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    Zoltán Pfund

    Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in... more
    Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in children with alternating hemiplegia of childhood. Clinical and neurodevelopmental data, as well as standard uptake values in 25 brain regions and whole-brain serotonin synthesis capacity (unidirectional uptake rate constant or K-complex), were assessed in six patients with alternating hemiplegia of childhood (three girls and three boys; mean age = 76/12 years) using α[ 11C]methyl-L-tryptophan positron emission tomography (PET). The PET studies were performed interictally in three patients, during the ictal state in two patients, and postictally in one patient. The PET data were compared to those obtained interictally from six age-matched patients with focal epilepsy (two girls and four boys; mean age = 78/12 years) and six non-age-matched apparently normal siblings of autistic children (two girls and four boys; mean age = 911/12 years). Patients with alternating hemiplegia of childhood studied in the ictal or postictal state showed increased serotonin synthesis capacity in the frontoparietal cortex, lateral and medial temporal structures, striatum, and thalamus when compared to controls, and subjects with alternating hemiplegia of childhood studied interictally. The involvement of these brain regions was consistent with the semiology of the hemiplegic attacks. In patients with interictal studies and in the controls, the PET scans revealed similar and bilaterally symmetric regional patterns of serotonin synthesis capacity. Increased whole-brain serotonin synthesis capacity (reported in migraine subjects without aura) was not found in the alternating hemiplegia of childhood group. There was no correlation between the neurodevelopmental scores and regional standard uptake values; however, patients with a larger estimated lifetime attack number showed greater delay in communication (P = .005) and daily living skills (P = .042). These studies suggest increased regional serotonergic activity associated with attacks in alternating hemiplegia of childhood. Furthermore, the attack number may have an effect on neurodevelopmental delay, thus supporting the notion that alternating hemiplegia of childhood may be a progressive disorder. (J Child Neurol 2002;17:253-260).
    Background: SturgeeWeber syndrome (SWS) with unilateral hemispheric involvement is a clinical model of early onset, chronic, often progressive hemispheric injury, resulting in variable neuro-cognitive impairment. Aims: To evaluate if... more
    Background: SturgeeWeber syndrome (SWS) with unilateral hemispheric involvement is a clinical model of early onset, chronic, often progressive hemispheric injury, resulting in variable neuro-cognitive impairment. Aims: To evaluate if abnormal diffusion and metabolism of the thalamus, a central relay station with extensive cortical connections, may serve as a simple imaging marker of neuro-cognitive dysfunction in SWS. Methods: We obtained both diffusion tensor imaging and FDG PET in 20 children (11 girls; age range: 3e12.4 years) with unilateral SWS. Diffusion parameters as well as FDG uptake were measured in thalami, compared to normal control values, and correlated with the extent of cortical hypometabolism, deep venous abnormalities and cognitive (IQ) as well as fine motor functions. Results: Children with SWS had significantly higher thalamic glucose metabolic asymmetry than controls ( p1⁄4 0.001). Thalamic metabolic asymmetries correlated positively with the asymmetry of thalam...
    The clinical data of 279 consecutive patients with brain tumors were analyzed pre- and postoperatively in the period of 1994-95. No headache had been recorded in the history of 115 patients, neither pre- nor postoperatively. Only in 139... more
    The clinical data of 279 consecutive patients with brain tumors were analyzed pre- and postoperatively in the period of 1994-95. No headache had been recorded in the history of 115 patients, neither pre- nor postoperatively. Only in 139 of the remaining 164 headache patients was there a probable connection between headache and intracranial neoplasm. In the headache group the most frequent findings were metastatic brain tumors and different astrocytomas. Hypophysis adenomas and glioblastoma multiforme were frequent in the no-headache group. Progressive headache was found in 110 patients (67% of the headache group). The progressive character of the headache showed a close relationship with the prevailing edema, but not with the size of the tumor. Infratentorial and intraventricular tumors were more frequently accompanied by headache than those located supratentorially, probably due to the disturbance of CSF circulation and midline dislocation with increased intracranial pressure. Only...
    TT: [Treatment of dystonia by deep brain stimulation: A summary of 40 cases] Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary gener- alized and segmental dystonia. In the present study we... more
    TT: [Treatment of dystonia by deep brain stimulation: A summary of 40 cases] Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary gener- alized and segmental dystonia. In the present study we eval- uated the results of our dystonia patients treated by DBS
    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.
    Poster: "ECR 2011 / C-2180 / Age-related changes of white matter structures in multiple sclerosis patients measured by diffusion MR imaging" by: "S. Nagy1, M. Aradi2, G. Orsi2, G. Perlaki2, Z. Pfund1, P. Bogner1;... more
    Poster: "ECR 2011 / C-2180 / Age-related changes of white matter structures in multiple sclerosis patients measured by diffusion MR imaging" by: "S. Nagy1, M. Aradi2, G. Orsi2, G. Perlaki2, Z. Pfund1, P. Bogner1; 1Kaposvar/HU, 2Pecs/HU"
    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...
    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.
