Agam Jung

Management of sialorrhoea in motor neuron disease: a survey of current UK practice

Abstract Our objective was to better understand UK-wide practice in managing sialorrhoea in motor neuron disease among specialist clinicians. We used a survey of neurologists in the UK with a special interest in motor neuron disease designed to establish clinicians’ attitudes towards treatment options and resources for sialorrhoea management. Twenty-three clinicians replied, representing 21 centres. Sixteen centres were specialist MND Care Centres. Clinicians estimated seeing a total of 1391 newly diagnosed patients with MND in 2011. One hundred and ninety-three patients were described. Forty-two percent of patients reviewed in clinicians’ last clinic had sialorrhoea and 46% of those with sialorrhoea had uncontrolled symptoms. Clinicians’ preferred drugs were hyoscine patches, amitriptyline, carbocisteine and botulinum toxin. Botulinum toxin was used in 14 centres. Risk of dysphagia and staff skills were identified as the main barriers to botulinum toxin use. This survey suggests that there may be as many as 1700 patients with MND in the UK who have symptoms of sialorrhoea and that symptoms may be poorly controlled in nearly half. Treatment strategies varied, reflecting the lack of evidence based guidelines. The use of specialist treatments was influenced by local infrastructure. This study highlights the need for further work to develop evidence based guidance.

A multicentre evaluation of oropharyngeal secretion management practices in amyotrophic lateral sclerosis

Abstract Failure to clear oral secretions can be debilitating for patients with amyotrophic lateral sclerosis (ALS), but the treatment of this symptom is poorly defined and there is no consensus on best practice. The objective of this study was to identify the treatments that are commonly prescribed, and to describe how experienced clinicians manage a patient with treatment resistant symptoms. Twenty-three clinicians were approached, of which 19 from 16 centres across the UK provided case report forms for a total of 119 ALS patients identified as having problematic oral secretions. The use of five anticholinergics, salivary gland botulinum toxin injections, conservative management approaches and carbocisteine were reported. Of the 72 patients who were evaluated following the initiation of a first anticholinergic, 61% had symptomatic improvement. Only 19% of patients achieved symptomatic improvement with the use of an alternative anticholinergic when an initial anticholinergic achieved no symptomatic improvement. Problems with thick and thin secretions often coexisted, with 37% of patients receiving treatment for both types of problem. In conclusion, a variety of treatment options are employed by expert clinicians for problematic oral secretions in ALS patients. The variation in management highlights the need for further prospective research in this area.

Incidence, aetiology, and sequelae of viral meningitis in UK adults: a multicentre prospective observational cohort study

Summary Background Viral meningitis is increasingly recognised, but little is known about the frequency with which it occurs, or the causes and outcomes in the UK. We aimed to determine the incidence, causes, and sequelae in UK adults to improve the management of patients and assist in health service planning. Methods We did a multicentre prospective observational cohort study of adults with suspected meningitis at 42 hospitals across England. Nested within this study, in the National Health Service (NHS) northwest region (now part of NHS England North), was an epidemiological study. Patients were eligible if they were aged 16 years or older, had clinically suspected meningitis, and either underwent a lumbar puncture or, if lumbar puncture was contraindicated, had clinically suspected meningitis and an appropriate pathogen identified either in blood culture or on blood PCR. Individuals with ventricular devices were excluded. We calculated the incidence of viral meningitis using data from patients from the northwest region only and used these data to estimate the population-standardised number of cases in the UK. Patients self-reported quality-of-life and neuropsychological outcomes, using the EuroQol EQ-5D-3L, the 36-Item Short Form Health Survey (SF-36), and the Aldenkamp and Baker neuropsychological assessment schedule, for 1 year after admission. Findings 1126 patients were enrolled between Sept 30, 2011, and Sept 30, 2014. 638 (57%) patients had meningitis: 231 (36%) cases were viral, 99 (16%) were bacterial, and 267 (42%) had an unknown cause. 41 (6%) cases had other causes. The estimated annual incidence of viral meningitis was 2·73 per 100 000 and that of bacterial meningitis was 1·24 per 100 000. The median length of hospital stay for patients with viral meningitis was 4 days (IQR 3–7), increasing to 9 days (6–12) in those treated with antivirals. Earlier lumbar puncture resulted in more patients having a specific cause identified than did those who had a delayed lumbar puncture. Compared with the age-matched UK population, patients with viral meningitis had a mean loss of 0·2 quality-adjusted life-years (SD 0·04) in that first year. Interpretation Viruses are the most commonly identified cause of meningitis in UK adults, and lead to substantial long-term morbidity. Delays in getting a lumbar puncture and unnecessary treatment with antivirals were associated with longer hospital stays. Rapid diagnostics and rationalising treatments might reduce the burden of meningitis on health services. Funding Meningitis Research Foundation and UK National Institute for Health Research.

