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    S. Komoly

    University of Pecs, Neurology, Faculty Member
    One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac... more
    One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure. We included 31 periictal video-EEG-ECG recordings of 31 patients with epilepsy who had consecutively undergone pre-surgical evaluation. Nine generalized tonic-clonic (GTCS), 15 complex partial, and seven simple motor seizures were included. HRV was evaluated by analyzing 5-min-long ECG epochs, sampling from baseline, direct preictal, early-postictal (<15 min after the seizure), and late-postictal (5-6 h after the seizure) periods. The heart rate was elevated immediately after the seizures, but 5-6 h postictally returned to the baseline level. Time-domain components of HRV decreased after the seizure and this decrease lasted for 5-6 h. Low-frequency power decreased in the early-postictal phase and high-frequency power of HRV dropped in the late-postictal phase. GTCS had an impact on short-term but not on long-term postictal HRV decrease. We found decreased HRV immediately after the seizures, which lasted at least 5-6 h postictally, indicating a long-term postictal disturbance of the autonomous nervous system. GTCS were accompanied by a more decreased HRV than other seizures. Our results may have relevance in explaining pathomechanism of SUDEP.
    It is unclear whether the hippocampal abnormality in temporal lobe epilepsy (TLE) is a consequence or the cause of afebrile or febrile seizures (FSs). We investigated whether hippocampal abnormalities are present in healthy... more
    It is unclear whether the hippocampal abnormality in temporal lobe epilepsy (TLE) is a consequence or the cause of afebrile or febrile seizures (FSs). We investigated whether hippocampal abnormalities are present in healthy adults>15 years after a simple FS. Eight healthy subjects (5 men) with a history of simple FS (FS+ group) and eight sex- and aged-matched control subjects (FS- group) were investigated by three MR methods: blinded visual inspection of the MRI pictures; automatic voxel-based volumetry; and T2 relaxation time measurements. The mean total volume of the two hippocampi was 5.36 +/- 1.33 cm(3)in the FS+ group and 6.63 +/- 1.46 cm(3)in the FS- group (p = 0.069). The T2 values in the anterior part of the left hippocampus (p = 0.036) and in the middle part of the right hippocampus (p = 0.025) were elevated in the FS+ subjects. The mean volume of the right hippocampus was 3.05 +/- 0.8 cm(3)in the FS+ men and 4.05 +/- 0.48 cm(3)in the FS- men (p = 0.043). The mean total volume of the two hippocampi was 5.38 +/- 1.4 cm(3)in the FS+ men and 7.48 +/- 1.14 cm(3)in the FS- men (p = 0.043). There were three FS+ men in whom hippocampal abnormalities including hippocampal sclerosis (HS) and dysgenesis were observed on visual inspection. A history of simple FS in childhood can be associated with hippocampal abnormalities in adults. These abnormalities are probably more pronounced in men. Simple FS may not be as a benign event as previously thought. Our findings suggest that hippocampal abnormalities associated with FS are not necessarily epileptogenic.
    Right-handedness and left-sided language lateralization is an unresolved mystery with unknown cause/effect relations. Most studies suggest that the language lateralization is related to a fundamental brain asymmetry: right-handedness may... more
    Right-handedness and left-sided language lateralization is an unresolved mystery with unknown cause/effect relations. Most studies suggest that the language lateralization is related to a fundamental brain asymmetry: right-handedness may be secondary. We analyzed the possibility of an opposite cause/effect relation: whether asymmetric hand usage (as a cause) can influence language lateralization (as a consequence). We determined language lateralization by functional magnetic resonance imaging in 15 subjects whose upper limb (UL) had been injured at birth because of unilateral damage of the brachial plexus. These subjects were able to use only one (the noninjured) UL perfectly. We found correlation between the severity of right-sided UL injuries and hand usage dysfunction and the degree of left-to-right shift of language lateralization. There was, however, not a complete switch of language lateralization. Right-sided UL injury can induce a left-to-right shift in language lateralization, suggesting that hand usage can influence language lateralization. These findings may contradict the broadly accepted theory that right-handedness is a secondary phenomenon caused by left-sided hemispheric language lateralization. However, the cause/effect problem between asymmetric hand usage and language lateralization is not resolved in this study. Our findings may support the theory that gestures had a crucial role in human language evolution and is a part of the language system even today.
    Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache... more
    Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache period. Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention. We report rapidly suppressed headache attacks with orally administered methylprednisolone in eight headache periods of three patients with idiopathic, episodic SUNCT syndrome. The remission was maintained until the period was over in all cases. Although the mechanism of methylprednisolone action is unclear, it is probably based on the anti-inflammatory effects of the drug.
    The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible... more
    The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible white matter signal abnormalities. Several attack-related pathomechanisms have been proposed in the lesion development, including the effect of repeated intracerebral hemodynamic changes. Supratentorial white matter hyperintensities of 17 migraine patients were investigated interictally with quantitative MRI, including quantitative single voxel spectroscopy, diffusion, and perfusion MRI at 3.0-Tesla. The findings were compared with data measured in the contralateral, normal-appearing white matter of migraineurs and in the white matter of 17 healthy subjects. Significantly higher apparent diffusion coefficient values, prolonged T2 relaxation times, and decreased N-acetyl-aspartate and creatine/phosphocreatine concentrations were found in the white matter hyperintensities. The cerebral blood flow and blood volume values were mildly decreased inside the hyperintensities. Differences were not present between the migraine patients' normal-appearing white matter and the white matter of healthy subjects. The MRI measurements denote tissue damage with axonal loss, low glial cell density, and an enlarged extracellular space with an increased extracellular water fraction. These radiological features might be the consequences of microvascular ischemic changes during migraine attacks.
    ... Monika Szots Arpad Szomor Ferenc Kover Laszlo Pajor Samuel Komoly Endre Kalman Eva Gomori Zsolt Illes ... Hematologica 92:486–492 3. Ponzoni M, Ferreri AJ, Campo E, Facchetti F, Mazzucchelli L, Yoshino T, Murase T, Pileri SA, Doglioni... more
    ... Monika Szots Arpad Szomor Ferenc Kover Laszlo Pajor Samuel Komoly Endre Kalman Eva Gomori Zsolt Illes ... Hematologica 92:486–492 3. Ponzoni M, Ferreri AJ, Campo E, Facchetti F, Mazzucchelli L, Yoshino T, Murase T, Pileri SA, Doglioni C, Zucca E, Cavalli F, Nakamura S ...
    Verapamil hydrochloride, a lipophilic calcium channel blocking agent was used in the treatment of 11 patients with multiple sclerosis. The rationale of the therapy was to improve the conduction capacity of the damaged nerve fibres. The... more
    Verapamil hydrochloride, a lipophilic calcium channel blocking agent was used in the treatment of 11 patients with multiple sclerosis. The rationale of the therapy was to improve the conduction capacity of the damaged nerve fibres. The therapy did not prove effective in a pilot trial.
    The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible... more
    The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible white matter signal abnormalities. Several attack-related pathomechanisms have been proposed in the lesion development, including the effect of repeated intracerebral hemodynamic changes. Supratentorial white matter hyperintensities of 17 migraine patients were investigated interictally with quantitative MRI, including quantitative single voxel spectroscopy, diffusion, and perfusion MRI at 3.0-Tesla. The findings were compared with data measured in the contralateral, normal-appearing white matter of migraineurs and in the white matter of 17 healthy subjects. Significantly higher apparent diffusion coefficient values, prolonged T2 relaxation times, and decreased N-acetyl-aspartate and creatine/phosphocreatine concentrations were found in the white matter hyperintensities. The cerebral blood flow and blood volume values were mildly decreased inside the hyperintensities. Differences were not present between the migraine patients' normal-appearing white matter and the white matter of healthy subjects. The MRI measurements denote tissue damage with axonal loss, low glial cell density, and an enlarged extracellular space with an increased extracellular water fraction. These radiological features might be the consequences of microvascular ischemic changes during migraine attacks.
