinion and knowledge about FND in light of new approaches to the illness. To do this, we evaluated the responses to a 13-item web-based survey completed by 133 GPs practicing in northern Italy. Psychological terms to describe FND were more frequently used than functional neurological disorder and mental illness was considered an important predictor of diagnosis. Referral to a neurologist rather than to a psychiatrist was largely preferred, while physiotherapy consultation was seldom recognized as a valuable approach to treating FND. Overall, the survey findings suggest that knowledge about novel approaches to FND is somewhat lacking. Currently, GPs appear to be transitioning from a classical psychological view of the disorder toward a more modern conceptualization, in which neurobiological, psychological, and social factors all play an important role. Professional education during this transition would be an advantageous way to optimize physician management of FND and to enhance diagnosis, explanation, and management across primary and secondary care pathways.

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The Arc de Siècle: functional neurological disorder during the ‘forgotten’ years of the 20th century

Brain ◽

10.1093/brain/awaa037 ◽

2020 ◽

Vol 143 (4) ◽

pp. 1278-1284

Author(s):

Max Fend ◽

Louise Williams ◽

Alan J Carson ◽

Jon Stone

Keyword(s):

20Th Century ◽

Neurological Disorders ◽

Neurological Disorder ◽

Oral Histories ◽

Pragmatic Approach ◽

Functional Neurological Disorders ◽

Functional Neurological Disorder

Using archived records, plus oral histories from retired neurologists, Fend et al. examine attitudes to functional neurological disorders in the mid-20th century. While frequently scathing in their correspondence, most neurologists of the time adopted a pragmatic approach with many referring patients to psychiatrists.

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You’ve made the diagnosis of functional neurological disorder: now what?

Practical Neurology ◽

10.1136/practneurol-2017-001835 ◽

2018 ◽

Vol 18 (4) ◽

pp. 323-330 ◽

Cited By ~ 13

Author(s):

Caitlin Adams ◽

Jordan Anderson ◽

Elizabeth N Madva ◽

W Curt LaFrance Jr ◽

David L Perez

Keyword(s):

Clinical Care ◽

Conversion Disorder ◽

Psychosocial Stressors ◽

Illness Beliefs ◽

Clinical Encounters ◽

Clinical Vignettes ◽

Outpatient Visits ◽

Functional Neurological Disorders ◽

Functional Neurological Disorder

Patients with functional neurological disorders (FND)/conversion disorder commonly present to outpatient clinics. FND is now a ‘rule in’ diagnosis based on neurological examination findings and semiological features. While neurologists may be more comfortable diagnosing patients with FND, there is only limited guidance as to how to conduct follow-up outpatient visits. Using clinical vignettes, we provide practical suggestions that may help guide clinical encounters including how to: (1) explore illness beliefs openly; (2) enquire longitudinally about predisposing vulnerabilities, acute precipitants and perpetuating factors that may be further elucidated over time; (3) facilitate psychotherapy engagement by actively listening for potentially unhelpful or maladaptive patterns of thoughts, behaviours, fears or psychosocial stressors that can be reflected back to the patient and (4) enquire about the fidelity of individual treatments and educate other providers who may be less familiar with FND. These suggestions, while important to individualise, provide a blueprint for follow-up FND clinical care.

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Decade of progress in motor functional neurological disorder: continuing the momentum

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2020-323953 ◽

2021 ◽

pp. jnnp-2020-323953

Author(s):

David L Perez ◽

Mark J Edwards ◽

Glenn Nielsen ◽

Kasia Kozlowska ◽

Mark Hallett ◽

...

Keyword(s):

Neurological Disorder ◽

Major Advance ◽

Limb Weakness ◽

Late 20Th Century ◽

Functional Movement ◽

The Past ◽

Control Circuits ◽

Functional Limb ◽

Randomised Controlled ◽

Functional Neurological Disorder

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.

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