Performance of an Arabic translation of the patient determined disease steps (PDDS) scale in Saudi patients with multiple sclerosis.

The Expanded Disability Status Scale (EDSS) is commonly used to measure and quantify disabilities in patients with multiple sclerosis (MS). The patient-determined disease steps (PDDS) scale is a patient-reported measure of disability that is useful in MS. However, the Arabic version of the PDDS has only been tested in Jordanian patients. Although both populations share similar Arabic languages, it is plausible that differences in dialects and educational systems could alter the generalizability of the tool. In this prospective study, patients with MS were asked to complete a printed translated version of the (PDDS), and the results were compared to their EDSS scores, functional system scores, and walking speed measures. Patients with relapsing or progressive MS were included in the study. Spearman rho rank-order correlation coefficients (P) were used to measure the correlation between the PDDS and other variables. We considered previously reported P values > .1, .3, and .5 as small, moderate, and strong correlations, respectively. A total of 79 patients completed the study. The PDDS showed a strong correlation with the EDSS (P = .69, 95% confidence interval 0.55-0.79, P < .001). PDDS is associated with cerebellar, pyramidal, and bladder dysfunctions. It was also moderately correlated with the timed-25-foot walk test and timed-up-and-go test. The Arabic version of the PDDS performed similarly to English and other languages when tested on a cohort of patients with MS.

Restless leg syndrome in multiple sclerosis: a case-control study.

Objectives: This study assessed the prevalence of restless leg syndrome (RLS) among patients with multiple sclerosis (pwMS) and the association between RLS and MS disease duration, sleep disturbance, and daytime fatigue. Methods: In this cross-sectional study, we interviewed 123 patients via phone calls using preset questionnaires containing the International Restless Legs Syndrome Study Group (IRLSSG) diagnostic criteria, Pittsburgh Sleep Quality Index (PSQI), and Fatigue Severity Scale (FSS) diagnostic criteria validated in both Arabic and English. The prevalence of RLS in MS was compared to a group of healthy controls. Results: The prevalence of RLS in pwMS, defined by meeting all four requirements included in the IRLSSG diagnostic criteria, was 30.3% compared to 8.3% in the control group. About 27.3% had mild RLS, 36.4% presented with moderate, and the remaining had severe or very severe symptoms. Patients with MS who experience RLS had a 2.8 times higher risk of fatigue compared to pwMS without RLS. pwMS with RLS had worse sleep quality, with a mean difference of 0.64 in the global PSQI score. Sleep disturbance and latency had the most significant impact on sleep quality. Conclusion: The prevalence of RLS among MS patients was significantly higher compared to the control group. We recommend educating neurologists and general physicians to increase their awareness of the increasing prevalence of RLS and its association with fatigue and sleep disturbance in patients with MS.

Clinical and economic evaluations of natalizumab, rituximab, and ocrelizumab for the management of relapsing-remitting multiple sclerosis in Saudi Arabia.

INTRODUCTION: The advent of new disease-modifying therapies (DMTs), such as monoclonal antibodies (mAbs), resulted in significant changes in the treatment guidelines for Multiple sclerosis (MS) and improvement in the clinical outcomes. However, mAbs, such as rituximab, natalizumab, and ocrelizumab, are expensive with variable effectiveness rates. Thus, the present study aimed to compare the direct medical cost and consequences (e.g., clinical relapse, disability progression, and new MRI lesions) between rituximab and natalizumab in managing relapsing-remitting multiple sclerosis (RRMS) in Saudi Arabia. Also, the study aimed to explore the cost and consequence of ocrelizumab in managing RRMS as a second-choice treatment. METHODS: The electronic medical records (EMRs) of patients with RRMS were retrospectively reviewed to retrieve the patients' baseline characteristics and disease progression from two tertiary care centers in Riyadh, Saudi Arabia. Biologic-naïve patients treated with rituximab or natalizumab or those switched to ocrelizumab and treated for at least six months were included in the study. The effectiveness rate was defined as no evidence of disease activity (NEDA-3) (i.e., absence of new T2 or T1 gadolinium (Gd) lesions as demonstrated by the Magnetic Resonance Imaging (MRI), disability progression, and clinical relapses), while the direct medical costs were estimated based on the utilization of healthcare resources. In addition, bootstrapping with 10,000 replications and inverse probability weighting based on propensity score were conducted. RESULTS: Ninety-three patients met the inclusion criteria and were included in the analysis (natalizumab (n = 50), rituximab (n = 26), ocrelizumab (n = 17)). Most of the patients were otherwise healthy (81.72%), under 35 years of age (76.34%), females (61.29%), and on the same mAb for more than one year (83.87%). The mean effectiveness rates for natalizumab, rituximab, and ocrelizumab were 72.00%, 76.92%, and 58.83%, respectively. Natalizumab mean incremental cost compared to rituximab was $35,383 (95% CI: $25,401.09- $49,717.92), and its mean effectiveness rate was 4.92% lower than rituximab (95% CI: -30-27.5) with 59.41% confidence level that rituximab will be dominant. CONCLUSIONS: Rituximab seems to be more effective and is less costly than natalizumab in the management of RRMS. Ocrelizumab does not seem to slow the rates of disease progression among patients previously treated with natalizumab.