    Atrial fibrillation is associated with higher mortality. Identification of causes of death and contemporary risk factors for all-cause mortality may guide interventions. In the Rivaroxaban Once Daily Oral Direct Factor Xa Inhibition... more
    Atrial fibrillation is associated with higher mortality. Identification of causes of death and contemporary risk factors for all-cause mortality may guide interventions. In the Rivaroxaban Once Daily Oral Direct Factor Xa Inhibition Compared with Vitamin K Antagonism for Prevention of Stroke and Embolism Trial in Atrial Fibrillation (ROCKET AF) study, patients with nonvalvular atrial fibrillation were randomized to rivaroxaban or dose-adjusted warfarin. Cox proportional hazards regression with backward elimination identified factors at randomization that were independently associated with all-cause mortality in the 14 171 participants in the intention-to-treat population. The median age was 73 years, and the mean CHADS2 score was 3.5. Over 1.9 years of median follow-up, 1214 (8.6%) patients died. Kaplan-Meier mortality rates were 4.2% at 1 year and 8.9% at 2 years. The majority of classified deaths (1081) were cardiovascular (72%), whereas only 6% were nonhemorrhagic stroke or syste...
    Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary generalized and segmental dystonia. In the present study we evaluated the results of our dystonia patients treated by DBS. The surgical results... more
    Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary generalized and segmental dystonia. In the present study we evaluated the results of our dystonia patients treated by DBS. The surgical results of forty consecutive dystonia patients underwent DBS implantation were analyzed (age: 43.7 +/- 17.7 years; sex: 22 men; etiology: 24 primary and 16 secondary dystonia; topography: 24 generalized, 12 segmental and four hemidystonia; disease duration: 16.1 +/- 9.3 years). Severity of dystonia measured by Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and health-related quality of life measured by EQ-5D scale were obtained preoperatively and compared to the scores obtained at postoperative six months and subsequent yearly follow-ups. The average follow-up lasted 2.5 years (median, 0.5-8 years). In all cases the BFMDRS scores were re-evaluated by a rater blinded to the treatment. Treatment responsiveness was defined as an at least 25% improvement on...
    Bilateral deep brain stimulation of the subthalamic nuclei (STN) is a well-established and cost-effective treatment in advanced PD. To quantitatively analyze the change in use of antiparkinsonian drugs one year after subthalamic deep... more
    Bilateral deep brain stimulation of the subthalamic nuclei (STN) is a well-established and cost-effective treatment in advanced PD. To quantitatively analyze the change in use of antiparkinsonian drugs one year after subthalamic deep brain stimulator (DBS) implantation in patients with idiopathic Parkinson's disease (PD). Eighteen consecutive patients with advanced PD underwent bilateral STN DBS implantation were involved in the study. The stimulation achieved a stable and clear clinical benefit in all of the cases. One year after the implantation, drug usage of patients was analyzed and correlated with the postoperative symptomatic improvement measured by the modified Hoehn-Yahr, Schwab and England, and Unified Parkinson's Disease Rating Scales. Because none of the investigated variables followed the normal distribution, non-parametric Wilcoxon signed-rank, McNemar and Kendell's T tests were applied. Preoperatively, the patients used 12.05 +/- 4.57 tablets a day out of ...
    Amyotrophic lateral sclerosis is a progressive neurodegenerative disease resulting in loss of upper and lower motoneurons. Here we analyzed the clinical and epidemiological data of 202 ALS patients admitted to our department between 1950... more
    Amyotrophic lateral sclerosis is a progressive neurodegenerative disease resulting in loss of upper and lower motoneurons. Here we analyzed the clinical and epidemiological data of 202 ALS patients admitted to our department between 1950 and 2004. Risk and protective factors influencing onset and progression of the disease were examined. We also analyzed the relationship between first clinical symptoms/signs and prognosis of the disease. Our data suggest the importance of toxic factors in the initiation of the disease and the effect of clinical phenotype on the progression of ALS.
    The membrane attack complex (MAC) assembles from C5b-9 complement components and has neoantigenic properties. Antihuman-MAC rabbit immunserum was applied in order to localize the MAC in myasthenic muscles. Using the indirect... more
    The membrane attack complex (MAC) assembles from C5b-9 complement components and has neoantigenic properties. Antihuman-MAC rabbit immunserum was applied in order to localize the MAC in myasthenic muscles. Using the indirect immunoperoxidase method MAC was demonstrated at the motor end-plates in eleven myasthenic patients who underwent thymectomy. This result provides direct evidence of antibody-dependent complement-mediated injury of acetylcholine receptors in myasthenia gravis.
