Regional Neuroscience Centre Research Articles

Illness perceptions, experiences of stigma and engagement in functional neurological disorder (FND): exploring the role of multidisciplinary group education sessions

BackgroundA critical first step in managing functional neurological disorder (FND) is a positive diagnosis and clear explanation using an understandable illness model. Multidisciplinary group education sessions are one way to...

Not always a tuberculoma: Case series from East Africa highlighting the importance of a multidisciplinary regional neurosciences centre in enhancing patient care

Not always a tuberculoma: Case series from East Africa highlighting the importance of a multidisciplinary regional neurosciences centre in enhancing patient care

Surgical management of anterior sacral meningoceles: an illustrated case series and review of the literature

Background Anterior sacral meningocele (ASM) is an uncommon variant of spinal dysraphism. Surgical correction for this condition is challenging and optimal corrective approaches are uncertain. Objective To share our experience of managing this rare condition using the posterior trans-sacral approach and provide a contemporary review of the literature. Methods Retrospective review of case notes, operative records, and imaging of eligible patients treated via the posterior trans-sacral approach between 2006 and 2020 at our regional neurosciences centre. Results Three patients, two females and one male with a mean age of 30 years (range 16–38), were treated. Presenting symptoms included lower abdominal pain and recurrent miscarriages. Patients underwent corrective surgery using the posterior approach involving a sacral laminectomy, durotomy and closure of the communicating fistula. A single patient required reoperation due to early recurrence. Another patient proved challenging because of a very large sacral fistula and required two procedures due to the development of high-pressure headaches secondary to a recurrence. All patients improved symptomatically postoperatively and remained symptom free at the last clinic follow-up and have been discharged. Following review of the literature, only two other non-syndromic cases have been described. Conclusions ASM is an uncommon congenital abnormality, typically presenting with mass effect symptoms secondary to a presacral cystic mass. Surgical management using a posterior approach to close the meningeal sac is feasible and less invasive than an anterior approach. Long term clinical outcomes in our series were satisfactory.

Review of the clinical spectrum of typical and atypical Lyme neuroborreliosis in southwest England

BackgroundLyme disease is endemic in the UK with a high incidence in southwest England. Neurological involvement occurs in approximately 5% of infected individuals. Previous reviews in southwest England have shown a consistent phenotype in most cases, however anecdotal review suggests a small number of atypical cases, with diverse presentations, which provide a diagnostic challenge, even to experienced neurologists.MethodsWe performed a retrospective, observational study of all identified cases (positive screening ELISA confirmed by immunoblot and neurological symptoms) of neuroborreliosis from six hospitals between January 2015 and December 2017. In addition, we reviewed atypical cases seen over the last decade in the regional neurosciences centre in depth.Results72 patients were included in the observational study. 83% had characteristics of Bannwarth’s syndrome (cranial neuropathy, radiculopathy and CSF pleocytosis). Unusual cases included mononeu- ritis multiplex.Central nervous system involvement was rare. We saw cases presenting with cerebrovascular events, meningitis and encephalitis. Complete resolution of symptoms was reported in 72% of patients. 28% of patients reported residual symptoms, most commonly fatigue and memory problems.ConclusionsThis is the largest UK review of neuroborreliosis. The presentation is usually typical, but atypical presentations vary and can be challenging. The majority of patients recover fully.amyrossrussell@gmail.com

Treatment of spontaneous intracranial hypotension: experiences in a UK regional neurosciences Centre

A robust treatment paradigm for spontaneous intracranial hypotension has yet to be agreed upon. We present retrospective data from the patient cohort at our UK regional neurosciences centre from 2010-2020 and describe our locally developed treatment pathway.Seventy-three patients were identified: 31 men and 42 women; mean age was 42 years. The majority presented with a headache of variable duration, and most had positive imaging. Very few patients (7%) responded to conservative treatment. Sixty-six underwent epidural blood patching, with 39 (59%) having a good response. Twenty-three patients underwent myelography and targeted treatment (injection of fibrin sealant at the leak site), with 13 (57%) showing a good response. One patient had successful surgery. The relapse rate after response to epidural blood patching was 10%, and after response to targeted treatment was 23%. Most patients who relapsed responded to repeated treatments.The outcome data for our diverse patient cohort shows the success of a staged approach to treatment. Relapse rates are low, and surgery is only rarely required. We use these data to inform our discussions with patients, and present them here to enable other centres to develop robust investigation and treatment paradigms of their own.

