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Introduction: The main purpose of the present study is to confirm Peripapillary Retinal Nerve Fiber Layer (pRNFL) thickness is a biomarker of axonal degeneration in patients with Multiple Sclerosis (MS) and to evaluate its relationship with Neurofilament heavy chain (NfH) and Nitrotyrosine (NT). Method: We quantified serum (s) and/or cerebrospinal fluid (CSF) NfH and NT levels in 30 relapsing-remitting MS patients (RRMS), 16 secondary progressive MS (SPMS) patients and in 29 control subjects matched for age and gender. Optical coherence tomography (OCT) measurements of pRNFL were performed in all subjects. Clinical outcomes were tested by Multiple Sclerosis Functional Composite (MSFC) and Expanded Disability Status Scale (EDSS). Results: RRMS patients exhibited significantly higher NfH/NT levels (99 pg/mL, 107.52 nM respectively) than controls (74 pg/mL, 48.72 nM) in CSF (p, Scientific Research Found of Ege University School of Medicine [12-TIP-003], This study was supported by the Scientific Research Found of Ege University School of Medicine (12-TIP-003).

Background: COVID-19 is a multisystemic infection with variables consequences depending on individual and comorbid conditions. The course and outcomes of COVID-19 during neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) are not clearly known. Objective methods: the aim of this study was to examine the features and outcomes of COVID-19 infection in NMOSD and MOGAD patients. The patients' demographic and clinical factors, disease modifying treatment (DMT) used and disease information of COVID-19 infection were recorded. Conditions leading to hospitalization and severe exposure to COVID-19 infection were also analyzed. Results: the study included 63 patients from 25 centers. Thirty-two patients (50.8%) belong to AQP-4 seropositive group, 13 (20.6%) and 18 (28.6%) were in MOG-positive and double-seronegative groups, respectively. Risk factors for severe COVID-19 infection and hospitalization were advanced age, high disability level and the presence of comorbid disease. Disease severity was found to be high in double-seronegative NMOSD and low in MOGAD patients. No statistically significant effect of DMTs on disease severity and hospitalization was found. Conclusion: in NMOSD and MOGAD patients, advanced age, high disability and presence of comorbid disease pose risks for severe COVID-19 infection. There was no direct significant effect of DMTs for COVID-19 infection., NA

PubMed: 32473575 Background: Although studies report a high prevalence rate of restless legs syndrome (RLS) among patients with multiple sclerosis (PwMS) ranging from 13.3 to 65.1%, little is known about the causes of this relationship. Methods: To ascertain the prevalence, features and impact of RLS among PwMS a nation-wide, multicenter, prospective and a cross-sectional survey, designed to reflect all of the PwMS throughout Turkey, was conducted in 13 centers. Exploring the relationship of the two conditions could possibly contribute to the understanding of the causes of the high and wide-ranging prevalence rates and the pathophysiology of both diseases. Results: Of the 1068 participants 173 (16,2%) found to have RLS [RLS(+)] and 895 (83,8%) did not [RLS(-)]. Among the RLS(+) 173, all but 8 patients (4,6%) were underdiagnosed in terms of RLS. More than half of the patients with RLS had ‘severe’ or ‘very severe’ RLS. The onset of RLS was before or synchronous with the onset of MS in about a half of our patients. Conclusion: We conclude that RLS should be meticulously investigated in PwMS and MS can be a direct cause of RLS at least in part of PwMS. Our data about the timing of the onset of MS and RLS, along with the high prevalence of RLS in PwMS suggest that the pathologic changes in the initial phases of MS can possibly trigger RLS symptoms. © 2020 Elsevier B.V.

Introduction: Multiple Sclerosis Knowledge Questionnaire (MSKQ) is a self-administered inventory assessing patients' knowledge about Multiple Sclerosis. In this study, we aimed to test the reliability and validity of MSKQ in Turkish patients. Methods: Patients with Multiple Sclerosis who attended an education seminar in our university hospital were enrolled in the study. Results: Fifty-eight patients completed and returned the questionnaire twice, before and after the seminar. Mean number of items that were answered correctly in the first round was 12.8 (5.2), which increased to 18.7 (3.2) in the second round after the seminar. This increase was found to be significant (p