    Besides the classical lesions, symmetric necrosis was found in the thalamus of pyrithiamine treated rats. The barrier systems of the two areas differ already in healthy animals and behave differently during the illness.
    The direct and indirect immunofluorescent methods were used to demonstrate the serum proteins in vasogenic brain edema on paraffin embedded formalin fixed human brains with carcinoma metastases. Applying these techniques extravasated... more
    The direct and indirect immunofluorescent methods were used to demonstrate the serum proteins in vasogenic brain edema on paraffin embedded formalin fixed human brains with carcinoma metastases. Applying these techniques extravasated albumin was detected within the tumor and in the surrounding neuropil. In the remote part of the peritumorous area the cytoplasma of neurons and astrocytes were stained specifically. The specific immunostaining clearly demonstrates the plasma origin of proteins in human peritumorous edema similarly to the experimental vasogenic brain edema. The immunofluorescent technique seems to be very useful to investigate the human brain edema on necropsy material as well.
    We aimed to determine the functional localisation of right-left discrimination (RLD) by functional MRI (fMRI). In this study, 16 male volunteers were examined. There were three task sessions: one active and two baseline tasks. During the... more
    We aimed to determine the functional localisation of right-left discrimination (RLD) by functional MRI (fMRI). In this study, 16 male volunteers were examined. There were three task sessions: one active and two baseline tasks. During the baseline tasks participants were instructed to show numbers with their fingers. The first baseline task was performed with the right hand, the second one with the left hand. During the active (RLD) task participants were also instructed to show numbers. The difference between baseline and active tasks was that during the active task the hand with which the participant should perform the instruction was assigned randomly. Thus, participants were unaware which hand should be used before the instruction command. During RLD, activations occurred in the right-sided frontal, precuneus, postcentral, angular, lingual, and superior temporal gyri. Activations also appeared in the left-sided temporal gyri and precuneus. Of the activations, 76.7% appeared in the right hemisphere, 23.3% in the left hemisphere. Conclusively, we found that RLD is mainly related to the right hemisphere, and requires activation of the parieto-temporo-occipital junction and the visual system including cuneus, precuneus, and gyrus lingualis.
    Rita Horváth Angela Abicht Eric A. Shoubridge Veronika Karcagi Csilla Rózsa Sámuel Komoly Hanns Lochmüller ... SK and by a NATO Collaborative Linkage Grant to HL and VK We thank Mrs. M. Czimbalmos and Mrs. M. Gogolák for ex-pert technical... more
    Rita Horváth Angela Abicht Eric A. Shoubridge Veronika Karcagi Csilla Rózsa Sámuel Komoly Hanns Lochmüller ... SK and by a NATO Collaborative Linkage Grant to HL and VK We thank Mrs. M. Czimbalmos and Mrs. M. Gogolák for ex-pert technical assistance, Dr. G. Jakab ...
    The authors report functional magnetic resonance imaging (fMRI) study data of a 60-year-old patient having short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Three consecutive... more
    The authors report functional magnetic resonance imaging (fMRI) study data of a 60-year-old patient having short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Three consecutive pain attacks were detected during the imaging session and strong brainstem activation was found. It was concluded that the brainstem can be involved in the pain signal transmission in SUNCT syndrome.
