Utility of Initial Arterial Blood Gas in Neuromuscular versus Non-Neuromuscular Acute Respiratory Failure in Intensive Care Unit Patients.

Background: The arterial blood gas (ABG) parameters of patients admitted to intensive care units (ICUs) with acute neuromuscular respiratory failure (NMRF) and non-NMRF have not been defined or compared in the literature. Methods: We retrospectively collected the initial ABG parameters (pH, PaCO2, PaO2, and HCO3) of patients admitted to ICUs with acute respiratory failure. We compared ABG parameter ranges and the prevalence of abnormalities in NMRF versus non-NMRF and its categories, including primary pulmonary disease (PPD) (chronic obstructive pulmonary disease, asthma, and bronchiectasis), pneumonia, and pulmonary edema. Results: We included 287 patients (NMRF, n = 69; non-NMRF, n = 218). The difference between NMRF and non-NMRF included the median (interquartile range (IQR)) of pH (7.39 (7.32−7.43), 7.33 (7.22−7.39), p < 0.001), PaO2 (86.9 (71.4−123), 79.6 (64.6−99.1) mmHg, p = 0.02), and HCO3 (24.85 (22.9−27.8), 23.4 (19.4−26.8) mmol/L, p = 0.006). We found differences in the median of PaCO2 in NMRF (41.5 mmHg) versus PPD (63.3 mmHg), PaO2 in NMRF (86.9 mmHg) versus pneumonia (74.3 mmHg), and HCO3 in NMRF (24.8 mmol/L) versus pulmonary edema (20.9 mmol/L) (all p < 0.01). NMRF compared to non-NMRF patients had a lower frequency of hypercarbia (24.6% versus 39.9%) and hypoxia (33.8% versus 50.5%) (all p < 0.05). NMRF compared to PPD patients had lower frequency of combined hypoxia and hypercarbia (13.2% versus 37.8%) but more frequently isolated high bicarbonate (33.8% versus 8.9%) (all p < 0.001). Conclusions: The ranges of ABG changes in NMRF patients differed from those of non-NMRF patients, with a greater reduction in PaO2 in non-NMRF than in NMRF patients. Combined hypoxemia and hypercarbia were most frequent in PPD patients, whereas isolated high bicarbonate was most frequent in NMRF patients.

Finger Flexor Weakness in Myasthenia Gravis.

Myasthenia gravis (MG) affects the ocular, bulbar, and proximal limb muscles. The involvement of distal limb muscles is uncommon. MG-related weakness that severely affects the finger flexors and spares finger extensors and intrinsic hand muscles have never been reported. Here, we report a 35-year-old woman with acetylcholine receptor-antibody positive generalised MG who presented with severe bilateral asymmetric (left worse than right) finger flexor weakness during an MG relapse. The remaining muscles including the median and ulnar intrinsic hand muscles were normal. Repetitive nerve stimulation test showed decremental responses of more than 10%. Magnetic resonance imaging showed short-T1 inversion recovery sequences and increased signal intensities in the volar forearm muscles. Needle electromyography revealed fibrillations and positive sharp waves, small amplitude, short-duration, and polyphasic early recruiting motor unit action potentials. Myositis-specific autoantibodies were negative. Muscle biopsy showed neurogenic features. The patient had a good recovery with immunotherapy. We conclude that clinicians should be aware that marked weakness of the finger flexors can occur as a result of an MG relapse and may require early aggressive therapy. Key Words: Electromyography, Finger flexors, Muscle, Biopsy, Myasthenia gravis.

Exacerbation Rate in Generalized Myasthenia Gravis and Its Predictors.

