Consensus recommendations for diagnosis and treatment of Multiple Sclerosis: 2023 revision of the MENACTRIMS guidelines.

With evolving diagnostic criteria and the advent of new oral and parenteral therapies for Multiple Sclerosis (MS), most current diagnostic and treatment algorithms need revision and updating. The diagnosis of MS relies on incorporating clinical and paraclinical findings to prove dissemination in space and time and exclude alternative diseases that can explain the findings at hand. The differential diagnostic workup should be guided by clinical and laboratory red flags to avoid unnecessary tests. Appropriate selection of MS therapies is critical to maximize patient benefit. The current guidelines review the current diagnostic criteria for MS and the scientific evidence supporting treatment of acute relapses, radiologically isolated syndrome, clinically isolated syndrome, relapsing remitting MS, progressive MS, pediatric cases and pregnant women. The purpose of these guidelines is to provide practical recommendations and algorithms for the diagnosis and treatment of MS based on current scientific evidence and clinical experience.

Impact on quality of life in multiple sclerosis patients: Which urinary symptoms are to blame?

AIM: To evaluate the correlation between the Expanded Disability Status Scale (EDSS) in multiple sclerosis (MS) subjects, and the severity of lower urinary tract symptoms (LUTS), the bother caused by these symptoms and subjects' quality of life (QoL). MATERIAL AND METHODS: This cross-sectional study included 50 subjects with persistent LUTS secondary to MS who were recruited from the registry of a national NGO, between October 2017 and November 2019. Subjects with a history of any disease besides MS that could otherwise explain the presence of LUTS, as well as those with other neurological conditions were excluded. Information including MS duration, subjects' EDSS, voiding and storage LUTS, voiding symptoms' subscore of the International Prostate Symptom Score (IPSS-V), Overactive Bladder Symptom Scores (OABSS), Urinary Bothersome Questionnaire in Multiple Sclerosis (UBQMS), and urologic QoL (SF-Qualiveen) was gathered. Correlations between these scores were assessed using Spearman's bivariate correlations. Wilcoxon's signed rank test was used to evaluate the difference of impact between voiding and storage LUTS on bother of subjects. RESULTS: The median disease duration was 7±5.8years and the predominant lower urinary symptom was urgency (82%). Median OABSS and IPSS-V were respectively 8±3.8 and 8±3. Subjects were significantly more bothered from storage than voiding symptoms (2 vs. 1.6; P=0.03), and their QoL was directly affected by storage LUTS. Urgency urinary incontinence had the highest positive correlation with SFQ (r=0.542; P<0.01). MS duration and urologic QoL measured by SF-Q were negatively correlated (r=-0.345; P=0.01). CONCLUSION: In MS patients with LUTS, urologic QoL is mainly affected by storage urinary symptoms. Physicians should use a holistic approach to reduce the risk of complications in these patients, by controlling both voiding and storage symptoms, in particular urgency urinary incontinence that mostly affects patient's QoL.

Restless legs syndrome among multiple sclerosis patients in Lebanon.

BACKGROUND: Multiple sclerosis (MS) is often associated with fatigue, with an increased prevalence of sleep disorders compared to the general population, notably restless legs syndrome (RLS). The aim of this study was to evaluate the prevalence and severity of RLS as well the co-occurrence of spinal demyelination lesions in patients with MS in Lebanon. METHODS: In this cross-sectional study, we consulted the MS database of the Lebanese association against Multiple Sclerosis and sent out questionnaires to 300 MS patients to screen then confirm the presence of RLS. The final sample included 28 MS participants with confirmed RLS. We conducted further questionnaires to collect demographic data, screen for comorbidities, gather spinal MRI results, and evaluate the severity of both diseases (using the EDSS and the JHRLSS). RESULTS: Prevalence of RLS was 15% among MS patients in our study. 46.4% of RLS-affected MS patients had spinal cord demyelination lesions on their MRIs. Participants with MRI lesions had a lower severity score on the JHRLSS (p = 0.088). No association was found between the EDSS results and JHRLSS, demographic data, or comorbidities. CONCLUSION: Restless legs syndrome is commonly found among patients with multiple sclerosis in Lebanon, is underdiagnosed, and ought to be systematically evaluated for in order to improve the patients' quality of life.