PO252 Swallow syncope

Swallow syncope is a rare neurally-mediated syncope syndrome. The majority of patients experience brady-arrhythmias on swallowing with the event leading to systemic hypotension. The diagnosis is suspected from the temporal relation between swallowing and the syncopal event, and confirmed by concomitant bradycardia. The diagnosis is suspected from the temporal relation between swallowing and the syncopal event, and confirmed by concomitant swallow-induced symptoms with bradycardia. Underlying oesophageal disease may be present in about 40% of patients necessitating investigations for oesophageal pathology. Recurrent syncopal symptoms can be prevented by placing a cardiac pacemaker. We present a case of swallow syncope and complete heart block successfully treated with a pacemaker.

CEREBRAL FAT EMBOLISM: SUSCEPTIBILITY-WEIGHTED IMAGING IS USEFUL AND PROGNOSIS CAN BE GOOD

We present two cases of cerebral fat embolism. Both patients were 21-years old, male and involved in high impact road traffic collisions with no immediate neurological deficits. Patient A underwent intra-medullary nailing of right tibia/fibular fractures within 12 hours, and had external fixation for bilateral distal femoral fractures. Post-operatively, GCS was 6. Subsequent intracranial MR imaging showed widespread cerebral hypointense punctate foci on SWI (susceptibility-weighted imaging), some with associated restriction on DWI (diffusion-weighted imaging). Despite initial period of coma lasting weeks and inpatient admission over 5 months, he has been discharged to a neuro-rehabilitation unit and continues to show physical and cognitive improvement (MRS score of 3). Patient B underwent intra-medullary nailing for a femoral shaft fracture within 24 hours. Post-operatively, GCS was 9, he developed a petechial rash and became hypoxic. MR brain showed bilateral centrum semiovale DWI restriction, and again, widespread SWI punctate abnormalities. He was discharged within weeks and currently lives at home with his parents (MRS of 2). These cases show the spectrum of radiological abnormalities, the utility of SWI, and highlight that although neurological injury may be severe, there is potential for significant recovery.

5 YEARS EXPERIENCE OF PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA (PCNSL): A RETROSPECTIVE PILOT AUDIT

Introduction Primary Central Nervous System lymphoma (PCNSL) is responsible for 5% of all primary brain tumours within the UK. Barriers to diagnosis include steroid-responsiveness/false-negative biopsy and poor CSF sensitivity. We performed a pilot audit reflecting 5 years experience of PCNSL diagnosis based on criteria derived from 2011 British Neuro-Oncology guidelines. Methods Retrospective case note and electronic patient record review for patients between 2008 to 2013 with biopsy-proven label of PCNSL. Imaging and staging results were reviewed to exclude secondary/metastatic disease. Results 20 patients were identified; median age of 58 and 60% were male. Top three clinical presentations were cognitive, paresis and ataxia. Median duration of symptoms was 21 days (range 3 to 180) and median time to biopsy was 14 days. Pre-biopsy 5 patients had negative CSF, 20% had steroids with documented clinical reason, 5% had record of HIV status checked and 88% had CT staging. Conclusion Despite limitations, this audit identified that the majority of these patients received steroids pre-biopsy, but in some cases, this may have reasonably been withheld. There is scope for a diagnostic algorithm to highlight safe withholding of steroids, early HIV testing and appropriate staging investigations, downplaying the importance of CSF sampling.

TIMELINESS OF MOTOR NEURONE DISEASE CARE– DIAGNOSIS TO SERVICE ACCESS

Background MND is a rapidly progressive incurable neurodegenerative condition. The unpredictable rate of deterioration poses challenges to timely seamless co-ordinated care delivery. We performed an audit mapping time intervals of the patients journey at the Leeds MND Centre to inform local service development. Objectives Evaluate the time period from symptom onset to referral, diagnosis and access to multidisciplinary services. Method Retrospective medical records review for newly diagnosed patients between 2010–2012. Time intervals were compared with MNDA Standards of Care and Kings MND pathway. Results 33 case notes were analysed. Median symptom onset to neurology referral was 36 weeks. Diagnosis was confirmed within 5 weeks. Post diagnosis nurse specialist review was within 2 weeks; however MDT review and consultant follow-up did not meet standards. Discussion This audit identified the lack of a standardised diagnostic and referral pathway. It resulted in significant service review and redesign. Measurement of specific time intervals can be a practical and relevant measure for assessing variation in quality of care. The limitations of a modest sample size and limited time frames can be overcome by a national prospective audit. This will identify delays in both diagnosis and multidisciplinary team access thus ensuring equitable provision of services for patients.

Teaching NeuroImages: MRI changes in superficial siderosis

A 41-year-old woman presented with 2 months of dysarthria. Her history included 6 years of unsteadiness resulting in multiple falls and 10 years of intermittent urinary incontinence. She had a history of significant trauma …