Cervical vestibular-evoked myogenic potentials (cVEMPs) are accepted to demonstrate the vestibulo-collic reflex. However, the brainstem pathway is still not fully understood. The aim of the study was to evaluate the contribution of cVEMPs to detection of brainstem involvement in multiple sclerosis (MS). Thirty patients fulfilling the criteria for definite MS were included in the study. All were newly diagnosed cases, admitted due to an attack with active lesions on MRI. Thirty-one age- and sex-matched healthy controls constituted the control group. The latencies of peaks p13 and n23 and peak-to-peak amplitude of p13–n23 were measured. Brainstem lesions on MRI were present in 13 of the patients (43.4%). Comparison of the overall results recorded from patients with the healthy controls did not reveal a statistically significant difference in any of the parameters studied (p > 0.05). A significant inter-side difference was not also present between groups (p > 0.05). When p13 and n23 latencies exceeding 2.5 standard deviations (SD) were taken into consideration, it was seen that there were seven patients (23.3%) with prolonged latencies mainly involving the p13 peak. Five of them had brainstem signs on examination and had brainstem lesions on MRI. In the other eight patients with abnormal MRI, normal results were recorded indicating that in only 38% of patients with brainstem lesions, cVEMPs were altered. Absence of a correlation between cVEMPs and brainstem clinical or MRI lesions defies their role in identifying lower brainstem involvement.

Purpose The study was purposed to investigate the effect of COVID-19 disease on fatigue, sleep quality, physical activity, quality of life, and psychological status in people with MS. Methods A total of 104 people with MS, including 46 of them with COVID-19 disease history, were enrolled in the study. All patients were evaluated with the Expanded Disability Status Scale (EDSS), Fatigue Severity Scale (FSS), Pittsburgh Sleep Quality Index (PSQI), International Physical Activity Questionnaire Short Form (IPAQ-SF), the EuroQoL Instrument (EQ-5D-3L), the Fear of COVID-19 Scale (FCV-19S) and the Coronavirus Anxiety Scale (CAS). Results People with MS in the COVID-19 positive group had a significantly lower IPAQ-Total score (p = 0.014). Besides, the FCV-19S scores of COVID-19 positive patients were significantly higher (p = 0.006). EQ-5D-3L Index and EQ-5D-3L VAS scores were higher in the group with COVID-19 (p1 = 0.021, p2 = 0.014, respectively). FCV-19S had a moderate correlation with EDSS (r = -0.362). IPAQ-Total was moderately associated with MS duration, EDSS and FSS (r1 = -0.471, r2 = -0.389, r3 = -0.388, respectively). The EQ-5D-3L Index was moderately correlated with FSS (r1 = -0.404). There was a weakly significant relationship between EQ-5D-3L Index and BMI, MS duration, PSQI and CAS (r1 = -0.471, r2 =-0.389, r3 = -0.388, r4 = -0.326, respectively). On the other hand, EQ-5D-3L was moderately associated with VAS and EDSS and PSQI (r1 = -0.393, r2 = -0.357, respectively). Conclusion COVID-19 negatively affected the people with MS's physical activity and coronavirus related fear parameters. However, the causality of this influence should be investigated in detail.

Adherence to medication treatment in patients with multiple sclerosis (MS) is important to increase its effectiveness, reduce patient disability, prevent attacks, and increase the quality of life.This study investigated factors that influence adherence to disease-modifying therapy in patients with MS.This descriptive study was conducted with 198 patients with MS who met the inclusion criteria and agreed to participate between July 2016 and February 2017. Data were collected using an Individual Identification Form that included sociodemographic characteristics, the Multiple Sclerosis Treatment Adherence Questionnaire, the Fatigue Severity Scale, the Self-Efficacy Scale, and the Brief COPE.We found that 59.6% of the patients were adherent to therapy. Patients were significantly more adherent to Avonex than other treatments, and "memory problems" was the most common reason for missing or forgetting medication in nonadherent patients. There was a significant difference between medication adherence and some sociodemographic characteristics and disease characteristics (P.05). There was no significant difference between coping attitudes, fatigue, and self-efficacy level and medication adherence (P.05).Patients' adherence to medication treatment was low and may be associated with social, physical, and cognitive measures.