    The progressive nature of Sturge-Weber syndrome is well known, but the mechanisms of focal cortical and subcortical degeneration in this disorder are poorly understood. In the present study, we assessed the structural and functional... more
    The progressive nature of Sturge-Weber syndrome is well known, but the mechanisms of focal cortical and subcortical degeneration in this disorder are poorly understood. In the present study, we assessed the structural and functional integrity of gray and white matter in unihemispheric Sturge-Weber syndrome using quantitative magnetic resonance imaging (MRI) volumetry and MRI-based partial volume correction of [18F]fluorodeoxyglucose positron emission tomographic (PET) images. Gray- and white-matter volumes and glucose metabolism were measured in three brain regions (parieto-occipital underneath the angioma, temporal, and frontal) in six children with Sturge-Weber syndrome (two infants, ages 6 and 9 months; four older children, ages 4 to 14 years), all with unilateral parieto-occipital leptomeningeal angiomatosis. The gray-matter volumes ipsilateral to the angioma were smaller in all children, with the posterior regions underneath the angioma the most affected. In the infants, the wh...
    The purpose of this study was to examine the relation between glucose metabolism and glutamate concentration in the human brain, in both the normal and diseased state. Regional values of glucose metabolism measured with... more
    The purpose of this study was to examine the relation between glucose metabolism and glutamate concentration in the human brain, in both the normal and diseased state. Regional values of glucose metabolism measured with 2-deoxy-2[F-18]fluoro-D-glucose positron emission tomography (FDG PET) studies and single-voxel proton magnetic resonance spectroscopy (1H MRS) measurements of the glutamate/glutamine/γ-aminobutyric acid (Glx) tissue concentration were determined in multiple brain regions in 11 patients (5 girls and 6 boys, mean age 7.5 years) with medically intractable partial epilepsy. FDG PET and 1H MRS studies were performed in the interictal state in seven patients and in the ictal/periictal state in four patients. Regions of interest were identified in epileptic cortex (determined by intracranial and/or scalp electroencephalography) and in contralateral normal brain regions. Lower glucose metabolism and lower Glx concentrations were found in the epileptic focus than in the cont...
    Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in... more
    Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in children with alternating hemiplegia of childhood. Clinical and neurodevelopmental data, as well as standard uptake values in 25 brain regions and whole-brain serotonin synthesis capacity (unidirectional uptake rate constant or K-complex), were assessed in six patients with alternating hemiplegia of childhood (three girls and three boys; mean age = 7 6/12 years) using alpha[11C]methyl-L-tryptophan positron emission tomography (PET). The PET studies were performed interictally in three patients, during the ictal state in two patients, and postictally in one patient. The PET data were compared to those obtained interictally from six age-matched patients with focal epilepsy (two girls and four boys; mean age = 7 8/12 years) and six non-age-matched apparently ...
    A 12-year-old girl developed acute erythromelalgia of distal extremities. Physical, imaging and laboratory examinations failed to find an infective, systemic autoimmune, metabolic, endocrine, and vascular origin. The severe pain and... more
    A 12-year-old girl developed acute erythromelalgia of distal extremities. Physical, imaging and laboratory examinations failed to find an infective, systemic autoimmune, metabolic, endocrine, and vascular origin. The severe pain and allodynia indicated small-fiber neuropathy but muscle weakness suggested an involvement of large myelinated nerve fibers. This was confirmed by electrophysiological testing. High-dose then slowly tapered methylprednisolone resulted in rapid remission
    Small fiber neuropathy develops due to the selective damage of the thin fibers of peripheral nerves. Many common diseases can cause this condition, including diabetes, infections, autoimmune and endocrine disorders, but it can occur due... more
    Small fiber neuropathy develops due to the selective damage of the thin fibers of peripheral nerves. Many common diseases can cause this condition, including diabetes, infections, autoimmune and endocrine disorders, but it can occur due to genetic alterations, as well. Eighty-five skin biopsy-proven small-fiber neuropathy cases were analyzed. Forty-one (48%) cases were idiopathic; among secondary types, hypothyreosis (9.4%), diabetes mellitus (7%), cryoglobulinemia (7%), monoclonal gammopathy with unproved significance (4.7%), Sjögren’s disease (3%), and paraneoplastic neuropathy (3%) were the most common causes. Two-thirds (68%) of the patients were female, and the secondary type started 8 years later than the idiopathic one. In a vast majority of the cases (85%), the distribution followed a length-dependent pattern. Intraepidermal fiber density was comparable in idiopathic and secondary forms. Of note, we found significantly more severe pathology in men and in diabetes. Weak corre...
    Voxel-based morphometry and functional magnetic resonance imaging demonstrated severe atrophy and decreased activation of visual attention areas and occipital lobes in a patient with early posterior cortical atrophy compared with healthy... more
    Voxel-based morphometry and functional magnetic resonance imaging demonstrated severe atrophy and decreased activation of visual attention areas and occipital lobes in a patient with early posterior cortical atrophy compared with healthy controls and patients with early Alzheimer's disease. Our complex approach indicates that structures responsible for attention can be damaged early in posterior cortical atrophy and may contribute to the characteristic decline in higher visual functions.
