Clinical Neuroscience

[Childhood sporadic type of hemiplegic migraine with arteria cerebri media hypoperfusion]

NAGY Csaba, BAJZIK Gábor, SKOBRÁK Andrea, CSORBA Eszter, LAJTAI Anikó, BALOGH Gábor, NAGY Ferenc, VAJDA Zsolt

SEPTEMBER 30, 2017

Clinical Neuroscience - 2017;70(09-10)

DOI: https://doi.org/10.18071/isz.70.0343

[Hemiplegic migraine is a rare subtype of migraine that is associated with reversible motor weakness in the aura phase. This is an uncommon form of migraine usually starting in childhood. The purpose of this case report is to highlight the differential diagnostic difficulty of the first attack. We describe a case, where the fluctuating unilateral motor weakness and aphasia suggested that the patient had ischaemic stroke. Nevertheless the brain MRI and MR angiography, the measured 5-hydroxyindole acetic acid (5-HIAA) concentration changes and the spontaneously improving clinical status proved the diagnosis of hemiplegic migraine. The MRI and MR angiography was very beneficial in establishing the correct diagnosis in this case. To distinguish between the familiar and sporadic type of hemiplegic migraine further genetic tests can be carried out.]

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Clinical Neuroscience

Correlation of vitamin D levels with electrophysiological findings and pain in the patients with Carpal Tunnel Syndrome

DEMIRYÜREK Enes Bekir, SENTÜRK Asli

Objective - This study aimed to assess the correlation between vitamin D deficiency and electrophysiological findings and pain level in patients with symptoms of carpal tunnel syndrome (CTS). Patients and method - A total of 131 patients with symptoms of CTS, 70 with vitamin D deficiency and 61 without vitamin D deficiency, were included in the study. Using demographic data and findings from electrophysiological examinations, the patients were divided into two groups based on their vitamin D level (Group 1: <20 ng/ml; Group 2: ≥20 ng/ml). The Boston Carpal Tunnel Syndrome Questionnaire was used to assess their CTS- related pain level. Results - Although the rate of CTS in the patients with a low vitamin D level was found to be high, no statistically significant correlation was observed between low vitamin D level and the frequency and severity of CTS. Additionally, the pain and functional loss ratio induced by CTS was found to be higher in the group with a lower vitamin D level than in the group with normal levels. Conclusion - Low vitamin D levels may increase the severity of CTS symptoms. Treatment of vitamin D deficiency in patients with CTS can play a role in reducing pain and disability.

Clinical Neuroscience

Does the comparison of median-to-ulnar nerve sensory conduction add an additional value in electrodiagnosis of carpal tunnel syndrome?

ÖZGÜR Selek, MURAT Alemdar

Background and purpose - Distal sensory onset latency (DSOL), conduction velocity (SCV) and nerve action potential (SNAP) amplitudes are used in electrodiagnosis of carpal tunnel syndrome (CTS) beside motor conduction data. The aim of our study is to search whether the comparison of median-to-ulnar nerve sensory conduction adds an additional diagnostic value in CTS or not. Methods - Median and ulnar nerve were stimulated on wrist, and SNAPs were recorded on second and fifth fingers, respectively. Best cut-off points for the searched parameters and their diagnostic efficiencies were determined. The cut off points were also stratified according to the age and gender, and their diagnostic efficiencies were calculated again. Results - The study includes 415 hands belong to 344 subjects. Best cut off points for median nerve DSOL and SCV were 2.7 msec and 49.0 m/sec with the diagnostic efficiencies of 87.7% and 88.7%, respectively. Best cut off points for DSOL difference and SCV difference were 0.62 msec and 4.0 m/sec, and efficiencies were 89.6% and 84.3%, respectively. Conclusion - Determining the relative elongation of median nerve DSOL to the ulnar nerve one has a little additional value in electrodiagnosis of CTS, whereas any additional value is not obtained from SCV comparison.