OBJECTIVE: The aim of the study was to estimate the exacerbation incidence rate (IR) in acetylcholine receptor antibody (AChR)-positive generalized myasthenia gravis (MG) and its predictors. METHODS: The primary outcome in this retrospective study was to estimate moderate-to-severe (M-S) exacerbations IR in the early course of generalized MG. The secondary outcome was to explore the predictors of MG exacerbations. RESULTS: Between 1999 and 2015, we identified 78 AChR-positive generalized MG patients and 37 M-S exacerbations over the first 6 years following the onset of generalized MG symptoms. The M-S exacerbation IR was 12.2 per 100 person years (95% confidence interval [CI] 8.8-16.8). Any exacerbation (including mild) IR was 24.4 per 100 person years (95% CI 19.4-30.7). After controlling for confounding factors, MG exacerbation IR predictors included gender, disease severity at onset, and prednisone dose reduction with risk ratio of 0.34 (male gender), 2.67, and 20.8, respectively (all p values <0.05). M-S exacerbation occurred in 25 cases (32.1%), while any exacerbation (mild or M-S) was detected in 45 cases (57.7%). CONCLUSION: More than half of newly diagnosed AChR + MG cases experience an exacerbation in the first 6 years. Gender, disease severity at onset and prednisone dose reduction are predictors that could inform clinical practice and future research.

Impact of neurological diseases on family planning: A single-center experience.

This cross-sectional study aimed to assess the impact of epilepsy, myasthenia gravis (MG), and multiple sclerosis (MS) on pregnancy and family planning decision-making in a cohort of Saudi women. Women with epilepsy, MG, and MS were recruited consecutively at the time of their follow-up visits at a neurology clinic. Data were collected using 3 standardized questionnaires, and presented using descriptive statistics. A logistic regression was performed to determine variables associated with decisions regarding abstaining from pregnancy and encouraging other women to conceive. A total of 272 (83 epilepsy, 69 MG, and 120 MS) women with a mean age of 29.9 ±â€Š8.0 years participated. The proportion of women who abstained from or postponed pregnancy was 41.2% and 31.4%, respectively. The concerns mentioned most often were disease worsening during pregnancy, peripartum and postpartum, side effects of medications on the unborn child, and inability to care for the child. Older age was independently associated with the decision to abstain from pregnancy (odds ratio [OR] 1.14, 95% confidence interval [CI] 1.04 - 1.25). Higher knowledge levels were independently associated with encouraging other women to have children (OR 1.3, 95% CI 1.11-1.53). Over 50% of women reported that they were not counseled on issues related to pregnancy and childbirth. In conclusion, we identified a major influence of epilepsy, MG, and MS on pregnancy and family planning. Comprehensive counseling programs are needed to help women with these neurological diseases make informed family-planning decisions.

The effects of fructose and metabolic inhibition on hepatocellular carcinoma.

Hepatocellular carcinoma is rapidly becoming one of the leading causes of cancer-related deaths, largely due to the increasing incidence of non-alcoholic fatty liver disease. This in part may be attributed to Westernised diets high in fructose sugar. While many studies have shown the effects of fructose on inducing metabolic-related liver diseases, little research has investigated the effects of fructose sugar on liver cancer metabolism. The present study aimed to examine the metabolic effects of fructose on hepatocellular carcinoma growth in vitro and in vivo. Fructose sugar was found to reduce cell growth in vitro, and caused alterations in the expression of enzymes involved in the serine-glycine synthesis and pentose phosphate pathways. These biosynthesis pathways are highly active in cancer cells and they utilise glycolytic by-products to produce energy and nucleotides for growth. Hence, the study further investigated the efficacy of two novel drugs that inhibit these pathways, namely NCT-503 and Physcion. The study is the first to show that the combination treatment of NCT-503 and Physcion substantially inhibited hepatocellular carcinoma growth in vitro and in vivo. The combination of fructose diet and metabolism-inhibiting drugs may provide a unique metabolic environment that warrants further investigation in targeting hepatocellular carcinoma.

Knowledge of and attitudes toward epilepsy surgery among neurologists in Saudi Arabia.

