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Clinical Neuroscience

MAY 30, 2020

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.

Lege Artis Medicinae

APRIL 18, 2020

[The relationship of traumatic experiences and eating disorders – therapeutical options]


[In the etiology of eating disorders (espe­cially bulimia nervosa and binge eating) traumatic experiences (sexual, physical, emo­tional abuse, neglect) play an important role. Traumatization can have a serious impact, which is influenced by the parameters of the traumatization, risk and protective factors, and the resiliency of the traumatized patient. The consequences may lead to the development of specific psychiatric and somatic disorders, and may cause lifetime revictimization. The exploration of data related to the traumatization is essential in eating disorders as well. If traumatic expericences can be detected in the back­ground of eating disorders, the targeted therapy of eating disorders while applying its specific elements should also follow the guide­lines of the general trauma-therapy. Providing safety in therapeutical relation­ship is fundamental. The therapeutic options are extensive. Along with psychodynamically oriented therapies, the newer methods based on cognitive-behavioral therapy (e.g., dialectic behavior therapy, integrative cognitive analytic therapy) are also proposed. Hyp­no­therapy can also be useful. ]

Clinical Neuroscience

JANUARY 30, 2016

[The propagation and semiology of focal epileptic seizures. case presentations to the insula. theoretical considerations]

BALOGH Attila, BALOGH Attila ifj.

[Study objectives – The developing of diagnostical examinations in epileptology provides new challenges in seizure semiology. On the analysis of seizures it is important to examine the mechanisms of their propagation. The brain connectivity (based on the neuroimaging), the shadowing of the movement of excessive neuronal activity (based on computerized EEG and MEG methods), the cognition of the physiological and pathological brain networks are the foot- stone of the epileptic seizure propagation. The investigators prove, by means of case demonstrations of the role of the network nodes and the role of the epileptic hubs in the seizure symptomatology. Methods – The preoperative, intra and postoperative data are analised of three insular and one parietal epileptic patients in point of view of their seizure symptomes. Complex neuroimaging, noninvasive and invasive electrophysiology, intensive long-term video-EEG monitoring, computerized EEG analysis, fuctional mapping, intraoperative corticography were used. The etiology were confirmed with hystology. Results – It is observed that on seizure semiology our patients plays the insula a double role. In some cases, it is the focus of insular seizures with their symptoms difficult to identify. However, in the majority of cases and as a consequence of its rich neural connections, the insula has a peculiar property in the evolution of the symptomatogenic features of seizures. This observations are developing new relationships between the mechanism of seizure propagation and its semiological consequences. Conclusions – On epileptological point of view there are brain structures which has peculiar role in the „designe” of propagation of the epileptic excitement. The numerous new methods in neuroimaging and neurophysiology allowed the connectomical examination of the epileptic networks. The role of the epileptic diathesis is approachable with the metholdology of the brain connectivity. Theoretically the node of the epileptic network consist of the potential pathes where the localised excessive excitement can propagete. The route where the actual seizure can go adhead is determined by the actual edpileptic propensity of the above mentioned potential pathes.]

Clinical Neuroscience

NOVEMBER 20, 2015

[A rare complication of a rare disease; stroke due to relapsing polychondritis]


[Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of cartilaginous structures. Its diagnosis is based primarily on clinical features such as laboratory parameters, biopsy. Neurological complications occur in 3% of the cases and are classified as an important cause of death. The cranial nerve disorders are most common but hemiplegia, ataxia, myelitis, polyneuritis, seizures, confusion, hallucination and headache can also happen. The aetiology of central nervous system involvement is still unknown. Moreover stroke has rarely reported in these patients. The diagnosis of stroke is challenging because of its rarity among these patients. Perhaps vasculitis is the common underlying mechanism. Also meningitis and encephalitis can occur during the course of RP. A 44 year-old woman was admitted with uncontemplated left hemiparesis, redness, swelling, and tenderness of the metacarpophalangeal and interphalangeal joints of the right hand and the cartilaginous portion. White blood cell count, C-reactive protein and the erythrocyte sedimentation rate were elevated. Vasculitis biomarkers were normal in our patient. Carotid and vertebral artery doppler ultrasonography, cranial and cervical MR Angiography were normal. Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed. Hemiparesis was her first neurological manifestation that led her to doctors for diagnosis. Our patient fulfilled the criteria of RP so no biopsy was needed. She was treated with oral prednisolone (80 mg/day) and aspirin (300 mg/day) and now she is on 10 mg prednisolone and 150 mg azathioprine. Two months later her physical and neurological symptoms returned to normal.]