Consensus recommendations for the diagnosis and treatment of multiple sclerosis: 2019 revisions to the MENACTRIMS guidelines.

With evolving diagnostic criteria and the advent of new oral and parenteral therapies for MS, most current diagnostic and treatment algorithms need revision and updating. The diagnosis of MS relies on incorporating clinical and paraclinical findings to prove dissemination in space and in time, and exclude alternative diseases that can explain the findings at hand. The differential diagnostic workup should be guided by clinical and laboratory red flags to avoid unnecessary tests. Appropriate selection of multiple sclerosis (MS) therapies is critical to maximize patient benefit. The current guidelines review the scientific evidence supporting treatment of acute relapses, radiologically isolated syndrome, clinically isolated syndrome, relapsing remitting MS, and progressive MS. The purpose of these guidelines is to provide practical recommendations and algorithms for the diagnosis and treatment of MS based on current scientific evidence and clinical experience.

Consensus recommendations for the diagnosis and treatment of multiple sclerosis: the Middle East North Africa Committee for Treatment and Research In Multiple Sclerosis (MENACTRIMS).

With evolving diagnostic criteria and the advent of new oral and parenteral therapies for MS, most current diagnostic and treatment algorithms need re-evaluation and updating. The diagnosis of MS relies on incorporating clinical and paraclinical findings to prove dissemination in space and in time, and exclude alternative diseases that can explain the findings at hand. The differential diagnostic workup should be guided by clinical and laboratory red flags to avoid unnecessary tests. Appropriate multiple sclerosis (MS) therapy selection is critical to maximize patient benefit. The current guidelines review the scientific evidence supporting treatment of acute relapses, radiologically isolated syndrome, clinically isolated syndrome, relapsing remitting MS, secondary progressive MS, and primary progressive MS. The purpose of these guidelines is to provide practical recommendations and algorithms for the diagnosis and treatment of MS based on current scientific evidence and clinical experience.

Leg ulcers in patients with ß-thalassaemia intermedia: a single centre's experience.

BACKGROUND: Leg ulcers in ß-thalassaemia intermedia (TI) patients are a relatively common occurrence that have an 8% prevalence. Both the pathophysiology and treatment of this condition have not been well-elucidated. This is mainly because of the rarity of the disease and the lack of well-structured studies. The goal of this study was to better explore the risk factors for the development of this condition along with the treatment options available. METHODS: We present 11 such cases that have occurred in 6 ß-TI patients over the course of 19 years who are followed up at the Chronic Care Center of Lebanon. RESULTS: Our patient population comprised three men and three women aged between 25 and 58, most of whom had iron overload and with an average lifetime haemoglobin ranging between 49 g/L and 77 g/L. Most of the patients were treated with blood transfusions with varying degrees of success. Nonetheless, some received Hydroxyurea, granulocyte macrophage colony-stimulating factor (GM-CSF) or topical antibiotics. CONCLUSION: Our results show that chelation therapy, hydroxyurea use and blood transfusions are beneficial in the treatment of this condition. Whether foetal haemoglobin is directly related to the development of the ulcers is not clear based on our results. Larger studies are needed to better explore the risk factors that predispose patients to this condition.

LSN MS guidelines for the management of multiple sclerosis.

OBJECTIVE: The prevalence of multiple sclerosis (MS) in Lebanon is unknown, as there are no available or reliable epidemiological studies to date. The circumstances of Middle East countries are different from those of Europe and North America in terms of differential diagnoses and disease management. The aim of the conference is to establish guidelines for diagnosis, treatment, follow-up and management of patients with MS in Lebanon. Another objective is to discuss and participate in research projects based on epidemiology, clinical trials and more fundamental aspects of the disease in the future. METHODS: Under the authority of the Lebanese Society of Neurology (LSN), a group of neurologists took the initiative to participate in this LSN MS committee with the purpose of establishing a consensus for the management of patients with MS, and under the supervision of a Coordinator (A.T.) designed by the LSN board. RESULTS: Diagnostic and therapeutic, follow-up and research recommendations were proposed with special emphasis on the specific needs and circumstances of Lebanon. The experts highlighted the importance of considering particular needs, the identification of patients at high risk of developing MS in order to maximize therapeutic opportunities, and cost-effective control of treatment efficacy, as well as global assessment of disability. CONCLUSIONS: The experts established guidelines concerning diagnosis, treatment, and follow-up of patients with MS in Lebanon. Furthermore, they recommended some clinical and fundamental research projects.

