Clinical, Demographic, and Radiological Characteristics of Patients Demonstrating Antibodies Against Myelin Oligodendrocyte Glycoprotein.

Background: Optic neuritis, myelitis, and neuromyelitis optica spectrum disorder (NMOSD) have been associated with antibodies against myelin oligodendrocyte glycoprotein-immunoglobulin G (anti-MOG-IgG). Furthermore, patients with radiological and demographic features atypical for multiple sclerosis (MS) with optic neuritis and myelitis also demonstrate antibodies against aquaporin-4 and anti-MOG-IgG. However, data on the diagnosis, treatment, follow-up, and prognosis in patients with anti-MOG-IgG are limited. Aims: To evaluate the clinical, radiological, and demographic characteristics of patients with anti-MOG-IgG. Study Design: Multicenter, retrospective, observational study. Methods: Patients with blood samples demonstrating anti-MOG-IgG that had been evaluated at the Neuroimmunology laboratory at Ondokuz Mayis University's Faculty of Medicine were included in the study. Results: Of the 104 patients with anti-MOG-IgG, 56.7% were women and 43.3% were men. Approximately 2.4% of the patients were diagnosed with MS, 15.8% with acute disseminated encephalomyelitis (ADEM), 39.4% with NMOSD, 31.3% with isolated optic neuritis, and 11.1% with isolated myelitis. Approximately 53.1% of patients with spinal involvement at clinical onset demonstrated a clinical course of NMOSD. Thereafter, 8.8% of these patients demonstrated a clinical course similar to MS and ADEM, and 28.1% demonstrated a clinical course of isolated myelitis. The response to acute attack treatment was lower and the disability was higher in patients aged > 40 years than patients aged < 40 years at clinical onset. Oligoclonal band was detected in 15.5% of the patients. Conclusion: For patients with NMOSD and without anti-NMO antibodies, the diagnosis is supported by the presence of anti-MOG-IgG. Furthermore, advanced age at clinical onset, Expanded Disability Status Scale (EDSS) score at clinical onset, spinal cord involvement, and number of attacks may be negative prognostic factors in patients with anti-MOG-IgG.

Retrospective analysis of effectiveness of fingolimod in real life setting in Turkey (REFINE).

BACKGROUND: During multiple sclerosis (MS) treatment different modes of action such as lateral (interferon beta to glatiramer acetate or glatiramer acetate to interferon beta) or vertical (interferon beta/glatiramer acetate to fingolimod) drug switch can be performed. This study aims to investigate the clinical effectiveness of switching from the first-line injectable disease modifying treatments (iDMTs) to fingolimod (FNG) compared to switching between first-line iDMTs. METHODS: This is a multicenter, observational and retrospective study of patients with relapsing-remitting MS who had lateral and vertical switch. The observation period included three key assessment time points (before the switch, at switch, and after the switch). Data were collected from the MS patients' database by neurologists between January 2018 and June 2019. The longest follow-up period of the patients was determined as 24 months after the switch. RESULTS: In 462 MS patients that were included in the study, both treatments significantly decreased the number of relapses during the postswitch 12 months versus preswitch one year while patients in the FNG group experienced significantly fewer relapses compared to iDMT cohort in the postswitch 12 months period. FNG cohort experienced fewer relapses than in the iDMT cohort within the postswitch 2 year. The mean time to first relapse after the switch was significantly longer in the FNG group. DISCUSSION: The present study revealed superior effectiveness of vertical switch over lateral switch regarding the improvement in relapse outcomes. Patients in the FNG cohort experienced sustainably fewer relapses during the follow-up period after the switch compared the iDMT cohort. Importantly, switching to FNG was more effective in delaying time to first relapse when compared with iDMTs.

Amplitude and velocity dependence of patellar pendulum triggered by T reflex in Parkinson's rigidity.