OBJECTIVE: To investigate the levels of knowledge and attitudes toward epilepsy surgery among neurologists in Saudi Arabia and evaluate the factors that affect the physicians` knowledge and attitudes. METHODS: A quantitative observational cross-sectional study conducted at King Saud University Medical City, Riyadh. The data were collected using a newly developed, self-administered online questionnaire. The questionnaire contained 3 sections: demographic information, knowledge, and attitudes which then sent to neurologist in Saudi Arabia from December 2016 to March 2017. RESULTS: A total of 106 neurologists met our inclusion criteria. Eighty percent of the participants had at least one epilepsy center in their city, and 78% indicated that they had access to adequate expertise and resources to enable the appropriate selection of epilepsy surgical candidates. Only 57.5% of the neurologists had a sufficient level of knowledge regarding epilepsy surgery. Neurologists with higher level of knowledge referred more patients to EMU and discussed epilepsy surgery more often with their patients. Overall, more than half of the neurologists (52.8%) had a positive attitude toward epilepsy surgery. There was a significantly positive correlation between the scores of knowledge and attitude (p less than 0.001). CONCLUSION: Neurologists in Saudi Arabia appear to have moderate knowledge of and positive attitudes toward epilepsy surgery. The place of the last neurology certificate, type of practicing hospital, and access to expertise and resources, affected their knowledge. Adequate knowledge was positively correlated with attitude.

Severity and depression can impact quality of life in patients with myasthenia gravis.

BACKGROUND: The revised 15-item Myasthenia Gravis (MG) Quality of Life Questionnaire (MGQoL15R) is a validated scale of quality of life in patients with MG. We aimed to study the factors causing the variability within the Arabic version of the MGQoL15R (MGQoL15R-A). METHOD: A standardized questionnaire was completed by 118 patients. Correlations and hierarchical regression analyses were used to assess the contribution of sociodemographic variables, clinical factors, Patient Health Questionnaire-9 (PHQ9-A), and Generalized Anxiety Disorder-7 (GAD7-A) to the variability in the MGQoL15R-A. RESULTS: The MGQoL15R-A was highly correlated with PHQ9-A (r = 0.76), and moderately correlated with GAD7-A (r = 0.52). Clinical factors and PHQ9-A independently explained 30.4% and 34.5% of the variability, respectively. Among the clinical factors, uncontrolled MG status, relapse within the past year, and a higher number of current MG therapies were significantly associated with a higher MGQoL15R-A score. CONCLUSIONS: MG severity and depressive symptoms (measured by PHQ9-A) can affect the MGQoL15R-A score.

Acute Paralytic Post-Bariatric Surgery Axonal Polyneuropathy: Clinical Features and Outcome.

INTRODUCTION: The syndrome of post-bariatric surgery axonal polyneuropathy (PAP) may present with various sensory and motor symptoms including paralysis. We aim to describe the diagnostic features and outcome of treatment of the acute paralytic form of PAP (acute paralytic PAP [APPAP]) as it was not described in a separate cohort previously. METHODS: We retrospectively reviewed medical charts and described the clinical, electrodiagnostic features, treatment, and outcome for patients who presented to our clinical neurophysiology unit with disabling weakness within 24 months post-bariatric surgery. The main outcome measure was the percent of patients who are able to walk independently at the last visit and comparing the group who resumed walking independently at 6 months to the group who did not, in regards to the use of intravenous immunoglobulin (IVIg). RESULTS: Thirteen patients were included (10 women and 3 men) with a mean age of 29.8 years (SD 12.5). All presented with loss of ambulation resembling Guillain-Barre Syndrome. The median time of onset was 4 months (interquartile range [IQR] 3-6) post-surgery, and the median time to weakness nadir was 3 weeks (IQR 3-3.5) with an average weight loss of 38.6 kg (SD 17.09). All patients regained their ability to ambulate; however, the ability to walk independently was achieved in 66.7% of patients. The percent of patients who were able to ambulate independently at 6 months were 16% with IVIg versus 66.7% without IVIg. CONCLUSION: The syndrome of -APPAP develops in the first-year post-bariatric surgery. The majority of patients regain independent ambulation.

