Clinical Neuroscience

[Prevention of invasive meningococcal infection, recognition and first treatment of the disease in primary care]

KOVÁCS Ákos, KULCSÁR Andrea, KALABAY László, TORZSA Péter

MAY 30, 2017

Clinical Neuroscience - 2017;70(05-06)

DOI: https://doi.org/10.18071/isz.70.0151

[Neisseria meningitidis, the meningococcus, is a Gram-negative diplococcal bacterium that is only found naturally in humans. The meningococcus is part of the normal microbiota of the human nasopharynx and is commonly carried in healthy individuals. In some cases systemic invasion occurs, which can lead to meningitis and/or septicemia. Invasive disease caused by Neisseria meningitidis is potentially devastating, with a high case fatality rate and high rates of significant sequelae among survivors after septicaemia or meningitis. Between 2006-2015 every year between 34 and 70 were the numbers of the registered invasive disease because of Neisseria meningitis, the morbidity rate was 0.2-0.7⁰⁄₀₀₀₀. Half of the diseases (50.7%) were caused by B serotype N. meningitidis, 23.2% were C serotype. In this article the authors summarise what you must do and must not do as primary care physician when suddenly meeting a young patients suspected of having meningococcus infection. ]

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Clinical Neuroscience

[Experience with natalizumab-treatment at Semmelweis University]

GOMBOS Barbara, ILJICSOV Anna, BARSI Péter, HEGEDÛS Katalin, SIMÓ Magdolna

[Multiple sclerosis is an autoimmune demyelinating disorder of the central nervous system. During the last two decades, numerous disease modifying drugs have been introduced for the treatment of the relapsing-remitting form of the disease. Since 2010, natalizumab (NTZ) treatment has been used as a second-line therapy for patients with breakthrough disease. In comparison to conventional immunomodulant drugs, NTZ has a more specific effect in that it prevents the entry of immune cells into the central nervous system without interfering with systemic immune response. The efficacy and the safety of NTZ have been confirmed by several studies. The most severe side-effect of NTZ is progressive multifocal leukoencephalopathy, which has been associated with an increased incidence in patients with anti-JCV antibody positivity, and in those who have been undergoing NTZ treatment for over two years and who have received prior immunosuppressive therapy. In the present study, our experience with natalizumab treatment of 37 patients at the Department of Neurology of Semmelweis University during the last 6 years is presented. We have observed a significant decrease of disease activity in our patients; in many cases the disease has become inactive both clinically (36/37) and radiologically (34/37). The patients’ quality of life has improved significantly during the treatment. In accordance with the literature, we confirm that NTZ is a highly effective treatment in a carefully selected patient group, and can be administered without significant inconvenience to the patient. ]

Clinical Neuroscience

Case report of a woman with anti amphiphysin positive stiff person syndrome

MANHALTER Nóra, GYÖRFI Orsolya, BOROS Erzsébet, BOKOR Magdolna, FAZEKAS Ferenc, DÉNES Zoltán, FABÓ Dániel, KAMONDI Anita, ERÕSS Loránd

Stiff person syndrome is a rare neuroimmunological disease, characterized by severe, involuntary stiffness with superimposed painful muscle spasms, which are worsened by external stimuli. The classical form is associated with high levels of antibodies against glutamic acid decarboxylase. One of the variant forms is associated with antibodies against amphiphysin. This entity is a paraneoplastic syndrome, caused primarily by breast cancer, secondarily by lung cancer. Symptomatic therapy of anti amphiphysin positive stiff person syndrome includes treatment with benzodiazepines and baclofen (including intrathecal baclofen therapy). The effect of immunological therapies is controversial. Treatment of the underlying cancer may be very effective. In this report, we describe a 68 year old female presenting with an unusally rapidly developing anti amphiphysin positive stiff person syndrome, which was associated with breast cancer. Her painful spasms abolished after intrathecal baclofen treatment was initiated. Her condition improved spontaneously and significantly after cancer treatment, which enabled to start her complex rehabilitation and the simultaneous dose reduction of the intrathecal baclofen. The bedridden patient improved to using a rollator walker and the baclofen pump could be removed 18 monthes after breast surgery. This highlights the importance of cancer screening and treatment in anti amphiphysin positive stiff person syndrome cases.