[Gait instability revealing a syndrome of inappropriate antidiuretic hormone secretion associated to pregabalin]. / Trouble de la marche révélant un syndrome de sécrétion inappropriée d'hormone antidiurétique associé à la prégabaline.

Pregabalin, a molecule with similar structure of GABA neurotransmitter, initially developed as an antiepileptic, is now commonly used in the treatment of painful peripheral neuropathies. We report an 82-year-old man who presented with confusion, urinary incontinence and gait instability. He was receiving pregabalin for a left L5 radicular pain. Laboratory tests at admission revealed a profound hyponatremia (117 mmol/L) that was corrected by fluid restriction and pregabalin withdrawal. According to the patient and laboratory outcome, we established the diagnosis of inappropriate antidiuretic hormone secretion due to pregabalin. The test of Naranjo demonstrated a probable imputability of pregabalin.

[Narcolepsy associated with systemic lupus erythematosus]. / Narcolepsie révélant un lupus érythémateux systémique.

Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus.

Survival and complications of beta-thalassemia in Lebanon: a decade's experience of centralized care.

beta-Thalassemia major is a debilitating disease with a considerable incidence in Lebanon (around 2-3% carriership). The present article describes our experience to this day with 214 patients, emphasizing the survival of beta-thalassemia major and development of complications among patients with different parameters. Fifteen deaths were reported. The most common cause of death was heart failure (60%). Patients with a ferritin level of 3,000 ng/ml showed better survival than those with a level >3,000 ng/ml (p < 0.006). In addition, patients with a ferritin level of 1,500 ng/ml showed less complication-free survival than those with a level >1,500 ng/ml (p < 0.024). High level of ferritin (1,500 ng/ml) is associated with increased risk of heart failure. Overall and complication-free survival were statistically different among patients classified according to birth cohort or ferritin level. The Chronic Care Center, a multidisciplinary center located in the suburbs of Beirut, led to an increase in complication-free as well as overall survival. Although patients are being diagnosed earlier and chelation therapy is being initiated at an earlier age, complications due to iron overload still persist. The introduction of new oral iron chelators and better iron overload quantitation methods will most likely modify this picture, and a follow-up study will examine their impact.

[Status epilepticus and gatifloxacin]. / Etat de mal épileptique et gatifloxacine.

INTRODUCTION: The epileptogenic potential of quinolones differs from one product to the other. The rare epileptic seizures induced by gatifloxacin were seen with the intravenous route. PATIENTS: We report two old ladies presenting with generalized status epilepticus after being treated orally with normal doses of gatifloxacin, with a good recovery. Past medical history of convulsion was the only factor found. Brain MR imaging was unremarkable. In one patient, EEG showed continuous bilateral paroxysmal discharges, predominantly frontal. CONCLUSION: Caution is warranted when using quinolones in elderly patients, especially those suffering from epilepsy.

Beta-thalassemia: the Lebanese experience.

Beta-thalassemia is a hereditary anemia that is quite prevalent in Lebanon. Most patients with beta-Thalassemia are treated and followed up mostly at a multidisciplinary center, located in the suburban area of Beirut: the Chronic Care Center (CCC), operational since 1994. We will review the experience with beta-Thalassemia accumulated through this institution. Four hundred and twenty five patients, aged 2 to 68 years are followed up at the CCC. Sixty four percent have thalassemia major (TM) while 36% have thalassemia intermedia (TI). Lebanese patients with TM receive periodic packed red cell transfusions to maintain a pre-transfusional hemoglobin level of 10 gm/dl at all times and desferrioxamine is the standard iron chelator in use. Since 1994, 12 patients with TM have died from complications of their disease, with heart failure being responsible for the majority of deaths. The incidence of cardiac, endocrinologic, and infectious complications will be reviewed. Finally, both current and prospective preventive measures will be discussed, specifically educational campaigns and premarital screening. The effects of prevention are starting to show as the number of newly diagnosed disease is diminishing.