Prevalence and Associated Factors of Depressive Symptoms in Patients with Myasthenia Gravis: A Cross-Sectional Study of Two Tertiary Hospitals in Riyadh, Saudi Arabia.

OBJECTIVES: This study is aimed at elucidating the prevalence of depression in patients with myasthenia gravis (MG) and examining the risk factors associated with depression. METHODS: We evaluated adult patients with MG who were recruited from two tertiary hospitals in the central region (Riyadh) of Saudi Arabia. Data were collected with a two-part standardized questionnaire: the first part included data on sociodemographic and clinical features of MG including disease type and duration, therapies, prednisolone dose, time of the last relapse, previous critical care unit admissions, MG status (controlled, partially controlled, or uncontrolled), and comorbid diseases; the second part included items from the previously validated Arabic version of the Patient Health Questionnaire-9 (PHQ-9). RESULTS: In total, 104/150 (69.3%) patients participated (72 females) with a mean age of 38.0 ± 16.0 years. The mean PHQ-9 score was 7.02 ± 6.1. Among all the participants, 27 (26.0%) patients had depression (PHQ-9 ≥ 10). Multiple logistic regression analysis revealed that uncontrolled MG status (OR = 12.31, 95%CI = 1.13-133.8, P = 0.04) was the only factor independently associated with depression. Collectively, the prevalence of depression among patients of the primary care clinics (PCC) as reported by 5 previous studies across multiple regions of the country was 15.8%. The odds of depression among MG patients were twofold higher than those among PCC patients (OR = 2.05, 95%CI = 1.30-3.22, P = 0.002). CONCLUSIONS: Approximately a quarter of MG patients have depression. Achieving a minimal manifestation or better MG status may decrease the depression rate in these patients.

Clinical features and outcomes of patients with myasthenia gravis.

OBJECTIVE: To report clinical and laboratory features and outcomes of patients with autoimmune myasthenia gravis (MG) recruited from a single center in Saudi Arabia. METHODS: We retrospectively reviewed prospectively collected data obtained from MG patients who have undergone examination and follow-up at our neuromuscular clinic between August 1, 2014 and January 31, 2019. RESULTS: Ninety-five patients (55 females) were included. The mean age of onset of MG was 40.5+/-18.1 years in males and 31.3+/-15 years in females (p=0.009). The mean duration of follow-up at our clinic was 34.7+/-14.1 months, while the mean duration since MG onset was 8.0+/-7.2 years. Of all patients, 92.6% had generalized MG, 82.1% had acetylcholine receptor (AChR) antibodies, 4.2% had muscle-specific tyrosine kinase (MuSK) antibodies, 78.9% had early-onset MG with no second peak after age of 50 years, 22.1% had myasthenia crisis, 12.6% had refractory MG, 31.6% had thymic hyperplasia, 10.5% had thymoma, and 61.1% required more than or equal 2 immunosuppressive therapies. At the last follow-up, 93 patients had achieved an optimal outcome (MG Foundation of America classification less than or equal II). No patient with double-seronegative (dSN)-MG had thymoma, needed rituximab or intravenous immunoglobulin maintenance therapy, or was classified as refractory MG. CONCLUSION: Contrary to other studies, we did not observe a second-peak of MG onset. Clinical outcomes were favorable in the majority of our patients.

Translation and validation of the arabic version of the myasthenia gravis activities of daily living scale.

