Clinical Neuroscience

The role of the insula in the parieto-frontomedial epileptic network. Clues from successful surgical treatment

BALOGH Attila, AIMEN Anwar, KELEMEN Anna, ERÕSS Loránd, FABÓ Dániel

MAY 30, 2017

Clinical Neuroscience - 2017;70(05-06)


We present a case of MRI negative SMA seizure with the seizure onset zone in the secondary leg area on the superior bank of the Sylvian fissure, localized with multiscale electro-clinical and neuroradiological examinations. The 34-year-old female patient’s intractable epilepsy started at age 14. She had diffuse pain aura in her left leg followed by tonic posturing with fully preserved consciousness suggesting parieto-fronto-medial seizure propagation. Her daily nocturnal SMA seizures became drug-resistant. Multiple 3T MRI images and neuropsychological evaluations were normal. Interictal PET detected a right parietal and insular FDG hypometabolism. The seizure onset zone and the symptomatogenic zone were localized by invasive electrophysiology. The insular deep electrode showed the propagation of ictal activity with an onset in the secondary sensory leg area through the insula to the fronto-medial surface. Eighteen spontaneous seizures, electrical cortical stimulation and cortical mapping confirmed the designated area of the resection, which was later proved macroscopically abnormal during surgery. The histological and immunohistological workup confirmed focal cortical dysplasia (IIb type). Postoperative postprocessing morphometry of the preoperative MRI study confirmed the lesion in the right inferior parietal lobe. The patient remained seizure free after surgery for more than 4 years, and medication free for the last two years. Our results concluded that the insula has a „relay” or „node” function in the parieto-opercular-fronto-medial epileptic network. The insular functional connectivity predisposed frontal propagation of the epileptic activity in the connectome of her epilepsy. The three-way insular structural connectivity has determining function on the seizure propagation.



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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

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


[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. ]

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.

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Clinical Neuroscience

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[A growing body of evidence suggests that sleep plays an essential role in the consolidation of different memory systems, but less is known about the beneficial effect of sleep on relational memory processes and the recognition of emotional facial expressions, however, it is a fundamental cognitive skill in human everyday life. Thus, the study aims to investigate the effect of timing of learning and the role of sleep in relational memory processes. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. 84 young adults (average age: 22.36 (SD: 3.22), 21 male/63 female) participated in our study, divided into two groups: evening group and morning group indicating the time of learning. We used the face-name task to measure relational memory and facial expression recognition. There were two sessions for both groups: the immediate testing phase and the delayed retesting phase, separated by 24 hours. Our results suggest that the timing of learning and sleep plays an important role in the stabilizing process of memory representation to resist against forgetting.]

Clinical Neuroscience

Evaluation of the effectiveness of transforaminal epidural steroid injection in far lateral lumbar disc herniations

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Clinical Neuroscience

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

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Lege Artis Medicinae

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