Clinical Neuroscience

A case of secondary SUNCT syndrome

GUL Gunay, KANDEMIR Melek, KARA Batuhan, SAKALLI Karagoz Nazan, EREN Sengul Fulya

SUNCT syndrome, a rare form of primary headaches, may be secondary to pituitary tumours. The secondary forms usually related with prolactinomas. The response of dopamin agonists could be variable. In this study, we reported a case of SUNCT syndrome secondary to prolactinoma. Cranial magnetic resonance imaging was performed for this patient because of the increase in pain severity and frequency. A hemorrhage was detected into the prolactinoma ipsilateral to the pain. The headache attacks were taken undercontrol and remission was ensured with cabergoline in a short time.

Clinical Neuroscience

[Diabetes, dementia, depression, distress]

SZATMÁRI Szabolcs, ORBÁN-KIS Károly, MIHÁLY István, LÁZÁR Alpár Sándor

[The number of people living with diabetes continues to rise. Therefore neurologists or other health care practitioners may be increasingly faced with comorbid neuropsychiatric disorders commonly presented by diabetic patients. More recently there has been an increasing research interest not only in the interactions between diabetes and the nervous system, the fine structure and functional changes of the brain, but also in the cognitive aspects of antidiabetic treatments. Patients with both types of diabetes mellitus may show signs of cognitive decline, and depression. Comorbid insomnia, anxiety, and distress may also occur. The bi-directional relationships between all these phenomena as well as their connection with diabetes can lead to further health and quality of life deterioration. Therefore it is important that all practitioners involved in the care of diabetic patients recognize the presence of comorbid neuropsychiatric disturbances early on during the healthcare process. Identifying higher risk patients and early screening could improve the prognosis of diabetes and may prevent complications.]

Clinical Neuroscience

Health anxiety mediates the connection between somatosensory amplification and self-reported food sensitivity

ELIESON M. Linn, DÖMÖTÖR Zsuzsanna, KÖTELES Ferenc

Background - The frequency of self-reported food sensitivity (SFS) is increasing, and has a negative impact on the well-being and everyday functioning of the affected people. A considerable proportion of SFS cannot be medically explained. The lack of knowledge of its origin and treatment causes further stress in those affected. Purpose - This study aims to get a better understanding of the psychological background of the condition. Methods - A non-representative community sample (N=335; age: 35.1±13.18 yrs; 75.8% female) completed an English on-line questionnaire assessing somatosensory amplification, health anxiety, modern health worries (MHWs), beliefs concerning the scientific validity of complementary and alternative medicine (CAM), holistic beliefs on health and illness. Results - In multiple binary logistic regression analyses, SFS were associated with CAM related beliefs, somatosensory amplification, and health anxiety after controlling for age and gender. The connection between somatosensory amplification and SFS were completely mediated by health anxiety. No differences between the two groups were found with respect to MHWs, worries about the harmful effects of various artificial components in food, and holistic health beliefs. Discussion: More positive attitudes toward CAM might be based on the lack of conventional treatment, rather than on higher levels of MHWs or a more holistic worldview. Both the existence of symptoms and the presence of health anxiety might be needed for the development and maintenance of SFS. Conclusions - The findings support the notion that somatosensory amplification and health anxiety might play a role in the development and maintenance of SFS.

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Clinical Neuroscience

Long-term follow-up results of concomitant chemoradiotherapy followed by adjuvant temozolomide therapy for glioblastoma multiforme patients. The importance of MRI information in survival: Single-center experience

LUKÁCS Gábor, TÓTH Zoltán, SIPOS Dávid, CSIMA Melinda, HADJIEV Janaki, BAJZIK Gábor, CSELIK Zsolt, SEMJÉN Dávid, REPA Imre, KOVÁCS Árpád