INTRODUCTION: We translated the myasthenia gravis (MG)-specific activities of daily living (MG-ADL) scale into Arabic (MG-ADL-A) and assessed its psychometric properties. METHODS: We assessed reliability using Cronbach's α, reproducibility using the intraclass correlation coefficient, and validity using Spearman's correlations with MG composite (MGC) score, MG-specific manual muscle test (MG-MMT), and MG quality-of-life revised Arabic version (MGQOL15R-A). Differences in MG-ADL-A scores among patients with different disease severity were evaluated by using the Kruskal-Wallis test. Sensitivity to change was examined by using the Wilcoxon signed-rank test. RESULTS: We recruited 87 patients. The mean MG-ADL-A score was 3.38 ± 3.38 (α = 0.77, ICC = 0.99). The correlation coefficients between the MG-ADL-A and MGQOL15R-A, MGC, and MG-MMT were 0.63, 0.74, and 0.61, respectively (P < 0.001). The MG-ADL-A discriminated between different severity groups and was responsive to clinical improvement at follow-up. DISCUSSION: The MG-ADL-A has rigorous psychometric properties and can be used with Arabic-speaking patients with MG. Muscle Nerve 59:583-583, 2019.

Multiple sclerosis and myasthenia gravis following severe weight loss.

Weight loss has substantial health benefits, but it is not risk-free. Various neurological disorders have been reported following bariatric surgery-induced weight loss. Here, we report 3 patients who developed multiple sclerosis (MS), one of whom also developed myasthenia gravis (MG), shortly after significant weight loss. Two patients lost weight by following a diet plan and one underwent bariatric surgery. There may be an association between significant weight loss and the development of an autoimmune neurological disorder such as MS or MG; a high index of suspicion is required.

Migraine and Headache Prevalence and Associated Comorbidities in a Large Saudi Sample.

BACKGROUND: We aimed to assess the prevalence of headache and migraine, along with comorbidities, in a large -Saudi sample. METHODS: Self-reported information was collected about headache, migraine and migraine comorbidities including depression, restless legs syndrome (RLS), syncope, bruxism, hypertension and ischaemic disease. The OR was estimated using logistic regression for any associations with headache and migraine. We then analyzed to find a trend of increasing migraine symptoms for each significant comorbidity. RESULTS: Out of 4,943 respondents, 4,158 (84.12%) had recurring headaches. Migraine was present in 1,333 (26.97%), with female predominance (ratio of 1: 2.9). There were statistically significant ORs between migraine and female sex, current smokers, higher income, hypertension, depression, syncope, RLS and bruxism. Non-migraine headaches were significantly associated with female sex, age, RLS and ischaemic disease. Migraine with aura was significantly associated with syncope, ischaemic disease, higher income and BMI. There was an overall significant trend of increasing migraine features in the presence of depression, syncope, RLS, bruxism and hypertension. CONCLUSIONS: Headache in general and migraine in particular are associated with multiple comorbidities in comparison to non-headache participants in our cohort, with an estimated prevalence similar to that of western countries.

Translation and validation of the arabic version of the revised 15-item myasthenia gravis quality-of-life questionnaire.

INTRODUCTION: We sought to translate, culturally adapt, and assess the Arabic version of the 15-item myasthenia gravis quality-of-life revised scale (MGQOL15R). METHODS: We assessed reliability with Cronbach α; reproducibility with intraclass correlation coefficient (ICC); validity with Spearman correlations for myasthenia gravis (MG)-specific activities of daily living (MG-ADL), MG composite (MGC) score, and MG manual muscle test (MG-MMT) and with MGQOL15R in patients with different disease severity through the Kruskal-Wallis test; and sensitivity to change with Wilcoxon signed-rank test. RESULTS: In 65 enrolled patients, the mean MGQOL15R score was 10.84 ± 8.11 (α = 0.94, ICC = 0.95). The correlation coefficients between MGQOL15R and MGC, MG-ADL, and MG-MMT scores were 0.75, 0.75, and 0.74, respectively (P < 0.001). MGQOL15R scores were significantly higher (worse) in patients with more severe disease at baseline and significantly lower (better) in improved patients at follow-up. DISCUSSION: The Arabic version of MGQOL15R is valid, reliable, stable, and sensitive to changes. Muscle Nerve 57: 581-585, 2018.