Clinical Neuroscience

MARCH 30, 2016

[Individual evaluation of loreta abnormalities in idiopathic generalized epilepsy]

CLEMENS Béla, PUSKÁS Szilvia, BESENYEI Mónika, KONKÁDOR István, HOLLÓDY Katalin, FOGARASI András, BENSE Katalin, EMRI Miklós, OPPOSITS Gábor, KOVÁCS Noémi Zsuzsanna, FEKETE István

[Background – Contemporary neuroimaging methods disclosed structural and functional cerebral abnormalities in idiopathic generalized epilepsies (IGEs). However, individual electrical (EEG) abnormalities have not been evaluated yet in IGE patients. IGE patients were investigated in the drug-free condition and after 3-6 month of antiepileptic treatment. To estimate the reproducibility of qEEG variables a retrospective recruited cohort of IGE patients was investigated. 19- channel resting state EEG activity was recorded. For each patient a total of 2 minutes EEG activity was analyzed by LORETA (Low Resolution Electromagnetic Tomography). Raw LORETA values were Z-transformed and projected to a MRI template. Z-values outside within the [+] 1. In drug-free condition, 41-50% of IGE patients showed abnormal LORETA values. 2. Abnormal LORETA findings showed great inter-individual variability. 3. Most abnormal LORETA-findings were symmetrical. 4. Most maximum Z-values were localized to frontal or temporal cortex. 5. Succesfull treatment was mostly coupled with disappearence of LORETA-abnormality, persistent seizures were accompanied by persistent LORETA abnormality. 1. LORETA abnormalities detected in the untreated condition reflect seizure-generating property of the cortex in IGE patients. 2. Maximum LORETA-Z abnormalities were topographically congruent with structural abnormalities reported by other research groups. 3. LORETA might help to investigate drug effects at the whole-brain level.]

Clinical Neuroscience

JANUARY 30, 2016

Long term follow-up of lesional and non-lesional patients with electrical status epilepticus in slow wave sleep


Objectives – A retrospective study has been done at the Bethesda Children’s Hospital Epilepsy Center with those patients whose EEG records fulfilled in one or more records the criteria of electrical status epilepticus in slow wave sleep (ESES) pattern, occupying at least 75% of NREM sleep with bilateral discharges, and had detailed disease history and long term follow-up data, between 2000 and 2012. Patients and methods – Thirty-three patients (mean 11.1±4.2 years of age) were studied by 171 sleep EEG records. Sleep was recorded after sleep deprivation or during spontaneous sleep at least for one hour length of NREM. From the 492 EEGs, 171 sleep records were performed (average five/patient). Average follow-up time was 7.5 years. Eighty-two ESES records have been analyzed in 15 non-lesional and 18 lesional (11 with dysgenetic and seven with perinatal - asphyxic or vascular origin) patients. Variability of seizure types, seizure frequency and frequency of status epilepticus was higher in the lesional group. Impairment of the cognitive functions was moderate and partial in the non-lesional, while severely damaged in the lesional group. Results – EEG records of 29 patients showed unihemispherial spike fields with a perpendicular axis (in anterior, medial and posterior variants) to the Sylvian fissure, regardless their lesional or non-lesional origin. Only three (1one non-lesional and two lesional) patients had bilateral synchronous spike-wave discharges with bilateral symmetric frontocentral spike fields. The individual discharges of the sleep EEG pattern were very similar to the awake interictal records except their extension in time and field, their increased number, amplitude, and continuity of them and furthermore in the increased trans-hemispheral propagation and their synchronity. Conclusions – Assumed circuits involved in the pathomechanism of discharges during NREM sleep in ESES are discussed based on our findings.

Clinical Neuroscience

MARCH 30, 2020

[Intracranial EEG monitoring methods]

TÓTH Márton, JANSZKY József

[Resective surgery is considered to be the best option towards achieving seizure-free state in drug-resistant epilepsy. Intracranial EEG (iEEG) is necessary if the seizure-onset zone is localized near to an eloquent cortical area, or if the results of presurgical examinations are discordant, or if an extratemporal epilepsy patient is MRI-negative. Nowadays, 3 kinds of electrodes are used: (1) foramen ovale (FO) electrodes; (2) subdural strip or grid electrodes (SDG); (3) deep electrodes (stereo-electroencephalographia, SEEG). The usage of FO electrode is limited to bitemporal cases. SDG and SEEG have a distinct philosophical approach, different advantages and disadvantages. SDG is appropriate for localizing seizure-onset zones on hemispherial or interhemispherial surfaces; it is preferable if the seizure-onset zone is near to an eloquent cortical area. SEEG is excellent in exploration of deeper cortical structures (depths of cortical sulci, amygdala, hippocampus), although a very precise planning is required because of the low spatial sampling. The chance for seizure-freedom is relatively high performing both methods (SDG: 55%, SEEG: 64%), beside a tolerable rate of complications.]

