Clinical Neuroscience

[How do temporal lobe seizures changeby age?]

FOGARASI András

DECEMBER 20, 2008

Clinical Neuroscience - 2008;61(11-12)

[Seizure semiology describes different - motor, sensory, autonomic, etc. - aspects of epileptic seizures. Several semiological studies showed already that different epilepsies - especially temporal lobe epilepsy - contain age-dependent features. In our researches, we tried to assess these subjective aspects with as objective methods as possible. We gave a comprehensive (preictal, ictal, and postictal) description of seizure semiology in patients fulfilling the gold standard criteria of semiological studies: being seizure free after temporal lobe resections. Our studies based on a large population, assess epileptic features at different levels of brain maturation. They help to understand why certain semiological axes show special characteristics at different ages. In this review, I summarize the most important results of our seizure semiology studies in temporal lobe epilepsy.]

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Clinical Neuroscience

[Pneumococcal meningitis in children - 9 1/2-year-experience at Szent László hospital, Budapest, Hungary ]

IVÁDY Balázs, LIPTAI Zoltán, ÚJHELYI Enikő, BALÁZS György

[Background and objective - No recent publications are available about pneumococcal meningitis in Hungarian children. The aim of this study was to collect data of epidemiological, clinical and prognostic features of pneumococcal meningitis in children treated at Szent László Hospital, Budapest, Hungary. Methods - We conducted a retrospective review of medical charts and follow-up records of patients aged 1 to 18 years admitted to our Pediatric and Pediatric Intensive Care Units due to pneumococcal meningitis between 1st Jan 1998 and 30th Jun 2007. Results - 31 children with 34 cases of pneumococcal meningitis were admitted to our hospital in the study period. Two children developed recurrent illness. The mean age was 6 years, 26% were under 1 year of age. The mean duration of hospital stay was 21 days, 97% required intensive care. Frequent clinical symptoms were fever (100%), nuchal rigidity and vomiting (78%), altered mental status (71%), Kernig's and Brudzinski's signs (58%) and seizures (41%). Otitis media, sinusitis, mastoiditis were present in 44%, 58%, 41%, respectively. Subdural effusion, parenchymal cerebral lesion and sinus thrombosis were documented in 5, 3 and 2 cases, respectively. One third of the patients recieved ceftriaxon, two thirds were administered ceftriaxon and vancomycin. Adjunctive therapy with dexamethason was given to 91% of the children. 70% of patients required mechanical ventillation. 9 patients (25%) required endoscopic sinus surgery. In 13 cases (38%) mastoidectomy, in 5 children (15%) neurosurgery was performed. The case fatality rate was 23.5%. 8 (23.5%) patients had mild or moderate, 1 child (3%) developed severe neurological sequelae. Conclusion - Pneumococcal meningitis in children remains a source of substantial morbidity and mortality in childhood. The long hospital stay, the frequent need for intensive care and severe neurologic sequelae emphasize the importance of early diagnosis, early treatment and prevention with pneumococcal conjugate vaccines.]

Clinical Neuroscience

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[Objective and background - Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by prolonged febrile hemiconvulsions or generalized seizures starting in the first year of life. Later on myoclonic, atypical absence, and complex partial seizures appear. When one of these seizure forms is lacking the syndrome of borderline SMEI (SMEB) is defined. Psychomotor delay resulting in mental retardation is observed during the second year of life. In most patients a de novo sodium channel alfa-1 subunit (SCN1A) mutation can be identified. By reviewing the clinical, laboratory, and neuroimaging data of our SMEI patients diagnosed between 2000 and 2008, we would like to share our experiences in this rare but challenging syndrome. Our results will facilitate the earlier and better diagnosis of Hungarian children with SMEI. Patients and methods - Clinical, EEG, MRI and DNA mutation data of 20 SMEI patients treated in the Bethesda Children’s Hospital (Budapest) were reviewed. Results - The first seizure appeared at age 6.3±3.0 months. At least one of the first two seizures were complex febrile seizures in 19/20 and unilateral seizures in 12/20 children. All children except for one showed hemiconvulsions at least once; all children had seizures lasting longer than 15 minutes. Eight of twenty patients had SMEB. DNA diagnostics identified an SCN1A mutation in 17 patients (6 missense, 4 nonsense, 4 frameshift, 2 splice site, 1 deletion) while 3 children had no mutation. Conclusion - Early diagnosis of SMEI is important for the avoiding unnecessary examinations and false therapies as well as for genetic counselling. Typical symptoms of SMEI are early and prolonged febrile hemiconvulsions with neurological symptoms, mental retardation and secondary seizure types later on. The presence of an SCN1A mutation supports the diagnosis. We propose the availability of molecular diagnostics and stiripentol therapy for SMEI children in Hungary.]

