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ObjectiveTo identify factors predictive of relapse risk and disability in myelin oligodendrocyte glycoprotein associated disease (MOGAD).SettingPatients were seen by the neuromyelitis optica spectrum disorders (NMOSD) service in Liverpool, UK, a national referral centre for adult patients with MOGAD, NMOSD and related conditions.ParticipantsPatients with MOGAD=76 from England, Northern Ireland and Scotland were included in this cohort study.ResultsRelapsing disease was observed in 55% (42/76) of cases. Steroid treatment >1 month (OR 0.2, 95% CI 0.05 to 0.80; p=0.022), transverse myelitis (TM) at first attack (OR 0.03, 95% CI 0.004 to 0.23; p=0.001) and male sex (OR 0.16, 95% CI 0.04 to 0.68; p=0.014) were associated with monophasic disease (area under the curve=0.85). Male sex (HR 0.46, 95% CI 0.24 to 0.89; p=0.011) and TM at disease onset (HR 0.42, 95% CI 0.22 to 0.82; p=0.011) were also associated with an increased latency to first relapse. 45% (32/71) of patients became MOG-antibody negative and in relapsing patients negative seroconversion was associated with a lower relapse risk (relative risk 0.11 95% CI 0.05 to 0.26; pConclusionsMale patients with spinal cord involvement at disease onset have a lower risk of relapsing disease and longer latency to first relapse. Steroid treatment for at least 1 month at first attack was also associated with a monophasic disease course. MOG-antibody negative seroconversion was associated with a lower risk of relapse and may help inform treatment decisions and duration.

Background Neuromyelitis optica spectrum disorder (NMOSD) is a rare, neurological disease that places a significant burden on patients, their carers, and healthcare systems. Objectives To estimate patient and carer health utilities and costs of NMOSD within the UK setting. Methods Patients with NMOSD and their carers, recruited via a regional specialist treatment centre, completed a postal questionnaire that included a resource use measure, the EuroQoL (EQ)-5D-5L, EQ-5D-VAS, Vision and Quality of Life Index (VisQoL), Carer Experience Survey (CES) and the Expanded Disability Status Scale (EDSS). The questionnaire asked about respondents’ use of health and community care services, non-medical costs, informal care and work capacity. Data were analysed descriptively. Uncertainties in costs and utilities were assessed using bootstrap analysis. Results 117 patients and 74 informal carers responded to the survey. Patients’ mean EQ-5D-5L and VisQoL health utilities (95% central range) were 0.54 (− 0.29, 1.00) and 0.79 (0.11, 0.99), respectively. EQ-5D-5L utility decreased with increasing EDSS score bandings, from 0.80 (0.75, 0.85) for EDSS ≤ 4.0, to 0.20 (− 0.29, 0.56) for EDSS 8.0 to 9.5. Mean, 3-month total costs were £5623 (£2096, £12,156), but ranged from £562 (£381, £812) to £32,717 (£2888, £98,568) for these EDSS bandings. Carer-reported EQ-5D-5L utility and CES index scores were 0.85 (0.82, 0.89) and 57.67 (52.69, 62.66). Mean, 3-month costs of informal care were £13,150 to £24,560. Conclusions NMOSD has significant impacts on health utilities and NHS and carer costs. These data can be used as inputs to cost-effectiveness analyses of new medicines for NMOSD.

BackgroundNeuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune inflammatory disorder of the central nervous system (CNS), and in most cases is associated with Aquaporin-4 antibodies (AQP4- IgG). Severe optic neuritis, longitudinally extensive myelitis and brain involvement is characteristic. Similarly presenting patients may not have detectable AQP4-IgG (seronegative NMOSD) or have Myelin Oligo- dendrocyte Glycoprotein IgG (MOG-IgG).MethodA retrospective study of AQP4-IgG, MOG-IgG, and seronegative NMOSD patients attending the highly specialised NMOSD service between September 2010-August 2019 was performed. In particular, the presence of coexistent autoimmunity was assessed.ResultsCoexistent autoimmunity in AQP4-IgG patients was higher as compared to MOG-IgG and seron- egative NMOSD (30% vs. 8% vs. 8%). Some AQP4-IgG patients had 2nd and 3rd autoimmune diseases (7% and 1% respectively). In contrast no patients with MOG-IgG or seronegative NMOSD had 2 or more other autoimmune diseases.ConclusionCoexistent autoimmunity in AQP4-IgG NMOSD is more common that in seronegative NMOSD and patients with MOG-IgG. It is reasonable to periodically assess for coexistent autoimmunity in AQP4-IgG NMOSD as it is present in almost a third of patients. Finally the detection of ≥2 autoimmune diseases in newly presenting cases provides a useful clue to AQP4-IgG NMOSD whilst antibody results are awaited.giovanna.campagna@thewaltoncentre.nhs.uk

Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been described in patients with neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 antibodies (AQP4-IgG). We aimed to identify the proportion of AQP4-IgG-negative NMOSD patients who are seropositive for MOG-IgG. In a cross sectional study, we reviewed all patients seen in the National NMO clinic over the last 4 years (after the availability of MOG-IgG testing), including clinical information, MRI, and antibody tests. 261 unique patients were identified. 132 cases satisfied the 2015 NMOSD diagnostic criteria. Of these, 96 (73%) were AQP4-IgG positive and 36 (27%) were AQP4-IgG negative. These 36 patients were tested for MOG-IgG and 15/36 (42%) tested positive. 20% (25/125) of the patients who did not satisfy NMOSD criteria had MOG-IgG. Approximately half of seronegative NMOSD is MOG-Ig seropositive and one in five of non-NMOSD/non-MS demyelination is MOG-IgG positive. Since MOG-associated demyelinating disease is likely different from AQP4-IgG disease in terms of underlying disease mechanisms, relapse risk and possibly treatment, testing for MOG-IgG in patients with AQP4-IgG-negative NMOSD and other non-MS demyelination may have significant implications to management and clinical trials.