Boz, Cavit/0000-0003-0956-3304; Altintas, Ayse/0000-0002-8524-5087; YUCEYAR, NUR/0000-0003-4590-6423; Sevim, Serhan/0000-0002-0518-3374; Efendi, Husnu/0000-0002-9143-3893, WOS: 000502098900008, PubMed: 31473488, Background: Fingolimod and teriflunomide are commonly used in the treatment of relapsing-remitting multiple sclerosis (RRMS). These have not been compared in controlled trials, but only in observational studies, with inconclusive results. Comparison of their effect on relapse and disability in a real-world setting is therefore needed. Objectives: the objective of this study was to compare the efficacy of fingolimod and teriflunomide in reducing disease activity in RRMS. Methods: This multicenter, retrospective observational study was carried out with prospectively collected data from 15 centers. All consecutive RRMS patients treated with teriflunomide or fingolimod were included. Data for relapses, Expanded Disability Status Scale (EDSS) scores and brain magnetic resonance imaging (MRI) scans were collected. Patients were matched using propensity scores. Annualized relapse rates (ARR), disability accumulation, percentage of patients with active MRI and treatment discontinuation over a median 2.5-year follow-up period were compared. Results: Propensity score matching retained 349 out of 1388 patients in the fingolimod group and 349 out 678 in the teriflunomide group for final analyses. Mean ARR decreased markedly from baseline after 1 and 2 years of treatment in both the fingolimod (0.58-0.17 after 1 year and 0.11 after 2 years, p < 0.001) and teriflunomide (0.56-0.29 after 1 year and 0.31 after 2 years, p < 0.001) groups. Mean ARR was lower in fingolimod-treated patients than in those treated with teriflunomide at years 1 (p = 0.02) and 2 (p = 0.004). Compared to teriflunomide, the fingolimod group exhibited a higher percentage of relapse-free patients and a lower percentage of MRI-active patients after 2.5-year follow-up. Disability worsening was similar between the two groups. Patients were less likely to discontinue fingolimod than teriflunomide (p < 0.001). Conclusion: Fingolimod was associated with a better relapse control and lower discontinuation rate than teriflunomide. the two oral therapies exhibited similar effects on disability outcomes.

WOS: 000473218600009, Objective: We aimed to investigate the correlations of urinary symptoms with urodynamic findings in patients with multiple sclerosis (MS) and their effects on quality of life. Methods: Nineteen MS patients with urinary symptoms were included. The data were obtained from files or with face-to-face interviews. Symptom types of patients were categorized as irritative, obstructive, and mixed. The Incontinence Quality of Life Scale, the King's Quality of Life Questionnaire (KQL) and the International Consultation on Incontinence Modular Questionnaire-Short Form were used to determine the effect of urinary symptoms on quality of life. The Extended Disability Status Scale (EDSS) was used to assess disability. All patients underwent urodynamic examination. Results: Of the 478 patients, 34.5% had visited a psychiatrist once in their lifetime, 13.6% were diagnosed with depression, and 13.8% did not know the diagnosis of mental disease; 32.2% were suggested medication by their doctor, 16.7% did not know the name of this medication, and 13.2% stated that the suggested drug was antidepressant. Moreover, 24.9% of the patients were known to use antidepressant at least once in their lifetime, and 16.3% were still using antidepressants. Among patients using antidepressants, 32% continued using this drug upon doctor's recommendation and 13.6% continued with the diagnosis of depression. Conclusion: The urinary symptoms significantly affect the quality of life in MS patients. Since symptoms do not always correlate with the underlying pathology, it is more appropriate to give the treatment based on the bladder type in urodynamic testing.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common, treatable, autoimmune peripheral neuropathy considered to produce imbalance by weakness and proprioceptive impairment rather than vestibular impairment. We measured semicircular canal vestibular function in 21 CIDP patients (15M/6F) by the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall Efficiency Scale, and the International Cooperative Ataxia Rating Scale. Of the 21 patients, 16 had vestibular impairment, ranging from mild-affecting just a single semicircular canal, to severe-affecting all 6 canals. Although the severity of the vestibular impairment did not correlate either with the severity of the postural imbalance or of the peripheral neuropathy, our data show that vestibular impairment is an additional challenge to balance that some CIDP patients will face.

Myosin storage myopathy (MSM) is a protein aggregate myopathy caused by the accumulation of myosin in muscle fibres and results from MYH7 mutation. Although MYH7 mutation is also an established cause of variable cardiomyopathy with or without skeletal myopathy, cardiomyopathy with MSM is a rare combination. Here, we update the clinical findings in the two brothers that we previously reported as having recessively inherited MSM characterized by scapuloperoneal distribution of weakness and typical hyaline-like bodies in type 1 muscle fibres. One of the patients, weak from childhood but not severely symptomatic until 28 years of age, had an unusual combination of MSM, severe dilated cardiomyopathy, and respiratory impairment at the age of 44 years. We identified homozygous missense mutation c.5458C>T (p.R1820W) in exon 37 in these patients as the second recessive MYH7 mutation reported to date.