Screening for functional neurological disorders by questionnaire

ObjectiveDiagnostic 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. MethodsThe 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. ResultsBlackouts, 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. ConclusionThe 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.

Cognitive Symptoms in Patients with Epilepsy: Role of Sleep and Mood Disturbance

Study background: Complaints of sleep disturbance are frequently encountered in patients with epilepsy, as are subjective memory complaints. Since the former may contribute to the latter, identification of sleep disturbance may contribute to diagnosis and treatment of subjective memory complaints in patients with epilepsy. Methods: Jenkins Sleep Scale (JSS), a validated sleep scale, was administered to consecutive new patients attending a dedicated epilepsy outpatient clinic based in a regional neuroscience centre, along with the five-point subjective memory complaint (SMC) Likert scale and a two-item mood screener. Results: Sleep disturbance identified using JSS was more frequent in patients with subjective memory complaint, as was mood disturbance (both p<0.02). Conclusion: Identifying sleep disturbance using a simple sleep screening scale may contribute to both diagnosis and treatment of subjective memory complaints in patients with epilepsy.

Functional seizures: An evaluation of the attitudes of general practitioners local to a tertiary neuroscience service in London.

SummaryObjectiveFunctional seizures are a common functional neurologic disorder. Given their chronic nature, and the biopsychosocial factors involved in their etiology, general practitioners (GPs) play a crucial role in the care of these patients. However, little is known about the attitudes of GPs toward, and knowledge of, functional seizures.MethodsThe Atkinson Morley Regional Neuroscience Centre in London provides a comprehensive service to patients with functional seizures. As part of a service evaluation we conducted an online survey among local GPs over a 1‐month period assessing their attitudes toward, and knowledge of, functional seizures.ResultsOne hundred twenty of 974 surveyed GPs replied to the survey (12.3%). Approximately 75% of GPs readily use the term “pseudoseizures,” and over 50% were not sure or did not think that functional seizures were involuntary. Nearly 30% believed, or were unsure as to whether, functional seizures occur only when patients are stressed. Despite approximately 50% of GPs expressing interest in getting involved in the management of these patients, a similar proportion do not feel confident in dealing with queries from patients with functional seizures. Although most GPs felt that neurology and psychiatry should be the primary caregivers in the diagnosis and management, respectively, of functional seizures, 50% were also of the opinion that neurology should be involved in the management of these patients.SignificanceThis survey highlights the attitudes of, and descriptive terms used by, GPs toward patients with functional seizures. Our findings suggest a need for better and clearer provision of information to GPs about this condition.

The clinical features and outcome of scan-negative and scan-positive cases in suspected cauda equina syndrome: a retrospective study of 276 patients

