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Clinical Neuroscience

SEPTEMBER 30, 2020

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Clinical Neuroscience

MARCH 30, 2019

EEG-based connectivity in patients with partial seizures with and without generalization

DÖMÖTÖR Johanna, CLEMENS Béla, EMRI Miklós, PUSKÁS Szilvia, FEKETE István

Objective - to investigate the neurophysiological basis of secondary generalization of partial epileptic seizures. Patients and methods - inter-ictal, resting-state EEG functional connectivity (EEGfC) was evaluated and compared: patients with exclusively simple partial seizures (sp group) were compared to patients with simple partial and secondary generalized seizures (spsg group); patients with exclusively complex partial seizures (cp group) were compared to patients with cp and secondary generalized seizures (cpsg group); the collapsed sp+cp group (spcp) was compared to those who had exclusively secondary generalized seizures (sg group). EEGfC was computed from 21-channel waking EEG. 3 minutes of waking EEG background activity was analyzed by the LORETA Source Correlation (LSC) software. Current source density time series were computed for 23 pre-defined cortical regions (ROI) in each hemisphere, for the 1-25 Hz very narrow bands (1 Hz bandwidth). Thereafter Pearson correlation coefficients were calculated between all pairs of ROI time series in the same hemisphere. Z-scored correlation coefficients were compared at the group level (t-tests and correction for multiple comparisons by local false discovery rate, FDR). Results - Statistically significant (corrected p<0.05) EEGfC differences emerged at specific frequencies (spsg > sg; cpsg > cp), and at many frequencies (sg > spcp). The findings indicated increased coupling between motor cortices and several non-motor areas in patients with partial and sg seizures as compared to patients with partial seizures and no sg seizures. Further findings suggested increased coupling between medial parietal-occipital areas (structural core of the cortex) and lateral hemispheric areas. Conclusion - increased inter-ictal EEGfC is associated with habitual occurrence of secondary generalized seizures.

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.]

Lege Artis Medicinae

OCTOBER 20, 2018

[Juvenile idiopathic arthritis: from diagnosis to treatment]

MOSDÓSI Bernadett

[Juvenile idiopathic arthritis is a heterogeneous group of diseases that is one of the most common chronic diseases of childhood. It begins before 16 years of age, the etiology is unknown, it is persisting for at least 6 weeks and other pathologic conditions could be excluded. Juvenile idiopathic arthritis includes several diseases categories, each of them have distinct methods of presentation, clinical symptoms, pathogenesis and treatment options. Recent advances in the understanding of the immunological pathogenesis of the diseases have led to dramatic improvements in treatment. Although the management is using a multidisciplinary approach, the disease often persist into adulthood and can cause significant long-term morbidity and physical disability. Our article discusses the classification, clinical manifestations, differential diagnosis, treatment and complications of juvenile idiopathic arthritis. ]

Lege Artis Medicinae

SEPTEMBER 20, 2018

[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]


[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion bet­ween experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]

Lege Artis Medicinae

AUGUST 30, 2018

[How to diagnose idiopathic pulmonary fibrosis. Part 2]


[Idiopathic pulmonary fibrosis is a severe irreversible lung disease with a progressive course. The disease onset is hard to discover due to the unspecific signs and symp­toms. It occurs mainly in elderly people. In the past decades its prevalence has increased continuously. Physical examination, restrictive pattern on lung function test with decreased diffusion capacity are characteristic features of the disease. Chest X-ray showing fibrotic pattern also points toward the diagnosis of idiopathic pulmonary fibrosis. Differential diagnosis is based on high resolution komputertomográfy. Diag­nosis of IPF is based on the appearance of usual interstitial pneumonia pattern together with the lack of external risk factors and autoimmune or other diseases also known to cause this pattern seen on chest imaging. If no firm diagnosis can be built lung biopsy is required. Multidis­ciplinary teams from clinician, radiologist and pathologist are set in predefined centres that could provide care with novel antifibrotic drugs. These can slow disease progression and are in the frontline in the treatment of the disease. Further research is required to understand the pathomechanism and foster the discovery of further treatment options. ]

Clinical Neuroscience

MAY 30, 2018

Assessment of mental health of carers according to patient stage of idiopathic Parkinson’s disease

YAZAR Olgun Hülya, YAZAR Tamer, DEMIR Yancar Esra, CANKAYA Soner, ENGINYURT Özgür

Purpose - In this study the aim was to collect data to assess the mental health of carers for patients with diagnosis of idiopathic Parkinson’s disease (IPD) according to disease stage and to examine precautions to reduce the patient and disease load on carers. Method - The study included 144 patients with staging according to modified Hoehn and Yahr criteria and 144 patient relatives who provided care support for patients every day, for some or all of the day, and who were over the age of 18 years and accepted participation in the research. Our prospective and cross-sectional study performed detailed neurological examination of patients, and after completing the ‘Personal Information Form’ with the interviewer every patient, with idiopathic Parkinson’s disease (IPD) according to ‘UK Brain Bank’ diagnostic criteria, had the ‘Unified Parkinson’s Disease Rating Scale (UPDRS)’ and ‘Modified Hoehn and Yahr scale (HYS)’ applied. Carers first completed the ‘Personal Information Form’ and then had the ‘Short Symptom Inventory (SSI)’ applied. Results - As the stage of disease increased, the points for all sub-scales of the Short Symptom Inventory increased. Conclusion - With the parallel increase in disease scores and UPDRS stage scores, the points obtained by carers on the SSI sub-scales increased. This data shows that with progressing disease stage, the load on the carer increases and mental health begins to be disrupted.