Saudi consensus recommendations on the management of multiple sclerosis: MS management in children and adolescents.

Multiple sclerosis (MS) most commonly presents in young adults, although 3-5% of patients develop MS prior to the age of 18 years. The new and comprehensive consensus for the management of MS in Saudi Arabia includes recommendations for the management of MS and other CNS inflammatory demyelinating disorders in pediatric and adolescent patients. This article summarizes the key recommendations for the diagnosis and management of these disorders in young patients. Pediatric and adult populations with MS differ in their presentation and clinical course. Careful differential diagnosis is important to exclude alternative diagnoses such as acute disseminated encephalomyelitis (ADEM) or neuromyelitis optica spectrum disorders (NMOSD). The diagnosis of MS in a pediatric/adolescent patient is based on the 2017 McDonald diagnostic criteria, as in adults, once the possibility of ADEM or NMOSD has been ruled out. Few data are available from randomized trials to support the use of a specific disease-modifying therapy (DMT) in this population. Interferons and glatiramer acetate are preferred initial choices for DMTs based on observational evidence, with the requirement of a switch to a more effective DMT if breakthrough MS activity occurs.

Fatigue, Depression, and Anxiety Among Ambulating Multiple Sclerosis Patients.

Background: Multiple sclerosis (MS) is an inflammatory disease associated with adverse effects: including depression, anxiety, fatigue, which may affect physical activity and the quality of life (QoL) among patients with MS (pwMS). Objective: This study aims to assess the prevalence of depression, anxiety, and fatigue among pwMS who have no physical disability in Saudi Arabia, and demonstrate any correlation between these factors and physical activity as well as the QoL. Methods: A cross-sectional study was conducted in the Neuroimmunology outpatient clinics in King Fahad Medical City (KFMC) and King Saud University Medical City (KSUMC) in Riyadh City, KSA. The Arabic version of the Hospital Anxiety and Depression Scale (HADS) was used to measure anxiety and depression levels. The HADS scores were then categorized into three levels according to the total points: normal (0-7 points), borderline (7-10 points), and anxiety/depression (11 - 21 points). The Arabic version of the Fatigue Severity Scale (FSS) was used to measure fatigue (cut-off point ≥5). The physical activity was measured by the Arabic version of the short form of the International Physical Activity Questionnaire (IPAQ), which measure time spent walking, moderate- and vigorous-intensity physical activity of at least 10 minutes duration. The QoL was also measured by the Arabic version of the EuroQOL five-dimensional (EQ-5D-3L) instrument (i.e., mobility, self-care, usual activities, pain/discomfort, and anxiety/depression). Results: A total of 323 pwMS participated in this study, 83 had scores that indicated anxiety (25.7%) and 44 had depression (13.6%). The majority of patients had scores with the normal range of depression and anxiety (70% and 57% respectively). The mean of EuroQol Group visual analogue scale (EQ-VAS) score was 80.43 (SD=19.8). 156 (48.3%) out of 323 pwMS reported fatigue while the remainder had no fatigue (n=167, 51.7%). The results indicate that only 143 patients (44.3%) had participated in vigorous physical activity during the last 70 days, with a median of 3 days per week (IQR= 5-3) and a median of 60 minutes per day 0 (Interquartile range: IQR = 60-30). Only 149 patients (49.2%) had patricpated in moderate physical activities during the previous week with a median of 3 days per week (IQR = 5-3) and a median of 40 minutes per day (IQR = 60-30). 194 patients had participated in walking activities (60.0%) with a median of 5 days per week (IQR = 7-3) and a median of 45 minutes per day (IQR = 60-30). The results revealed that fatigue was positively correlated with depression (r = 0.407, p-value < 0.001) and anxiety (r = 0.289, p-value < 0.001). Conclusion: The current study shows depression, anxiety, and fatigue tend to be correlated and clustered together among pwMS in our cohort. However, fatigue is not associated with the intensity of physical activity undertaken. The results of this study are important for the improvement of the clinical management of MS patients.