Introduction - Glioblastoma multiforme (GBM) is the most common malignant primary anomaly of central nervous system. The GBM infiltrates the nearly sturctures from the initial tumor and its metastatic attribution is well known. The aim of our single-centered retrospective study was to introduce the importance of postoperative medical imaging confirmation of total tumor resection for patient with GBM combined concomitant and adjuvant chemoradiotherapy on a 10 year long patient follow up. Methods - From January 2006 to April 2015 we registered 59 patients with newly diagnosed GBM at the University of Kaposvár Health Center Institute of Diagnostic Imaging and Radiation Oncology. The histological diagnosis was confirmed by a proficient neuropathologist (World Health Organisation WHO; grade IV astrocytoma). According to histological status if the ECOG performance status of patients allowed it the mutidisciplinary oncoteam recommended adjuvant chemoradiotherapy all features strictly by Stupp protocol. (60 Gy dose on the gross tumor volume and 2-3 cm margin for the clinical target volume with parallel 75 mg/m2 TMZ. Four weeks after monotherapial phase patients had to recieve 6 cycles of TMZ first cycle with 150 mg/m2 up to 200 mg/m2). The irradiation was carried out by a conformal three dimensional planning system. Results - 59 patients with the median age of 63 (range 17-84) year. Our sample counted 34 male patients and 25 woman patients. 14 patients underwent gross total tumor resection while, 39 patients underwent partial resection and the rest from our sample 6 patients passed through biopsy. Statistical analysis showed a lengthier survival among males than females, with a median survival of 13 months for males and females, the OS of 26.209 for males, meanwhile 15.625 for females. However, the difference is not considerable (log-rank p=0.203). Our study found that the estimated survival of patients at least 50 years old is significantly shorter at a median survival of 12 months (log rank p=0.027) than that of patients below 50 years of age at a median survival of 23 months. The longest estimated median survival was calculated with patients of ECOG '0' condition (16 months). However, no significant difference was found in the estimated survival of patients of different ECOG conditions (log-rank p=0.146). Based on the extent of surgery, complete resection resulted in the longest average survival of 36.4 months, followed by 21.5 months among patients with biopsy, and 15.8 months among patients with partial resection. Different surgical procedures, however, did not result in significant differences in survival (log-rank p=0.059). The overal survival of patients who had complete resection confirmed by MRI compared with the overal survival of patients with residual tumor confirmed by MRI as well we can estimate that there is significant difference between these two groups (p=0,004). Conclusion - Despite complex and intense treatment, recurrence is inevitable and causes relatively rapid death. In our analysis complete resection, as defined from the neurosurgeon’s report and postoperative MRI, resulted in an independently significant improvement in OS. Our results are the evidences that the treatment of patients with glioblastoma multiforme in Hungary is at least on the same level as any other developed European countries.

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments II]

KINCSES Zsigmond Tamás, TÓTH Eszter, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, BENCSIK Krisztina, VÖRÖS Erika, CSOMOR Angéla, PALKÓ András, VÉCSEI László

[The paraclinical examinations, principally the MRI have an increasing significance in the diagnosis of multiple sclerosis. However, MRI markers also have a prominent role in monitoring of the disease-course and activity, and also in the planning of possible therapeutic changes. In accordance with previously published international guidelines, in this article we propose a protocol for the monitoring the treatment efficacy in multiple sclerosis. This could be the basis of a consensus based guideline to be implemented in Hungary.]

Hungarian Radiology

[Breast malignancies: review of the year 2008/2009 radiological diagnostics and therapy news - Onco Update, 2010]

FORRAI Gábor

[Systematic review of the recent articles of the years 2008/2009 about breast tumours’ radiological diagnostics and guided therapy, the actual place of the imaging and interventional methods are presented.]

Hungarian Immunology

[Diagnostic value of MRI in patients with juvenile dermatomyositis]

CONSTANTIN Tamás, PONYI Andrea, BALÁZS György, SALLAI Ágnes, DANKÓ Katalin, FEKETE György, KARÁDI Zoltán