A 55-year-old man presented in October 2004 with general unease, vomiting, and gait disturbance. Initially diagnosed with an inner ear infection, the patient's symptoms did not improve and he was evaluated further. MRI revealed a cerebellar lesion, which led to the suspicion of a posterior circulation stroke, and he was started on antiplatelet drugs. However, an angiogram suggested no vascular pathology. In March 2011, he had another episode of ataxia and MRI showed a right brainstem lesion. Cryptogenic stroke was reconsidered. In March 2013, the patient developed paraparesis with urinary and bowel symptoms. MRI revealed myelitis of the midthoracic spinal cord. Oligoclonal bands were present in both serum and CSF and steroids were begun. In February 2014, he was admitted with expressive aphasia and right hemiparesis. MRI (figure 1) confirmed a new T2-hyperintense lesion in the left frontal lobe and he was given IV methylprednisolone and subsequently underwent plasma exchange. Aquaporin 4 (AQP4) IgG was negative. The working diagnosis was tumefactive multiple sclerosis. He was started on glatiramer acetate in August 2014. A few months later, he developed right-sided weakness, left optic neuritis (ON), and confusion. MRI (figure 2) showed left occipital and left parietal cortical and subcortical white matter lesion spreading across the corpus callosum to the opposite side. There were changes bilaterally in the cerebellum, cerebral peduncles, and along the corticospinal tracts on the left side. Further scans showed swelling of the splenium of the corpus callosum. He was put back on prednisolone 60 mg. Markers for systemic inflammation (erythrocyte sedimentation rate, C-reactive protein) were normal and autoantibody testing (antinuclear antibodies, extractable nuclear antigen, paraneoplastic antineuronal antibodies) was negative. The diagnosis of fulminant seronegative NMO was re-entertained but AQP4 antibodies were negative. However, MOG antibodies returned positive (October 2014, live cell-based assay, John Radcliffe Hospital, Oxford, UK). Azathioprine 125 mg was begun in February 2015.

Background: The international panel for neuromyelitis optica (NMO) diagnosis has proposed diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD). Objectives: We assessed the impact of these criteria on diagnostic rates in a large cohort of patients. Methods: We identified and applied the 2006 and 2015 criteria to all patients ( n = 176) seen in the NMO and non-multiple sclerosis central nervous system demyelination clinic (part of the UK NMO service) from January 2013 to May 2015. Results: The 2006 criteria classified 63 of 176 (36%) patients as NMO. A total of 42 patients (67%) were aquaporin 4 (AQP4) immunoglobulin G (IgG) +ve and 21 (33%) AQP4 IgG −ve. The 2015 criteria classified 111 of 176 (63%) patients as NMOSD, of which 81 (73%) were AQP4 IgG +ve and 30 (27%) were AQP4 IgG −ve. There was an increase of 48 patients (76%) diagnosed as NMOSD using the new criteria. Conclusion: Application of the 2015 criteria led to a rise in diagnosis of NMOSD by 76%. The rise in the AQP4 IgG +ve group contributed 62% and the seronegative group contributed 14%.

Objective Our primary objective was to examine the neuropsychological and psychopathological profile of patients with neuromyelitis optica (NMO) and compare these to multiple sclerosis (MS) and healthy control (HC) groups. We also examined for relationships between cognitive and psychiatric variables and clinical factors including accumulated neurological disability and disease duration. Methods A neuropsychological test battery was administered along with a structured psychiatric interview and quantitative measures of mood symptoms. Results 42 NMO, 42 MS and 42 HC participants were assessed. Cognitive impairments were observed in 67% of NMO patients. The prevalence and profile of cognitive impairments and lifetime prevalence of depression was similar between NMO and MS groups. However, significantly higher rates of recurrent depression and suicidality were observed in NMO patients. Correlational analyses revealed higher levels of anxiety symptoms were associated with shorter disease duration in NMO, while higher depression symptom levels were associated with higher neurological disability and poorer cognition. Conclusions Our results demonstrate substantial cognitive and psychiatric comorbidities in NMO patients. Similar rates of lifetime and current depression between NMO and MS appear to mask greater underlying psychiatric burden in NMO and further understandings of the course of neurobehavioural comorbidities is required to better comprehend the additional morbidity in NMO. Our data support a role for cognitive and psychiatric assessments in the comprehensive care of NMO patients.

Background Seasonal variation in incidence and exacerbations has been reported for neuroinflammatory conditions such as multiple sclerosis and acute disseminated encephalomyelitis (ADEM). It is unknown whether seasonality also influences aquaporin-4 antibody (AQP4-Ab) disease and myelin-oligodendrocyte antibody (MOG-Ab) disease. Objective We examined the seasonal distribution of attacks in AQP4-Ab disease and MOG-Ab disease. Methods Observational study using data prospectively recorded from three cohorts in the United Kingdom. Results There was no clear seasonal variation in AQP4-Ab or MOG-Ab attacks for either the onset attack nor subsequent relapses. In both groups, the proportion of attacks manifesting with each of the main phenotypes (optic neuritis, transverse myelitis, ADEM/ADEM-like) appeared stable across the year. This study is the first to examine seasonal distribution of MOG-Ab attacks and the largest in AQP4-Ab disease so far. Conclusion Lack of seasonal distribution in AQP4-Ab and MOG-Ab disease may argue against environment factors playing a role in the aetiopathogenesis of these conditions.

Background Neuromyelitis optica (NMO) is an auto-immune disease that can cause severe visual and mobility impairments. Research on health-related quality of life (HRQoL) in NMO is scarce, limiting knowledge on factors influencing HRQoL and support needs. Aim This study provides the first qualitative exploration of HRQoL in NMO, conducted to provide a conceptual framework for the development of an NMO patient-reported outcome measure. Method Fifteen people with NMO (aged 18–74; 11 women, 4 men) participated in semi-structured interviews; data were analysed using constant comparative analysis. Results HRQoL in NMO is a multifaceted concept incorporating highly subjective perceptions of normality and meaning. Four major themes were identified: impact of physical symptoms on daily living, utilizing support to achieve independence, expectations for life and meaningful roles in life and purpose. Discussion Themes highlighted the importance of perceived normality, and its relationship to attaining life goals comparable to peers, as underpinning evaluations of HRQoL. Many people with severe disability reported a high HRQoL, suggesting the inappropriateness of assuming a negative HRQoL on the basis of an individual's neurological impairment. Conclusions These findings further the conceptual understanding of HRQoL in NMO, informing patient-care approaches and the development of an NMO-specific patient-reported outcome measure.