Clinical Neuroscience

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[Authors summerized the etiology of convulsions in neonatal period and infancy (hypoxy, intracranial hemorrhage, infections of central nervous system, metabolic background, chromosomal abnormalities, brain developmental abnormalities, benign neonatal convulsions, benign neonatal familial convulsions, drug withdrawal, inborn error of metabolism). They suggest screening examinations after convulsion, summerized the basic princpile of tandem examination and review a proposal at suspicion of inborn error of enzym defects (aminoacidemias, defects of fatty acid oxydation, organic acidemias). They present case history of two patients suffered in extraordinary inborn error of enzym defect (SCO2 gene mutation, propionic acidemia). Diagnosis originated in Heim Pál Hospital (settlement Madarász Hospital) with a Hungarian and international cooperation.]

Clinical Neuroscience

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[Recent medical genetic research has identified a number of novel, or previously known, but rare conditions, caused by private founder mutations. The Finnish and Ashkenazi Jew populations provide the best examples for identifying genes in unique genetic disorders. In these populations, research efforts and high-level medical services resulted in intense improvements of medical care and in organization of population- based screening programs. Hereditary disorders of the Roma populations are known for a long time. The genetic background of these diseases has been established by extensive molecular genetic studies. The Romas represent 6% of the Hungarian population and live under extremely bad health conditions. Therefore, our aim was to map the incidence of the hereditary neuromuscular disorders among the Hungarian Roma population. Moreover, we intended to provide proper information, genetic counseling and possible prevention strategies for the families at risk, which should represent a primer task in public health. Because of our experience in neuromuscular disorders, we choose six, frequent, autosomal recessive disorders for these clinical and genetic studies: hereditary motor and sensory neuropathy type Lom (HMSNL), hereditary motor and sensory neuropathy type Russe (HMSNR), congenital cataracts facial dysmorphism syndrome (CCFDN), Limb- Girdle muscular dystrophy 2C (LGMD2C), congenital myasthenic syndrome (CMS) and spinal muscular atrophy (SMA). Following identification of the founder mutations, the possibility of prenatal diagnosis and carrier screening for family members will contribute to the decrease of the recurrence risk for these severe, mostly untreatable disorders.]

Clinical Neuroscience

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Clinical Neuroscience

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Purpose - Methylation is a key epigenetic modification of DNA and regarding its impact on epilepsy, it is argued that “DNA methylation may play an important role in seizure susceptibility and maintenance of the disorder”. DNA methylation status of KCC2 (SCL12A5) and NKCC1 (SCL12A2) associated with refractory temporal lobe epilepsy was investigated in our study. Materials and methods - Thirty-eight patients with temporal lobe epilepsy (TLE) who were diagnosed by video EEG monitoring and 32 healthy control subjects were included in the study. Twenty-three patients in TLE group were men and the remaining 15 were women. Among them, 27 had unilateral temporal focus (9 with right; 18 with left) and 11 patients had bilateral TLE. We analyzed promoter region methylation status of the KCC2 (SCL12A5) and NKCC1 (SCL12A2) genes in the case and control groups. Gene regions of interest were amplified through PCR and sequencing was accomplished with pyro-sequencing. Results - We found a significant relationship between TLE and methylation on the NKCC1. However, there was no association between TLE and methylation on the KCC2 gene. Also, we found no association between right or left and unilateral or bilateral foci of TLE. There was no relationship between TLE and methylation on the NKCC1and KCC2 genes in terms of mesial temporal sclerosis in cranial MRI, head trauma or febrile convulsions. Conclusion - The methylation of NKCC1 can be a mecha­nism of refractory temporal lobe epilepsy. There are limited findings about DNA methylation in TLE. Therefore, further studies with large sample sizes are necessary.

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Clinical Neuroscience

[PREDICTIVE FACTORS OF TEMPORAL LOBE SURGERY]

KELEMEN Anna, RÁSONYI György, SZŰCS Anna, FABÓ Dániel, HALÁSZ Péter

[The most effective type of epilepsy surgery in adults is temporal lobe epilepsy (TLE) surgery. Three quarter of the patients become seizure free, however the remaining patients experience seizures after resection. In our study we analyzed retrospectively the possible electro-clinical, neuroimaging and surgery-related outcome predictors in 94 adult patients who had anterior temporal lobectomy (ATL) from the material of Epilepsy Centre of the National Institute of Psychiatry and Neurology, Budapest since the beginning of the surgery program in 1989 until 2001. Three outcome endpoints were chosen: the seizure status at the last visit, the longest seizure free period and the time to the first non-acute postoperative seizure. The predictors were assessed by multivariate and Cox regression methods. After one year of surgery 72% of the patients were seizure free, after two years 67% and after five years 59%. Factors predicting favorable outcome in TLE surgery were: typical temporomesial aura, strictly unilateral interictal anterotemporal spikes, unilateral ictal onset, slow contralateral propagation, hippocampal sclerosis (HS) as etiology. Factors predicting poor outcome in TLE surgery were: increase in seizure frequency in the last two preoperative years, presence of preoperative psychiatric disturbances, ictal contralateral propagation, MRI lesion distant from the surgery site, incongruency of data of preoperative investigations, postoperative sequels and non-HS type MR residuum.]