WOS: 000373736400014, PubMed ID: 25936385, Objective: To determine the frequency and severity as well as the diagnosis and treatment of overactive bladder problems in patients with multiple sclerosis (MS) followed up at five centers in Turkey. Design: Survey study. Setting: Outpatient tertiary clinics of physical medicine and rehabilitation and neurology. Participants: Consecutive MS patients scheduled for outpatient follow-up (n=309). Intervention: MS patients were asked to complete a questionnaire regarding the frequency and severity, as well as the diagnosis and treatment of their overactive bladder problems. Results: The mean age +/- SD was 39.3 +/- 10.6 years. Urinary urgency was the most common urinary symptom (62%), followed by frequency (50.4%), urge incontinence (44.7%) and nocturia (33%). Residual urine volume was measured using a portable ultrasound instrument in 13.3% of the patients and by catheterization in 16.2% of them. Urodynamic investigations and urinary tract ultrasound were performed on 26.5% and 35.3% of the patients, respectively. Anticholinergic medications were prescribed for 27.5% of the patients. Intermittent catheterization and indwelling catheterization were used on 8.1% and 1.9% of the patients, respectively. The overactive bladder symptom score (OABSS) was significantly higher in patients who had had residual urine measurement (P

Optic neuritis (ON) is highly associated with multiple sclerosis (MS) and conversion rate has been reported to be around 50% over a period of 15 years. We revised the risk factors for developing MS after an attack of ON, in western Turkey. One hundred and twenty-four patients with acute unilateral ON, who were on follow-up for at least 5 years (range 62 to180 months, mean 85 months) were enrolled in the study. Gender, age at onset, seasonal occurrence, severity of visual loss, presence of pain, optic disc appearance, treatment, degree of recovery, presence or absence of lesions on magnetic resonance imaging (MRI) during the initial ON attack and recurrence were considered. Of the 124 patients, 89 (71.8%) were women, 35 (28.2%) were men, with a mean age of 28.7 ± 8.6 years at the time of the initial ON attack. During follow-up, 70 patients (56.5%) developed MS within 17.14 months, ranging from 1 to 125 months. Female gender (p = 0.021), experiencing the attack before 40 years of age (p = 0.02), normal-appe...

MS patients frequently present with urinary system symptoms, which have a negative effect on the quality life. The aim of our study was to demonstrate the validity and reliability of the Turkish King’s Health Questionnaire (KHQ) in MS patients. The study included 37 patients. For analysis of test–retest reliability, the Turkish version of the KHQ developed and the “translation-back translation” method was performed. To assess the validity of these results, Multiple Sclerosis Quality of Life Scale (MQoL-54) and Expanded Disability Status Scale (EDSS) were used. The internal consistency (intra-class correlation coefficient: 0.59–0.94) and test–retest reliability (Cronbach’s α-score 0.59–0.94) of KHQ were found to be high (p < 0.05). A significant correlation was detected between most of the KHQ subscores and the physical and mental MQoL-54 and EDSS (total and bowel/bladder, p < 0.05) subscores. The KHQ may be used to determine the effect of incontinence on the quality of life for MS patients.

Hashimoto's encephalopathy (HE) is a rare immune-mediated encephalopathy developing in patients with high serum concentrations of anti-thyroid antibodies usually in an euthyroid or hypothyroid state. We report a 31-year-old female patient with thyrotoxic HE whose daughter has been followed up with the same diagnosis. Suboptimal response was observed with intravenous methylprednisolone (IVMP), intravenous immunoglobulin (IVIG) and plasmapheresis. Reduction of the anti-thyroid auto-antibody concentrations marked the patient's improvement in each episode. She relapsed under oral immunosuppressive therapy. After removing the thyroid tissue, full recovery has been achieved for the last 18 months. These data may contribute to clarification of the pathogenetic role of anti-thyroid antibodies in HE. Thyroidectomy can be considered as one of the treatment options especially in thyrotoxic HE patients with uncontrolled relapses. Our patient is the first reported HE case with a family history. Genetic background can underlie the etiopathogenesis of HE as is the case in other autoimmune disorders.

The nature of the oropharyngeal dysphagia in polymyositis/dermatomyositis (PM/DM) has been investigated by EMG methods. Nineteen patients with PM/DM were studied. The oropharyngeal phase of swallowing was evaluated by the electrophysiological methods measuring the laryngeal relocation time, pharyngeal transit time and the triggering of the pharyngeal phase of swallowing reflex. The EMG of cricopharyngeal muscle of the upper esophageal sphincter was also recorded in 10 patients. The patients have been compared with a group of 22 healthy controls matched with age and gender. Dysphagia limit was also measured for all patients and control subjects. Fourteen out of 19 patients could not swallow 20 ml or less amount of water at one go and divided the bolus into two or more pieces (piecemeal deglutition) in comparison to normal subjects. In PM/DM patients, the triggering of the swallowing reflex for the voluntarily initiated swallow was normal while the pharyngeal phase of swallowing was significantly prolonged. The cricopharyngeal sphincter muscle EMG demonstrated severe abnormalities in halves of the patients investigated. These findings demonstrated the weakness of the striated oropharyngeal muscles. Cricopharyngeal sphincter muscle was affected less frequently and showed either hyperreflexic or hyporeflexic states during swallowing. It is concluded that the pharyngeal stage of oropharyngeal swallowing is mainly involved in patients with PM/DM.