Background Transverse myelitis (TM) associated with Neuromyelitis Optica (NMO) can be severe and is well known to reduce mobility early in the disease. However the burden of bladder and bowel dysfunction is unknown and overlooked. We studied the frequency of bladder and bowel dysfunction and their impact on quality of life. Methods A cross-sectional study of 60 patients who had AQP4-IgG positive NMO associated TM was performed using the Bladder Control Scale, Lower Urinary Tract Quality of Life, Bowel Control Scale and Neurogenic Bowel Score, Short-Form-36 Health Survey and EDSS. The relationships between the variables were analysed with multiple linear regression. Results Fifty women and 10 men participated. 78% (47/60) patients reported bladder symptoms and a similar number reported bowel problems. 87% (52/60) patients reported either bladder or bowel dysfunction. 65% (39/60) developed residual symptoms after the first episode of myelitis and the remaining by the second episode. Both bladder and bowel dysfunction reduced quality of life and required modification of lifestyle in 83% (39/47) and 70% (33/47) respectively. Conclusion Bladder and bowel dysfunction is very common in NMO associated myelitis developing early in the disease and significantly affects quality of life.

Importance: Antibodies to myelin oligodendrocyte glycoprotein IgG (MOG-IgG) are increasingly detected in patients with non–multiple sclerosis–related demyelination, some of whom manifest a neuromyelitis optica (NMO) phenotype. Cortical involvement, encephalopathy, and seizures are rare in aquaporin 4 antibody (AQP4-IgG)–related NMO in the white European population. However, the authors encountered several patients with seizures associated with MOG-IgG disease. Objective: To compare incidence of seizures and encephalitis-like presentation, or both between AQP4-IgG–positive and MOG-IgG–positive patients. Design, Setting, and Participants: Retrospective case series of all patients who were seropositive for MOG-IgG (n = 34) and the last 100 patients with AQP4-IgG disease (NMO spectrum disorder) seen in the NMO service between January 2013 and December 2016, and analysis was completed January 4, 2017. All patients were seen in a tertiary neurological center, The Walton Centre NHS Foundation Trust in Liverpool, England. Main Outcomes and Measures: The difference in seizure frequency between the AQP4-IgG–positive and MOG-IgG–positive patient groups was determined. Results: Thirty-four patients with MOG-IgG disease (20 female) with a median age at analysis of 30.5 years (interquartile range [IQR], 15-69 years), and 100 AQP4-IgG–positive patients (86 female) with a median age at analysis of 54 years (IQR, 12-91 years) were studied. Most patients were of white race. Five of the 34 patients with MOG-IgG (14.7%) had seizures compared with 1 patient with AQP4-IgG (2-sided P

Though pain in neuromyelitis optica (NMO) has been described in two recent reports, the proportion with true neuropathic pain (NP), its features, impact on activities of daily living (ADL) and quality of life has not been well characterised. A cross-sectional study of 50 NMO patients with transverse myelitis was performed using Douleur Neuropathique 4, Brief Pain Inventory, Extended Disability Status Scale and Short Form 36. NP was identified in 62% of patients. Pain was constant in 68% affecting most ADL. Pain was associated with significant reduction of the SF36 Mental Composite Score. The high prevalence of NP and associated disability necessitates an in-depth enquiry in patients with NMO.

Neuromyelitis optica typically presents at a median age of 40-50 years. The myelitis is usually of acute onset, long (>3 vertebral segments) and causes severe sensorimotor and bladder and bowel disturbances. We describe a 73-year-old Caucasian woman with aquaporin-4 antibody-positive neuromyelitis optica whose index event was intermittent paroxysmal tonic spasms (and no other myelitis features) that recurred for 6 months and was associated with a short spinal cord lesion on MRI. This case reiterates recent observations that neuromyelitis optica can occur in older persons, and its myelitis can be 'short' and clinically mild.

Neuromyelitis optica (NMO) is an uncommon, demyelinating disease that causes long-term disability in adults. Though much has recently been learned about its pathogenesis, there are still only a few studies regarding the epidemiology of NMO. The aim of the study was to describe the epidemiology of NMO among adults in the Merseyside county of the United kingdom. Multiple overlapping sources of data were used including hospital records of The Walton Centre for Neurology and Neurosurgery in Liverpool, regional district general hospital data, central Aquaporin-4 antibody testing laboratory data and the British Neurological Surveillance Unit- to identify adults with a first-ever-in-a-lifetime diagnosis of NMO. As of December 31, 2010, there were eight cases (five NMO; three NMO spectrum disorder), indicating a prevalence of 7.2/million (95 % CI 3.1-14.2). Four incident cases of NMO and three incident cases of NMO spectrum disorder were identified in this period, indicating a minimum combined average annual incidence rate of 0.8/million (95 % CI 0.3-1.6). NMO still remains an uncommon condition, but the prevalence is rising with early diagnosis.

Objective:To study the effect of pregnancy on the frequency of neuromyelitis optica spectrum disorder (NMOSD) relapse and evaluate rates of pregnancy-related complications in an international multicenter setting.Methods:We administered a standardized survey to 217 women with NMOSD from 7 medical centers and reviewed their medical records. We compared the annualized relapse rate (ARR) during a baseline period 2 years prior to a participant's first pregnancy to that during pregnancy and to the 9 months postpartum. We also assessed pregnancy-related complications.Results:There were 46 informative pregnancies following symptom onset in 31 women with NMOSD. Compared to baseline (0.17), ARR was increased both during pregnancy (0.44; p = 0.035) and during the postpartum period (0.69; p = 0.009). The highest ARR occurred during the first 3 months postpartum (ARR 1.33). A total of 8 of 76 (10.5%) with onset of NMOSD prior to age 40 experienced their initial symptom during the 3 months postpartum, 2.9 times higher than expected.Conclusions:The postpartum period is a particularly high-risk time for initial presentation of NMOSD. In contrast to published observations in multiple sclerosis, in neuromyelitis optica, relapse rate during pregnancy was also increased, although to a lesser extent than after delivery.