Assessment of factors associated with depression and anxiety among pwMS in Saudi Arabia.

BACKGROUND: Multiple sclerosis (MS) is an inflammatory chronic disease that is characterized by an increased prevalence of adverse mental health outcomes in patients with MS (pwMS). The main aim of this study is to investigate the factors of depression and anxiety in pwMS in the Kingdom of Saudi Arabia (KSA). MATERIALS AND METHODS: This is a cross-sectional study conducted in KSA during the period from March to June 2020. Participants were recruited from the Neuroimmunology clinics in King Fahad Medical City (KFMC) and King Saud University medical city (KSUMC)in Riyadh City, KSA. The Hospital Anxiety and Depression Scale (HADS) was used to measure depression and anxiety. Fatigue Severity Scale (FSS) was used to measure fatigue in pwMS. A simple random sampling technique was utilized to select participants and the data were analyzed using SPSS v.24.0. RESULTS: A total of 529 participants participated in this study with a response rate of 53.1%. The prevalences of anxiety and depression were 35.3% and 19.7%, respectively. The findings also revealed that depression was more likely to be significantly affected by being male, low education, unemployment, physical inactivity, and fatigue but the anxiety was significantly affected by region, unemployment, short duration since last MS relapse, physical inactivity, and fatigue. CONCLUSION: Anxiety and depression are not uncommon in pwMS. Given their impact on the lives of affected patients, early detection and management of these symptoms and their associated factors are crucial.

Waterpipe Tobacco Smoking and Other Multiple Sclerosis Environmental Risk Factors.

INTRODUCTION: There are a number of well-established risk factors for multiple sclerosis (MS). Other factors, however, showed conflicting or inconsistent results. Here, we examine some factors that are unique to or more practiced in Saudi Arabia (SA) and the Arab region such as waterpipe tobacco smoking (WTS), face veiling, raw milk (RM) and camel milk (CM) consumption, and tuberculosis (TB) infection in addition to other traditional factors. METHODS: This is a sex- and age-matched case-control study in which we used a structured questionnaire to examine the relation between a number of factors and exposures and the risk of MS. Three hundred MS patients and 601 controls were included. Data were analyzed across different statistical models using logistic regression adjusting for age, sex, marital status, duration of breastfeeding, age first joining school, coffee consumption, and face exposure. RESULTS: Cigarette smoking (OR = 1.79, [95% CI: 1.01-3.17], p = 0.047), WTS (OR = 2.25, [95% CI: 1.21-4.15], p = 0.010), and CM consumption (OR = 2.50, [95% CI: 1.20-5.21], p = 0.014) increased the risk of MS, while performing hajj (OR = 0.47, [95% CI: 0.34-0.67], p = 0.001), TB infection (OR = 0.29, [95% CI: 0.11-0.78], p = 0.015), face veiling (OR = 0.32, [95% CI: 0.23-0.47], p = 0.001), and coffee consumption (OR = 0.67, [95% CI: 0.49-0.89], p = 0.008) appeared to be associated with decreased risk. No association was found between fast food, processed meat, soft drinks, animal milk (other than camel), or RM consumption and the risk of MS. CONCLUSION: The results of this case-control study confirm that different means of tobacco smoking are associated with increased risk of MS. It also sheds more light on the complex association between infections and MS.

Real-World Comparative Cost-Effectiveness Analysis of Different Classes of Disease-Modifying Therapies for Relapsing-Remitting Multiple Sclerosis in Saudi Arabia.

