Clinical Neuroscience

[Atlas of Microsurgery of the Lateral Skull Base]

VERES Róbert és munkatársa

JUNE 02, 2009

Clinical Neuroscience - 2009;62(05-06)

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Clinical Neuroscience

[Genetics and present therapy options in Parkinson’s disease: a review]

BEREZNAI Benjámin, MOLNÁR Mária Judit

[In the past years, six monogenic forms of Parkinson disease have clearly been associated with this movement disorder. The most frequent forms are LRRK2- and Parkin-associated Parkinson disease. Currently, a genetic diagnosis does not change the therapy, the genes involved in genetic Parkinson disease help to understand the underlying pathophysiologic mechanisms of Parkinson disease. Beside the overview of the molecular-genetic basis, we give a review about genetic testing, pharmacological and other multidisciplinary treatment options.]

Clinical Neuroscience

[In memoriam Richter Hugó (1885-1945)]

EMED Alexander

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.]

Clinical Neuroscience

[The 25 years of the epileptological workshops in Gyõr (1984-2008)]

OSTORHARICS-HORVÁTH György

Clinical Neuroscience

[Severe intracranial hypotension in an adolescent with Marfan syndrome. Case report]

ROSDY Beáta, KOLLÁR Katalin, MÓSER Judit, VÁRALLYAY György, KORDÁS Mariann

[Spontaneous intracranial hypotension is a rare complication of connective tissue disorders. One of them is Marfan syndrome. It predisposes the patient to meningeal diverticula. Possibly after minor unrecognised head trauma or secondary to Valsalva manoeuvre cerebrospinal fluid leak from meningeal diverticula can happen. It causes postural headache. We describe a 15 year old adolescent female newly diagnosed with Marfan syndrome, who presented with intractable postural headache. Our patient's brain MRI showed bilateral frontal subdural effusion, narrow ventricles, downward displacement of the brainstem, enlarged cervical venous plexi, dural ectasias and wide nerve root sleeves. Radionuclide cisternography demonstrated CSF leaks at multiple sites. She could not be treated conservatively, but was successfully treated by epidural saline injections. Control brain and cervical MRI confirmed her healing, too. At the two and half year follow up visit, she was completely well. So we recommend this easiest technic to use before epidural autolog blood patches.]

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Clinical Neuroscience

Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

ÖZTOP-CAKMAK Özgür, VANLI-YAVUZ Ebru, AYGÜN Serhat, BASTAN Birgül, EGEMEN Emrah, SOLAROGLU Ihsan, GURSOY-OZDEMIR Yesemin

Introduction – Although the involvement of the hypoglossal nerve together with other cranial nerves is common in several pathological conditions of the brain, particularly the brainstem, isolated hypoglossal nerve palsy is a rare condition and a diagnostic challenge. Case presentation – The presented patient arrived to the hospital with a history of slurred speech and an uncomfortable sensation on his tongue. Neurological examination showed left-sided hemiatrophy of the tongue with fasciculations and deviation towards the left side during protrusion. Based on the clinical and MRI findings, a diagnosis of hypoglossal nerve schwannoma was made. Discussion – Hypoglossal nerve palsy may arise from multiple causes such as trauma, infections, neoplasms, and endocrine, autoimmune and vascular pathologies. In our case, the isolated involvement of the hypoglossal nerve was at the skull base segment, where the damage to the hypoglossal nerve may occur mostly due to metastasis, nasopharyngeal carcinomas, nerve sheath tumors and glomus tumors. Conclusion – Because of the complexity of the region’s anatomy, the patient diagnosed with hypoglossal nerve schwannoma was referred for gamma knife radiosurgery.

Clinical Neuroscience

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.

Hypertension and nephrology

[Efficient, cardiovascular risk-dependent therapy of patients with hypertension according to the data from database of the Hungarian Hypertension Registry]