OBJECTIVES: The relation between increase of tonus and joint movement velocity is controversial in Parkinson's rigidity. It is accepted that the increase of tonus in rigidity is constant during joint movement, and does not change within all limits of movement. However, there is thoughtful evidence that the change in tonus in rigidity has a correlation with joint movement velocity and amplitude of movement. The pendulum movement that is formed by triggering of the patellar T reflex allows the examination of phasic stretching reflexes and physiological changes of passive stretching. Therefore, the velocity and amplitude properties of tonus in Parkinson's rigidity can be scanned together. MATERIALS AND METHODS: Patellar T reflex-triggered patellar pendulum was recorded in 40 Parkinson's patients. The velocity and amplitude changes in the pendulum were observed according to the rigidity scale. Muscle action potentials were recorded from the rectus femoris muscle and biceps femoris muscles simultaneously via superficial recording electrodes. Knee joint angle changes were recorded with a goniometer. The kinesiological and electromyographic features were compared with those of the control subjects. RESULTS: The number of pendulums decreased significantly, the angle of joint movement decreased, the peak time decreased and the angular velocity slowed down significantly in the Parkinson's group. While the latency of the patellar T reflex did not change significantly, its amplitude decreased, and the onset time of joint movement measured by accelerometer was prolonged. CONCLUSIONS: Parkinson's rigidity has a velocity-dependent component, and this correlates negatively with the rigidity scale.

Evaluation of cardiac autonomic functions in myasthenia gravis.

OBJECTIVE: We aimed to comprehensively evaluate cardiac autonomic function in patients with MG and to investigate the relationship between this disorder and disease duration, thymoma and acetylcholine receptor antibody positivity in cases of cardiac autonomic disorder. METHODS: The study included 30 patients with MG and 30 age-matched healthy control subjects. Haemodynamic parameters (heart rate, systolic and diastolic blood pressure) and autonomic parameters (low frequency [LF], high-frequency [HF], sympathovagal balance [LF/HF], baroreceptor reflex sensitivity [BRS]) of the patients were automatically measured at rest and in a tilted position with the Task Force Monitor. RESULTS: The mean systolic and diastolic blood pressure measurements obtained at rest and during the tilt test were higher in patients with MG. Sympathovagal balance has been disturbed in favour of sympathetic tone, and parasympathetic insufficiency has become more prominent. When baroreceptor sensitivity was used as the second parameter to evaluate autonomic heart functions, BRS at rest and during the tilt test was lower in the MG group compared with the control group. DISCUSSION: These results suggest that sympathovagal balance has been disturbed in favour of sympathetic tone and that parasympathetic insufficiency has become more prominent. The current findings support the presence of cardiac autonomic involvement in patients with MG. The determination of cardiac autonomic function via noninvasive methods among patients with MG has high predictive value. The identification of autonomic dysfunction at an early stage and the early treatment of cardiovascular diseases can reduce morbidity and mortality.

The interaction between breathing and swallowing in amyotrophic lateral sclerosis.

The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus. Laryngeal and chest movement sensors, pulmonary function tests, submental, and diaphragm electromyography activity were used to evaluate the relationship between swallowing and respiratory phase. Of the 27 patients included in the study, 14 were dysphagic and 13 were non-dysphagic. Tests showed normal respiratory function in 11 of the non-dysphagic patients and 3 of the dysphagic patients. There was a high correlation between the dysphagia limit and Amyotrophic Lateral Sclerosis Functional Rating Scale swallowing parameters. Non-dysphagic patients were able to swallow during inspiration but only six patients in the dysphagic group were able to swallow during inspiration. The occurrence of dysphagia in ALS is related to piecemeal deglutition and respiration consistency during swallowing. Detecting the timing of disturbances in the relationship between swallowing and respiration may be a way of identifying dysphagia. Dysphagia limit may be a useful, complementary test for assessing swallowing disturbances in amyotrophic lateral sclerosis.

Impact of sympathetic dysfunction in the etiology of overactive bladder in women: A preliminary study.