Clinical Neuroscience

[EEG-based cerebral networks in 14 neurological disorders]

DÖMÖTÖR Johanna, CLEMENS Béla, CSÉPÁNY Tünde, EMRI Miklós, FOGARASI András, HOLLÓDY Katalin, PUSKÁS Szilvia, FEKETE Klára, KOVÁCS Attila, FEKETE István

[Background - Brain networks have not been systematically investigated yet in most neurological disorders. Purpose - To investigate EEG functional connectivity (EEGfC) networks in 14 neurological disorders. Patients - Potentially eligible patients were collected from clinical and EEG databases. All the available clinical data and EEG records were critically revised. All the patients who suffered of a single neurological disorder (out of the 14) and had a good quality EEG recording entered the study. Confoundig factors as comorbidity and CNS-active drug effects were eliminated as far as possible. EEG analysis - Three minutes of resting-state, waking EEG activity were selected for analysis. Current source density (CSD) values were computed for 2394 cortical voxels by Low Resolution Electromagnetic Tomography (LORETA). Thereafter, Pearson correlation coefficients were computed between all pairs of 23 cortical regions of interest (ROI) in each hemisphere (LORETA Source Correlation, LSC software). Computation was carried out for conventional EEG broad bands and very narrow bands (1 Hz bandwidth) between 1 and 25 Hz as well. Correlation coefficients of each group were statistically compared to our normative EEG (LSC) database by two-talied t-tests. Bonferroni-corrected p<0.05 values were accepted as statistically significant, and were graphically displayed as topographical networks. Results and conclusion - Group-specific networks were demonstrated. However, non-specific networks, charasteristic for most groups, were detected as well. Common finding were: decreased connectivity in the alpha band and increased connectivity in the delta, theta bands and upper-beta band. Decreased alpha-band connectivity presumably reflected primary lesional effects and on the other hand, non-specific vulnerability of “rich club connections”. Increased connectivity in the slow bands presumably indicated adaptive-compensatory activity of brain homeostasis. ]

Clinical Neuroscience

Independent validation of the Quality of Life in Essential Tremor Questionnaire (QUEST)

KOVÁCS Márton, MAKKOS Attila, JANSZKY József, KOVÁCS Norbert

Quality of Life in Essential Tremor Questionnaire (QUEST) was specially developed for essential tremor population to measure the health-related quality of life. Besides the development of the Hungarian version, we performed an independent testing of the scale adding further information on its clinimetric properties. In this study 133 ET patients treated at University of Pécs, Hungary, were enrolled. Besides QUEST, we assessed Patient’s Global Impression-Severity (PGI-S) and Fahn-Tolosa-Marin Tremor Rating Scales. After the independent validation in accordance to the Classic Theory of Tests, we evaluated cut-off values for detecting clinically meaningful ET-related disabilities based on receiver operating characteristics analysis. Cronbach’s a was 0.897. QUEST demonstrated high convergent validity with PGI and divergent validity with disease-duration, positive family history, need for deep brain stimulation surgery, and the presence of depression and anxiety. Presence of moderate ET-related disabilities was identified by scores > 11.25 points on QUEST-SI (sensitivity: 77.4%, specificity: 83.3%); whereas scores > 20.35 points indicated severe ET-related disabilities (sensitivity: 83.3%, specificity: 59.1%). We demonstrated that the fundamental clinimetric properties of the QUEST are satisfactory.

Clinical Neuroscience

[Non-contrast brain ct based systemic thrombolysis of two wake up ischemic stroke patients in rural settings]

POZSEGOVITS Krisztián, RENCZ László, CSÚSZ Lajos, SZABÓ Géza

[Background and presentation - Conventionally the acute ischemic stroke patients who wake up with symptomes (WUS - wake up stroke) cannot benefit from systemic thrombolysis due to the uncertainty of the exact onset time of the cerebrovascular event. Perfusion brain imaging could be used as patient selection tool but the method is not available in many settings. Simple non-contrast CT scan is easily accessible and reliable as it shows the different stages of the evolving ischemia with high accuracy. Early brain CT scan results of WUS patients have the same characteristics as the ones who are surely within therapeutic window. The intravenous thrombolysis with recombinant tissue plasminogen activator (rTPA) of WUS patients seems to be similarly successful as the ones with known onset time, the treatment does not come with excess complications, higher rate of symptomatic intracranial hemorrhage was not found in previous reports. Purpose - In this report we present two systemic thrombolysis cases of acute ischemic stroke patients who woke up with stroke symptoms. Methods - In 2014 and 2015 we performed systemic thrombolysis for one wake up stroke patients, respectively. Both patients had large vessel occlusion. Indication was based on favourable non-contrast brain CT scan results. Results - Treatment of these two patients with rTPA proved to be safe, no hemorrhage occurred after treatment. Conclusion - We presented two acute ischemic stroke patients with symptomes at early wake up who were treated intravenously with recombinant tissue plasminogen activator based on non-contrast CT alone without complications and some moderate improvement at 90 days in the settings of a rural town hospital in a middle income country.]