The very fact that multiple sclerosis (MS) is incurable and necessitates life-long care makes it one of the most burdensome illnesses. The aim of this study was to compare the cost-effectiveness of orally administered medications (e.g., fingolimod, dimethyl fumarate, and teriflunomide), interferon (IFN)-based therapy, and monoclonal antibodies (MABs) (e.g., natalizumab and rituximab) in the management of relapsing-remitting multiple sclerosis (RRMS) in Saudi Arabia using real-world data. This was a retrospective cohort study in which patients with RRMS aged ≥18 years without any other chronic health conditions with non-missing data for at least 12 months were recruited from the electronic health records of a university-affiliated tertiary care center. Multiple logistic regressions controlling for age, sex, and duration of therapy were conducted to examine the odds of disability progression, clinical relapse, MRI lesions, and composite outcome (e.g., relapse, lesion development on MRI, disability progression). The number of patients who met the inclusion criteria and were included in the analysis was 146. Most of the patients were female (70.51%) and young (e.g., ≤35 years of age). There were 40 patients on the orally administered agents (e.g., dimethyl fumarate, teriflunomide, fingolimod), 66 patients were on IFN-based therapy (e.g., Rebif®), and 40 patients were on monoclonal antibodies (e.g., rituximab and natalizumab). Patients on MABs had lower odds of the composite outcome (OR = 0.17 (95% CI: 0.068-0.428)). The use of orally administered agents was dominant (e.g., more effective and less costly), with average annual cost savings of USD -4336.65 (95% CI: -5207.89--3903.32) and 8.11% higher rate of effectiveness (95% CI: -14.81-18.07) when compared with Rebif®. With regard to the use of MABs in comparison to Rebif®, MABs were associated with higher cost but a better rate of effectiveness, with an average additional annual cost of USD 1381.54 (95% CI: 421.31-3621.06) and 43.11% higher rate of effectiveness (95% CI: 30.38-61.15) when compared with Rebif®. In addition, the use of MABs was associated with higher cost but a better rate of effectiveness, with an average additional annual cost of USD 5717.88 (95% CI: 4970.75-8272.66) and 35% higher rate of effectiveness (95% CI: 10.0-42.50) when compared with orally administered agents. The use of MABs in the management of RRMS among the young patient population has shown to be the most effective therapy in comparison to both IFN-based therapy (e.g., Rebif®) and orally administered agents, but with higher cost. Orally administered agents resulted in better outcomes and lower costs in comparison to IFN-based therapy. Future studies should further examine the cost-effectiveness of different disease-modifying therapies for the management of RRMS using more robust study designs.

Conversion disorder upon emergence from general anesthesia-A case report and review of literature.

Conversion disorder (CD) is a neurological symptom that is not related to any medical or neurological disease. Symptoms can range from sensory complaints to loss of consciousness. Psychological stressors such as surgery and anesthesia are considered a precipitating factors. Early diagnosis is crucial to prevent unnecessary interventions. Here, we report a case of a patient who developed CD upon emergence from general anesthesia.

Registry of patients with multiple sclerosis and COVID-19 infection in Saudi Arabia.

BACKGROUND: The outbreak of coronavirus disease 2019 (COVID-19) has rapidly spread and developed as a pandemic threatening global health. Patients with multiple sclerosis (MS)-an autoimmune demyelinating inflammatory disease of the central nervous system (CNS)-are predominantly treated with immunomodulatory/immunosuppressive disease-modifying therapies (DMTs), which can increase the risk of infection. Therefore, there is concern that these patients may have a higher risk of COVID-19. In response to growing concerns of neurologists and patients, this study aimed to determine the prevalence, severity, and possible complications of COVID-19 infection in patients with MS in Saudi Arabia (SA). METHODS: In this prospective cohort study, demographic and clinical data were obtained from patients residing in SA with MS who had a positive result for COVID-19 per reverse transcription-polymerase chain reaction test or viral gene sequencing, using respiratory or plasma samples. Comparison of COVID-19 severity groups was performed using one-way ANOVA or Kruskal-Wallis test for numerical variables and Chi-squared test for categorical variables. RESULTS: Seventy patients with MS and COVID-19 (71% female) were included in this analysis. Of the 53 (75.7%) patients receiving a DMT at the time of COVID-19 infection, the most frequently used DMTs were fingolimod (25%) and interferon-beta (25%). Nine (13%) patients had MS relapse and were treated with intravenous methylprednisolone in the four weeks before COVID-19 infection. The most common symptoms at the peak of COVID-19 infection were fever (46%), fatigue (37%), and headache (36%). Symptoms lasted for a mean duration of 8.7 days; all symptomatic patients recovered and no deaths were reported. COVID-19 severity was categorized in three groups: asymptomatic (n = 12), mild-not requiring hospitalization (n = 48), and requiring hospitalization (n = 10; two of whom were admitted to the intensive care unit [ICU]). Between the three groups, comparison of age, body mass index , Expanded Disability Severity Score , MS disease duration, and DMT use at the time of infection showed no significant differences. A higher percentage of patients who were admitted to hospital or the ICU (40%; p = 0.026) presented with an MS relapse within the prior four weeks compared with those who were asymptomatic or had a mild infection (both 8.3%). CONCLUSION: These findings present a reassuring picture regarding COVID-19 infection in patients with MS. However, patients with MS who have had a relapse in the preceding four weeks (requiring glucocorticoid treatment) may have an increased risk of severe COVID-19.