IMPORTANCE: Chronic, intractable neuropathic pain is a common and debilitating consequence of neuromyelitis optica spectrum disorder (NMOSD) myelitis, with no satisfactory treatment; few studies have yet to explore its aetiology. OBJECTIVE: To establish if myelitis-associated chronic pain in NMOSD is related to the craniocaudal location of spinal cord lesions. METHOD: (1) Retrospective cohort of 76 aquaporin 4-antibody (AQP4-Ab)-positive patients from Oxford and Liverpool's national NMOSD clinics, assessing current pain and craniocaudal location of cord lesion contemporary to pain onset. (2) Focused prospective study of 26 AQP4-Ab-positive Oxford patients, a subset of the retrospective cohort, assessing current craniocaudal lesion location and current pain. RESULTS: Patients with isolated thoracic cord myelitis at the time of pain onset were significantly more disabled and suffered more pain. Cervical and thoracic lesions that persisted from pain onset to 'out of relapse' follow-up (current MRI) had highly significant (p CONCLUSIONS: Persistent, caudally located (ie, thoracic) cord lesions in AQP4-Ab-positive patients associate with high postmyelitis chronic pain scores, irrespective of number of myelitis relapses, lesion length and lesion burden. Although disability correlated with pain in isolation, it became an insignificant predictor of pain when analysed alongside craniocaudal location of lesions.

Introduction Around 70–90% of typical NMO have aquaporin-4 antibodies (AQP4-Ab). More recently, an association between myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and monophasic NMO has been reported. Methods We present 5 AQP4-Ab negative cases with relapsing CNS demyelinating disease (4 with NMO phenotype) associated with MOG-Ab. Results All patients were Caucasian. Four patients presented with typical NMO phenotype. One patient had a long myelitis and a further brainstem event. Median age at onset was 17 (16–39) years. The first episode of optic neuritis and longitudinally extensive myelitis developed simultaneously in 2 patients while 2 patients had inter-attack interval 1.1 and 7 years. All except one patient had a normal brain MRI. EDSS (visual) scores after disease duration (number of relapses) of 1.3 (2), 1.6 (2), 3.4 (6), 8.3 (7) and 12 (13) years were 5 (0), 4 (3), 3 (5), 4 (0) and 4 (6) respectively. Conclusions MOG-Ab can be seen in relapsing NMO and is not restricted to the monophasic subtype. The clinical and diagnostic role of MOG-Ab is still evolving.

Background: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). Objective: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. Methods: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. Results: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. Conclusion: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

Background: Azathioprine (AZA) is a common immunosuppressive drug used for relapse prevention in neuromyelitis optica (NMO). Objectives: The objective of this paper is to assess efficacy, tolerability and retention of AZA in a large NMO cohort. Methods: We conducted a retrospective review of medical records of 103 aquaporin-4 antibody-positive NMO and NMO spectrum disorder (NMOSD) patients treated with AZA. Results: This is the largest reported cohort of AQP4-Ab positive patients treated with AZA. Eighty-nine per cent ( n = 92) had reduction in median annualised relapse rates from 1.5 (IQR 0.6–4.0) to 0 (IQR 0–0.27, p < 0.00005) with treatment. Sixty-one per cent ( n = 63) remained relapse free at a median follow-up of 18 months. Neurological function improved or stabilised in 78%. At last follow-up, treatment was discontinued in 46% ( n = 47). Of these, 62% ( n = 29) were because of side effects, 19% ( n = 9) because of death, 15% ( n = 7) because of ongoing disease activity, and 2% ( n = 1) because of pregnancy. Using Kaplan-Meyer curves, we estimate that 73%, 58%, 47% and 33% of patients will remain on AZA for longer than one, three, five and 10 years, respectively, after initiation of treatment. Conclusions: AZA is a modestly effective treatment for NMO. However, many patients discontinue AZA over time and this seems to reflect poor tolerability more than lack of efficacy.

Aim: Neuromyelitis optica (NMO) is a rare neuro-inflammatory condition characterized by acute relapses causing severe visual or physical disability. The impact on family members and their experiences have not been studied. The study aims were to explore the lived experience of partners of people with NMO and to investigate potential carer burden in this population.Method: A mixed-method design was used; 11 partners of people with NMO completed semi-structured interviews; 54 partners completed Zarit Burden Interview and Hospital Anxiety and Depression Scale.Results: Three qualitative themes influenced partners’ quality of life (QoL): role/relationship; it’s all about them; and the impact of NMO. Life changed dramatically for participants after the first NMO attack, necessitating responsibility for physical, financial, social, and emotional support. As NMO symptoms improved and stabilized, freedom and QoL for spouses also improved, albeit with on-going worries regarding the impact of potential devastating future relapses. Quantitative findings showed mild/moderate carer burden (46%), mild/moderate anxiety (59%), and mild/moderate depression (24%). No partner indicated severe carer burden, anxiety, or depression.Conclusion: Participants regarded themselves as partners rather than carers whom require assessment and support for their emotional and health well-being. Health-care professionals need to acknowledge the important role partners play in the dynamics of the family unit, through greater discussion and inclusion.Implications for RehabilitationNMO has a strong impact on couples, resulting in both physical caregiving needs and anxiety regarding the unpredictability of potential devastating relapses.Partners do not necessarily experience clinically significant “burden”, anxiety or depression, and tools which screen for this may not capture the nature of their experiences.Health-care professionals need to acknowledge, consult, and respect the experience of partners during assessment and implementation of action plans.Partners should be individually assessed based upon the physical and emotional dependency created by NMO to improve their health and well-being.

Background: Neuropathic pruritus (itch) is an uncommon, but well described, symptom in neurology. There are itch-specific neurons in the dorsal horn of the spinal cord. We noted excessive pruritus in patients with neuromyelitis optica (NMO). Objective: We aimed to explore the characteristics of pruritus in NMO patients. Methods: We reviewed case records of a well-defined cohort of 45 serial aquaporin-4 antibody-positive patients visiting the national NMO service. All patients were interviewed. Results: Of the 45 antibody-positive NMO patients, 44 had myelitis and 12 of those 44 (27.3%) patients reported pruritus within a week of other symptoms of transverse myelitis with central cord involvement. In three patients, pruritus was the first symptom of a relapse, while in one case, pruritus was the very first symptom of the index episode of NMO. Conclusion: Neuropathic pruritus seems to be a common, but under-recognised symptom of myelitis associated with NMO.

Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder of the central nervous system. Relapses typically affect the optic nerves (uni/bilateral optic neuritis) and the spinal cord (myelitis). Disability often accrues with relapses and can occur early in the disease course. While there are many similarities with multiple sclerosis (MS), the identification of an antibody Aquaporin-4, with the water channel aquaporin-4 (AQP4) as its main target antigen, has led to advances in the understanding of NMO as an independent disease. These antibodies have been shown to be a highly specific and sensitive serum biomarker for NMO. This article summarizes the epidemiology, clinical features, diagnostic criteria, pathogenesis and treatment of NMO.