Autoimmune mechanisms have been implicated in the pathogenesis of headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL). Pooled sera of five HaNDL patients and 30 controls (10 multiple sclerosis patients, 10 migraine patients, 10 healthy controls) were screened by protein macroarray. All sera were also individually subjected to immunoprecipitation with neuroblastoma cells and the bound antigens were identified by mass spectrometry. Antibodies to three DNA repair proteins (mitogen-activated protein kinase-4, DNA-dependent protein kinase catalytic subunit, DNA excision repair protein ERCC-6) were identified by both macroarray and immunoprecipitation methods in 3/5 HaNDL sera, but in none of the controls. The presence of DNA repair protein antibodies indicates DNA damage and provides further support for the inflammatory etiology of HaNDL.

WOS: 000335016500001, PubMed ID: 25944978, Objective. The aim of our study was to investigate the frequency and pattern of cognitive impairment in patients with clinically isolated syndromes and definite diagnosis of multiple sclerosis within the last 2 years. Methods. We assessed the cognitive status of 46 patients aged 18-49 years with clinically isolated syndromes or definite diagnosis of multiple sclerosis who have onset of their symptoms within the last 2 years. Patients were matched with 40 healthy participants for age, sex, and educational level. Neuropsychological assessment was performed by stroop test, paced auditory serial addition test (PASAT), controlled oral word association test (COWAT), clock drawing test, trail making test (TMT), faces symbol test (FST). Hamilton Depression Scale and Modified Fatigue Impact Scale were used to quantify the severity of any depression and fatigue the subjects might suffer. Results. 19.6% of early MS/CIS group failed at 4 and more tests and had significant cognitive impairment focused on attention, executive functions, memory, and learning. No significant relationship was found between cognitive impairment and disability and fatigue scores. Discussion. Cognitive impairment can be present from the earliest stage of multiple sclerosis. It should be considered among the main manifestations of MS even in the earliest stages of the disease.

Nitric oxide (NO) has been implicated in immune mediated cellular cytotoxicity and inflammatory processes including multiple sclerosis (MS). We aimed to assess NO production in MS patients and to delineate its involvement in different stages. The stable end-products of NO; nitrite(NO2 − ) and nitrate(NO3 − ) were analysed both in serum and CSF (cerebrospinal fluid) of patients with MS and non-inflammatory neurological diseases. Nitrite levels were quantified by calorimetric assay based on the Griess reaction. Nitrate levels were examined spectrophotometrically. MS patients exhibited significantly increased serum and CSF levels of NO2 − +NO3 − compared with the control subjects. CSF NO2 − + NO3 − levels were raised significantly in MS patients with both relapsing remitting (RR) and secondary progressive (SP) course. There was no significant difference between RR and SP MS patients with regard to NO metabolites. No significant correlation was found between NO metabolites and disability score, disease progression index, MRI (magnetic resonance imaging) activity and development of cortical atrophy on MRI. This study provides further evidence for excessive NO production both in CSF and peripheral blood of MS patients. Excessive CSF NO2 +NO3 levels being more increased than the levels in sera supports pathological inflammatory process within CNS (central nervous system) in both stages of MS. Another implication for the role of NO and INOS inhibitors in the treatment of MS patients with both RR and SP courses was also suggested. © 2001 Elsevier Science B.V. All rights reserved.

WOS: 000085528600013, PubMed ID: 10611127, We investigated the pathophysiological mechanisms of dysphagia in amyotrophic lateral sclerosis, Forty-three patients with sporadic amyotrophic lateral sclerosis were examined by clinical and electrophysiological methods that objectively measured the oropharyngeal phase of voluntarily initiated swallowing, and these results were compared with those obtained from 50 age-matched control subjects. Laryngeal movements were detected by a piezoelectric sensor and EMG of submental muscles, and needle EMG of the cricopharyngeal muscle of the upper oesophageal sphincter of both the amyotrophic lateral sclerosis and control groups was recorded during swallowing. Amyotrophic lateral sclerosis patients with dysphagia displayed the following abnormal findings. (i) Submental muscle activity of the laryngeal elevators, which produce reflex upward deflection of the larynx during wet swallowing, was significantly prolonged whereas the laryngeal relocation time of the swallowing reflex remained within normal limits. (ii) The cricopharyngeal sphincter muscle EMG demonstrated severe abnormalities during voluntarily initiated swallows. The opening of the sphincter was delayed and/ or the closure occurred prematurely, the total duration of opening was shortened and, at times, unexpected motor unit bursts appeared during this period. (iii) During voluntarily initiated swallows there was significant lack of co-ordination between the laryngeal elevator muscles and the cricopharyngeal sphincter muscle. These results point to two pathophysiological mechanisms that operate to cause dysphagia in amyotrophic lateral sclerosis patients. (i) The triggering of the swallowing reflex for the voluntarily initiated swallow is delayed and eventually abolished, whereas the spontaneous reflexive swallows are preserved until the preterminal stage of amyotrophic lateral sclerosis, (ii) The cricopharyngeal sphincter muscle of the upper oesophageal sphincter becomes hyper-reflexic and hypertonic. As a result, the laryngeal protective system and the bolus transport system of deglutition lose their co-ordination during voluntarily initiated swallowing, We conclude that these pathophysiological changes are related mainly to the progressive degeneration of the excitatory and inhibitory corticobulbar pyramidal fibres.