    Wilson's disease shows a wide heterogeneity in its symptoms. In this case report we would like to call attention on a not yet described symptom of Wilson's disease. Case report: The male patient's complaints of fatigue,... more
    Wilson's disease shows a wide heterogeneity in its symptoms. In this case report we would like to call attention on a not yet described symptom of Wilson's disease. Case report: The male patient's complaints of fatigue, decreased level of concentration, ambivalence, and highly increased demand of sleeping started at his age of 21 years. The complaints did not improved throughout two months, when he went to see the doctor. No abnormality was found at physical examination. A moderate elevation in ALT (83U/1) was found, but all the other laboratory findings were within the normal range. The viral hepatitis (A,B,C,EB,CMV) markers were negative, and neither toxic nor infectious disease was detectable. Neurologic examinations including EEG, liquor tests, CT, MRI scans were negative. The marked hypersomnia was verified by 24-hour EEG holter-monitoring. He spent nearly 2/3 of the day sleeping. The sleeping pattern contained abnormally elevated ratio of REM-periods. The seminological picture was like in narcolepsy. However, narcolepsy was excluded by negative HLA DR-2 examination and the lack of the narcoleptic tetrade. Wilson's disease was diagnosed. The diagnosis was based on decreased serum coeruloplasrnin and copper level, on increased urinary copper excretion and later confirmed also by gene mutation. Kayser-Fleischer ring was not detectable. After four-week treatment with zinc-sulphate, D-penicillamin treatment was introduced. The serum copper level became normal and the urinary copper excretion significantly increased. The patient's complaints gradually resolved within some months and the clinical improvement was confirmed by repeated 24-hours EEG holter monitoring. The elevated ALT returned to the normal value. Three years later, the patient is currently on penicillamine treatment and he is free of any symptom. He was found to be heterozygous for the His1069Gln mutation by PCR examination. Conclusion: Although hypersomnia was not a known symptom of Wilson's disease, in this case the causality is quite probable. The hypersomnia was verified as a real hypersomnia and all the other possible causes were excluded by detailed examinations. The relationship between hypersomina and Wilson's disease was further supported by the verified clinical improvement after the therapy. This work was supported in part by ETF 510/96 and OTKA T022453 grants.
    OBJECTIVES: To perform a pilot study assessing possible efficacy, safety, and optimal dosage of pramipexole in essential tremor. METHODS: Twenty-nine patients with essential tremor were enrolled into this 16-week-long study. After... more
    OBJECTIVES: To perform a pilot study assessing possible efficacy, safety, and optimal dosage of pramipexole in essential tremor. METHODS: Twenty-nine patients with essential tremor were enrolled into this 16-week-long study. After recording baseline condition, 2 different dosages of immediate-release formulation of pramipexole were evaluated (1.05 mg/d and 2.1 mg/d in 3 identical dosages). Subsequently, immediate- and extended-release formulations were compared. The Fahn-Tolosa-Marin Tremor Rating Scale, Activities of Daily Living, the EuroQol instrument for detecting health-related outcome, and Clinical Global Impression of Improvement Scale were obtained. After completing the study, a rater blinded to the treatment reassessed the tremor rating scales based on video recordings. RESULTS: Twenty-four patients (82.6%) completed the study. Causes for discontinuation were adverse effects of pramipexole: intolerable nausea (n = 3), daytime sleepiness (n = 1), and anxiety (n = 1). Twenty-one patients had a score of less than 3 on the Clinical Global Impression of Improvement Scale (treatment responders, 72.4%). All the major outcome values demonstrated significant improvement. The severity of tremor was reduced by 52.0% (43.7 vs 20.8 points, Fahn-Tolosa-Marin Tremor Rating Scale), and the EuroQol instrument for detecting health-related outcome score improved from 0.69 to 0.91 (P < 0.01). The dose of 2.1 mg was more effective than that of 1.05 mg; however, both the immediate- and extended-release formulations were equally efficacious. After completion of the study, 15 patients (51.7% of the enrolled patients) wanted to remain on pramipexole treatment. CONCLUSIONS: This pilot study suggests that pramipexole may be effective in the treatment of essential tremor. However, further controlled studies are required.
    A normal cell count as well as normal CSF pressure levels were found in both classic and common migraine patients during and between attacks. Total protein content was significantly lower in the migraine patients than in the controls, but... more
    A normal cell count as well as normal CSF pressure levels were found in both classic and common migraine patients during and between attacks. Total protein content was significantly lower in the migraine patients than in the controls, but no changes were found in the CSF protein fractions. The CSF 5-hydroxyindoleacetic acid level of the migraine patients proved to be higher than in the controls, whereas the homovanillic acid concentration was within the control limits.

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