Family Planning for People with Multiple Sclerosis in Saudi Arabia: an Expert Consensus.

More than half of all patients with multiple sclerosis (MS) in the Kingdom of Saudi Arabia (KSA) are women of childbearing age. Raising a family is an important life goal for women in our region of the world. However, fears and misconceptions about the clinical course of relapsing-remitting MS (RRMS) and the effects of disease-modifying drugs (DMDs) on the foetus have led many women to reduce their expectations of raising a family, sometimes even to the point of avoiding pregnancy altogether. The increase in the number of DMDs available to manage RRMS and recent studies on their effects in pregnancy have broadened management options for these women. Interferon beta now has an indication in Europe for use during pregnancy (according to clinical need) and can be used during breastfeeding. Glatiramer acetate is a further possible option for women with lower levels of RRMS disease activity who are, or about to become, pregnant; natalizumab may be used up to 30 weeks in patients with higher levels of disease activity. Where possible, physicians need to support and encourage women to pursue their dream of a fulfilling family life, supported where necessary by active interventions for RRMS that are increasingly evidence based.

Shared breastfeeding & other early multiple sclerosis risk factors: A case-control study.

BACKGROUND: Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease. Etiology is thought to be multifactorial with genetic and environmental factors interplay. Our objective in this study is to evaluate culture specific and other early life risk factors for MS. We examined the association between MS and breastfeeding including shared breastfeeding, parental consanguinity, being born abroad or living abroad during childhood, prematurity, vaccination, tonsillectomy, rank among siblings, number of siblings, number of household members (HHM) at birth, and age first time joining school. METHODS: This is an age and sex matched case-control study that was conducted in Riyadh, Kingdom of Saudi Arabia (KSA). We enrolled 300 cases and 601 controls. A structured questionnaire about demographics, consanguinity and potential environmental factors was answered by participants. Data was analyzed using logistic regression adjusting for covariates occurring later in life such as waterpipe smoking and performing Hajj. RESULTS: About two thirds of the cases and the controls were females. Mean age was 34.8 (9.2) for the cases and 33.6 (10.6) for the controls. We found that shared breastfeeding (OR=0.58; 95% CI, 0.35-0.96, p = 0.033), and older age first joining school (OR=0.83; 95% CI, 0.73-0.94, p = 0.005) were associated with decrease risk of MS. While longer duration of breastfeeding by biological mother (OR=1.03; 95% CI, 1.01-1.04, p = 0.001), rank among siblings of ≥6 (OR=1.69; 95% CI, 1.11-2.56, p = 0.014), and larger number of HHM at birth (OR=2.32; 95% CI, 1.64-3.28, p = 0.001) were associated with increased risk. Patients with MS were less likely to receive formula with breastfeeding than controls (OR=0.72; 95% CI, 0.51-0.99, p = 0.046). No association was found with breastfeeding by biological mother, number of siblings, prematurity, being born abroad or living abroad during childhood, vaccination, consanguinity, or tonsillectomy. CONCLUSION: The findings of this case-control study add to the accumulating evidence that early life factors could modify the risk of developing MS. Among these, novel associations with shared breastfeeding and number of HHM at birth are suggested. Future studies are needed to verify the observed results.

Electrodiagnostic reference data for sensory nerve conduction studies in Saudi Arabia.