This article presents a commentary on the benefits that participation in quality of life research can offer prequalification members, using the case study of a qualitative investigation into quality of life in a rare neurological condition, neuromyelitis optica.

An audit project was undertaken by a group of multiple sclerosis (MS) specialist nurses who were seeking to establish whether or not they were providing appropriate care. The authors wanted to ascertain if the care being provided met the patients' requirements and whether their input had a direct impact on the quality of life of people with MS. An audit tool was collectively designed to enable them to conduct a retrospective evaluation of service users who have regular contact with an MS specialist nursing service, identify key ways MS nurses contribute to the outcomes and overall quality of patient care, identify patient opinions and experiences of the MS specialist nursing service, and to identify where improvements can be made. There was a response rate of 55% from 1350 questionnaires, and results showed that 94.9% of patients responded positively to ten statements relating to their involvement with MS specialist nurses, ranging from emotional support to educational and clinical input in a timely manner. Additionally, 83% reported preferring to contact their MS nurse for MS-related problems rather than any other health professional.

Background People are not expected to die from multiple sclerosis although, as the condition progresses over a period of time, some people become increasingly disabled and will require assistance with all activities of daily living. Their partners invariably carry out these tasks. Objective To gain a deeper understanding of the experiences of the partner living with and caring for a spouse disabled by multiple sclerosis. Methodology In a qualitative study, eight partners who live and care for a person with multiple sclerosis were interviewed using a semi-structured questionnaire to explore their experience of their role. Results The interview transcripts were analyzed using a thematic framework approach. Codes, themes and five categories were identified, which were worry, planning, frustrations, commitment to marriage and coping strategies. These categories were examined using quotes from the transcripts as evidence. Conclusion This qualitative study identified that these couples had been married for at least 20 years before disability of the spouse significantly affected their lifestyle. Partners felt obliged to continue in their caring role due to a sense of duty and commitment of marriage. Partners felt a sense of loss as they prioritized the health and needs of their spouse above their own and, finally, partners lost their identity as husband/wife as they were called ‘the carer’. Partners felt out of control due to the unpredictable and progressive nature of MS and because it consumed their life 24 hours every day. Partners often felt guilty at not being satisfied with their life and wanting some independence

Multiple sclerosis (MS) affects approximately 85000 people in the UK and is the commonest cause of disability in young adults. About 80% of patients begin with relapsing-remitting MS, where relapses are separated by periods of stability or improvement. A lifetime condition such as MS requires long-term treatment. Therefore, an important concern is to have a treatment that will not only remain effective over many years, but also one that patients find acceptable to use. This article focuses on the use of glatiramer acetate (Copaxone) in the management of MS. Copaxone was licensed in the UK in August 2000 for the reduction of relapse frequency in ambulatory patients with relapsing-remitting MS. This discussion considers the implications for patients′ relapse rates and disease progression of a 10-year clinical trial of glatiramer acetate.

BackgroundAntibodies against glycine receptors (GlyR Ab) have been strongly linked to progressive encephalomyelitis with rigidity and myoclonus (PERM). Their association with other neurological disorders is poorly understood.MethodsWe looked retrospectively at all patients who were tested for (GlyR Abs) in the Walton Centre between 2010–2014.Results138 patients were tested. The pre-test diagnoses (n) were transverse myelitis (34), NMO (22, (7 AQP4 IgG+ve and 15 AQP4 IgG-ve), optic neuritis (17), MS (22), ADEM (4), other atypical demyelination (4), encephalitis (11), epilepsy (4), dementia (4), parkinsonism (3), functional disorders (3) and others (10). 53.6% (74) had a relapsing course6/138 (4%) were positive for GlyR Ab. The diagnoses (n) were optic neuritis (2) one of which was AQP4 IgG+ve, NMO-AQP4-IgG negative (1), transverse myelitis (1), tumefactive demyelination (1) and undiagnosed spastic ataxic syndrome with normal imaging (1). 5/6 had a relapsing course and are on immunosuppressants.ConclusionAntibodies against GlyR are not common and seem to be associated with some non-PERM inflammatory CNS diseases, with a relapsing course. Larger studies are required to understand the clinical and prognostic significance of these early findings.

Introduction In the UK, rituximab is a second line treatment for relapse prevention in neuromyelitis optica (NMO), after conventional immunosuppressant medications, primarily due to cost and lack of high level evidence. Method Retrospective analysis of all patients of the UK NMO Service in Liverpool initiated on rituximab within the last 10 years. Results 50 patients were identified from total cohort of 183. 36 were AQP4-IgG positive, 3 were MOG-IgG positive and 11 were seronegative. Median follow-up was 21 months on rituximab (range 2.1–112 months). Rituximab reduced the pre-treatment annualised relapse rate (ARR) by 83% (95% CI 73%–89%, p Conclusion Rituximab is highly effective in preventing relapses in NMO. Considering the greatly disabling nature of NMO relapses there is an urgent need to commission it as a standard first line agent for NMO or for an RCT to establish unambiguous efficacy.

Background NMOSD is well known to cause physical disability. However the impact on a patient’s life is poorly quantified. One important aspect of this is loss of employment – but no data exists on the proportion of patients affected. Method A cross-sectional study of 103 patients (84 female, 19 male), median age 53 years (range 18–87) diagnosed aquaporin-4 antibody positive NMOSD, were assessed regarding their employment status. Results 91/103 patients were of working age when they had their first attack. 72/91 (79%) patients were employed at the time of their first attack. At last follow up 38/72 (53%) were not working; 27/38 (71%) had not worked since their first attack. Of those who continued to work- 34/72 (47%), 22/34 (65%) were in part time work; only 12/34 (35%) patients were in full time work. Conclusion 60/72 (83%) patients either stopped work or reduced hours following the onset of NMOSD. This has a major impact upon financial, family and social life besides the already known physical effects, highlighting the need for early aggressive treatments.