AIMS: We investigated the effects of autonomic sympathetic dysfunction in the etiology of overactive bladder (OAB) in women, by assessing the sympathetic skin response (SSR). METHODS: In total, 40 women with OAB and 15 volunteers were enrolled. Group 1 (n = 20) consisted of patients who benefitted from oral anticholinergics, Group 2 (n = 20) consisted of patients who were refractory to oral anticholinergics, and the volunteers (n = 15) were the control group. All patients were asked to complete OAB-SF questionnaires. The absence of SSR was accepted as significant. Detailed physical examinations including neurological examinations were performed. All patients underwent SSR tests on the skin of the hands, feet, and genital area in our neurology department using electromyography, and SSR responses were recorded. Statistical significance was accepted at P < 0.05. RESULTS: The mean age was 47.2 ± 12.4 years. In total, 55 women were evaluated (20 in Group 1, 20 in Group 2, and 15 in the control group). Mean OAB scores were similar between groups 1 and 2, although urgency was significantly higher in Group 2 than 1. The absence of SSR was significantly higher in Group 2 than 1 (P = 0.01). Impaired SSR was observed more commonly in Group 2 than 1. All controls had normal SSRs. CONCLUSIONS: Regional dysfunction in the autonomic nervous system may be part of OAB's etiology. SSR may be a candidate test for determining early OAB, and may also be useful for predicting antimuscarinic-refractory patients.

Biceps femoris late latency responses and the "notching sign" in spasticity.

BACKGROUND: Spasticity is a motor impairment due to lesions in the brain and spinal cord. Despite being a well-known problem, difficulties remain in the assessment of the condition. The electrophysiological and kinesiological characteristics of the patellar pendulum changes during the movement triggered by the patellar T reflex could be used to assess spasticity. METHODS: Features of the patellar pendulum during the patellar T reflex were considered using a goniometric approach in spastic patients evaluated with the Ashworth scale. Medium and late latency responses in the rectus and biceps femoris muscles were examined electrophysiologically. For each pendulum, the maximum angle extension during an oscillation of the knee joint, maximal extension time, angular velocities of extensions of the knee joint and frequency of motion due to the patellar reflex were calculated. The damping of the amplitude in the pendulum was calculated. RESULTS: The spasticity group consisted of 65 patients (38 males and 27 females) with a mean age of 47.6 ± 14.0 years. The normal control group consisted of 25 individuals (19 males and six females) with a mean age of 32.1 ± 10 years. The biceps and rectus femoris long latency late responses were not observed in the normal cases. The biceps femoris medium latency response was observed only in 24 % of healthy individuals; conversely, late responses were observed in 84 % of patients. Activation of the antagonist muscles at a certain level of spasticity created a notching phenomenon. Amplitude of the reflex response and mean angular velocity of the first oscillation present in a dichotomic nature in the spasticity groups. Frequency of the first pendular oscillation increased with the increase of the Ashworth scale, while the damping ratio decreased with increasing scale. The Ashworth scale showed a correlation with the damping ratio. The damping ratio strongly distinguished the spastic subgroups and showed a strong negative correlation with the Ashworth scale. CONCLUSIONS: The Ashworth scale presents a good correlation with kinesiological parameters, but it is only possible to differentiate normal and spastic cases with electrophysiologic parameters. Furthermore, the notching phenomenon could be evaluated as a determinant of spasticity.

One-minute exercise is best for evaluation of postexercise exhaustion in myasthenia gravis.

INTRODUCTION: To find the best exercise duration for postexercise exhaustion by decrement (PEE-D) in myasthenia gravis (MG). METHODS: In 32 tests in 32 MG patients, repetitive nerve stimulation was performed in the abductor digiti quinti muscle. The 3 Hz responses for 2 s were obtained with supramaximal stimulation at rest, and immediately after (PE0), 30 s after (PE30s), and 1, 2, 3, and 4 min after 10-s, 30-s, and 1-min exercises. RESULTS: Compared with the decrement at rest, a significantly greater decrement was found at PE2m and PE3m after 30-s exercise, and at PE2m, PE3m, and PE4m after 1-min exercise. In 11 patients who showed a decremental response only with exercise, PEE-D was observed in 5 after 30-s exercise and in 8 after 1-min exercise. CONCLUSIONS: One-minute exercise is best for evaluation of PEE-D in MG.

Sensorimotor polyneuropathy in patients with SMA type-1: electroneuromyographic findings.

