Measuring persistent somatic symptom related stigmatisation: Development of the Persistent Somatic Symptom Stigma scale for Healthcare Professionals (PSSS-HCP).

OBJECTIVE: Persistent somatic symptoms (PSS) describe recurrent or continuously occurring symptoms such as fatigue, dizziness, or pain that have persisted for at least several months. These include single symptoms such as chronic pain, combinations of symptoms, or functional disorders such as fibromyalgia or irritable bowel syndrome. While stigmatisation by healthcare professionals is regularly reported, there are limited measurement instruments demonstrating content validity. This study develops a new instrument to measure stigmatisation by healthcare professionals, the Persistent Somatic Symptom Stigma scale for Healthcare Professionals (PSSS-HCP). METHODS: Development was an iterative process consisting of research team review, item generation and cognitive interviewing. We generated a longlist of 60 items from previous reviews and qualitative research. We conducted 18 cognitive interviews with healthcare professionals in the United Kingdom (UK). We analysed the relevance, comprehensibility and comprehensiveness of items, including the potential for social desirability bias. RESULTS: After research team consensus and initial feedback, we retained 40 items for cognitive interviewing. After our first round of interviews (n = 11), we removed 20 items, added three items and amended five items. After our second round of interviews (n = 7), we removed four items and amended three items. No major problems with relevance, comprehensibility, comprehensiveness or social desirability were found in remaining items. CONCLUSIONS: The provisional version of the PSSS-HCP contains 19 items across three domains (stereotypes, prejudice, discrimination), demonstrating sufficient content validity. Our next step will be to perform a validation study to finalise item selection and explore the structure of the PSSS-HCP.

Pain and functional neurological disorder: a systematic review and meta-analysis.

BACKGROUND: Functional neurological disorder (FND) is characterised by neurological symptoms, such as seizures and abnormal movements. Despite its significance to patients, the clinical features of chronic pain in people with FND, and of FND in people with chronic pain, have not been comprehensively studied. METHODS: We systematically reviewed PubMed, Embase and PsycINFO for studies of chronic pain in adults with FND and FND in patients with chronic pain. We described the proportions of patients reporting pain, pain rating and timing, pain-related diagnoses and responsiveness to treatment. We performed random effects meta-analyses of the proportions of patients with FND who reported pain or were diagnosed with pain-related disorders. RESULTS: Seven hundred and fifteen articles were screened and 64 were included in the analysis. Eight case-control studies of 3476 patients described pain symptoms in a higher proportion of patients with FND than controls with other neurological disorders. A random effects model of 30 cohorts found that an estimated 55% (95% CI 46% to 64%) of 4272 patients with FND reported pain. Random effects models estimated diagnoses of complex regional pain syndrome in 22% (95% CI 6% to 39%) of patients, irritable bowel syndrome in 16% (95% CI 9% to 24%) and fibromyalgia in 10% (95% CI 8% to 13%). Five studies of FND diagnoses among 361 patients with chronic pain were identified. Most interventions for FND did not ameliorate pain, even when other symptoms improved. CONCLUSIONS: Pain symptoms and pain-related diagnoses are common in FND. Classification systems and treatments should routinely consider pain as a comorbidity in patients with FND.

Anomalies in the review process and interpretation of the evidence in the NICE guideline for chronic fatigue syndrome and myalgic encephalomyelitis.

Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is a disabling long-term condition of unknown cause. The National Institute for Health and Care Excellence (NICE) published a guideline in 2021 that highlighted the seriousness of the condition, but also recommended that graded exercise therapy (GET) should not be used and cognitive-behavioural therapy should only be used to manage symptoms and reduce distress, not to aid recovery. This U-turn in recommendations from the previous 2007 guideline is controversial.We suggest that the controversy stems from anomalies in both processing and interpretation of the evidence by the NICE committee. The committee: (1) created a new definition of CFS/ME, which 'downgraded' the certainty of trial evidence; (2) omitted data from standard trial end points used to assess efficacy; (3) discounted trial data when assessing treatment harm in favour of lower quality surveys and qualitative studies; (4) minimised the importance of fatigue as an outcome; (5) did not use accepted practices to synthesise trial evidence adequately using GRADE (Grading of Recommendations, Assessment, Development and Evaluations trial evidence); (6) interpreted GET as mandating fixed increments of change when trials defined it as collaborative, negotiated and symptom dependent; (7) deviated from NICE recommendations of rehabilitation for related conditions, such as chronic primary pain and (8) recommended an energy management approach in the absence of supportive research evidence.We conclude that the dissonance between this and the previous guideline was the result of deviating from usual scientific standards of the NICE process. The consequences of this are that patients may be denied helpful treatments and therefore risk persistent ill health and disability.