Objective To compare cognitive functioning in aquaporin-4 (AQP4) antibody positive and myelin oligodendrocyte glycoprotein (MOG) antibody positive neuromyelitis optica (NMO patients) matched by age, education and disease duration with matched healthy control (HC) participants. Method 15 NMO AQP4+, 15 NMO MOG +and 15 HC participants were administered a battery of neuropsychological tests. Test results were combined to give a single composite measure for cognitive functioning. Results Participants did not significantly differ on age, education level or disease duration. A significant overall effect of group was found on the cognitive composite measure. Post hoc analysis revealed NMO AQP4 +patients scored significantly worse than HCs (Tukey HSD; p=0.05), with no significant difference between NMO MOG+and HCs. Analysis of cognitive subtests identified significant differences on executive functioning testing between the NMO AQP4 +patients and HCs (Tukey HSD; p=0.02) but not between NMO MOG+patients and HCs. Conclusions Initial data confirms previous findings of evidence for cognitive impairments in NMO patients compared to HCs and indicates that cognitive dysfunction may be greater in AQP4 +patients compared to MOG+ patients, whom did not substantially differ from HCs.

Background NMOSD is well known to cause physical disability, however the impact on a patient’s life and work is poorly quantified. One important aspect of this is being unable to drive. No data exists on the proportion of patients affected. Method A cross-sectional study was conducted in 103 (84 female) patients with aquaporin-4 antibody positive NMOSD, of driving age (median age 53). Each patient was questioned about their driving status before onset of NMOSD and at present. Results 71/103 (73.1%) patients were driving before first attack. 6/103 (6%) patients never held a licence due to disability from NMOSD. Of those who were driving, 33/71 (46%) stopped. Of these 19/33 (58%) of patients said this was due to the effects of transverse myelitis (TM), 10/33 (30%) attributed it to optic neuritis (ON) whilst 2/33 (6%) reported it was due to both and a further 2/33 (6%) cited confidence issues. Conclusion 38% of patients are not able to drive due to the direct effects of NMOSD, with significant impacts on work, lifestyle and independence, highlighting the need to aggressively treat the disease early on.

Background The international panel for Neuromyelitis optica diagnosis (IPND) has published consensus diagnostic criteria for NMOSD. MOG IgG is present in some seronegative NMO. Objectives Assess impact of new criteria on diagnostic rates of NMO and frequency of MOG IgG in AQP4 IgG-ve NMO Methods All eligible patients seen in the NMO and atypical CNS demyelination clinic from January 2013 to Dec 2016 were tested for AQP4 IgG. Both the 2006 and 2015 criteria were applied. MOG IgG was tested in all seronegative NMO/SD. Results 233 patients were included. The 2006 criteria classified 75 patients as NMO (49 were AQP4 IgG +ve and 26 AQP4 IgG-ve). The 2015 criteria classified 130 patients as NMOSD (94 AQP4 IgG +ve and 36 AQP4 IgG-ve) an increase of 55 patients (73% rise). MOG IgG was present in 10/26 (38%) AQP4 IgG-ve NMO cases as per 2006 criteria and 15/36 (44%) of AQP4-IgG negative cases as per 2015 criteria. Conclusion Application of the 2015 IPND criteria increases the diagnosis of NMOSD by 73%. MOG IgG is found in 38%–44% of NMO/SD cases without AQP4 IgG.

Pruritus, better known as itch, is ‘an unpleasant cutaneous sensation provoking the desire to scratch’.1 It is typically caused by pruritogens that activate nerve endings in cutaneous and transitional tissues (eg, conjunctivae, anal mucosa) and serves a protective function against noxious stimuli. Itch can also occur with diseased or malfunctioning pruritogenic neurones in the absence of a pruritogen—a symptom termed neuropathic pruritus or neuropathic itch.2 Neuropathic pruritus is a well described but under-recognised symptom of neurological disorders. The site of involvement may be (i) peripheral nerve and root, (ii) spinal cord or (iii) brain. It may accompany a variety of peripheral and central neurological disorders, including postherpetic neuralgia, peripheral neuropathy, trigeminal neuralgia, traumatic nerve injury, complex regional pain syndrome, multiple sclerosis and stroke.3 Neuromyelitis optica is a relapsing inflammatory astrocytopathic disorder affecting predominantly the optic nerves and spinal cord. It is associated with antiaquaporin-4 immunoglobulin G (AQP4-IgG) in up to 70% of patients. Spinal cord involvement typically presents as a longitudinally-extensive transverse myelitis with associated sensorimotor and sphincter dysfunction. Sensory symptoms such as numbness, dysaesthesia, pain and tonic spasms are common.4 This report highlights neuropathic pruritus as an under-recognised feature of neuromyelitis optica. A 45-year-old woman with no previous medical history presented with a 4-day history of unexplained nausea, vomiting, hiccup and vertigo. On the fourth day, she developed bilateral forearm itching. Over the following 3 days, she developed rapidly progressive weakness from the chest down, ultimately resulting in complete paraplegia, sensory loss and loss of both bladder and bowel function. She also developed weakness in both arms, worse on the left side. She was admitted 3 days after the onset of her nausea and vomiting, which subsided a day later. However, the itching …

Neuromyelitis optica (NMO) is a rare autoimmune condition characterised by acute relapses of optic neuritis and extensive transverse myelitis. The aim of this qualitative study was to develop an improved understanding of the experiences of people living with NMO.Fifteen participants completed a semi-structured interview and data were interpreted using a constant comparative method. Participants were recruited from the Northern UK NMO Service.Five major themes were identified: diagnosis and treatment, symptoms, adjustment, identity and support.Results suggest NMO is a difficult condition to live with due to the unpredictability of relapses and accrued disability of visual or spinal symptoms occurring with each relapse. Poor vision, reduced mobility, bladder dysfunction and pain affected participants' independence and experience of living with NMO. Participants reported that during relapse and recovery they would "put their life on hold". They identified the importance of periods of stability to enable them to adjust to their condition and therefore aim for "normality" of life that they believed was comparable to their peers. Implications for Rehabilitation Disability due to NMO has a major impact on an individual's life, abilities and identity. Timely treatment of relapse is essential to reduce or prevent disability. Health care professionals need to coordinate care locally and nationally. Multi-disciplinary work is vital to promote adjustment, coping strategies and support for people living with NMO.

Prompt treatment of neuromyelitis optica (NMO) relapses with steroids or plasma exchange (PLEX) often prevents irreversible disability. The objective of this study is to report the use of intravenous immunoglobulins (IVIG) as treatment for acute relapses in NMO. A retrospective review of 10 patients treated with IVIG for acute relapses was conducted. IVIG was used in the majority of cases because of lack of response to steroids with/without PLEX. Improvement was noted in five of 11 (45.5%) events; the remaining had no further worsening. One patient, a 79-year-old woman, had a myocardial infarction seven days after IVIG. IVIG may have a role in treating acute NMO relapses.