Clinical Neuroscience

[The propagation and semiology of focal epileptic seizures. case presentations to the insula. theoretical considerations]

BALOGH Attila, BALOGH Attila ifj.

[Study objectives – The developing of diagnostical examinations in epileptology provides new challenges in seizure semiology. On the analysis of seizures it is important to examine the mechanisms of their propagation. The brain connectivity (based on the neuroimaging), the shadowing of the movement of excessive neuronal activity (based on computerized EEG and MEG methods), the cognition of the physiological and pathological brain networks are the foot- stone of the epileptic seizure propagation. The investigators prove, by means of case demonstrations of the role of the network nodes and the role of the epileptic hubs in the seizure symptomatology. Methods – The preoperative, intra and postoperative data are analised of three insular and one parietal epileptic patients in point of view of their seizure symptomes. Complex neuroimaging, noninvasive and invasive electrophysiology, intensive long-term video-EEG monitoring, computerized EEG analysis, fuctional mapping, intraoperative corticography were used. The etiology were confirmed with hystology. Results – It is observed that on seizure semiology our patients plays the insula a double role. In some cases, it is the focus of insular seizures with their symptoms difficult to identify. However, in the majority of cases and as a consequence of its rich neural connections, the insula has a peculiar property in the evolution of the symptomatogenic features of seizures. This observations are developing new relationships between the mechanism of seizure propagation and its semiological consequences. Conclusions – On epileptological point of view there are brain structures which has peculiar role in the „designe” of propagation of the epileptic excitement. The numerous new methods in neuroimaging and neurophysiology allowed the connectomical examination of the epileptic networks. The role of the epileptic diathesis is approachable with the metholdology of the brain connectivity. Theoretically the node of the epileptic network consist of the potential pathes where the localised excessive excitement can propagete. The route where the actual seizure can go adhead is determined by the actual edpileptic propensity of the above mentioned potential pathes.]

Clinical Neuroscience

[Sturge-Weber syndrome: clinical and radiological correlates in 86 patients]

FOGARASI András, LODDENKEMPER Tobias, MELLADO Cecilia, TUXHORN Ingrid, EVERS Georg, SARCO Dean, BURGESS C. Richard, HALÁSZ Péter, BARSI Péter, GYORSOK Zsuzsanna, GYIMESI Csilla, KÓBOR Jenõ, SIEGLER Zsuzsanna, JANSZKY József, JAKUS Rita, RÁSONYI György, EBNER Alois, WOERMANN Friedrich, SAHIN Mustafa

[Backgrounds and purpose - To correlate the extent of the leptomeningeal angiomatosis with clinical features in Sturge- Weber syndrome (SWS). Methods - The study group consisted of 86 consecutive patients aged two months to 56 (mean 7.9±10.3) years with SWS and epilepsy. Clinical and MRI data were analyzed. Results - Based on the extent of leptomeningeal angiomatosis, patients were divided into two subgroups: 43 patients had hemispheric angiomatosis and atrophy, whereas, another 43 had focal involvement. Nine of the 43 hemispherial patients (10%) showed bilateral involvement: all of these bilateral cases demonstrated dominance in a single side with hemispheric leptomeningeal angiomatosis and contralateral focal extension. Hemispheric and focal subgroups were clinically different. Patients with hemispheric SWS were younger at the age of epilepsy onset (p<0.001) and age at MRI examination (p<0.05). Neither gender, lateralization, duration of epilepsy, appearance of secondarily generalized seizures, nor seizure frequency revealed a significant difference between subgroups. Conclusion - Bilateral involvement is frequent and occurs in cases with a hemisperic involvement on one side. The age of epilepsy onset is related to the extent of leptomeningeal angiomatosis. Patients with hemispheric form of SWS presented with earlier age of seizure onset. Focal pial angiomatoses do not tend to progress (a longer duration is not associated with more frequent hemispheric involvement). Other variables including seizure frequency and secondary generalized tonic-clonic seizures are not associated with the extent of angiomatosis.]