BackgroundThe majority of patients presenting with suspected clinical cauda equina syndrome (CES) have no identifiable structural cause for their symptoms (‘scan-negative’ CES). Understanding these patients aids clinical differentiation and management in CES.MethodsA retrospective electronic note review was undertaken of patients presenting with suspected CES, defined as ≥ 1 of acute bladder, bowel, sexual dysfunction or saddle numbness, to a regional neurosciences centre. We investigated radiology, clinical features, psychiatric and functional disorder comorbidities and outcome of patients with ‘scan-negative’ CES and patients with MRI confirmed compression of the cauda equina (‘scan-positive’ CES).Results276 patients were seen over 16 months. There were three main radiologically defined patient groups: (1) ‘scan-positive’ CES (n = 78, mean age 48 years, 56% female), (2) ‘scan-negative’ CES without central canal stenosis but with lumbosacral nerve root compression not explaining the clinical presentation (n = 87, mean age 43 years, 68% female) and (3) ‘scan-negative’ CES without neural compromise (n = 104, mean age 42 years, 70% female). In the two ‘scan-negative’ groups (no neural compromise and nerve root compression), there were higher rates of functional disorders (37% and 29% vs. 9%), functional neurological disorders (12% and 11% vs 0%) and psychiatric comorbidity (53% and 40% vs 20%). On follow-up (mean 13–16 months), only 1 of the 191 patients with ‘scan-negative’ CES was diagnosed with an explanatory neurological disorder (transverse myelitis).ConclusionsThe data support a model in which scan-negative cauda equina syndrome arises as an end pathway of acute pain, sometimes with partly structural findings and vulnerability to functional disorders.

In-house development of a dedicated data acquisition and monitoring system for intracranial pressure, patient posture and patient symptoms in a regional neurosciences centre

Management of traumatic brain injury and cerebrospinal fluid (CSF) flow disorders can be aided by measurement and monitoring of intracranial pressure (ICP). In addition to pressure measurement, knowledge of patient symptoms and posture during monitoring are also valuable, particularly in the management of CSF flow disorders. ICP monitoring systems have been developed in this centre to meet clinical needs in the absence of commercially available solutions. An early system (mark I) was developed and the technical challenges in its design are described, along with limitations to this system that motivated the development of a new mark II system. The mark II system is then described.

STOP-Bang: Screening for Obstructive Sleep Apnoea in a Cognitive Disorders Clinic

Study background: Obstructive sleep apnoea may be one of the few reversible causes of cognitive impairment. Hence its reliable identification in patients referred to dedicated cognitive disorders clinics is important. Method: STOP-Bang, a validated questionnaire for obstructive sleep apnoea, was administered to consecutive new patients attending a dedicated cognitive disorders clinic based in a regional neuroscience centre. Results: Almost half of those completing the STOP-Bang were screen positive, although on clinical grounds the diagnosis of obstructive sleep apnoea was seldom considered likely; many were diagnosed with other explanations for their cognitive impairment. Conclusion: STOP-Bang is likely a very sensitive screening instrument which may generate large numbers of false positives in a cognitive disorders clinic, which might have significant service implications for onward referral to sleep disorders clinics.

AN EVALUATION OF THE USEFULNESS OF PERIPHERAL NERVE BIOPSIES

Peripheral nerve biopsy may be useful in selected patients, but practice varies, partly due to continued uncertainty over usefulness of biopsy. An audit was completed of all patients who had undergone nerve biopsy over a 3 year period (2009–2012) at a tertiary regional neuroscience centre. 61 patients were identified and medical information of 54 patients was obtained (33 male, 21 female patients; mean age 63.6 years). The sural nerve was the most commonly biopsied nerve (94.4%). Complications (pain, stitch extrusion, dysaesthesia, infection) occurred in 7.4%. Neurophysiological pre-biopsy diagnosis was axonal in 74.1% (37.5% vasculitic); demyelination in 14.8% (all CIDP); axonal and demyelinating in 9.3%, and MND in 1.9%. Overall, biopsy led to diagnostic confirmation in 14.8% of patients, change of diagnosis in 11.1%, and change to treatment in 9.3%. As some patients were represented in more than one of these groups, overall 72.2% of biopsies yielded neither further diagnostic information, nor led to change in diagnosis or treatment. Nerve biopsy was of clinical benefit to 1 in 4 patients and management was directly altered in 1 in 11 patients. Nerve biopsy remains of use, and has an acceptable complication rate in diseases with significant burden from ineffective or inappropriate treatment.