[Epileptic seizures and vardenafil]. / Crise d'épilepsie et vardenafil.

INTRODUCTION: Epileptic seizures complicating treatment with selective inhibitors of phosphodiesterase type 5 are scarcely reported. CASE REPORT: A previously non-epileptic 78-year-old patient presented with a partial epileptic seizure following oral intake, for the second time, of 10mg of vardenafil (Levitra). The brain MRI failed to show any preexisting lesion. To our knowledge, only 2 cases of generalized tonic-clonic seizures induced by sildenafil (Viagra) use have been reported. In our patient, the seizure could be due to the epileptogenic potential of the drug or to its vascular complications. CONCLUSION: Further studies are needed to elucidate the association of phosphodiesterase inhibitors use and epileptic seizures.

[Megalencephalic leucoencephalopathy with subcortical cysts: a study of a Lebanese family and a review of the literature]. / La leucoencéphalopathie mégalencéphalique avec kystes sous-corticaux: étude d'une famille libanaise et revue de la littérature.

INTRODUCTION: Megalencephalic leukoencephalopathy with subcortical cysts is a rare disease with autosomal recessive inheritance. MATERIALS AND METHODS: Two brothers born from a consanguineous marriage, presenting with the phenotype of the disease, their parents, brothers and sisters were examined. Magnetic resonance imaging of the brain was performed for the two patients. Sequence analysis of MLC1 (GenBank mRNA accession no. NM_OI5166) was performed for the patients using intronic primers. PCR restriction fragment length polymorphism analysis was done in patients, their parents and in 100 Lebanese controls in order to exclude gene polymorphism. RESULTS: The clinical features were characteristic of the disease, consisting of an early-onset macrocephaly followed by slowly progressive ataxia, pyramidal tract involvement and epileptic seizures. In one patient, the clinical manifestations were aggravated by a trivial brain trauma. In his brother and in one female cousin, a status epilepticus was precipitated by a febrile syndrome. The diffuse cerebral white matter lesions and the subcortical temporo-polar and frontal cysts, best seen on MRI, allowed making the diagnosis. Molecular genetics revealed a new mutation involving the MLC1 gene (263G-->T, exon 3). As a consequence, it affects the second transmembrane domain predict (G88V) of the MLC protein (protein sequence NP_055981). The mutation was confirmed by PCR restriction fragment length polymorphism analysis. CONCLUSION: Megalencephalic leucoencephalopathy with subcortical cysts may be individualized on clinical and radiological basis and confirmed by molecular genetics. In this Lebanese family, a new mutation of the MLC1 gene is reported.

Five-year trial of deferiprone chelation therapy in thalassaemia major patients.

Twelve thalassaemia major patients have been given deferiprone 75 mg/kg body weight daily as iron chelation therapy for 5 years. Their ages ranged from 18 to 34 years (mean 24.2) at the end of the study. Two patients were hepatitis C virus (HCV) mRNA positive and a further 5 were positive for HCV antibody. The mean serum ferritin level fell significantly from 4,302 +/- 2,245 microg/l SD at baseline to 3,032 +/- 1,155 microg/l at 2 years (p = 0.037) and 2,229 +/- 1,070 microg/l (p = 0.007) at 5 years. At the end of the study, liver iron ranged from 3.59 to 23.7 mg/g dry weight (mean 11.9 +/- 5.4), 3 patients having levels >15 mg/g. There was no significant change in serum AST levels, but ALT levels fell significantly at 2 years (p = 0.019) and 5 years (p = 0.001). Liver biopsy at the end of the study showed no evidence of hepatic fibrosis caused by deferiprone. Cardiac studies showed no overall change in left ventricular ejection fraction but a significant improvement in isovolumic relaxation time (p = 0.045). We conclude that in this albeit small group of thalassaemia major patients, deferiprone was a safe long-term method of iron chelation. In a minority, higher doses of deferiprone or a combination with desferrioxamine would be needed to lower liver iron below 15 mg/g.