Long-term mental wellbeing and functioning after surgery for cauda equina syndrome.

INTRODUCTION: Cauda Equina Syndrome (CES) can cause persisting life-changing dysfunction. There is scarce literature regarding the long-term assessment of CES symptoms, and rarer still is the impact of these symptoms on mental wellbeing investigated. This study assessed the long-term patient reported mental wellbeing outcomes of post-operative CES patients. METHODS: Patients who underwent surgery for CES between August 2013 and November 2014 were identified using an ethically approved database. They then completed validated questionnaires over the telephone assessing their mental and physical functioning (Short-Form 12 Questionnaire), generating the Physical Component Summary (PCS) and Mental Component Summary (MCS). Bladder, bowel and sexual function were also assessed using validated questionnaires. MCS scores were compared to both the Scottish mean and previously published cut-offs indicating patients at risk of depression. Correlations of MCS with bladder, bowel, sexual and physical dysfunction were examined and multifactorial regression to predict MCS from these variables analysed. Independent t-tests assessed the mean difference in MCS between patients presenting with incomplete CES (CES-I) and CES with retention (CES-R) and between those with radiologically confirmed and impending CES. RESULTS: Forty-six participants with a mean follow-up time of 43 months completed the study. The mean (±SD) MCS was 49 (±11.8) with 22% demonstrating poor mental health related quality of life in comparison to the Scottish mean. Overall, 37% had scores consistent with being at risk for depression with in the last 30 days, and 45% within the last 12 months. MCS was significantly correlated with Urinary Symptoms Profile (USP) score (-0.608), NBDS score (-0.556), ASEX score (-0.349) and PCS score (0.413) with worse bladder, bowel, sexual and physical dysfunction associated with worse MCS score. Multifactorial regression analysis demonstrated both urinary (USP score p = 0.031) and bowel function (NBDS score p = 0.009) to be significant predictive variables of mental health related quality of life. There were no significant mean differences in MCS between those presenting with CES-I and CES-R or those with radiologically complete and impending CES. DISCUSSION: This study demonstrates a high frequency of being at risk for depression in patients with CES and identifies outcome measures (physical, sexual and more so bladder and bowel dysfunction) associated with poorer mental wellbeing. Our large cohort and long follow-up highlight that CES patients should be considered at risk of depression, and the need to consider mental health outcomes following CES surgery.

Validation of the PHQ-9 in adults with dissociative seizures.

BACKGROUND: The PHQ-9 is a self-administered depression screening instrument. Little is known about its utility and accuracy in detecting depression in adults with dissociative seizures (DS). OBJECTIVES: Using the Mini - International Neuropsychiatric Interview as a reference, we evaluated the diagnostic accuracy of the PHQ-9 in adults with DS, and examined its convergent and discriminant validity and uniformity. METHODS: Our sample comprised 368 people with DS who completed the pre-randomisation assessment of the CODES trial. The uniformity of the PHQ-9 was determined using factor analysis for categorical data. Optimal cut-offs were determined using the area under the curve (AUC), Youden Index, and diagnostic odds ratio (DOR). Convergent and discriminant validity were assessed against pre-randomisation measures. RESULTS: Internal consistency of the PHQ-9 was high (α = 0.87). While the diagnostic odds ratio suggested that a cut-off of ≥10 had the best predictive performance (DOR = 14.7), specificity at this cut off was only 0.49. AUC (0.74) and Youden Index (0.48) suggested a ≥ 13 cut-off would yield an optimal sensitivity (0.81) and specificity (0.67) balance. However, a cut-off score of ≥20 would be required to match specificity resulting from a cut-off of ≥13 in other medical conditions. We found good convergent and discriminant validity and one main factor for the PHQ-9. CONCLUSIONS: In terms of internal consistency and structure, our findings were consistent with previous validation studies but indicated that a higher cut-off would be required to identify DS patients with depression with similar specificity achieved with PHQ-9 screening in different clinical and non-clinical populations.