The documentation of consent and disclosure of neurogenetic testing outside clinical genetics

Neurogenetic tests are increasingly requested by clinical neurologists without any formal training in clinical genetics. The aim of our study was to assess the documentation of consent and disclosure of genetic test results in a large regional clinical neuroscience centre. Documentation of some form of consent was evident in only 26/132 (20%) of tests. However, the higher proportion of both positive and negative results disclosed (50/132, 38%) suggest that the former figure may underestimate actual rates of undocumented consent within the clinical setting. Our findings highlight the need for a review of established practices surrounding consent in clinical neurology.

Spontaneous intracranial hypotension and venous sinus thrombosis

Spontaneous intracranial hypotension (SIH) is an uncommon but well-recognised cause of orthostatic headache. Subdural effusions or haemorrhage are well-known complications of SIH. However, that SIH may lead to venous sinus...

The patient experience of fatigue in motor neurone disease

Aims: This paper is a qualitative investigation that aims to investigate the lived experience of fatigue in patients with motor neurone disease—a progressive and fatal neurological condition.Background: Fatigue is a disabling symptom in motor neurone disease (MND) that affects a large number of patients. However, the term “fatigue” is in itself imprecise, as it remains a phenomenon without a widely accepted medical definition. This study sought to investigate the phenomenon of fatigue from the perspective of the MND patient.Methods: Ten patients with MND participated in semi-structured recorded interviews at a regional neuroscience center in Liverpool, UK. Transcripts analysis was broadly informed by the principles of interpretative phenomenological analysis (IPA).Findings: Fatigue was unanimously explained to be disabling and progressive phenomenon. Participants described two forms of fatigue: whole-body tiredness or use-dependent reversible muscle weakness related to exertion of limb and bulbar muscles. Both weakness and whole-body tiredness could be experienced simultaneously, and patients used the terms “fatigue” and “tiredness” interchangeably. Alongside descriptions of fatigue themes of Adaptation, Motivation, Avoidance, Frustration and Stress were revealed. Fatigue could be defined as “reversible motor weakness and whole-body tiredness that was predominantly brought on by muscular exertion and was partially relieved by rest.”Conclusion: The results of this study support a multi-dimensional model of fatigue for patients with MND. Fatigue appears to be experienced and explained in two ways, both as an inability to sustain motor function and as a pervasive tiredness. Fatigue was only partially relieved by rest and tended to worsen throughout the day. It is crucial that MND care practitioners and researchers appreciate the semantic dichotomy within fatigue.

Outcome of Aneurysmal Subarachnoid Haemorrhage in the Elderly: Retrospective Study of a Contemporary Cohort Treated in a Regional Neuroscience Centre

Outcome of Aneurysmal Subarachnoid Haemorrhage in the Elderly: Retrospective Study of a Contemporary Cohort Treated in a Regional Neuroscience Centre The incidence of subarachnoid haemorrhage (SAH) increases with age, and together with the elderly constituting a significant and growing proportion of the population in developed countries, this poses unique challenges to the healthcare system. Treatment of SAH in the elderly is traditionally underrepresented due to pessimism toward its outcome. With recent advances in management techniques, it has been suggested that elderly patients with SAH may benefit from treatment.

VOLTAGE-GATED POTASSIUM CHANNEL (VGKC) ANTIBODIES IN EPILEPSY: DIAGNOSTIC YIELD AND THERAPEUTIC IMPLICATIONS

BackgroundRecent studies have identified serum VGKC antibodies in various cohorts of patients with epilepsy, and have suggested a potential aetiological role for VGKC antibodies in epilepsy.MethodsWe have undertaken systematic testing...

Acute subarachnoid haemorrhage: Is a negative CT angiogram enough?