Background Transverse myelitis (TM) associated with neuromyelitis optica (NMO) has many unusual characteristics compared with TM in Multiple Sclerosis (MS): long centrally located lesions that cause significant motor-sensory (including tonic spasms) and autonomic dysfunction. We wish to report a symptom not previously described in neurological literature, neuropathic post ejaculatory pain (PEP). Methods We will present videos of 3 male patients with AQP4-IgG +ve NMO describing post-ejaculatory pain (PEP). Results All patients were recovering from long thoracic TM and had autonomic dysfunction at nadir. None had preexisting urological symptoms. The pain occurred immediately after orgasm and ejaculation and was severe, brief ( Conclusion PEP has not been described in neurological disorders and maybe a unique feature of NMO associated myelitis.

Objective This study outlines the development of a patient-reported outcome measure (PROM), an instrument to obtain self-reported health status for neuromyeltis optica (NMO), a disabling neurological condition. Design Development was conducted in accordance with international guidance for PROMs including systematic review of existing literature, item generation guided by qualitative interviews, health-related quality of life conceptual framework and clinical expert panel and cognitive interviews with NMO patients. Setting Participants were identified through a national NMO clinic in a tertiary NHS neurosciences service. Participants 15 individuals with NMO participated in cognitive interviews requiring review and ranking of proposed PROM items and qualitative feedback on content, layout and response options. Results Participants endorsed the draft instrument as reflecting their experience of the condition and as being easy to understand. Rating and ranking of item relevance and importance reduced the draft instrument from 106 to 48 items. Participant feedback on overlapping items eliminated a further 2 items and resulted in a preliminary instrument of 46 items. As a direct result of participant feedback ordering of the 10 domains was revised, a 4 option Likert scale was employed and a 4-week recall period for impact of symptoms was selected. Conclusions A 46-item instrument developed in accordance with international PROM development guidelines through literature review, developed by subject matter experts and refined through pretesting examining content validity provides a preliminary measure for assessing patient-report of health status in NMO. Further evaluation is proposed including sensitivity to clinical change, and international contributions to evaluating the measure are encouraged.

Multiple sclerosis (MS) typically affects people aged 20–40years and, by its very nature, is characterized by unpredictability, uncertainty and variability, and is therefore bound to have an impact on children who have a parent with MS. There has been little work done to assess the needs of young people living with MS in their family. A series of workshops specifically aimed at 9–14-year-olds who have a parent with MS have been successfully conducted. This article reviews the background to setting up the workshop and identifies implications for future MS specialist nurses to help this potentially vulnerable group of people. By breaking the silence and talking about MS with young people, the myths can be removed and the anxieties reduced.

Patients who have had a stroke are often unable to support their full weight when moving. Recent guidelines on patient handling stress the risk of injury to nurses' backs if they lift patients or support their body weight. Nurses in one stroke rehabilitation unit who used a 'pivot' transfer had to reconsider their practice and explore other options for moving patients.

Background The impairment of mobility due the severe myelitis in NMO is well known, but the effect on bladder, bowel and sexual function is not reported. We report the frequency of these problems in NMO. Method 50 patients [42 females) with aquaporin-4 antibody positive NMO spectrum disorder, all with myelitis, were studied. They completed validated organ-specific questionnaires: Bladder Control Scale (BLCS), Bowel Control Scale (BWCS), Sexual Satisfaction Scale (SSS). Results Bladder symptoms were reported in 78% and 44% experienced severely restricted lifestyle due to these. Similarly 80% had high bowel scores (54% constipation) and 24% experienced lifestyle impairment. Of the 29 patients in a relationship 12 (41%) were dissatisfied with their sexual life, four of five men had erectile dysfunction. Conclusion This first ever report on the extent of pelvic organ dysfunction in NMO identified significant and oft hidden morbidity. The importance of assessment of these symptoms is emphasised by the availability of treatments for them. It also highlights the importance of treating and preventing relapses in NMO.

Background Longitudinally extensive myelitis (>3 vertebral segments) is considered the sine-qua-non of spinal cord involvement in NMO and short spinal cord lesions typical of MS. Similarly, small callosal lesions and U fibre involvement are thought to be characteristic of MS and uncommon in NMO. We present 2 cases that are exceptions to these. Results Case 1 : A 22 year-old man on azathioprine for aquaporin-4 antibody positive NMO, reported slight worsening of his pre-existing visual symptoms. He was already known to have other brain MRI changes supportive for NMO. Further MRI showed small callosal lesions, juxtacortical lesions in the U-fiber layer, which on their own would appear typical for MS. Case 2 A 38 year-old Asian woman, on azathioprine for aquaporin-4 antibody positive NMO presented with new onset sensory symptoms in the arms. MRI showed new small enhancing lesions at T4 and T6/7 of one segment length only. She was promptly started on steroids with resolution of symptoms. Conclusions MS-typical brain and spinal cord lesions can be found in NMO. As our knowledge of the spectrum of NMO expands many more unusual clinical and radiological findings are likely to emerge. Prompt recognition and imaging of relapses may influence such the findings.

Background Cognitive impairment has been reported in patients with neuromyelitis optica (NMO) but little is known about the impacts of cognitive symptoms on well-being in patients with the condition. Methods NMO patients completed a neuropsychological battery and self-report measures of cognitive symptoms, mood and well-being. Results 31 patients with a mean age of 47.03 years (SD 15.26), disease duration 9.13 years (SD 8.95) and EDSS 3.74 (SD 2.03) participated. Although patients self-reported substantial cognitive difficulties, only objective cognitive performance and not subjective cognitive complaints were significantly associated with quality of life (QoL) ratings (r=−0.39, p=0.04 vs r=0.25, p=0.20). The Symbol Digit Modalities Test was the only cognitive test individually associated with QoL (r=0.52, p=0.01) but was not significantly associated with levels of mood symptoms. In contrast, degree of cognitive impairment (based on number of domains impaired) was significantly associated with overall QoL (r=−0.39, p=0.04) and HADS depression (r=0.49, p=0.01) and anxiety (r=0.38, p=0.04). Conclusions Our results suggest that objective cognitive impairments are associated with reduced quality of life and increased mood symptoms but are not readily identified through patient self-report of cognitive symptoms. Patient self-report may not capture the extent of cognitive dysfunction in NMO.

