Clinical Neuroscience - 2020;73(01-02)

Clinical Neuroscience

JANUARY 30, 2020

[Current questions of multiple sclerosis: the secunder progressive form of the disease]

VÉCSEI László

[Recent data suggest that long-term worsening is common in relapsing-remitting multiple sclerosis patients and is largely independent of relapses or new lesion formation on brain MRI. The current definition of secunder progressive multiple sclerosis is worsening of disability independent of relapses over at least 6-month interval. Early focal inflammatory disease activity and spinal cord lesion are predictors of very-long term disease outcomes in relapse - onset multiple sclerosis. The potential of PET imaging to visualize hidden inflammation in MS brain in vivo is an important contribution for better understanding the progression of the disease. Therefore, PET imaging is a promising tool in detecting the conversion from relapsing remitting multiple sclerosis to secunder progressive form of multiple sclerosis. Furthermore, neuro-axonal damage is the pathological substrate of permanent disability in different neurological disorders including multiple sclerosis. The neurofilament proteins have promise in this context because their levels rise upon neuro-axonal damage not only in the cerebrospinal fluid but also in blood. Patients with increased serum levels of neurofilament at baseline, independent of other clinical and MRI variables, experience significantly more brain and spinal cord volume loss over 2 years and 5 years of follow-up. The kynurenine-pathway abnormalities may be associated with the swich from early-mild stage multiple sclerosis to debilitating progressive forms of the disease. Analysis of these metabolites in serum may have application as multiple sclerosis disease biomarkers. Free radical action has been suggested as a causal factor in the illness. Increased free radical production and consumption of the scavenger molecules were found during the active phase of the disease. Based on the clinical findings (EXPAND Study) and pathomechanism of the disease siponimod is approved by the US Food and Drug Administration for the treatment of relapsing remitting forms of multiple sclerosis, to include secunder progressive multiple sclerosis with active disease, relapsing-remitting multiple sclerosis and clinically isolated syndrome.]

Clinical Neuroscience

JANUARY 30, 2020

[The quality of life of the cluster headache patients during the active phase of the headache]

DIÓSSY Mária, BALOGH Eszter, MAGYAR Máté, GYÜRE Tamás, CSÉPÁNY Éva, BOZSIK György, ERTSEY Csaba

[Introduction - Cluster headache (CH), which affects 0.1% of the population, is one of the most painful human conditions: despite adequate treatment, the frequent and severe headaches cause a significant burden to the patients. According to a small number of previous studies, CH has a serious negative effect on the sufferers’ quality of life (QOL). In the current study, we set out to examine the quality of life of the CH patients attending our outpatient service between 2013 and 2016, using generic and headache-specific QOL instruments. Methods - A total of 42 CH patients (16 females and 26 males; mean age: 39.1±13.5 years) completed the SF-36 generic QOL questionnaire and the headache- specific CHQQ questionnaire (Comprehensive Headache- related Quality of life Questionnaire), during the active phase of their headache. Their data were compared to those of patients suffering from chronic tension type headache (CTH) and to data obtained from controls not suffering from significant forms of headache, using Kruskal-Wallis tests. Results - During the active phase of the CH, the patients’ generic QOL was significantly worse than that of normal controls in four of the 8 domains of the SF-36 instrument. Apart from a significantly worse result in the ‘Bodily pain’ SF-36 domain, there were no significant differences between the CH patients’ and the CTH patients’ results. All the dimensions and the total score of the headache-specific CHQQ instrument showed significantly worse QOL in the CH group than in the CTH group or in the control group. Conclusion - Cluster headache has a significant negative effect on the quality of life. The decrease of QOL experienced by the patients was better reflected by the headache-specific CHQQ instrument than by the generic SF-36 instrument. ]

Clinical Neuroscience

JANUARY 30, 2020

The effects of the level of spinal cord injury on life satisfaction and disability

GULSAH Karatas, NESLIHAN Metli, ELIF Yalcin, RAMAZAN Gündüz, FATIH Karatas, MÜFIT Akyuz

Introduction - Spinal Cord Injury (SCI) may often lead to significant disability in affected individuals and reduce life satisfaction. Herein we aimed to investigate the effects of the level of injury on disability and life satisfaction as well as the relation between life satisfaction and disability. Methods - Patients with at least one-year history of SCI were included. Demographic-clinical data of patients were recorded. The Craig Handicap Assessment and Reporting Technique-Short Form (CHART-SF) was used for quantifying the degree of patients’ disability. Life satisfaction was assessed by the Satisfaction with Life Scale (SWLS). Results - Of the 76 patients, 21 (27.6%) were tetraplegic and 55 (72.4%) were paraplegic. SWLS was found to be similar in tetraplegic vs. paraplegic patients (P=0.59), whereas CHART parameters such as physical independence, mobility, occupation, and total CHART value were significantly higher in paraplegic patients (P=0.04, P=0.04, P=0.001 and P=0.01, respectively). Social integration was found similarly high in both groups. There was a positive correlation between elapsed time after the injury and CHART physical independence, occupation and the level of economic sufficiency (P<0.01, P<0.01, P=0.01). Excluding the economic sufficiency (P=0.02), there was not any other association between the rest of CHART parameters and SWLS. Conclusions - According to our findings, although the level of injury seems to be influential on disability, it seems to have no significant effect on life satisfaction. Since the only thing that positively affects life satisfaction is economic sufficiency, more emphasis should be placed on regulations that increase the return to work in patients.

