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Highlights • Our study reveals that our study population with MS had decreased heart rate variability compared to healthy controls. • There is no significant association between cardiac autonomic impairment and number of attack, EDSS or MSFC test in patients with MS. • There is no significant relationship between findings on cranial, cervical and thoracic spinal MRI and the HRV analysis in patients with MS. Abstract Background Multiple sclerosis can cause cardiovascular autonomic dysfunction. It is assumed that is caused by multiple demyelinating plaques localized in the brain stem and spinal cord. Previous studies have determined this using tilt table test, heart rate responses to Valsalva maneuver and deep breathing and heart rate variability analysis with 24 h Holter monitoring. However there is not a consensus regarding the presence of the relationship between autonomic dysfunction and severity of multiple sclerosis, type of multiple sclerosis and expanded disability status scale. The aim of the study is comparison of heart rate variability between recently diagnosed patients with relapsing-remitting multiple sclerosis and healthy controls by using 24 h Holter monitoring. Also we intended to investigate relationship between Expanded Disability Status Scale score, Multiple Sclerosis Functional Composite scores and cranial and spinal magnetic resonance imaging findings and hearth rate variability. Method Fifty-one patients with newly diagnosed relapsing-remitting multiple sclerosis and 44 age- and sex-matched healthy controls were compared in this study. Patients with multiple sclerosis, who were already under immunomodulatory or immunosuppressive treatment, were excluded from the study. Echocardiography and hearth rate variability analysis using 24 h period Holter monitoring were performed in all of the subjects. Echocardiography was used to detect the presence of cardiac pathology. One multiple sclerosis patient with right ventricular dilatation and mobile intratrial septum was excluded from the study. All the patients underwent cranial and cervical spinal magnetic resonance imaging to determine the relationship between autonomic abnormalities and magnetic resonance imaging. Results Our results showed that hearth rate variability values were significantly lower in patients with multiple sclerosis when compared with healthy controls: SDNN index (the mean of all the 5 min standard deviations of normal RR intervals during the 24 h period) (59.80 ± 17.33 vs. 67.20 ± 21.28, p = 0,044), the root-mean-square successive difference (rMSSD) (34.40 ± 17.50 vs. 38.25 ± 12.95, p = 0,042), spectral hearth rat variability total power (3738.84 ± 2085.51 vs. 4427.44 ± 1965.71, p = 0,037), spectral hearth rate variability low frequency (852.03 ± 450.54 vs. 1011.75 ± 370.06, p = 0,018). Ten patients (20%) had brainstem lesion, 25 patients (50%) had cervical lesions and 10 patients (20%) had thoracic spinal lesions on magnetic resonance imaging. There was no significant relationship between location of the lesions and heart rate variability analyses. Also there was no significant relationship between hearth rate variability values and Expanded Disability Status Scale score, Multiple Sclerosis Functional Composite scores or number of multiple sclerosis attack (p > 0,05). Conclusion These findings reveals that our study population with multiple sclerosis had decreased heart rate variability compared to healthy controls. This was reflected by dysfunction of both parasympathetic and sympathetic parameters of hearth rate variability analysis. However, there is no significant relationship between hearth rate variability analysis and the findings on cranial, cervical, thoracic spinal magnetic resonance imaging findings, number of attack, Expanded Disability Status Scale score or Multiple Sclerosis Functional Composite scores in patients with multiple sclerosis. [ABSTRACT FROM AUTHOR]

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Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The nervous system involvement of BD, neuro-Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was mana - ged with an uncommon aggressive immunosuppressive combination therapy. The patient first presented six years ago with vertigo and difficulty in talking and walking. On examination, he had oral ulcers, acneiform lesions on the torso, geni tal ulcer scar, dysarthria, and ataxia. Along with the magnetic resonance imaging (MRI) findings, the patient was diagnosed as NBD. After pulse methylpred nisolone (1g/day, 3 days) and 8 courses of 1g/month iv cyclophosphamide therapy, he was put on azathioprine and oral methylprednisolone. On the 4th year of the maintenance therapy, he was admitted with NBD relapse which was treated with 3 days of iv 1g pulse methylprednisolone. One year after the last relapse, the patient voluntarily stopped medications and presented with global aphasia, right hemihypoesthesia and quadriparesis. MRI findings were sugges tive of NBD relapse. After exclusion of infection, pulse methylprednisolone was started but no improvement was observed. Considering the seve rity of the NBD, the patient was put on methylpredniso lone (1mg/kg/day), iv cylophosphamide (1g) and adalimumab 40 mg/14 days subcutaneously with appro - priate tuberculosis prophylaxis. Neurological examination and MRI findings after 4 weeks showed dramatic improvement however patient developed pulmonary tuberculosis. Methylprednisolone dose was decreased (0.5mg/kg/day) and quadruple antituberculosis therapy was started. Patient was dischar ged with 5/5 muscle strength in extremities without any respiratory symptoms 2 months after the first presentation. Prompt introduction of immunosuppressive therapy is crucial in NBD. Although combination of TNF inhibitors and cyclophosphamide is a rare therapeutic approach, it may be life-saving. However a higher awareness is required for opportunistic infections. [ABSTRACT FROM AUTHOR]