Abnormal glutamate metabolism is implied in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS) and cerebrospinal fluid (CSF) glutamate levels appear to be elevated. Since nitric oxide (NO) inhibits glutamate transport, excessive amounts of nitric oxide could underlie the glutamate induced neurotoxicity in ALS. Stable metabolites of NO (NO2- + NO3-) levels were determined in serum and CSF of sporadic ALS patients and control subjects. NO2- + NO3- levels were higher in ALS, in males and in serum samples compared to controls, females and CSF, respectively. Furthermore, while the difference between serum and CSF NO2- + NO3- levels was significant in males (higher in serum) no such difference was observed in females. Our results suggest that nitric oxide may be involved in the pathogenesis of ALS directly or indirectly and in a sexually dimorphic manner.

WOS: 000077418200009, PubMed ID: 9854960, Objective-To evaluate dysphagia at the oropharyngeal stage of swallowing and to determine the pathophysiological mechanisms of dysphagia in patients with myasthenia gravis. Methods-Fifteen patients with myasthenia gravis with dysphagia and 10 patients without dysphagia were investigated by a combined electrophysiological and mechanical method described previously. Laryngeal movements were detected by a piezoelectric transducer and the related submental EMG (SM-EMG) and sometimes the EMG of cricopharyngeal muscle of the upper esophageal sphincter (CP-EMG) were recorded during dry or wet swallowing. The results of these electrophysiological variables were compared with those of normal age matched control subjects. Results-In patients with myasthenia gravis with dysphagia, it was found that the time necessary for the larynx to remain in its superior position during swallowing and swallowing variability in successive swallows increased significantly compared with normal subjects and with patients with myasthenia gravis without dysphagia. The total duration of SM-EMG activity was also prolonged in both groups but more severely in the dysphagic patients. Electromyographic activity of the CP sphincter was found to be normal in the dysphagic patients investigated. All the patients with myasthenia gravis with dysphagia had pathological dysphagia limits (

Necrotizing autoimmune myopathy (NAM) is a relatively newly recognized subgroup of idiopathic inflammatory myopathies. Classically, patients present with a subacute severe proximal myopathy, associated with a markedly elevated creatine kinase level, usually greater than 10 times the upper limit of normal, and electromyography shows myopathic changes. Muscle biopsy is required for diagnosis, revealing active necrosis of the muscle fibers without inflammatory invasion and variable upregulation of MHC class 1. The other causes of necrotizing myopathy should be excluded such as toxic myopathies, muscular dystrophies. The clinical presentation is highly variable, often similar to the other inflammatory myopathies. We herein report the clinical and histopathological features of 6 patients with NAM. Age of onset ranged from 35 to 69 years (mean 56.8 years), and 5 of them were female. All of the patients presented with subacute onset proximal limb weakness, 4 with myalgia. Serum CK levels ranged from 1041 to 7900 IU/L. Needle EMG revealed myopathic changes in all. Muscle biopsies showed mainly necrotic and/or regenerative muscle fibers in all 6 patients. There were focal (in 4) or diffuse (in 2) major histocompatibility complex class 1 expression in sarcolemma or cytoplasm of muscle fibers among necrotic muscle fibers. Screening for cancer revealed negative or normal results in all. Two patients received statins previously. Three patients had diabetes mellitus and 1 patient had SLE. All treated with steroids and/or immunosuppressive drugs. The clinical characteristics of these patients, pathologic findings, associated risk factors and response to immunosuppressive therapies are discussed.