INTRODUCTION: Generally, spinal muscular atrophy (SMA) is believed to be a pure motor neuron disease. We retrospectively evaluated our electrodiagnostic findings in SMA type 1 patients to demonstrate co-existence of sensorimotor neuropathies. METHODS: Electroneuromyographic (ENMG) studies in 15 patients (11 boys, 4 girls) were reviewed independently by 2 neurophysiologists. Upper extremity findings were compared with normal right arm controls. RESULTS: Patient ages ranged from 1.5 to 26 months. Four SMA patients (26.7%) had decreased sensory nerve action potentials (SNAPs) or sensory nerve conduction velocities. Of them, median SNAPs could not be elicited in 3, and sural SNAPs could not be elicited in 2. Compound muscle action potential amplitudes were severely decreased in 14 (93.3%) and normal in 1. CONCLUSIONS: Survival motor neuron 1 (SMN1) gene analysis should be considered if clinical features are consistent with SMA, even if pathological or electrophysiological findings demonstrate peripheral sensorimotor polyneuropathies.

The interaction between breathing and swallowing in healthy individuals.

In this article, we aimed at investigating the interaction between breathing and swallowing patterns in normal subjects. Ten healthy volunteers were included in the study. Diaphragm EMG activity was recorded by a needle electrode inserted into the 7th or 8th intercostal space. Swallowing was monitored by submental EMG activity, and laryngeal vertical movement was recorded by using a movement sensor. A single voluntary swallow was initiated during either the inspiration or expiration phases of respiration, and changes in EMG activity were evaluated. When a swallow coincided with either inspiration or expiration, the duration of the respiratory phase was prolonged. Normal subjects were able to voluntarily swallow during inspiration. During the inspiration phase with swallowing, diaphragmatic activity did not ceased and during the expiration phase with swallowing, there was a muscle activity in the diaphragm muscle.

Medium-latency reflex response elicited from the flexor carpi radialis by radial nerve stimulation.

The H reflex obtained from the flexor carpi radialis muscle by median nerve stimulation is a well-known monosynaptic reflex. However, the origin of the late responses is still contentious. Radial nerve stimulation was performed through the spiral groove, and EMG recording was obtained from the flexor carpi radialis (FCR) and extensor carpi radialis (ECR) muscles. An M response followed by an F response was achieved from the ECR by radial nerve stimulation; the antagonistic FCR muscle elicited a late response. A total of 25 cases were included in this study. In 22 of these cases, a response with a latency of 40.97 ± 5.35 ms was obtained from the FCR by radial nerve stimulation. When extension of the hand was restricted, the response disappeared in five of nine cases. Application of cold markedly suppressed the response and prolonged the latency of the FCR medium-latency reflex response (FCR-MLR). Oral tizanidine considerably suppressed the FCR-MLR response. Two out of eight cases did not exhibit any response. No response could be recorded from a patient with complete amputation of the right hand. The FCR-MLR is the reflex caused by stretching of the FCR muscle from radial nerve stimulation, and it is greatly influenced by group II afferents.

Flexor reflexes elicited by magnetic and electric stimulation of the sural nerve.

OBJECTIVES: We have investigated whether magnetic stimulation of the sural nerve can evoke a flexor reflex recorded from the ipsilateral short head of the biceps femoris muscle. METHODS: The sural nerve was subjected to magnetic stimulation as well as by single-pulse electrical stimulation in healthy subjects. RESULTS: In 87% of the participants, a reflex response was elicited from the short head of biceps femoris muscle by magnetic stimulation of the sural nerve. In terms of latency and amplitude, this reflex response was similar to the flexor reflex response evoked from the same muscle by single-pulse electrical stimulation of the sural nerve. DISCUSSION: Findings indicate that flexor reflexes can easily be evoked from the short head of the biceps femoris muscle by magnetic stimulation of the sural nerve. The late component of the flexor reflex may not only be elicited via nociceptive afferents but may also involve non-nociceptive afferents.

VEGF polymorphisms and serum VEGF levels in Parkinson's disease.

Accumulated data within the recent years demonstrate that reduced levels of VEGF which is a well known angiogenic molecule might cause neurodegeneration in part by impairing neural tissue perfusion, vasoregulation and normal functioning of perivascular autonomic nerves. Additionally, VEGF has been reported to support neuroprotection in dopaminergic neurons by indirect and direct mechanisms and suppress apoptosis in dopaminergic neurons in vitro. The aim of the current study is first to demonstrate whether there is an association between the three common VEGF polymorphisms (-2578C/A, -634C/G and 936C/T) in the VEGF gene and idiopathic Parkinson's disease (IPD) which is a neurodegenerative disease caused by the progressive degeneration of nigrostriatal dopaminergic neurons, and second to see if the serum levels of VEGF is reduced in the patients with IPD. We screened the genotype and allele frequencies of three common functional polymorphisms of VEGF, namely -2578C/A, -634C/G and 936C/T in DNA samples of 126 patients with IPD and healthy control subjects and also we compared the median serum levels of VEGF between these two groups. No association was found between the inspected VEGF polymorphisms and IPD and also no difference was found between the serum VEGF levels of both groups. The current study failed to support the hypothesis that VEGF polymorphisms and/or reduced serum VEGF levels are likely contributors to the neurodegenerative process in IPD.