A Review and Expert Opinion on the Neuropsychiatric Assessment of Motor Functional Neurological Disorders.

Functional neurological (conversion) disorder (FND) is a prevalent and disabling condition at the intersection of neurology and psychiatry. Advances have been made in elucidating an emerging pathophysiology for motor FND, as well as in identifying evidenced-based physiotherapy and psychotherapy treatments. Despite these gains, important elements of the initial neuropsychiatric assessment of functional movement disorders (FND-movt) and functional limb weakness/paresis (FND-par) have yet to be established. This is an important gap from both diagnostic and treatment planning perspectives. In this article, the authors performed a narrative review to characterize clinically relevant variables across FND-movt and FND-par cohorts, including time course and symptom evolution, precipitating factors, medical and family histories, psychiatric comorbidities, psychosocial factors, physical examination signs, and adjunctive diagnostic tests. Thereafter, the authors propose a preliminary set of clinical content that should be assessed during early-phase patient encounters, in addition to identifying physical signs informing diagnosis and potential use of adjunctive tests for challenging cases. Although clinical history should not be used to make a FND diagnosis, characteristics such as acute onset, precipitating events (e.g., injury and surgery), and a waxing and waning course (including spontaneous remissions) are commonly reported. Active psychiatric symptoms (e.g., depression and anxiety) and ongoing psychosocial stressors also warrant evaluation. Positive physical examination signs (e.g., Hoover's sign and tremor entrainment) are key findings, as one of the DSM-5 diagnostic criteria. The neuropsychiatric assessment proposed emphasizes diagnosing FND by using "rule-in" physical signs while also considering psychiatric and psychosocial factors to aid in the development of a patient-centered treatment plan.

Does a history of lumbar spine surgery predict radiological cauda equina compression in patients undergoing MRI for suspected cauda equina syndrome?

Background: The clinical symptoms and signs of Cauda equina syndrome (CES) are non specific and poorly predictive of cauda equina compression on MRI. We aimed to establish whether a history of lumbar spine surgery predicts cauda equina compression on MRI in those presenting with suspected CES.Methods: A retrospective electronic record review was undertaken of 276 patients referred with clinically suspected CES who underwent a lumbosacral spine MRI. Those with a history of prior lumbar surgery were compared to those without. The likelihood of cauda equina compression was compared between the two groups.Results: About 78/276 (28%) patients with suspected CES had radiological compression of the cauda equina and went on to surgical decompression. A total of 54 (20%) patients had undergone prior lumbar surgery. Patients with a history of lumbar surgery were less likely to have cauda equina compression on MRI (χ2 - p = .035). Twenty six (9%) patients presented more than once with suspected CES. Patients with a history of lumbar surgery were more likely to re-present with suspected CES (χ2 - p = .002).Conclusions: Prior lumbar surgery was associated with a higher frequency of re-presentation with clinically suspected CES but a lower frequency of radiological cauda equina compression.

Screening for functional neurological disorders by questionnaire.