We studied the various physiological aspects of oropharyngeal swallowing in Parkinson's disease (PD). Fifty-eight patients with PD were investigated by clinical and electrophysiological methods that measured the oropharyngeal phase of swallowing. All patients except 1 had mild to moderate degree of disability score. Dysphagia was demonstrated in 53% of all patients in whom the test of dysphagia limit was abnormal. All PD patients with or without dysphagia displayed the following abnormalities: (1) the triggering of the swallowing reflex was prolonged probably due to inadequate bolus control in the mouth and tongue and/or a specific delay in the execution of the swallowing reflex; (2) the duration of the pharyngeal reflex time was extremely prolonged due to slowness of the sequential muscle movements, especially those of the suprahyoid-submental muscles; (3) cricopharyngeal muscle of the upper oesophageal sphincter was found to be electrophysiologically normal; and (4) the electrophysiological phenomena in PD patients could not be strongly correlated with the degree of the disability and clinical score of the PD. It was concluded that various motor disorders of PD have considerable influence on oropharyngeal swallowing: hypokinesia, reduced rate of spontaneous swallowing, and the slowness of segmented but coordinated sequential movements rather than any abnormalities in the central pattern generator of the bulbar center. Some compensatory mechanisms in the course of PD may explain the benign nature of swallowing disorder until the terminal stage of the disease. Similarly, the swallowing problems of PD are not only related with the dopamine deficiency; some other nondopaminergic mechanisms may also be involved.

Carbendazim (C9H9N3O2; methyl 1-H-benzimidazol-2-yl carbamate) is widely used as an agricultural and horticultural fungicide and pesticide around the world.1No hazardous effects of carbendazim on the health of humans has yet been reported. However, in animals, especially in rats, adverse effects, including testicular atrophy, infertility, ascendant paralysis, respiratory failure, and muscle cramps due to chronic carbendazim exposure have been well documented.2-4 Recently, we have treated a 60 year old female farmer who had been affected by excessive exposure to carbendazim. She was admitted to our clinic 3 days after the intoxication, which occurred when she disinfected nits with mitrasol, which contains carbendazim. She was exposed to carbendazim for 6 hours and at night, after returning from work, she slowly developed ptosis and dysphagia for especially liquid. She was admitted to the local state hospital and given atropine for 3 days because of suspicion of organophosphorus intoxication. However, her complaints persisted …

WOS: 000167163100015, PubMed ID: 11181860, Objective-Oropharyngeal dysphagia is a common feature of patients with myotonic dystrophy and is not usually perceived due to their emotional deficits and lack of interest. The aim was to show the existence and frequency of subclinical electrophysiological abnormalities in oropharyngeal swallowing and to clarify the mechanisms of dysphagia in myotonic dystrophy. Methods-Eighteen patients with myotonic dystrophy were examined for oropharyngeal phase of swallowing by clinical and electrophysiological methods. Ten patients had dysphagia whereas 11 patients had signs and symptoms reflecting CNS involvement. Four patients with myotonia congenita and 30 healthy volunteers served as controls. Laryngeal movements were detected by means of a piezoelectric sensor. EMG activities of the submental muscle (SM-EMG) and needle EMG of the cricopharyngeal muscle of the upper eosophageal sphincter (CP-EMG) were also recorded during swallowing. Results-In about 70% of the patients with myotonic dystrophy, the existence of oropharyngeal dysphagia was indicated objectively by means of the technique of "dysphagia limit" and by clinical evaluation. Duration of the swallowing reflex as defined by the laryngeal relocation time (0-2 time interval) and submental muscle excitation as a part of the swallowing reflex (A-C interval) were significantly prolonged in patients with myotonic dystrophy, especially in dysphagic patients. Triggering time of the swallowing reflex (A-0 interval) also showed significant prolongation, especially in the patients having both dysphagia and CNS involvement. During swallowing, CP muscle activity was abnormal in 40% of the patients with myotonic dystrophy. Conclusion-Both myopathic weakness and myotonia encountered in oropharyngeal muscles play an important part in the oral and the pharyngeal phases of swallowing dysfunction in myotonic dystrophy. It was also suggested that CNS involvement might contribute to the delay of the triggering of the swallowing reflex and some abnormal EMG findings in the CP sphincter, resulting in oropharyngeal dysphagia in myotonic dystrophy.