    Our aim was to characterize bi-exponential diffusion signal changes in normal appearing white matter of multiple sclerosis (MS) patients. Diffusion parameters were measured using mono-exponential (0-1000 s/mm(2)) and bi-exponential... more
    Our aim was to characterize bi-exponential diffusion signal changes in normal appearing white matter of multiple sclerosis (MS) patients. Diffusion parameters were measured using mono-exponential (0-1000 s/mm(2)) and bi-exponential (0-5000 s/mm(2)) approaches from 14 relapsing-remitting subtype of MS patients and 14 age- and sex-matched controls after acquiring diffusion-weighted images on a 3T MRI system. The results were analyzed using parametric or nonparametric tests and multiple linear regression models. Mono-exponential apparent diffusion coefficient (ADC) slightly increased in controls (P=.09), but decreased significantly in MS as a function of age, nonetheless an elevated ADC was observed with increasing lesion number in patients. Bi-exponential analyses showed that the increased ADC is the result of decreased relative volume fraction of slow diffusing component (f(s)). However, the fast and slow diffusion components (ADC(f), ADC(s)) did not change as a function of either age in controls or lesion number and age in MS patients. These data demonstrated that the myelin content of the white matter affects diffusion in relapsing-remitting subtype of multiple sclerosis that is possibly a consequence of the shift between different water fractions.
    To compare the white matter lesions seen in multiple sclerosis and migraine using monoexponential and high b-value biexponential diffusion measurements. Diffusion-weighted images were acquired on a 3.0-Tesla magnetic resonance imaging... more
    To compare the white matter lesions seen in multiple sclerosis and migraine using monoexponential and high b-value biexponential diffusion measurements. Diffusion-weighted images were acquired on a 3.0-Tesla magnetic resonance imaging system. Diffusion parameters were estimated using monoexponential (0-1000 s/mm(2) ) and biexponential (0-5000 s/mm(2) ) approaches from 15 multiple sclerosis patients, 15 patients with migraine and 15 healthy control subjects. The study was performed in accordance with the approval of the Regional Research Ethics Committee. The apparent diffusion coefficient (ADC) values were measured in the lesions and the normal-appearing white matter of patients and in the white matter of controls. High lesional ADCmono values were detected in both patient groups without significant differences between the groups (10.72 and 9.86 × 10(-4) mm(2) /s for MS and migraine respectively, P = 0.2134). The biexponential measurements showed significantly higher ADCfast , ADCslow , and Pslow values in the migraine lesions than in the multiple sclerosis lesions (16.47 versus 14.29, 1.41 versus 0.76, and 20.34 versus 12.01 all values in 10(-4) mm(2) /s; P = 0.0344, P = 0.0019, P = 0.0021, respectively). Biexponential diffusion analysis may help to differentiate multiple sclerosis-related white matter lesions from migraine-related ones. J. Magn. Reson. Imaging 2015;41:676-683. © 2014 Wiley Periodicals, Inc.
    Drug-resistant partial epilepsy in children often has a major impact on cognitive development, and early surgical intervention has been advocated to prevent adverse neurobehavioral effects of seizures in such patients. We report a... more
    Drug-resistant partial epilepsy in children often has a major impact on cognitive development, and early surgical intervention has been advocated to prevent adverse neurobehavioral effects of seizures in such patients. We report a 5-year-old boy who had cryptogenic partial epilepsy of right parietal origin as documented by ictal electroencephalogram (EEG) and glucose metabolism positron emission tomographic (PET) scan. His atonic seizures could not be controlled by multiple antiepilepsy drugs; therefore, cortical resection was scheduled. However, his seizures remitted spontaneously after 1 year of failed medical treatment. The epileptiform abnormality disappeared on the follow-up EEGs, and a glucose PET scan also normalized. This boy has fully retained his cognitive and motor functions and has remained seizure free in the past 4z\x years off medications.
    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.
    Migraine is a risk factor for the formation of silent brain white matter lesions (WMLs) that are possibly ischemic in nature. Although dysfunction of the L-arginine/nitric oxide (NO) pathway has been associated with oxidative stress and... more
    Migraine is a risk factor for the formation of silent brain white matter lesions (WMLs) that are possibly ischemic in nature. Although dysfunction of the L-arginine/nitric oxide (NO) pathway has been associated with oxidative stress and endothelial dysfunction in migraine, its role in WML development has not been specifically investigated. Thus, this prospective study aimed to measure the serum concentrations of the NO substrate L-arginine, the NO synthase inhibitor asymmetric dimethylarginine (ADMA), and the L-arginine transport regulator symmetric dimethylarginine (SDMA) in migraine patients in a headache-free period. All participants underwent MR imaging to assess for the presence of WMLs on fluid-attenuated inversion recovery imaging. Altogether 109 migraine patients (43 with lesions, 66 without lesions) and 46 control individuals were studied. High-performance liquid chromatography was used to quantify L-arginine, ADMA and SDMA serum concentrations. Migraine characteristics wer...