Stroke recurrence rates among patients with symptomatic intracranial vertebrobasilar stenoses: systematic review and meta-analysis.

BACKGROUND: A recent randomized trial of patients with primarily anterior circulation intracranial artery stenosis showed that intensive medical therapy was superior to intracranial stenting in preventing recurrent stroke. The rate of stroke recurrence or death in symptomatic intracranial vertebrobasilar stenosis with medical therapy alone may be especially high, and rates compared with endovascular therapy need further study. METHODS: We conducted a systematic review and meta-analysis of studies reporting the rates of stroke recurrence or death (the primary outcome) in symptomatic intracranial vertebrobasilar stenosis with medical or endovascular treatment over a minimum follow-up period of 6 months. We included all studies in any language indexed in MEDLINE or EMBASE, supplemented by bibliography searches and by contacting the authors. The secondary endpoints were stroke recurrence, and basilar artery and vertebral artery stroke recurrence rates. RESULTS: 23 studies (592 medical treatment patients and 480 endovascular treatment patients) were included. The risk of combined stroke recurrence or death was 14.8 per 100 person-years (95% CI 9.5 to 20.1) in the medical group compared with 8.9 per 100 person-years (95% CI 6.9 to 11.0) in the endovascular group. The incidence rate ratio was 1.3 (95% CI 1.0 to 1.7). The stroke recurrence rate was 9.6 per 100 person-years (95% CI 5.1 to 14.1) in the medical group compared with 7.2 per 100 person-years (95% CI 5.5 to 9.0) in the endovascular group. CONCLUSIONS: Our results showed that the risk of stroke recurrence or death or the risk of stroke recurrence alone was comparable between the medical and endovascular therapy groups. A small preventive effect of endovascular therapy may exist, particularly if the 30 day postprocedural risk is reduced.

Non alcoholic steatohepatitis a precursor for hepatocellular carcinoma development.

Hepatocellular carcinoma (HCC) is increasing in prevalence and is one of the most common cancers in the world. Chief amongst the risks of attaining HCC are hepatitis B and C infection, aflatoxin B1 ingestion, alcoholism and obesity. The later has been shown to promote non alcoholic fatty liver disease, which can lead to the inflammatory form non alcoholic steatohepatitis (NASH). NASH is a complex metabolic disorder that can impact greatly on hepatic function. The mechanisms by which NASH promotes HCC are only beginning to be characterized. Here in this review, we give an overview of the recent novel mechanisms published that have been associated with NASH and subsequent HCC progression. We will focus our discussion on inflammation and gut derived inflammation and how they contribute to NASH driven HCC.

Diagnostic yield and cost-effectiveness of investigations in patients presenting with isolated lower motor neuron signs.

Our objective was to investigate the yield and cost-effectiveness of investigations and therapeutic trials of intravenous immunoglobulin (IVIg) in patients presenting with isolated lower motor neuron (LMN) signs. We performed a retrospective chart review of cases diagnosed between January 2007 and September 2013. Investigation results and their impact on outcome, and outcome of IVIg treatment trials were abstracted. Cost was calculated in Canadian dollars (C$). Fifty-nine of 333 patients presented with isolated LMN signs. The majority of patients (61%) evolved to amyotrophic lateral sclerosis (ALS) within 36 months of presentation, while 37.3% remained with progressive muscular atrophy (PMA) with mean follow-up 29.6 months. Of the 1210 tests performed, 4.9% were abnormal. The diagnosis was changed in only one patient where a muscle biopsy revealed a distal myopathy. Fourteen patients received therapeutic trials of IVIg to rule out an IVIg-responsive inflammatory motor neuropathy with no objective clinical benefit. Total group cost was C$630,484.72 (C$10,686.18/patient). IVIg represented 58.7% of total costs. In conclusion, extensive investigations and treatment trials of IVIg have low yield in the work-up of patients with isolated LMN signs and are not cost-effective when clinical features do not suggest an alternative diagnosis to PMA.