KISS István, PAKSY András, KÉKES Ede, KERKOVITS Lóránt

[Over 3.5 million people have hypertension in Hungary, although with only 40-45% of them have the target blood pressure of under 140/90 been reached thanks to the non-medication and medication therapies. The reason of this can be several folds as an improperly chosen blood pressure lowering therapy, not sufficient care, insufficient doctor-patient cooperation and the incompetent information of the patients. According to the Hungarian Hypertension Register’s database of 2015 it is confirmed that the reaching of target blood pressure significantly differs and in the case of the hypertension disease coincide with the morbidity and mortality differences of the regions. These regional differences can be explained with not only the social, cultural and economic dissimilarities, but with the diversity of the quality of the healthcare and the professional work. Analyzation of the biggest risk factors of hypertension as the diabetes, ischemic heart failure and chronic kidney disease showed that when all of them are extant, the reaching of the target blood pressure is only successful in the case of 26% of the male and 33% of the female’s patients. According to the Register it turned out that the leading cause of the unsuccessful reaching of the target blood pressure is the peripheral vascular disease in the case of female patients which is followed by in turn with the disorder of lipid metabolism, the disorder of uric acid metabolism and obesity. The leading cause in the case of male patients is lipid metabolism which is followed by in turn with smoking, alcohol abuse and peripheral vascular disease. According to the summed-up results, 88% of the patients received combined treatment with the medication suggested by the professional guidelines. The proportion responsible for the unsuccessful reaching of the target blood pressure can be explained by the insufficient patient concordance and adherence. Both patient information and the care involved in the doctor-patient relationship have to be corrected and improved. One possible solution is the transmission of some competence of the doctors to the well-trained assistants and nurses who have a decisive role in the risk assessment and the base medical examination. It is also important to draw the pharmacists in too to the care of patients with applying more telemedicinal methods. It can be concluded from the results that came from the processing of the Register’s database that the population-level knowledge is important and the continuation of the data entry into the Register is necessary.]

Clinical Neuroscience

[New surgical technique for atlanto-axial instability: C1-2 dorsal fixation with the screw-rod system]

LIPÓTH László, BANCZEROWSKI Péter, VERES Róbert

[Objective - It is still challenging to perform the operation for the instability of the C1-C2 junction because of the limited cases, unique anatomical landmarks, the potential or real injury of the neurological elements, the serious clinical state and the special technical and human background is demanding. With the aim of minimalize the risk the following method provide sufficient stability, successful and simple. Method - The authors used the dorsally implanted screwrod systems for operating 34 patient with C1-2 instability resulting clinically signs and symptoms. Depending the anatomical landmarks and the technical possibilities, the screw insertion method and the reinforced wire cable use was selected. Meaning the indication of the surgical treatment, the neurological signs, compromise of the spinal canal and pain resisting the conservative treatment was presented. Result - The C1-2 dorsally fixation was performed to 34 patients. Both sided lateral mass screw was inserted in 30 cases (88.3%). Because of the anatomical landmarks in four cases (11.7%) the one sided screwing was made by the Magerl technique. Pure bone quality detecting intraoperatively demand reinforcement with titanium cables for three cases (8.8%). Pain resulted of instability was recovered. The clinical and neurorariological follow-up present perfect result for 26 patients (76%), good result for six patients, there was respectable result for two patients. Most of the patients, 31 cases (91%) were satisfied, and three patients (9%) consider their condition acceptable. Conclusion - Performing the represened techique, the risk and the time of the operation was decreased reducing the rate of the complications. The method providing full stability resulted immediate axial painless, no outer support and early mobilization is possibile. Summarizing this technique is safe, reliable and cost effective.]

Hungarian Immunology

[Ocular myositis]

KISS Emese, FACSKÓ Andrea, DÉVÉNYI Katalin, DANKÓ Katalin, ZEHER Margit

[INTRODUCTION - Dermato-/polymyositis is an autoimmune disorder, which belongs to the idiopathic inflammatory myopaties. It involves skeletal muscles in form of weakness and inflammatory infiltrates. Characteristic skin lesions are present in dermatomyositis. Other organs may also be affected mainly in the presence of myositis specific autoantibodies. The inflammation usually involves the proximal muscles of extremities. CASE REPORT - In the present work we report the case of a 52-year-old woman. In the previous history the removal of rectal adenocarcinoma was remarkable in 1994. After that she received chemotherapy. She complied for severe headache and pain in the right eye in 2000 October, therefore a skull CT was performed, indicating thickening of rectus medalis muscle within orbital cavity. There was an enhancement of contrast material in the muscle. Glaucoma was excluded. Neurologist suspected the presence of myositis and indicated 0.5 mg/kg corticosteroid therapy. Soon after the left eye became painful, but due to the corticosteroid treatment both eyes became painless. A control orbital CT was completely negative in 2000 November. Immunology consultancy revealed a mild proximal muscle atrophy in both lower extremities, but CPK and LDH enzyme levels were normal, EMG was characteristic for mild chronic nerve lesion. The biopsy, taken from the involved proximal muscle of lower extremity, did not show inflammatory infiltration. Complete screening for cancer was negative. Thyroid gland disease could be excluded. Immune laboratory data were negative, autoantibodies, including anti-Jo1, could not be detected. Based on the results a rare disease, ocular myositis was diagnosed. Considering the clinical improvement, the withdrawal of corticosteroid therapy was offered. Stringent immunology and oncology follow-up is required. CONCLUSION - In relation to our case report, we discuss clinical symptoms of orbital myositis, diagnostic procedures to identify the disease and also differential diagnostic considerations.]