To determine the negative predictive value of 16 channel multisection computed tomography angiography (CTA) for detecting aneurysms in spontaneous subarachnoid haemorrhage (SAH), using digital subtraction angiography (DSA) as the reference standard. The prospectively collected cerebral angiogram database of Department of Neuroradiology, Atkinson Morley Regional Neuroscience Centre was used to identify 200 consecutive patients who had undergone DSA for SAH. Of these, 176 had undergone CTA prior to DSA. Clinical details and radiology reports were correlated and images of positive investigations reviewed. DSA showed one or more cerebral aneurysms in 105 (60%) patients. These were correctly reported on CTA in 100. CTA was reported negative for aneurysms in 74 patients. Of these five were false negative and had aneurysms detected on DSA. In the CTA/DSA negative group, 11 (16%) patients had classical perimesencephalic clinical syndrome and blood distribution. There were two false positives at CTA. For ruptured cerebral aneurysms, CTA had 95.2% sensitivity, 97.2% specificity, 98.1% positive predictive value, and 93.2% negative predictive value. The sensitivity and negative predictive value of CTA for ruptured aneurysms remains imperfect. Continued use of DSA is recommended in most patients with a negative CTA after acute SAH. Confirmation of a negative CTA result with DSA may not be routinely required in patients with perimesencephalic syndrome.

P9 Impact of neuropsychiatry screening in neurological inpatients: comparison with routine clinical practice

Objective To compare the rate of detection of psychiatric illness in routine clinical practice, with the prevalence of mental illness established, using a dedicated screening programme at a regional neuroscience centre; and to assess if the screening programme had any enduring impact on routine clinical practice after its completion. Method Consecutive admissions to a neurology ward in the 3-month period before (n=160) and after (n=158) a dedicated neuropsychiatric screening program was carried out were identified. Case notes were then reviewed to establish if symptoms of mental illness were identified by the treating neurologists and if patients were referred for neuropsychiatric assessment. Rates of detection of neuropsychiatric problems and rates of referral for treatment were compared with those identified during the screening programme. Results In routine clinical practice, over two 3-month study periods, psychiatric symptoms were identified in 23.7% of patients and only 10.6% received neuropsychiatric interventions. This is much lower as compared to rates identified (51.3%) and treated (51.3%) during dedicated screening. Detection of mood symptoms decreased from 14.7% pre-screening to 3.8% in the post-screening period. Conclusion Rate of detection and treatment of neuropsychiatric problems remains low in neurology inpatients in routine clinical practice. Neuropsychiatric Screening is effective but does not have sustained effect once it stops. Hence we suggest active ongoing screening should be incorporated into routine practice.

Efficiency of hospital reporting systems in detecting head injury admissions

Objectives. To examine the completeness of data on admission with head injury at a Regional Neuroscience Centre. Design. A comparative study using retrospective and prospective data sources. Subjects. All adults admitted to all specialties with all severities of head injury. Methods. Adult admissions with ‘head injury’ in a single month were identified from two sources: (a) prospective ED reports using ED codes for head injury + site ‘Head/Face’ and ‘Reason for attendance’, (b) retrospective ICD-10 coding reports of codes S00-09. Data from both sources were compared and reasons for non-capture analysed. Admissions from both reports were combined to obtain a more complete number of admissions with head injury. Results. A total of 112 admissions with head injury were identified from both sources. Completeness from ED reports was 70% (n = 78) and 83% (n = 93) from ICD-10 reports. In total, 53% (n = 59) of admissions were captured in both reports. The main reasons for non-capture in the ED reports were non-coding of head injury (56%, n = 19), and admission via another routes, for example, transfers (44%, n = 15). The main reasons for non-capture in the ICD-10 reports were non-coding of head injury or not the primary diagnosis (74%, n = 14). Conclusions. Reliable epidemiological data is required for planning and commissioning services, however, identification of head injury admissions is difficult due to ambiguity in terminology and patient population. More than one source is necessary for completeness of data, however, data issues such as comprehensiveness, reliability and inclusion criteria/possible bias need to be considered.