Aim: We aim to describe the demographic characteristics, etiology, neurophysiology, imaging findings, treatment, prognosis, and prognostic factors of acute flaccid myelitis., Methods: The clinical data, laboratory test and, magnetic resonance imaging (MRI) results of pediatric patients diagnosed with acute flaccid myelitis according to the Centers for Disease Control criteria between August 1, 2016, and December 31, 2018, from 13 centers in Turkey were reviewed., Results: Of the 34 cases identified, 31 were confirmed (91.2%). Eighteen patients (55.9%) were boys. The median patient age was 4 years (interquartile range 2.5-6.9 years). Most of the patients were admitted in 2018 (n = 27). A preceding history of a febrile illness was reported in all patients, with a median of 4 days (interquartile range 3-7 days) before symptom onset. Thirty-one patients had T2 hyperintensity on spinal MRI, and 18 patients had cerebrospinal fluid pleocytosis. The most common infectious agents were entero/rhinoviruses (n = 5) in respiratory specimens. All patients except one received immunotherapy either alone or in combination. Among 27 patients with follow-up data 24 had persistent weakness. Involvement of four limbs together with an abnormal brain MRI at onset were associated with a poor prognosis., Conclusion: The number of patients with acute flaccid myelitis increased since 2012, spiking with every 2-year interval, largely in the pediatric population. The median age decreases with every outbreak. Clinicians should be aware of the clinical picture for early collection of specimens and early start of rehabilitation programs. Further studies are needed to better characterize the etiology, pathogenesis, risk factors, and treatment of this rare condition., Competing Interests: Declaration of competing interest The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2020 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Background: The aim of this study was to establish reference jitter values for the voluntary activated sternocleidomastoid (SCM) muscle using a concentric needle electrode (CNE)., Methods: The study included 39 healthy participants (20 female and 19 male) aged 18-77 y. Jitter was expressed as the mean consecutive difference (MCD) of 80-100 consecutive discharges. Filters were set at 1 and 10 kHz. The mean MCDs for all participants were pooled, and the mean value +2.5 SD was accepted as the upper limit for the mean MCD. The upper limit for individual MCD was calculated using +2.5 SD of the upper 10th percentile MCD for individual participants., Results: Mean age of the participants was 45 ± 14.5 y. Mean MCD was 16.20 ± 2.23 μs (range: 12-21 μs), and the upper limit of normal for mean MCD was 21.8 μs. The mean value for 823 individual jitters was 23.3 ± 4.61 μs (range: 6.6-36.9 μs), and the upper limit of normal for each individual jitter was 34.6 μs., Conclusions: The present findings indicate that upper normal limit for mean MCD is 22 μs and for individual data it is 35 μs., (© 2020 Wiley Periodicals LLC.)

Purpose of the Study: We proposed a new electrophysiological parameter medial plantar (MP)-to-radial amplitude ratio (MPRAR), similar to sural-to-radial amplitude ratio (SRAR), in the diagnosis of distal sensory polyneuropathy (DSP), based on the concept that distal nerves are affected more and earlier than proximal nerves in axonal neuropathies. We aimed to investigate the diagnostic sensitivity of this parameter in diabetic DSP, together with sensitivities of SRAR and MP nerve action potential (NAP) amplitude., Materials and Methods: In 124 healthy controls and 87 diabetic patients with clinically defined DSP and normal sural responses, we prospectively performed sensory nerve conduction studies (NCS), and evaluated the MP NAP amplitude, MPRAR and SRAR values. We determined the lower limits of normal (LLN) of these parameters in the healthy controls and calculated their sensitivities and specificities in detecting DSP in diabetic patients., Results: MP nerve amplitude and MPRAR values were significantly lower in the patient group, compared to controls. However, SRAR values did not differ significantly between the two groups. The LLN of MP NAP amplitude was found to be 4.1 μV. The cutoff values for SRAR and MPRAR were determined as 0.24 and 0.16, respectively. MPRAR was abnormal in 21.8% of patients. However, the most sensitive parameter in detection of DSP was MP NAP amplitude, which showed a sensitivity of 31% and a specificity of 100%., Conclusions: Although MPRAR is more sensitive than SRAR in detecting DSP, it does not provide additional diagnostic yield to the assessment of MP NCS alone in diabetic DSP patients with normal sural responses.

