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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, 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

MARCH 10, 2020

[Diagnosis and treatment of the overactive bladder]


[The Overactive Bladder Syndrome (OAB) is a symptomatic diagnosis featured mainly by urgency of urine discharging. This condition can be triggered by a number of etiological factors, most of which are idiopathic in origin. Regardless of gender and age, the prevalence is ca. 16% and has a serious impact on the quality of life of the patients. In­ves­tigations are mostly performed through usual baseline examinations; second-line invasive examinations are rarely required. The broad spectrum of treatment options ran­ges from lifestyle changes and elimina­tion of triggering factors, through be­ha­vio­ural therapy and medication to the mi­ni­mally invasive (botulinum toxin, neuro­mo­dulation, percutaneous stimulation of the tibial nerve) - and rarely - invasive thera­pies. ]

Clinical Neuroscience

NOVEMBER 30, 2019

Vestibular evoked myogenic potential responses in Parkinson’s disease


Background - Our objectives were to determine the differences in the vestibular evoked myogenic potential (VEMP) responses in patients diagnosed with early staged idiopathic Parkinson’s disease (PD) compared to the normal population and evaluate the vestibular system disorder causing balance-posture disorders. Second aim of this study was to investigate caloric test responses particularly in early staged PD compared to normal popu­lation. Material and methods - Thirty patients (14 females and 16 males; mean age, 60.6 ± 13.1 years) diagnosed with idiopathic PD and 28 healthy subjects (20 males and 8 females; mean age, 59.1 ± 6.4 years) were included. The patient and control groups were subdivided according to their age, gender and the patient group was subdivided according to onset time of the Parkinson symptoms, Hoehn-Yahr staging. The subgroups were compared for VEMP and caloric test responses. Results - There were no significant differences between the study and control groups for right and left VEMP measurements. Patients over 60 years and under 60 years did not show significant differences in terms of right and left mean VEMP measurements. However, P1 amplitude was significantly lower in patients over 60 years old (P = .004). Gender, disease duration, BERG balance scale and Hoehn-Yahr stage had no effect on the VEMP amplitudes. There was no significant correlation with the side of Parkinsonian symptoms to the side of canal paresis (P = .566) and the side on which no VEMP response was obtained in caloric test. Conclusion - VEMP responses were not different between PD and healthy subjects. VEMP P1 amplitude was decreased with age in PD group. Canal paresis and symptoms side were not statistically correlated in caloric test.

Clinical Neuroscience

SEPTEMBER 30, 2019

[Newer studies on the strong link between sleep and epilepsy: Epilepsy as an epileptic transformation of sleep plastic functions]


[Aims - Overview of the new data about the strong link of sleep and epilepsy and conjoining cognitive impairment. Methods - Search for relevant references and summary of our own research activity on the topic. Results - Strong interrealtionship exists between epilepsy and plastic brain functions (memory processing and synaptic homeostasis) and the working modes of NREM sleep. In the most frequent childhood and adult epilepsy networks responsible for plastic functions can be derailed to an epileptic level of excitability, and suffer a transitory or permanent epileptic transformation. Exampling on the three big epilepsies: absence epilepsy; medial temporal lobe epilepsy; and childhood idiopathic focal age dependent epilepsy spectrum we demonstrate the most important features of this epileptic transformation. The association of cognitive impairment to certain sleep dependent epilepsies gains explanation by the epilepsy caused interference with slow wave decline (ICFE) and memory consolidation (MTLE) during NREM sleep. This paper serves also to introduce the concept of sleep dependent system epilepsies. Conclusions - We provide evidences about shared mechanisms among sleep related epilepsies being the derailment of sleep plastic funcions toward exaggerated excitability determined by the inherent possibilities of the signal transduction properties. ]

Lege Artis Medicinae

JULY 20, 2019

[Severe polymyositis associated with multiplex pulmonary abscesses]