Clinical Neuroscience

JANUARY 30, 2020

[Correction of the physiological artefacts at pre-surgical clinical functional MR]

KISS Máté, GÁL Andor Viktor, KOZÁK Lajos Rudolf, MARTOS János, NAGY Zoltán

[Introduction/aim of the study - Pre-surgical functional MRI (fMRI) is an important modality of examinations before brain surgery. There are several artefacts (e.g. motion, susceptibility) which may hinder the evaluation of fMRI data. Physiological artefacts (breathing, pulsation) also affect the sensitivity and specificity of anatomical localization. The aim of this study is to demonstrate the efficiency of physiological artefact identification and removal methods for presurgical evaluation. Materials and methods - Siemens Magnetom Verio 3T MRI scanner was used to collect data. The physiological parameters (breathing, pulse) were recorded with the MRI system’s built-in devices. Data from fourteen patients - with primary brain tumour - were evaluated with SPM12 utilizing the RETROICOR/RVHR tool to detect and decrease the effect of physiological artefacts. We compared the statistical maps obtained with and without the physiological correction using the Jaccard similarity coefficient, and ROI analyses. Results - Significant differences were found in the mean ROI values (p<0.0016) and the extensions of eloquent activations (p<0.0013), when using the physiological correction (RETORICOR/RVHR) based on convolution method. On the other hand, no significant differences were found between the ROIs’ standard deviations (F=0.28). The RETROICOR/ RVHR method helps to define the precise localisation of eloquent areas (p<0.009). The number of irrelevant (non-significant) voxels were increased (p<0.001). Conclusions - Minimising of physiological artefacts in fMRI data calculations, the (RETROICOR/RVHR) method based on convolution has been successfully adapted. This algorithm could be helpful before neurosurgical intervention. The activity pattern became more reliable. ]

Clinical Neuroscience

JANUARY 30, 2020

Neurocognitive functions in patients with hepatitis C infection

HORVÁTH Gergely, KELETI Teodóra, MAKARA Mihály, UNGVARI S Gabor, GAZDAG Gábor

Background - With improving treatment options, more attention is being paid to the neurocognitive symptoms related to hepatitis C infection (HCI). While HCI-related neurocognitive impairments are frequently subclinical, they can influence patients’ quality of life and fitness to work. Objective - The aim of this study was to assess HCI patients’ neurocognitive functions and explore the correlations between disease variables and neurocognitive symptoms. Method - The study was conducted between January 1, 2013 and December 31, 2015. All patients with HCI were included in the study who were registered at the Hepatology Outpatient Clinic of Szent István and Szent László Hospitals, met inclusion criteria and volunteered to participate. Patients’ sociodemographic data and medical history were recorded in a questionnaire designed for the study. The 21-item Beck Depression Inventory was used to detect depressive symptoms. Six computerized tests were used to evaluate patients’ neuropsychological functions. Results - Sixty patients participated in the study. In comparison with general population standards, patients demonstrated poorer performance in several neurocognitive tests. Neuropsychological performance was correlated with age, sex, length of time since HCI diagnosis, Fibroscan score and the number of previous antiviral treatments. Conclusions - The study’s main finding is that compared to general population standards, patients with hepatitis C virus-related disease exhibit impaired neuropsychological functioning in visuomotor and visuospatial functions, working memory, executive functions, and reaction time. Executive functions and reaction time were the most sensitive indicators for the length and severity of disease. Deterioration in these functions has a major negative effect on work performance particularly in certain occupations.

Clinical Neuroscience

JANUARY 30, 2020

Delirium due to the use of topical cyclopentolate hydrochloride

MAHMUT Atum, ERKAN Çelik, GÜRSOY Alagöz

Introduction - Our aim is to present a rare case where a child had delirium manifestation after instillation of cyclopentolate. Case presentation - A 7-year old patient was seen in our outpatient clinic, and cyclopentolate was dropped three times at 10 minutes intervals in both eyes. The patient suddenly developed behavioral disorders along with gait disturbance, and complained of visual hallucinations 20-25 minutes after the last drop. The patient was transferred to intensive care unit and 0.02 mg/kg IV. physostigmine was administered. The patient improved after minutes of onset of physostigmine, and was discharged with total recovery after 30 minutes. Conclusion - Delirium is a rare systemic side effect of cyclopentolate. The specific antidote is physostigmine, which can be used in severely agitated patients who are not responding to other therapies.