Clinical Neuroscience

MARCH 30, 2020

[New applications of conventional EEG analysis ]


[Neurophysiological research suggests that the so-called “standard” EEG analysis has been confronted with new diagnostic challenges. The findings mainly concern the occurrence, the neurophysiological and clinical significance of epileptiform EEG discharges in several neurological and psychiatric disorders. In addition to well-known interictal and ictal discharges, a growing number of recently recognized epileptiform phenomena have been described. The first reports suggested that they might be relevant for the comprehensive description of epileptic dysfunction and might contribute to diagnosis and treatment as well. However, considerable improvement of present-day “standard” EEG technique is necessary to give an appropriate answer to most challenges. Reliable registration and quantitative assessment of well-known epileptiform transients require extended electrode coverage of the head (high-density EEG) and long-term recordings including waking and sleep states to estimate frequency and dyna­mics of targeted activities. Computer-based automatic event detection is preferable to spare time and cost of the evaluation. The authors review recent progress concerning epidemiology, neurophysiology and clinical impact of well-known epileptiform transients and candidate epileptiform activities in neurological and psychiatric conditions. However, recent results need confirmation in large patient populations; therefore, research should not be restricted to a few central laboratories.]

Lege Artis Medicinae

MARCH 10, 2020

[Summary data of Hungary's comprehensive health screening program (MAESZ) 2010-2019]

BARNA István, KÉKES Ede, HALMY Eszter, BALOGH Zoltán, KUBÁNYI Jolán, SZŐTS Gábor, NÉMETH János, PÉCSVÁRADY Zsolt, MAJOROS Attila, DAIKI Tenno, ERDEI Ottilia, DANKOVICS Gergely

[The comprehensive screening program of Hun­gary (MAESZ) 2010-2020-2030 is a unique initiative in Hungary and worldwide too. This largest humanitarian program provides by the latest technology free scree­ning tests for all residents in Hungary. The program developed by 76 pro­fessional organizations offers 38 scree­ning tests to every participants free of charge, in a special designed screening truck. Screening program performed by MAESZ includes cardiovascular, ophthalmologic, dermatologic, gynecologic, and neurologic investigations, lab tests, audiometry, blood pressure and arterial stiffness measurements, and venous Doppler ultrasound examinations. More­over, screening tests for lactose intolerance, colon malignancy, inflammatory bowel disease, reflux disease, urine incontinency, prostatic cancer and physical activity level were evaluated. Starting 2020, a dental screening station will be added to the mobile unit for early detection of oral cancers. Beyond screening tests, special attention is paid to assess health threatening risk factors, such as smoking, alcohol con­sumption, physical inactivity, un­healthy nutrition, and obesity. The program demonstrates the key elements of first aid from reanimation to bandage of burns in cooperation with professional and civil organisations. Furthermore, during the waiting time, participants get lifestyle recommendations and a health booklet with a bar code enabling the immediate computer analysis of test outcomes. Since the 2018/2019 school year the official prevention program for children entitled “Travel around the Empire of Health” was started. During its 10 years, the MAESZ performed 7 million free of charge screening tests on 1,886 scenes, enrolled 560,000 participants, invested 16,000 hours for prevention, handed out 1,200,000 health booklets and 391,000 prevention info packages to thousands of fami­lies. More than 20,000 health professionals (GPs, nurses, dietetics, health development agents, public health government officials, Accident Prevention Committee of National Police Headquarters, General Directorate of Social Affairs and Child Protection and non-governmental organizations) have been participated. The program designed to improve social health aims to help more and more Hun­garian citizens to be informed about their health status and to reminds them of the importance of prevention. ]

Lege Artis Medicinae

JANUARY 20, 2020

[The Veszprém model, alias a different approach of psychiatric care]


[This study describes an integration of a county-level psychiatric care unit with two elements of primary social care, namely the community psychiatric care and the daycare for psychiatric patients via mediation of a specific foundation. Adequate trainings make the connection and dual employment possible in the presented system. On the health care side development is characterized by extended psychiatric care with effective psychosocial interventions. Adding specialized group psychotherapies and sociotherapies to the activity of the social care also brought a quality change, extension of which provided self-help and the consumers' applicability as therapy leaders in sociotherapy group. Based on our ten years of practice we may declare that recovery-based psychiatric rehabilitation can be realized more effectively in this community-based biopsychosocial model of care. ]