OBJECTIVE: Diagnostic screening for functional neurological disorders (FNDs) continues to pose a challenge. Simple symptom counts fail clearly to discriminate patients with FND but there is increasing recognition of 'positive' features which are useful diagnostically during face-to-face assessments. A self-completed questionnaire evaluating specific features of FNDs would be useful for screening purposes in clinical and research settings. METHODS: The Edinburgh Neurosymptoms Questionnaire (ENS) is a 30-item survey of presence and nature of: blackouts, weakness, hemisensory syndrome, memory problems, tremor, pain, fatigue, globus, multiple medical problems, and operations constructed via literature review and expert consensus. We conducted a pilot of the ENS on new general neurology clinic attendees at a large regional neuroscience centre. Patients were grouped according to consultant neurologist impression as having symptoms that were 'Not at all', 'Somewhat', 'Largely' or 'Completely' due to a functional disorder. RESULTS: Blackouts, weakness and memory questions provided reasonable diagnostic utility (AUROC = 0.94, 0.71, 0.74 respectively) in single symptom analysis. All other symptoms lacked discriminating features. A multivariate linear model with all symptoms predicted functional classification with moderate diagnostic utility (AUROC = 0.83), specificity of 0.97, sensitivity of 0.47. Pain and blackout scores provided the most accurate predictor of functional classification. CONCLUSION: The ENS questionnaire provides some utility in differentiating patients presenting with functional blackouts but failed to provide diagnostic value in other types of FND, highlighting the limitations of this self-report tool.

Understanding foreign accent syndrome.

OBJECTIVE: Foreign accent syndrome (FAS) is widely understood as an unusual consequence of structural neurological damage, but may sometimes represent a functional neurological disorder. This observational study aimed to assess the prevalence and utility of positive features of functional FAS in a large group of individuals reporting FAS. METHODS: Participants self-reporting FAS recruited from informal unmoderated online support forums and via professional networks completed an online survey. Speech samples were analysed in a subgroup. RESULTS: Forty-nine respondents (24 UK, 23 North America, 2 Australia) reported FAS of mean duration 3 years (range 2 months to 18 years). Common triggers were: migraine/severe headache (15), stroke (12), surgery or injury to mouth or face (6) and seizure (5, including 3 non-epileptic). High levels of comorbidity included migraine (33), irritable bowel syndrome (17), functional neurological disorder (12) and chronic pain (12). Five reported structural lesions on imaging. Author consensus on aetiology divided into, 'probably functional (n=35.71%), 'possibly structural' (n=4.8%) and 'probably structural' (n=10.20%), but positive features of functional FAS were present in all groups. Blinded analysis of speech recordings supplied by 13 respondents correctly categorised 11 (85%) on the basis of probable aetiology (functional vs structural) in agreement with case history assignment. CONCLUSIONS: This largest case series to date details the experience of individuals with self-reported FAS. Although conclusions are limited by the recruitment methods, high levels of functional disorder comorbidity, symptom variability and additional linguistic and behavioural features suggest that chronic FAS may in some cases represent a functional neurological disorder, even when a structural lesion is present.

Clinicopathological case: progressive somnolence and dementia in an accountant: when the shine rubs off the gold standard.

A 63-year-old accountant developed progressive somnolence, cognitive decline, gait disturbance and cerebellar dysfunction with autonomic features. This report documents the clinicopathological conference at the 39th Edinburgh Advanced Neurology Course 2017.

Fowler's syndrome of urinary retention: A retrospective study of co-morbidity.

AIMS: To study the frequency of pain, psychological, or functional disorders in patients with Fowler's syndrome. METHODS: We carried out a retrospective chart review of patients with a diagnosis of Fowler's syndrome attending the Uro-Neurology centre at the National Hospital for Neurology and Neurosurgery between 2009 and 2013 looking at triggering events, physical and psychological comorbidities. RESULTS: Of 62 patients with clinical and electromyographic diagnosis of Fowler's syndrome, 31 (50%) had unexplained chronic pain syndromes, 12 (19%) of these were taking opiates. Fifteen (24%) had "functional" neurological symptoms. Abdominopelvic surgery with general anesthesia was the leading trigger (n = 21, 35%). CONCLUSIONS: We found high levels of co-morbidity with patients having some form of pain (50%), a probable functional disorder (24%), or psychological symptoms (31%). There are several potential explanations for this association including the effect of developing an apparently unexplained distressing condition, confounding effect of opiate use or referral bias. The findings suggest a need for prospective systematic study of comorbidity for this disabling condition. Neurourol. Urodynam. 35:601-603, 2016. © 2015 Wiley Periodicals, Inc.

Leukoencephalopathy with calcifications and cysts: a purely neurological disorder distinct from coats plus.