SZABÓ Katalin, VINCZE Anett, NAGY-VINCZE Melinda, DANKÓ Katalin, GRIGER Zoltán

[INTRODUCTION – Idiopathic inflammatory myopathies are heterogeneous autoimmune diseases characterized by immune mediated inflammation of the skeletal muscles. CASE REPORT – A case of a 62-year-old male patient with severe proximal muscle weakness, elevated creatine kinase and swallowing difficulity is presented. Electromyography showed myogenic pattern, thus probable polymyositis was diagnosed. Radiological examination has confirmed bilateral multiplex lung lesions, which were caused by the possibility of tumor, tuberculosis, vasculitis and abscess as well. The condition of the patient deteriorated, nasogastric feeding, high dose steroid treatment was initiated, which reduced the patient's creatinine kinase values, but muscle strength was not changed. Based on the results of various investigations, the condition of the patient was finally confirmed by the development of myositis, resulting dysphagia, chronic aspiration, and multiplex lung abscess. Antibiotic therapy, steroid treatment was continued and finally intravenous immunoglobulin treatment was administered. The condition of the patient gradually improved, the swallowing dysfunction disappeared, and the lung abscesses were resolved. As a result of physiotherapy and rehabilitation treatment, the patient could walk again. CONCLUSIONS – Nasogastric feeding is recommended to prevent aspiration in the case of myositis-associated dysphagia. In case of steroid refractory therapy, the use of intravenous immunoglobulin may be effective. ]

Clinical Neuroscience

MAY 30, 2019

Somatosensory amplification absorption contribute to electrosensitivity


Background - Two trait-like characteristics, somatosensory amplification and absorption, have been associated with symptom reports and idiopathic environmental intolerances in past research. Purpose - As the two constructs are not connected with each other, their independent contribution to symptom reports and electromagnetic hypersensitivity, as well as their interaction can be expected. Methods - On-line questionnaire. Patients - 506 college students completed an on-line questionnaire assessing absorption, somatosensory amplification, negative affect, somatic symptoms, and electromagnetic hypersensitivity. Results - Somatosensory amplification (β = 0.170, p < 0.001) and absorption (β = 0.128, p < 0.001) independently contributed to somatic symptoms after controlling for gender and negative affect (R2 = 0.347, p < 0.001). Similarly, somatosensory amplification (OR = 1.082, p < 0.05) and absorption (OR = 1.079, p < 0.01) independently contributed to electromagnetic hypersensitivity after controlling for somatic symptoms, gender, and negative affect (Nagelkerke R2 = 0.134, p < 0.001). However, no interaction effects were found. Discussion - Somatosensory amplification and absorption independently contribute to symptom reports and electromagnetic hypersensitivity. Conclusion - The findings suggest that psychological mechanisms underlying symptom reports and electromagnetic hypersensitivity might be heterogeneous.

Clinical Neuroscience

NOVEMBER 30, 2018

Evaluation of symptom severity, functional status and anxiety levels in patients with carpal tunnel syndrome with different electrophysiological stages

SEVINC Gürses Eftal, TEKESIN Aysel, TUNC Abdulkadir

Objective - The aim of this study was to evaluate the relationship between electrophysiological stage, symptom severity, functional status and anxiety levels in patients with idiopathic carpal tunnel syndrome (CTS). Materials and methods - This study included 130 patients in the 25-79 age group who were classified as clinically and electrophysiologically idiopathic carpal tunnel syndrome in our electromyography (EMG) laboratory. Visual Analog Scale (VAS) was used to assess pain during rest and activity. The Boston Carpal Tunnel Scale (BCTS) was used to evaluate symptom severity and functional status. Symptom Severity Scale (SSS) and Functional Capacity Scale (FCS) were assessed separately as a part of BCTS. Beck Anxiety Inventory (BAI) was used for anxiety assessment. Results - A total of 130 patients (25 males and 105 females) were enrolled to this prospective study. The mean age of the patients was 46.95 ± 10.57 years. When the electrophysiological stage was increased, it was found that SSS score and FCS score were increased (p <0.001). No significant correlation was detected between electrophysiological stage and VAS or BAI score. There was a positive correlation between VAS scores and SSS, FCS and BAI scores (p <0.001). Symptom severity and functional status were correlated with anxiety scores (p <0.001). SSS and FCS values of stage III and above patients were significantly higher than Stage I and II CTS patients (p <0.01). Conclusion - In conclusion, our study showed a significant correlation between symptom severity, functional status and anxiety in CTS patients. This can be interpreted as the mental deterioration of individuals with more severe symptoms. On the other hand, additional psychiatric support options should be recommended in cases of moderate findings but anxious symptoms. Electrophysiological findings shouldn’t be sufficient to measure the effect of the disease on the person.

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.]