Clinical Neuroscience

JANUARY 30, 2020

Review of electrode placement with the Slim Modiolar Electrode: identification and management

DIMAK Balazs, NAGY Roland, PERENYI Adam, JARABIN Andras Janos, SCHULCZ Rebeka, CSANÁDY Miklós, JÓRI József, ROVÓ László, KISS Geza Jozsef

Background - Several cochlear implant recipients experience functionality loss due to electrode array mal-positioning. The application of delicate perimodiolar electrodes has many electrophysiological advantages, however, these profiles may be more susceptible to tip fold-over. Purpose - The prompt realization of such complication following electrode insertion would be auspicious, thus the electrode could be possibly repositioned during the same surgical procedure. Methods - The authors present three tip fold-over cases, experienced throughout their work with Slim Modiolar Electrode implants. Implantations were performed through the round window approach, by a skilled surgeon. Standard intraoperative measurements (electric integrity, neural response telemetry, and electrical stapedial reflex threshold tests) were successfully completed. The electrode position was controlled by conventional radiography on the first postoperative day. Results - Tip fold-over was not tactilely sensated by the surgeon. Our subjects revealed normal intraoperative telemetry measurements, only the postoperative imaging showed the tip fold-over. Due to the emerging adverse perception of constant beeping noise, the device was replaced by a CI512 implant after 6 months in one case. In the two remaining cases, the electrode array was reloaded into a back-up sheath, and reinserted into the scala tympani successfully through an extended round window approach. Discussion - Future additional studies using the spread of excitation or electric field imaging may improve test reliability. As all of these measurements are still carried out following electrode insertion, real-time identification, unfortunately, remains questionable. Conclusion - Tip fold-over could be reliably identified by conventional X-ray imaging. By contrast, intraoperative electrophysiology was not sufficiently sensitive to reveal it.

Clinical Neuroscience

JANUARY 30, 2020

Myasthenia gravis, Guillain-Barré syndrome, or both?

ERDOGAN Cagdas, TEKIN Selma, ÜNLÜTÜRK Zeynep, GEDIK Korkut Derya

Myasthenia gravis (MG) and Guillain-Barré syndrome (GBS) are autoimmune disorders that may cause weakness in the extremities. The coexistence of MG and GBS in the same patient has rarely been reported previously. A 52-year-old male presenting with ptosis of the left eye that worsened with fatigue, especially toward evening, was evaluated in our outpatient department. His acetylcholine receptor antibody results were positive, supporting the diagnosis of MG. His medical history revealed a post-infectious acute onset of weakness in four extremities, difficulty in swallowing and respiratory failure, which was compatible with a myasthenic crisis; however, his nerve conduction studies and albuminocytologic dissociation at the time were compatible with GBS. With this case report, we aimed to mention this rare coincidental state, discuss possible diagnoses and review all other similar cases in the literature with their main features.

Clinical Neuroscience

JANUARY 30, 2020

Marchiafava-Bignami disease: Report of three cases

GUNAY Gul, MESUDE Özerden, ZEYNEP Ozdemir, CAHIT Keskinkiliç, HAKAN Selçuk, BATUHAN Kara, AYSUN Soysal

Marchiafava-Bignami disease (MBD) is a rare alcohol-associated disorder characterized by demyelination and necrosis of the corpus callosum. We report three patients who had history of chronic alcoholism, different clinical presentation and MRI findings consistent with the diagnosis of Marchiafava-Bignami disease.

Clinical Neuroscience

JANUARY 30, 2020

Valproic acid associated pleuropericardial effusion: case report

DEMIR Figen Ulku

Introduction - Valproic acid is an effective antiepileptic and mood stabilizer used in the treatment of many neurological and psychiatric disorders. Although there are frequently seen side effects, effusions between layers of pleural and pericardial membranes are rare to be seen. Case - Pleuropericardial effusion was detected in a 23 years old woman who was under valproic acid treatment because of epileptic seizure. After 1 year of valproic acid treatment, patient complained of dyspnea. As all the researches intended on etiology were usual, valproic acid has been thought to be responsible for the matter. Control examination after 1.5 months regarding the end of treatment revealed complete recovery of pleuropericardial effusion. Discussion - Pleural and pericardial effusions are rarely seen complications related to the use of valproic acid. It must also be kept in mind that valproic acid causes a potential for such side effects which can be blamed etiologically when the other possibilities for patients are excluded.