Clinical Oncology

FEBRUARY 20, 2019

[Molecular subtypes and the evolution of treatment decisions in metastatic colorectal cancer]

RODRIGO Dienstmann, RAMON Salazar, JOSEP Tabernero

[Colorectal cancer (CRC) has clinically-relevant molecular heterogeneity at multiple levels: genomics, epigenomics, transcriptomics and microenvironment features. Genomic events acquired during carcinogenesis remain drivers of cancer progression in the metastatic setting. For example, KRAS and NRAS mutations defi ne a population refractory to EGFR monoclonal antibodies, BRAFV600E mutations associate with poor outcome under standard therapies and response to targeted inhibitors in combinations, while HER2 amplifi cations confer unique sensitivity to double HER2 blockade. Multiple rare gene alterations driving resistance to EGFR monoclonal antibodies have been described with signifi cant overlap in primary and acquired mechanisms, in line with a clonal selection process. In this context, sequential analysis of circulating tumor DNA has the potential to guide drug development in a treatment refractory setting. Rare kinase fusion events and complex alterations in genes involved in DNA damage repair have been described, with emerging evidence for targetability. On the other hand, transcriptomic subtypes and pathway activation signatures have also shown prognostic and potential predictive value in metastatic CRC. These markers refl ect stromal and immune microenvironment interactions with cancer cells. For example, the microsatellite instable (MSI) or POLE ultramutant CRC population is particularly sensitive to immune checkpoint inhibitors, while tumors with a mesenchymal phenotype are characterized by activation of immunosuppressive molecules that mandate stratifi ed development of novel immunotherapy combinations. In this manuscript we review the expanding landscape of targetable oncogenic alterations and signatures in metastatic CRC and discuss the clinical implementation of novel molecular diagnostic tests.]

Clinical Neuroscience

MARCH 30, 2019

The yield of electroencephalography in syncope

NALBANTOGLU Mecbure, TAN Ozturk Ozlem

Introduction - Syncope is defined as a brief transient loss of consciousness due to cerebral hypoperfusion. Although the diagnosis of syncope is based on a thorough history and examination, electroencaphalography (EEG) is also an important investigational tool in the differential diagnosis in this group of patients. In this study we aimed to identify the diagnostic value of EEG in patients with syncope. Methods - We retrospectively examined EEG recordings of 288 patients with the diagnosis of syncope referred to the Cankiri State Hospital EEG laboratory, from January 2014 to January 2016. The EEG findings were classified into 6 groups as normal, epileptiform discharges (spike and sharp waves), generalized background slowing, focal slowing, hemispherical asymmetries, and low amplitude EEG tracing. The EEGs were separated according to gender and age. Results - Total of 288 patients were included in this study, 148 were females (51.4%) and 140 (48.6%) were males. Among all the EEG reports, 203 (70.5%) were normal, 8 of them (2.8%) showed generalized background slowing and 7 (2.4%) demonstrated focal slow waves. Epileptiform discharges occured among 13 patients (4.5%). Hemispherical asymmetries were detected in 10 patients (3.5%) and low amplitude EEG tracing in 47 patients (16.3%). There was no significant difference between age groups in EEG findings (p=0.3). Also no significant difference was detected in EEG results by gender (p=0.2). Discussion - Although the diagnosis of syncope, epilepsy and non-epileptic seizures is clinical diagnosis, EEG still remains additional method

Clinical Neuroscience

NOVEMBER 30, 2018

[Neuropsychological rehabilitation following acquired brain injury]

TAMÁS Viktória, KOVÁCS Noémi, TASNÁDI Emese

[Neuropsychological rehabilitation or rehabilitation neuropsychology is a field within applied neuropsychology. It originally diverges from applied clinical and functional neuropsychology, although it could not be entirely differed from them. The unique nature of this area over the complexity is given by its process-controlled and system-approach aspects. In Hungary the number of neurorehabilitation centres and departments requiring neurocognitive rehabilitation has been continually increasing. Nevertheless, the number is still low; accordingly in our country this field is relatively young and isn’t well known. Authors of this review would like to draw attention to the importance of rehabilitation of patients with acquired brain injury and improvement of their quality of life with the theoretical and practical knowledge, as well as the necessity of future alterations and challenges emphasizing the need of a significant change of this narrow domain. ]