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Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

[The role of anaerobic bacteria in brain abscesses: a literature review]

URBÁN Edit, GAJDÁCS Márió

[Brain abscesses are potentially serious, life-threatening diseases that pose a complex diagnostic challenge not only to neurosurgeons but also to clinical microbiologists, neurologists, psychiatrists, infectologists. The etiology of brain abscess is usually polymicrobial, most commonly involving a variety of aerobic and obligate anaerobic bacteria. Epidemiological studies on the anaerobic etiology of brain abscesses are common between the time period of 1960s and 1980s, but today there are very few new publications on the subject. The role of anaerobic bacteria in this disease was presumably underdiagnosed for a very long time, as many laboratories did not have the adequate laboratory capabilities for their cultivation and identification. The purpose of this review is to summarize the available literature on the etiology of obligate anaerobic bacteria in brain abscesses, including their prevalence and current therapeutic recommendations.]

Clinical Neuroscience

[Pneumococcal meningitis in children - 9 1/2-year-experience at Szent László hospital, Budapest, Hungary ]

IVÁDY Balázs, LIPTAI Zoltán, ÚJHELYI Enikő, BALÁZS György

[Background and objective - No recent publications are available about pneumococcal meningitis in Hungarian children. The aim of this study was to collect data of epidemiological, clinical and prognostic features of pneumococcal meningitis in children treated at Szent László Hospital, Budapest, Hungary. Methods - We conducted a retrospective review of medical charts and follow-up records of patients aged 1 to 18 years admitted to our Pediatric and Pediatric Intensive Care Units due to pneumococcal meningitis between 1st Jan 1998 and 30th Jun 2007. Results - 31 children with 34 cases of pneumococcal meningitis were admitted to our hospital in the study period. Two children developed recurrent illness. The mean age was 6 years, 26% were under 1 year of age. The mean duration of hospital stay was 21 days, 97% required intensive care. Frequent clinical symptoms were fever (100%), nuchal rigidity and vomiting (78%), altered mental status (71%), Kernig's and Brudzinski's signs (58%) and seizures (41%). Otitis media, sinusitis, mastoiditis were present in 44%, 58%, 41%, respectively. Subdural effusion, parenchymal cerebral lesion and sinus thrombosis were documented in 5, 3 and 2 cases, respectively. One third of the patients recieved ceftriaxon, two thirds were administered ceftriaxon and vancomycin. Adjunctive therapy with dexamethason was given to 91% of the children. 70% of patients required mechanical ventillation. 9 patients (25%) required endoscopic sinus surgery. In 13 cases (38%) mastoidectomy, in 5 children (15%) neurosurgery was performed. The case fatality rate was 23.5%. 8 (23.5%) patients had mild or moderate, 1 child (3%) developed severe neurological sequelae. Conclusion - Pneumococcal meningitis in children remains a source of substantial morbidity and mortality in childhood. The long hospital stay, the frequent need for intensive care and severe neurologic sequelae emphasize the importance of early diagnosis, early treatment and prevention with pneumococcal conjugate vaccines.]

Lege Artis Medicinae

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DARVAI László, RIHMER Zoltán, BALCZÁR Lajos, CSERHÁTI Zoltán, TÚRI Gergő, BÉLTECZKI Zsuzsa, KALABAY László, TORZSA Péter

[There is a complex and multi-directional relationship between alcoholism, smoking, depression, anxiety disorders, cardiovascular disorders and type 2 diabetes. They are risk factors not only for each other but - because of their relationship with depression - also for suicide. The effect of successful therapy of depression and anxiety plays a role not only in reducing suicide but also in reduction of alcoholism, cardiovascular morbidity and mortality, and smoking. Therefore, the effect of the appropriate specific treatments appears (even to a lesser extent) also in the field of another medical discipline. On the other hand, the reduction of smoking may be - in addition to decreasing mortality and morbidity of chronic obstructive pulmonary disease, lung cancer, ischemic heart disease, and so on - a causal factor in decreasing suicidal morbidity and mortality, as well as in decreasing morbidity of depression. The purpose of this article is to raise awareness of the relevant professions and healthcare financiers and decision-makers to these hidden benefits (appearing in other fields of expertise). The presented data justify not only the better funding for Mental Health Services but also the extension of the competence of primary care physicians to the mild and moderate (not psychotic and not suicidal) depression and anxiety disorders. ]

LAM Extra for General Practicioners

[Changes in infectology over the past two decades]

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[Infectious diseases and various infections are the major causes of morbidity and mortality in developing as well as in industrialised countries. Despite the advances in the past decades in our understanding of microbes, efficient treatment of diseases and preventive approaches, more than 13 million people die every year due to infectious diseases. In the past two decades, more and more new pathogens and infections diseases have been emerging and old diseases that were almost forgotten have re-emerged. There are many new diseases for which we do not have or have hardly any efficient antimicrobial drugs and no efficient vaccines. Despite an increasing frequency of multi- and panresistant microbes, the development of new antibiotics to be used against these infections is unlikely to occur in the near future. The big pharmaceutical companies have stopped the research of antibiotics. In this situation, the only option we have is to use antibiotics rationally and to take prevention and control of infections seriously, both in the outpatient system and in hospitals. Preserving the effectiveness of currently used antibiotics is in everyone’s interest and is everyone’s responsibility]