Clinical Neuroscience

[28 years in the service of the cooperation of national clinical neurosciences]

RAJNA Péter, TAJTI János

SEPTEMBER 30, 2018

Clinical Neuroscience - 2018;71(09-10)

DOI: https://doi.org/10.18071/isz.71.0303

[Two leading managers of the scientific journal currently publishing its 71st volume give an overview on the history and content- and form-related development of the journal. Recognizing the constantly decreasing role of paper-based literature products, the value system and the priorities of the journal were established more than 20 years ago: 1) preservation of high standards by achieving and maintaining international registration and annual qualification (i.e. impact factor); 2) enabling publication in Hungarian language for scientists living in Hungary and abroad; 3) displaying an ever broader spectrum of clinical neurosciences in the publications; 4) presentation of cutting-edge findings (related to the etiology, diagnostics, therapy, and care) of the most frequent and most relevant diseases in order to assure and enhance the quality of national clinical practice; 5) providing the possibility for the highest possible standard of scientific publication for Hungarian clinical neuroscientist; and finally 6) maintaining a readily available interaction surface and debate forum for the involved professionals in clinical questions of public interest. With respect to the above aspects, an outline is given of the efforts of the nearly three decades by listing editorial, publication, and other activities introduced throughout the history of the journal. The presumed strengths and weaknesses of the journal are summarized, the opportunities and limitations of the established objectives are highlighted, based on which the editors outline the most important tasks (SWOT analysis).]

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Clinical Neuroscience

[Diagnosis of multiple sclerosis: A review of the 2017 revisions of the McDonald criteria]

CSÉPÁNY Tünde

[The revolutionary progress of research in neuroimmu­nology has led to the introduction of disease modifying therapies in multiple sclerosis at the end of the last century. The International Panel on Diagnosis of Multiple Sclerosis originally proposed the 2001 McDonald criteria to facilitate the diagnosis of MS in patients with the first objective neurological symptom(s) suggesting demyelinating event, when magnetic resonance imaging is integrated with clinical and other paraclinical diagnostic methods. New terms have been introduced to substitute clinical information by MRI: dissemination in space - indicating a multifocal central demyelinating process and dissemination in time - indicating the development of new CNS lesions over time. The criteria for diagnosis of Multiple Sclerosis have continuously evolved, they were modified in 2005 and 2010 allowing for an earlier and more accurate diagnosis of MS over time, and they provided the most up-to-date guidance for clinicians and researchers. The last recommended revisions relied entirely on available evidence, and not on expert opinion thereby reducing the risk of the misdiagnosis. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical, clinically isolated syndrome. In this review, we provide an overview of the recent 2017 revisions to the criteria of dissemination in space and time with the importance of the presence of CSF-specific oligoclonal bands; keeping fully in mind that there is no better explanation for symptoms than diagnosis of MS. In the future, validation of the 2017 McDonald criteria will be needed in diverse populations. Further investigations are required on the value of new MRI approaches, on optic nerve involvement, on evoked potential and optical coherence tomography, in order to assess their possible contribution to diagnostic criteria.]

Clinical Neuroscience

Acute motor and sensory axonal neuropathy associated with Sjögren’s syndrome

ETHEMOGLU Ozlem, KOCATÜRK Özcan, TARINI Zeynep Emine

Sjögren’s syndrome (SS) is an autoimmune disease with mononuclear cell infiltration and destruction of the lacrimal gland and salivary glands, which cause dryness of the eyes and mouth. The most common neurological condition seen in SS is peripheral neuropathy. Initial manifestation of SS as an acute fulminant peripheral neuropathy is extremely rare. We report a 42-year-old patient presenting with acute motor sensory-axonal neuropathy in the presence of SS. She showed partial response to intravenous immunoglobulin but favourable clinical improvement was seen after initiation of corticosteroid treatment.

Clinical Neuroscience

[The evaluation of paroxysmal events in neonates and infants]

NAGY Eszter, FARKAS Nelli, HOLLÓDY Katalin

[Introduction - Differential diagnosis of neonatal and infantile seizures based only on inspection poses a challenge even for specialists. Aims - To investigate the evaluations of neonatal and infantile paroxysmal events based only on inspection. Research question - Is there any difference in the opinion of neonatologists, paediatric neurologists and neurologists about the assessment of common paroxysmal events in infancy? Patients and methods - Video recordings about paroxysmal movements of 15 neonates or infants (aged 2 days- 5 months) were displayed for 47 paediatric neurologists, 35 neonatologists and nurses working in Neonatal or Perinatal Intensive Care Units and 43 neurologists. They had to decide without knowing the past medical history or EEG results whether events presented were epileptic or nonepileptic in nature. Results - Answers of neonatologists and paediatric neurologists were correct in 67% of cases (824/1230), no significant difference was found between their results. The largest uncertainty was in the judgement of discrete hand movements and very rapid clonus with epileptic origin, they were judged correctly by only one third of participants. The result of neurologists was only slightly, but not significantly different from that of paediatric neurologists. Conclusion - In most cases, the correct diagnosis of neonatal and infantile paroxysmal events requires video-EEG recording. No significant difference was revealed between the evaluation of neonatologists and paediatric neurologists about the differential diagnosis of movements. The ongoing cooperation of paediatric neurologists and neurologists going back to several decades facilitates the shaping of a common perspective.]