Importance Neuromyelitis optica is associated with severe neurodisability if not recognized and treated promptly. Several autoimmune disorders are associated with this condition and may vary in their presentation. It is essential that clinicians are aware of the uncommon presenting features of neuromyelitis optica and associated autoimmune conditions. Observations A 53-year-old woman presented with nausea and vomiting and was noted to have an asymptomatic elevated creatinine kinase level, which improved with conservative management. She had a history of iron-deficiency anemia due to long-standing celiac disease that was managed with a gluten-free diet. She then presented with recurrent transverse myelitis and a vesicobullous rash over her arms and feet that was pruritic and excoriating. Skin biopsy results confirmed a clinical diagnosis of dermatitis herpetiformis and antibody test findings against aquaporin-4 were positive, leading to a diagnosis of neuromyelitis optica spectrum disorder. She was treated with methylprednisolone sodium succinate, plasma exchange, and azathioprine and has remained in remission. Conclusions and Relevance This report highlights the association of neuromyelitis optica with dermatitis herpetiformis, which can present even without clinical features of celiac disease. Nausea, vomiting, and asymptomatic hyperCKemia should be recognized as rare presenting features of neuromyelitis optica.

Background Neuromyelitis optica (NMO) is a relapsing inflammatory demyelinating disorder associated and mediated by aquaporin–4 antibody (AQP4–Ab), affecting predominantly the optic nerves, spinal cord (as longitudinally extensive myelitis) and often the brainstem. Relapses of NMO are usually severe and disabling. Respiratory failure, often due to brainstem or high cervical cord involvement, is a feared complication of NMO. We aimed to determine the causes and frequency of respiratory failure in NMO. Methods Retrospective medical record review of all patients with a diagnosis of NMO fulfilling criteria (2006) seen by the Walton Centre NMO service, UK till March 2012, were reviewed. Results We identified 85 patients (66 women, 69 with AQP4–Ab) with a median age of 51 (13.1–80.9) years. Between them they had 417 relapses over their disease course. Respiratory dysfunction that occurred after onset of NMO was reported in 19/85 (22.3%, 16 women, 17 with AQP4–Ab) patients. In 14/85 patients (16%) of these patients it was related to a relapse. This accounted for 3.36% (14/417) of all relapses. 6/85 (7%) patients required invasive ventilation. One patient died during the brainstem relapse. Conclusions Though only a minority of relapses in NMO cause respiratory failure, about 20% of all NMO patients, will have some respiratory dysfunction during the course of their illness.

Background Neuromyelitis Optica (NMO) is an autoimmune inflammatory demyelinating disorder of the central nervous system that affects the optic nerve and spinal cord: frequent relapses and the absence of full remission leads to accumulation of deficits and rapid progression of disability. Despite severe weakness and other features of myelitis, patients often describe pain as their most disabling symptom. The aim of the study was to assess any disparity between neurology clinical consultation (and its documentation) and a dedicated interview focussing on the presence and quality of pain in a specialist NMO service. Method A retrospective case note review of all patients attending the national NMO clinic at the Walton Centre, Liverpool UK, April- July 2011 was performed in all patients for documentation of pain by an investigator (SZ). A second independent investigator (KM) performed a structured interview and administered the Brief Pain Inventory (BPI) to assess neuropathic pain. Results 61 patients and their respective records were assessed by both investigators: 45 were diagnosed with NMO and 16 NMO spectrum (only patients who had at least one episode of myelitis were included). AQP4 antibody was present in 72% patients. MRI showed lesions in the cervical or upper-thoracic cord most commonly. The case note review documented neuropathic pain in 23/61 (38%) patients. In these patients documentation of the location of pain was 100%, whilst duration (21%), quality (62%), severity (26%), temporality (31%) and exacerbating/relieving factors (45%) were less frequently recorded. However using the BPI, 37/61 (61%) patients reported neuropathic pain (ie. pain in 38% of patients was not documented to have been recognised): 23/37 (62%) had constant pain. Areas of worst pain were in the legs in 81% (predominantly bilateral), arms (35%), back of trunk (73%), front of trunk (22%) and base of skull/neck (19%). On a scale of 0 to 10, mean severity for worst pain experienced in the last 24 hours was 5.3 (±3.2 SD), with the average pain scored at 3.6 (±2.8 SD). Response to analgesia gave a mean pain reduction of 46% (±35% SD). Pain significantly affected QoL (defined as >5 on a scale of 0 to 10) in terms of: general activity (44%), mood (38%), walking ability (38%), enjoyment of life (32%), normal work (29%), relation with others (29%) and sleep (15%). Conclusion Though neuropathic pain is an important symptom of NMO, the clinician faced with gamut of symptoms associated with typical myelitis (weakness, stiffness, numbness incontinence, immobility, social issues) can overlook pain in many patients (38% of documented cases) in this study. The study also confirms that pain can be severe, is often constant, and significantly affects QoL. The use of a validated questionnaire such as the BPI is recommended and is now part of the routine assessment of all new NMO cases referred to in the Walton centre multidisciplinary NMO clinic.

Background Pruritis (itch) is primarily a dermatologic problem. ‘Neurogenic pruritus’ (NP) has been described in some neurological diseases including MS and in a case with neuromyelitis optica spectrum disorder (NMOSD). Though the physiology of NP is uncertain ascending pathways or central processing could be involved. Aim To assess the characteristics of NP in NMOSD patients. Method Retrospective case note review followed by prospective telephone/clinic interviews of all AQP4 positive patients who attended National NMO clinic after 1 January 2011. Results 14/45 patients reported significant pruritus during the course of NMO and were interviewed. After excluding patients with only optic neuritis or an obvious skin/systemic cause 12 (26.7%) had NP occurring within a median of 3(0–90) months from onset of NMO. The median severity on visual analogue score was 6 (2–9). In three cases pruritus developed at a median of 5 (2–28) days before onset of motor-sensory symptoms of transverse myelitis (TM); most of the remaining patients developed it within 1 month after the onset of TM. A long cervical cord lesion was seen in 9 of 12 cases and correlated well with the dermatomal distribution of NP. Conclusions NP seems to be a common symptom associated with the TM of NMOSD.

Background: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described. Objective: To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs). Methods: We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population. Results: Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) ( pConclusions: This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.