Introduction: We aimed to assess central and peripheral nervous system involvement in systemic lupus erythematosus (SLE) patients without any neurological signs and symptoms by performing electrophysiological investigations., Methods: Thirty-eight SLE patients and 35 healthy volunteers participated in this study. Peripheral nerve conduction and brainstem reflexes were evaluated by performing nerve conduction studies (NCSs) and blink reflex (BR) and masseter inhibitory reflex (MIR) recordings., Results: Eleven patients (29%) had an abnormality in at least 1 NCS parameter, and 1 (2.6%) patient was diagnosed with polyneuropathy. The number of patients with abnormal BR and MIR was 23 (60.5%) and 14 (37%), respectively. The contralateral R2 latency of BR and the silent period 1 (SP1) latency of MIR were significantly prolonged in the patients compared with the controls (p=0.015 and p<0.001, respectively)., Conclusion: This study showed that irrespective of peripheral nervous system involvement, brainstem reflexes could be affected in SLE patients even without clinical neurological findings. Brainstem reflex abnormalities suggested that the functional integrity of the inhibitory or excitatory interneurons in the lateral caudal pons and lateral medulla is disturbed in SLE patients., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors.

Abstract/ Resumo Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The nervous system involvement of BD, neuro-Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was managed with an uncommon aggressive immunosuppresive combination therapy. The patient first presented six years ago with vertigo and difficulty in talking and walking. On examination, he had oral ulcers, acneiform lesions on the torso, genital ulcer scar, dysartria, and ataxia. Along with the magnetic resonance imaging (MRI) findings, the patient was diagnosed as NBD. After pulse methylprednisolone (1g/day, 3 days) and 8 courses of 1g/month iv cylophosphamide therapy, he was put on azathioprine and oral methlyprednisolone. On the 4th year of the maintenance therapy, he was admitted with NBD relapse which was treated with 3 days of iv 1g pulse methlyprednisolone. One year after the last relapse, the patient voluntarily stopped medications and presented with global aphasia, right hemihypoesthesia and quadriparesis. MRI findings were suggestive of NBD relapse. After exclusion of infection, pulse methylprednisolone was started but no improvement was observed. Considering the severity of the NBD, the patient was put on methylprednisolone (1mg/kg/day), iv cylophosphamide (1g) and adalimumab 40 mg/14 days subcutaneously with appropriate tuberculosis prophylaxis. Neurological examination and MRI findings after 4 weeks showed dramatic improvement however patient developed pulmonary tuberculosis. Methylprednisolone dose was decreased (0.5mg/kg/day) and quadruple antituberculosis therapy was started. Patient was discharged with 5/5 muscle strength in extremities without any respiratory symptoms 2 months after first presentation. Prompt introduction of immunosuppressive therapy is crucial in NBD. Although combination of TNF inhibitors and cyclophoshamide is a rare therapeutic approach, it may be life-saving. However a higher awareness is required for opportunistic infections.

Objective: This study aimed to assess palmar cutaneous branch of the median nerve (PCBm) conduction in patients with clinically diagnosed carpal tunnel syndrome (CTS), to compare PCBm conduction with that of the median and ulnar nerves, and to determine the PCBm conduction abnormality rate in patients with CTS., Materials and Methods: The study included 99 hands of 60 patients with clinical CTS and 38 hands of 38 healthy controls. Sensory nerve conduction study (NCS) was performed on the median nerve, ulnar nerve, and PCBm, and onset latency, conduction velocity and amplitude were recorded. Additionally, differences in latency and velocity between the median nerve and PCBm, and the difference in latency between the median and ulnar nerves were calculated., Results: In all, 56% of the patients with CTS had abnormal PCBm conduction. Additionally, in 7 of 8 hands with abnormal sensation--both in the thenar eminence and abnormal sensory distribution along the main branch--NCS of the PCBm was also abnormal., Conclusions: The PCBm is not ideal as a comparator nerve for the neurophysiological diagnosis of CTS. The frequency of PCBm abnormality in CTS patients may be related to the concomitant damage in both of these nerves. Additionally, the present findings may help explain, at least in part, why patients with CTS often exhibit sensory involvement beyond the classical median nerve sensory borders.

Postcoital artery dissection is a rare condition. Here we report a 40-year-old male patient with painful Horner syndrome related to postcoital internal carotid artery (ICA) dissection. In neurologic examination of the patient, semiptosis, enophthalmus, and myosis were observed on the left side. There were no carotid bruits. On T1-weighted and fat-suppressed cranial MRI, hyperintensity consistent with intramural hematoma was observed within cervical and temporal petrous segments of left ICA. On cervical and cranial MRA, marked decrease in the calibration of C1 and C2 segments of the left ICA was remarkable. The patient was diagnosed as left ICA dissection and anticoagulant therapy was initiated. A prominent improvement was noted in clinical findings during two months of followup period.