OBJECTIVE: To estimate reference data for the commonly performed sensory nerve conduction studies (NCS) using a cohort of healthy subjects from Saudi Arabia. METHODS: This is a cross-sectional study conducted between May 2015, and June 2019. Sensory nerve action potential (SNAP) amplitude, conduction velocity (CV), and peak latency (PL) were recorded. Associations between these parameters and the covariates (age, sex, height, weight, and body mass index) were tested with Pearson correlations. Reference data were then derived using the lowest percentile that could be reliably determined for SNAP amplitude and CV. Reference data were derived using the highest percentile for PL. RESULTS: Upper and lower limb sensory NCS were performed in 127 and 137 participants, respectively. Age was the only covariate that required adjustment for estimation of SNAP amplitude. Therefore, a prediction model was generated for each nerve. Percentile estimation for PL and CV did not require adjustment for any of the covariates. Hence, it was derived for all the subjects pooled together. CONCLUSION: The sensory NCS reference data were comparable to the data from other countries. However, minimal differences were observed. Further studies are required with a focus on the older age group.

Electrodiagnostic reference data for motor nerve conduction studies in Saudi Arabia.

OBJECTIVE: To determine nerve conduction studies (NCS) reference data for motor nerves and F-waves in the upper and lower limbs of healthy subjects in Saudi Arabia. METHODS: This is a cross-sectional study conducted between May 2015 and June 2019. Healthy subjects without neurological or systemic diseases were recruited. Motor NCS were performed following a standard protocol. Pearson correlations were employed between NCS parameters and age, gender, height, weight, and body mass index. Reference data were generated using the percentile method. RESULTS: A total of 127 subjects were recruited for the upper limb studies and 137 for the lower limb studies. Quantile regression models were generated to estimate compound muscle action potential amplitude (adjusted for age), as well as F-wave minimal latency (adjusted for height). The estimated reference limits of distal motor latency (ms) and conduction velocity (m/s) for the different nerves were, respectively, 3.7 and 50 for the median nerve, 3.3 and 50 for the ulnar nerve, 5.8 and 40 for the tibial nerve, and 5.0 and 40 for the fibular nerve. CONCLUSION: The reference data for motor NCS parameters and F-wave minimal latency are generally comparable with those of Western countries. However, minimal differences were observed. The underrepresentation of the older age group warrants future studies. The reference data for motor NCS parameters and F-wave minimal latency are generally comparable with those of Western countries. However, minimal differences were observed. The underrepresentation of the older age group warrants future studies.

Deep learning segmentation of orbital fat to calibrate conventional MRI for longitudinal studies.

In conventional non-quantitative magnetic resonance imaging, image contrast is consistent within images, but absolute intensity can vary arbitrarily between scans. For quantitative analysis of intensity data, images are typically normalized to a consistent reference. The most convenient reference is a tissue that is always present in the image, and is unlikely to be affected by pathological processes. In multiple sclerosis neuroimaging, both the white and gray matter are affected, so normalization techniques that depend on brain tissue may introduce bias or remove biological changes of interest. We introduce a complementary procedure, image "calibration," the goal of which is to remove technical intensity artifacts while preserving biological differences. We demonstrate a deep learning approach to segmenting fat from within the orbit of the eyes on T1-weighted images at 1.5 and 3 â€‹T to use as a reference tissue, and use it to calibrate 1018 scans from 256 participants in a study of pediatric-onset multiple sclerosis. The machine segmentations agreed with the adjudicating expert (DF) segmentations better than did those of other expert humans, and calibration resulted in better agreement with semi-quantitative magnetization transfer ratio imaging than did normalization with the WhiteStripe1 algorithm. We suggest that our method addresses two key priorities in the field: (1) it provides a robust option for serial calibration of conventional scans, allowing comparison of disease change in persons imaged at multiple time points in their disease; and (ii) the technique is fast, as the deep learning segmentation takes only 0.5 â€‹s/scan, which is feasible for both large and small datasets.

Treatment of Myasthenia Gravis in the Aged.