Evaluation of the lateral instability of the ankle by inversion simulation device and assessment of the rehabilitation program.

OBJECTIVES: To assess the correctibility of the muscle atrophy, proprioceptive loss, and slowing of the reflex arc around the ankle after ankle sprain with rehabilitation. METHODS: The study group consisted of 20 cases with chronic instability who had at least two episodes of ankle sprains (mean 20.6 years, range 16-32 years); control group consisted of 20 patients with same demographic characteristics but without instability. Isokinetic muscle strength measurements and proprioceptive evaluations were made using the Cybex device before and 1.5-month after rehabilitation period. Additionally, the inversion simulation device, which was developed together with the mechanical engineering department of our university, was correlated with the EMG device, and response periods of muscles to stimulation were measured. RESULTS: The proprioceptive loss present in all cases with ankle instability before treatment significantly improved after effective rehabilitation (p=0.001). It was detected that lengthened peroneal latent periods shortened with effective rehabilitation (p=0.001). Cross-interaction of rehabilitation was shown with the preservation of the difference between the pathologic and normal sides regarding proprioception and peroneal latent periods before and after treatment, without any difference between the control group and the pathologic sides. CONCLUSION: After ankle sprains, especially in patients with chronic instability, strengthening of the muscles around the ankle with well-planned proprioceptive exercises helps the patients return to normal living and sports activities, and prevents unnecessary surgery, especially in cases with functional instability.

Electrophysiologic and neuropsychologic evaluation of patients with centrotemporal spikes.

The present study was designed to evaluate neurocognitive functions with endogenous potentials and neurophysiologic tests in patients with centrotemporal spikes who were not on any medication. Of the patients, 85.7% had seizures, 9.5% had pavor nocturnes, and 4.8% had atypical headache. The patients, especially who had atypical seizures or left-sided epileptic activity, were found to have significant visuomotor function impairment (p <.05). In P300 test, N2P3 amplitude was lower in the patients, particularly who had left sided epileptic activity (p <.05). MMN and LDN results were normal. Serial evaluations of such patients with endogenous potentials and neuropsychological tests may be helpful to show development of neurocognitive impairment.

Effect of strength and endurance training on cognition in older people.

The purpose of this study was to investigate the effect of moderate strength and endurance training on cognition evaluated by event-related potentials (ERP) in older people. Thirty-six adults, aged 60-85 years, were randomly divided into three groups: sedentary control (C), strength training (ST), and endurance training (ET). Participants performed functional fitness tests and ERP data were recorded before and after nine weeks of training. Training involved three sessions per week. Functional fitness test performance improved significantly in the ST and ET groups. The latencies of the N1, N2, and P2 components and the amplitudes of the N1P2, P2N2, and N2P3 components differed significantly between groups (p < 0.05). After training, the latencies of the P2 and N2 components at the Fz and Cz sites, decreased significantly, and the amplitudes of the N1P2, P2N2, and N2P3 components at the Fz site and the N1P2 and N2P3 components at the Cz site, increased significantly in the ST group compared with the ET group. After training, the latencies of N1, N2, and P2 components shortened significantly, and the amplitudes of the N1P2, P2N2, and N2P3 components increased significantly in the ST group compared with the C group. The latencies of the N2 and P2 components shortened significantly in the ET group compared with the C group, although the amplitudes of the ERP recordings did not differ significantly between groups. These data suggest that strength training might facilitate early sensory processing and cognitive functioning in older individuals. Key PointsStrength training may have facilitating effects on early information processing and cognition in older people.It is interesting that only small improvements in functional fitness affected cognitive performance.More research is needed to determine how the different exercise regimens contribute to discrete changes in CNS functioning and how such changes affect the P3 component of the ERP.