OBJECTIVE: With the identification of mutations in the conserved telomere maintenance component 1 (CTC1) gene as the cause of Coats plus (CP) disease, it has become evident that leukoencephalopathy with calcifications and cysts (LCC) is a distinct genetic entity. PATIENTS AND METHODS: A total of 15 patients with LCC were identified from our database of patients with intracranial calcification. The clinical and radiological features are described. RESULTS: The median age (range) at presentation was 10 months (range, 2 days-54 years). Of the 15 patients, 9 presented with epileptic seizures, 5 with motor abnormalities, and 1 with developmental delay. Motor abnormalities developed in 14 patients and cognitive problems in 13 patients. Dense calcification occurred in the basal ganglia, thalami, dentate nucleus, brain stem, deep gyri, deep white matter, and in a pericystic distribution. Diffuse leukoencephalopathy was present in all patients, and it was usually symmetrical involving periventricular, deep, and sometimes subcortical, regions. Cysts developed in the basal ganglia, thalamus, deep white matter, cerebellum, or brain stem. In unaffected areas, normal myelination was present. No patient demonstrated cerebral atrophy. CONCLUSION: LCC shares the neuroradiological features of CP. However, LCC is a purely neurological disorder distinguished genetically by the absence of mutations in CTC1. The molecular cause(s) of LCC has (have) not yet been determined.

Physical precipitating factors in functional movement disorders.

BACKGROUND: A traditional explanation for functional (psychogenic) neurological symptoms, including functional movement disorders (FMD), is that psychological stressors lead to unconsciously produced physical symptoms. However, psychological stressors can be identified in only a proportion of patients. Patients commonly reported a physical event at onset of functional symptoms. In this study, we aim to systematically describe physical events and surrounding circumstances which occur at the onset of FMD and discuss their potential role in generation of functional symptoms. METHODS: We recruited 50 consecutive patients from a specialized functional movement disorders clinic. Semi-structured interviews provided a retrospective account of the circumstances in the 3 months prior to onset of the FMD. Questionnaires to assess mood disturbance and life events were also completed. RESULTS: Eleven males and 39 females were recruited. Forty (80%) patients reported a physical event shortly preceding the onset of the FMD. The FMD occurred after an injury in 11 patients and after an infection in 9. Neurological disorders (n=8), pain (n=4), drug reactions (n=3), surgery (n=3) and vasovagal syncope (n=2) also preceded the onset of the functional motor symptom. 38% of patients fulfilled criteria for a panic attack in association with the physical event. CONCLUSIONS: In our cohort, physical events precede the onset of functional symptoms in most patients with FMD. Although historically neglected in favour of pure psychological explanation, they may play an important role in symptoms development by providing initial sensory data, which along with psychological factors such as panic, might drive subsequent FMD.

'A Leg to Stand On' by Oliver Sacks: a unique autobiographical account of functional paralysis.

Oliver Sacks, the well known neurologist and writer, published his fourth book, 'A Leg to Stand On', in 1984 following an earlier essay 'The Leg' in 1982. The book described his recovery after a fall in a remote region of Norway in which he injured his leg. Following surgery to reattach his quadriceps muscle, he experienced an emotional period in which his leg no longer felt a part of his body, and he struggled to regain his ability to walk. Sacks attributed the experience to a neurologically determined disorder of body-image and bodyego induced by peripheral injury. In the first edition of his book Sacks explicitly rejected the diagnosis of 'hysterical paralysis' as it was then understood, although he approached this diagnosis more closely in subsequent revisions. In this article we propose that, in the light of better understanding of functional neurological symptoms, Sacks' experiences deserve to be reappraised as a unique insight in to a genuinely experienced functional/psychogenic leg paralysis following injury.

Comment on "Detection of an infectious retrovirus, XMRV, in blood cells of patients with chronic fatigue syndrome".

Lombardi et al. (Reports, 23 October 2009, p. 585) reported an association between the human gammaretrovirus XMRV and chronic fatigue syndrome. However, their results may be misleading because of various potential sources of bias and confounding. If real, the association may lack generalizability because of the specific characteristics of the cases studied and could be due to reverse causality.