Clinical Neuroscience

Anterior cerebral artery infarcts; two years follow-up study

LÜTFÜ Hanoglu, ELMIR Khanmammadov, SEMA Demirci, ÜMMÜHAN Altin, DURSUN Kirbaş, TAHA Hanoglu, BURAK Yulug

Objectives – Anterior cerebral infarct (ACA) infarcts are reported very rare that is due to the compensatory collateral circulation provided by the anterior communicating artery. There are very few studies reporting the long-term follow-up results of ACA infarcts regarding their aetiology, clinical features and prognosis. Most studies reported in the literature vary between several months to one year. Patients and methods – A total of 27 patients with ACA infarcts were registered (14 women and 13 men). The mean age of the patients was 68.5 (age range: 45–89 years). Results – Bilateral ACA infarcts were reported in four patients (14.8%), right ACA infarct in 11 (40%) patients and left ACA infarct in 12 patients (44%). During the initial examination 15 patients (55.5%) were found to have apathy, 13 patients (48%) had incontinence, nine patients (33.3%) had primitive reflexes, 11 patients (40.7%) had aphasia, while six patients (22.2%) were found to suffer from neglect. At the end of one-year follow-up, five patients (22.7%) were reported to have apathy, 6 patients (27.2%) had incontinence, one patient (4.5%) had primitive reflexes, while one patient (4.5%) was found to have permanent aphasia, and no patients was found to suffer from neglect. Conclusion – Here we present our clinical data regarding the aetiology, specific clinical characteristics (including the speech disorders) and prognosis of 27 patients with ACA infarcts during a relatively longer follow-up period (3 months – 30 months) in compared to previous literature. We show that there are differences in the etiological factors of ACA infarcts between the Asian and European communities. Regarding speech disorders which are frequently reported during ACA infarcts, our study results are in agreement with other studies suggesting that this clinical picture is more than a real aphasia and associated with general hypokinesia and reduction in psychomotor activity.

Clinical Neuroscience

A multidisciplinary clinical approach to facioscapulohumeral muscular dystrophy

CAKMAK Öztop Özgür, EREN Ilker, ASLANGER Ayca, GÜNERBÜYÜK Caner, KAYSERILI Hülya, OFLAZER Piraye, SAR Cüneyt, DEMIRHAN Mehmet, ÖZDEMIR Gürsoy Yasemin

Background - Impaired shoulder function is the most disabling problem for daily life of Fascioscapulohumeral muscular dystrophy (FSHD) patients. Scapulothoracic arthrodesis can give a high impact to the functionality of patients. Here we report our experience with scapulothoracic arthrodesis and spinal stenosis surgery in FSHD patients. Patients and methods - 32 FSHD patients were collected between 2015-2016. Demographical and clinical features were documented. All the patients were neurologically examined. The Medical Research Council (MRC) and the FSHD evaluation scale was used to assess muscle involvement1. Scapulothoracic arthrodesis and spinal stenosis surgeries were performed in eligible patients. Results - There were 16 male and 16 female (mean age 34.4 years; range 12-73) patients. 6 shoulders of 4 patients aged between 2132 years underwent scapulothoracic arthrodesis (two bilateral, one left and one right sided). Only one 63 years old female patient with severe hyperlordosis had spinal fusion surgery. All of the patients undergoing these corrective surgeries have better functionality in daily life, as well as superior shoulder elevation. Conclusion - Until the emergence and clinical use of novel therapeutics, surgical interventions are indicated in carefully selected patients with FSHD to improve arm movements, the posture and the quality of life of patients in general. Scapulothorosic arthrodesis is a management with good clinical results and patient satisfaction. In selected cases other corrective orthopedic surgeries like spinal fusion may also be considered.

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Clinical Neuroscience

Cases of inborn errors of metabolism diagnosed in children with autism

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Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

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Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Comparison of direct costs of percutaneous full-endoscopic interlaminar lumbar discectomy and microdiscectomy: Results from Turkey

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Microdiscectomy (MD) is a stan­dard technique for the surgical treatment of lumbar disc herniation (LDH). Uniportal percutaneous full-endoscopic in­terlaminar lumbar discectomy (PELD) is another surgical op­tion that has become popular owing to reports of shorter hos­pitalization and earlier functional recovery. There are very few articles analyzing the total costs of these two techniques. The purpose of this study was to compare total hospital costs among microdiscectomy (MD) and uniportal percutaneous full-endoscopic interlaminar lumbar discectomy (PELD). Forty patients aged between 22-70 years who underwent PELD or MD with different anesthesia techniques were divided into four groups: (i) PELD-local anesthesia (PELD-Local) (n=10), (ii) PELD-general anesthesia (PELD-General) (n=10), (iii) MD-spinal anesthesia (MD-Spinal) (n=10), (iv) MD-general anesthesia (MD-General) (n=10). Health care costs were defined as the sum of direct costs. Data were then analyzed based on anesthetic modality to produce a direct cost evaluation. Direct costs were compared statistically between MD and PELD groups. The sum of total costs was $1,249.50 in the PELD-Local group, $1,741.50 in the PELD-General group, $2,015.60 in the MD-Spinal group, and $2,348.70 in the MD-General group. The sum of total costs was higher in the MD-Spinal and MD-General groups than in the PELD-Local and PELD-General groups. The costs of surgical operation, surgical equipment, anesthesia (anesthetist’s costs), hospital stay, anesthetic drugs and materials, laboratory wor­kup, nur­sing care, and postoperative me­dication diffe­red significantly among the two main groups (PELD-MD) (p<0.01). This study demonstrated that PELD is less costly than MD.