Objective: Swallowing mechanisms and neurogenic dysphagia have not been systematically studied by the EMG technique. It is desirable to evaluate neurogenic dysphagia for diagnostic and possibly for therapeutic purposes using electrophysiological methods. Results: The following methods were described: mechanical upward/downward movements of the larynx were detected using a piezoelectric sensor, while submental integrated EMG activity was recorded during dry and wet swallowing. The EMG activity of cricopharyngeal muscle of the upper oesophageal sphincter was also recorded in some normal subjects and patients. Piecemeal deglutition and the dysphagia limit were determined in all patients to detect dysphagia objectively. In this study 75 normal subjects and 177 neurological patients with various degrees of dysphagia were investigated. Results: Voluntarily triggered oropharyngeal swallowing was commonly pathological in the majority of patients, with or without overt dysphagia. The dysphagia limit appeared to be an objective measure of the degree of dysphagia in more than 90% of patients. Pathophysiological mechanisms were different in at least three groups of patients with neurogenic dysphagia. In the group of patients with muscular disorders, laryngeal elevators were involved while the CP-sphincter was intact. The second group included patients with the clinical signs of corticobulbar fibre involvement such as amyotrophic lateral sclerosis and pseudobulbar palsy. In these patients, there was incoordination between paretic laryngeal elevators and hyperreflexic CP-sphincter. In the third group (patients with Parkinson’s disease), the swallowing reflex was delayed and prolonged. Conclusions: EMG methods described in the present study are very useful for the diagnosis of neurogenic dysphagia, objectively and quickly. They are important to understand the physiological mechanisms for deglutition and its disorders. © 1998 Elsevier Science Ireland Ltd. All rights reserved

A case of dermatomyositis associated with Behcet’s disease (BD) is described. A 51-year-old male with a 12-year history of BD, was currently on remission with colchicine therapy (0.5 mg/day), presented with acute onset difficulty in walking and skin lesions. His neurological examination revealed proximal muscle weakness in both the upper and lower extremities. No myalgia was noted. He had cutaneous lesions typical for dermatomyositis on his face, neck, upper trunk and hands. Laboratory tests demonstrated normal findings except elevated ESR and serum CK level (639 U/L). Muscle MRI of lower extremities revealed multiple patchy hyperintense lesions in pelvic and thigh muscles on STIR images. There was no muscle atrophy or fat replacement and it was commented as muscle inflammation. The clinical and laboratory findings were compatible with the diagnosis of dermatomyositis. He responded well to IV methylprednisolone within days. He continued on prednisolone therapy orally with gradual tapering. This case of Behcet’s disease associated with dermatomyositis (DM) is a very rare one. Muscular involvement in BD is rarely described but DM in association with BD has been never reported. The underlying cause of BD and the precise pathogenic mechanisms responsible for DM are unknown. The association of BD and DM may provide insights into genetic predisposition and pathogenesis of these complex and multisystemic autoimmune disorders.

WOS: A1996VT28700013, PubMed ID: 8937344, Objective-Before the advanced evaluation of deglutition and selection of a treatment method, objective screening methods are necessary for patients with dysphagia. In this study a new electroclinical test was established to evaluate patients with dysphagia. Methods-This test is based on determining piecemeal deglutition; which is a physiological phenomenon occurring when a bolus of a large volume is divided into two or more parts which are swallowed successively. The combined electrophysiological and mechanical method used to record laryngeal movements detected by a piezoelectric transducer, and activities of the related submental integrated EMG (SM-EMG)-and sometimes the cricopharyngeal muscle of the upper oesophageal sphincter (CP-EMG)-were performed during swallowing. Thirty normal subjects and 66 patients with overt dysphagia of neurogenic origin were investigated after detailed clinical evaluation. Twenty patients with a potential risk of dysphagia, but who were normal clinically at the time of investigation, were also evaluated to determine the specificity of the test. All subjects were instructed to swallow doses of water, gradually increasing in quantity from 1 ml to 20 ml, and any recurrence of the signals related to swallowing within the eight seconds was accepted as a sign of dysphagia Limit. Results-In normal subjects as well as in the patients without dysphagia, piecemeal deglutition was never seen with less than 20 mi water. This volume was therefore accepted as the lower limit of piecemeal deglutition. In patients with dysphagia, dysphagia limits were significantly lower than those of normal subjects. Conclusion-The method is a highly specific and sensitive test for the objective evaluation of oropharyngeal dysphagia even in patients with suspected dysphagia of neurogenic origin. It can also be safely and simply applied in any EMG laboratory.

WOS: 000331415100007, PubMed ID: 24291332, We describe a 34-year old man presenting with subacute generalized myasthenic symptoms. His clinical features and laboratory investigations demonstrated both myasthenia gravis and myotonic dystrophy type 1. The computerized tomography of chest revealed anterior mediastinal mass. The lymphocyte-rich thymoma was removed surgically and he received radiotherapy. Recent observations suggested that the patients with myotonic dystrophy may have an increased risk of benign and malignant tumours but its coexistence with thymoma is very rare. The risk of thymoma associated with myotonic dystrophy is unknown. (C) 2013 The Authors. Published by Elsevier B.V. All rights reserved.