[Diagnosis of juvenile dermatomyositis is based on the presence of proximal muscle weakness, characteristic skin lesions, muscle enzyme elevation in the serum, and may requires the performance of invasive procedures such as electromyography and/or muscle biopsy. Magnetic resonance imaging (MRI) is considered to be an objective non-invasive tool to detect muscle involvement for diagnosis as well as for follow-up studies. We report a case of a 12 years old girl with definitive juvenile dermatomyositis. She received glucocorticoid therapy and achieved remission of the disease. After a long-term relapse free period, she was presented with severe proximal muscle weakness and normal creatinine kinase levels. The laboratory studies did not reveal acute inflammation or infection. In this case MRI was diagnostic to the relapse of juvenile dermatomyositis, with an increased STIR (short tau inversion recovery) signal of proximal muscles. The muscle involvement detected by MRI correlated with functional ability. After she achieved clinical remission, further follow-up MRI scans demonstrated that the affected muscles had returned to normal signal intensity. Findings of dermatomyositis on MRI scans include increased signal intensity in the affected muscles, perimuscular edema, chemical-shift artifact, and increased signal intensity in subcutaneous tissue. MRI is a sensitive technique and proposed to be a good indicator for an early diagnosis of the disease. MRI may also help to guide the muscle biopsy and may enhance the sensitivity of histological examination. After completion of therapy, MRI may be used for monitoring the progress of the disease as signal intensity of affected muscles returns to normal. MRI is also helpful, if the diagnosis is suspected but has not been formally evaluated.]

Clinical Neuroscience

[99-mTc-HMPAO single photon emission computed tomography examinations in genetically determined neurometabolic disorders]

ARANKA László, AMBRUS Edit, VÖRÖS Erika, SVEKUS András, KÓBOR Jenõ, BEREG Edit, PALATKA János, PÁVICS László

[The aim of our study was to determine regional cerebral blood flow (rCBF) abnormalities in different types of enzymopathies. Patients and methods - Among the patients with genetically determined enzymopathies 3 patients had aminoacidopathies, and 11 had different types of encephalopathies, from which 10 had mitochondrial encephalomyopathy (MEMP), and 1 patient had hyperuricaemic encephalopathy. Besides the mentioned 14 patients, 1 had ceroid lipofuscinosis and another patient had tuberous sclerosis. The further distribution of the MEMP patients’ group was the following - 5 patients had MEMP with lactic acidosis, 5 had Leigh’s disease (subacute necrotizing encephalopathy), from which 1 had cytochrome-c-oxidase deficiency (COX). Additionally in all patients were performed cerebral MRI and SPECT examination 10 min. after intravenous administration of 20 Mbq/kg 99 mTc-HMPAO. Results - Fourteen out of 16 SPECT findings were pathologic, showing decreased focal frontal/temporal/temporoparietal cerebral blood perfusion. Aminoacidopathic group - all the 3 patients revealed pathologic signs from the aminoacidopathic patients’ group. Among them the ornithine transcarbamylase (OTC) heterozygous female patient with left-sided hemiparesis caused by hyperammonemic stroke at 10 month-age, showed right sided temporoparietal, occipital and left frontal hypoperfusion, nearly 6 years after the cerebral vascular attack. This finding might be resulted because of diaschisis. Mitochondrial encephalo-myopathic (MEMP) group - all the four patients with MEMP and lactic acidosis showed focal hypoperfusion in the temporal region, while the perfusion was normal in the COX deficient patient and in 2 Leigh’s disease (subacute necrotizing encephalopathy) patients. In the remaining 1 Leigh’s patient frontotemporal hypoperfusion was found. In all patients there were non specific structural abnormalities detected by MRI - cortical and subcortical atrophy, and scattered demyelination foci. In the case of ceroid lipofuscinosis the MRI showed cerebral atrophy and cerebellar hypoplasia, and the SPECT showed right frontal and occipital hypoperfusion, bilateral parietal physiological riping process. The patient with tuberous sclerosis showed bilateral temporo-occipital hypoperfusion. Conclusion - 1. SPECT images demonstrated hypoperfusion rCBF changes in 14 out of all 16 patients. 2. Regional cerebral/cerebellar hypoperfusion was detected by SPECT in mitochondrial encephalomyopathies, with lactate acidosis and aminoacidopathies giving high informative value about the cerebral perfusion.]