Myasthenia gravis is diagnosed at a progressively later age and the incidence continuously increases in the aged with a clear male predominance. People above the age of 65 constitute more than 50% of the newly diagnosed. Commonly, patients present with focal (ocular or bulbar) weakness. A high index of suspicion is needed to achieve early diagnosis and improve prognosis. Management options include acetylcholinesterase inhibitors, steroids, and immunosuppressants. Of the immunosuppressants, azathioprine is one of the most widely used due to its demonstrated effect and favorable side-effect profile. Others include cyclosporine, tacrolimus, mycophenolate mofetil, and cyclophosphamide. The use of mycophenolate mofetil is still controversial. Monoclonal antibodies, like rituximab, eculizumab, and belimumab, are relatively new therapeutic options in autoimmune diseases. Rituximab is of special interest in muscle-specific tyrosine kinase antibody-positive patients. A novel skeletal muscle activator and stem cell transplantation are being studied. There is no place for standard thymectomy in the older age groups except for thymomas and with computer-assisted endoscopy. Generally, the disease responds well to treatment at this age but comorbidities make medication choice more complex. Successful treatment is associated with improved survival, and quality of life can be remarkably improved during the whole survival period. As the median age of onset is 65 years and has been increasing steadily, it appears necessary to review the subject of treatment of myasthenia gravis in the aged.

Amyotrophic lateral sclerosis presentation of a human T-lymphotropic virus type-1 myelopathy--insight into pathogenesis.

A 41-year-old human T-lymphotropic virus type 1-positive woman developed a syndrome with upper and lower motor neuron signs sometime after bilateral vertebral artery dissections. Over 2 years, she developed a progressive myelopathy affecting predominantly the motor system. She had the picture of a 'person in a barrel' and died from complications. At autopsy, spinal cord revealed inflammatory infiltrates and extensive gliosis involving mainly the anterior horns. The vertebral arterial dissections may have permitted the entry of infected lymphocytes and macrophages, secreting cytokines and metalloproteinases, into the medulla progressing to the spinal cord. Few cases with pathological correlation have been reported.

Ischaemia due to a vascular malformation causing focal myositis.

Focal myositis secondary to an intramuscular vascular malformation has rarely been reported in the literature. We describe a 21-year-old woman presenting with left thigh pain. Imaging of the thigh muscles showed a vascular malformation and muscle biopsy demonstrated focal changes diagnosed initially as myositis. Ischaemia is thought to be the responsible mechanism.

Comparison of monoclonal gammopathy of undetermined significance-associated neuropathy and chronic inflammatory demyelinating polyneuropathy patients.

OBJECTIVES: There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes. METHODS: We performed a retrospective chart review of MGUSN (n = 56) and CIDP (n = 67) patients. Data extracted included: demographics, neurological examination, and nerve conduction studies (NCS) at baseline and last visit. Clinical status was rated as 0 = worse, 1 = unchanged, 2 = stabilized after a declining course, or 3 = improved. The electrophysiology data were rated as 0 = worse, 1 = stable, or 2 = improved. Statistical analyses were performed using JMP (version 9.0.2 for Macintosh, from SAS). RESULTS: Seventy percent were males, aged 68.1 ± 12.6 years with neuropathy for 9.8 ± 6.8 years and follow-up of 4.0 ± 3.2 years. CIDP patients had more severe neuropathy, and were more likely to receive treatment and to respond. The clinical neuropathy status remained unchanged in 52.8 % of the MGUSN and 24.2 % of the CIDP patients, and stabilized in 7.6 % of MGUSN and 30.3 % of CIDP patients. IgM-MGUSN patients did not differ from other immunoglobulin subclasses in response to treatment. The clinical severity and the number of abnormal NCS parameters were greater in the demyelinating MGUSN in comparison to the axonal group. CONCLUSION: MGUSN patients have less severe neuropathy than CIDP patients, but among the MGUSN patients the severity is greater in the demyelinating and the IgM groups. MGUSN patients may do well without treatment and exposure to potential adverse effects.

Late-onset myasthenia gravis: a review when incidence in older adults keeps increasing.

We define late-onset myasthenia gravis (LOMG) when symptoms appear at ≥65 years of age. There has been a continuous increase in the incidence of LOMG with a clear male predominance. Commonly, patients present with focal (ocular or bulbar) weakness. A high index of suspicion required to achieve early diagnosis and to improve prognosis. Management options include acetylcholinesterase inhibitors, steroids, and immunosuppressants. The most controversial issue in treatment is thymectomy, because not enough data are available. Successful treatment is associated with improved survival, and death is often secondary to comorbidities.