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J Janszky

Publications and source records attributed to J Janszky.

At least 19 recordsLinked to original sources
PubMed2001

[Surgically treatable epilepsy--a review].

20-25% of epileptic patients do not become seizure free on adequate drug therapy. In 25-50% of patients with intractable epilepsy, the brain area responsible for seizures is well localizable and does not involve eloquent regions. In these patients, the surgical excision of the epileptic focus may lead to relief from seizures. In Hungary, there may be 5-6000 patients who needs an epilepsy surgery, but till now only 200 patients with chronic epilepsy underwent a surgical procedure. In the surgically remediable epilepsies, the operation is not a "ultima ratio". Concerning these syndromes, if 2-3 adequate antiepileptic drugs do not lead to seizure freedom within 1-3 years after the epilepsy onset, then a presurgical evaluation is necessary. The most common surgically remediable epilepsy is the temporal lobe epilepsy in which 60-90% of drug-resistant patients could be surgically cured. In lesional neocortical epilepsies 50-80% of patients become postoperatively seizure free. In childhood hemispheric epilepsies, the surgery could lead to seizure freedom in 70-80% of patients. The basic tools of the presurgical evaluation are the detailed history, the high resolution-MRI, the video-EEG monitoring, and the neuropsychological assessment. These investigation methods are usually enough to evaluate the necessity of the surgery and the postoperative outcome as well as to plan the localization and the extension of the resection. In some cases, ictal SPECT, PET, or video-EEG monitoring with intracranial electrodes could also be necessary in order to localize the epileptic focus.

Algorithms↗
PubMed2001

Seizures accelerate forgetting in patients with left-sided temporal lobe epilepsy.

Ten patients with refractory temporal lobe epilepsy performed a word-position association learning task every 24 hours during video EEG monitoring. On 55 occasions recall performance was tested 30 minutes and 24 hours after the initial learning phase. Patients with left- but not right-sided temporal lobe epilepsy exhibited impaired retention of word position if a seizure had occurred during the preceding 24-hour interval. Seizures may impair the consolidation of memory in patients with left-sided temporal lobe epilepsy beyond the chronic memory deficits caused by the underlying pathology.

Adult↗
PubMed2001

Lateralizing value of unilateral motor and somatosensory manifestations in frontal lobe seizures.

PURPOSE: To evaluate the lateralizing value of unilateral somatosensory aura, unilateral tonic posturing, head version, non-forced head turning, ictal cloni, dystonic posturing, and postictal nose wiping in seizures originating in the frontal lobe. METHODS: We included patients who had consecutively undergone presurgical evaluation with ictal video-EEG monitoring at our institution, had had resective epilepsy surgery involving the frontal lobe, and had remained seizure-free >1 year after operation. Twenty-seven patients aged 1-42 years (mean 18) met the inclusion criteria. Fifteen patients had right-sided, 12 patients had left-sided epileptogenic regions. Seizures recorded during EEG-video monitoring were re-evaluated by two investigators in order to identify lateralization signs in frontal lobe seizures. One of the investigators was blind to patients' clinical data. RESULTS: We analyzed 153 seizures of 27 patients. The most common unilateral phenomenon was the unilateral tonic posturing occurring in 48% of all the patients and in 25% of all seizures. Somatosensory aura and head version appeared exclusively contralateral whereas clonus occurred in 92% and unilateral tonic posturing in 89% of seizures contralateral to the epileptogenic region. Ictal non-forced head turning and postictal nose wiping showed no lateralizing significance. Dystonic posturing did not occur. CONCLUSIONS: Somatosensory aura, head version, ictal cloni, and tonic posturing are reliable lateralizing signs in frontal seizures. These signs may help in identifying the epileptogenic region during presurgical evaluation of patients suffering from frontal lobe epilepsy.

Adolescent↗
PubMed2001

A detailed analysis of frontal lobe seizure semiology in children younger than 7 years.

PURPOSE: We sought to analyze semiology of seizure onset and evolution in young children with frontal lobe epilepsy (FLE), compare this with adult reports, and assess age-related differences. METHODS: We analyzed 111 videotaped seizures from 14 patients with FLE based on focal cortical dysplasia aged 3-81 months (mean, 30 months). Ictal events were categorized into behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included tonic, clonic, epileptic spasm, and myoclonic seizure components. We developed a time-scaled datasheet to record each epileptic event as onset, very early, early, or late manifestation. RESULTS: Patients had a high seizure frequency with up to 40 attacks/day; half of them showed a cluster tendency. Forty-seven percent of the seizures started in sleep. Mean duration of seizures was short (29 s). Most common seizure components were motor manifestations, mostly tonic-clonic seizures, and epileptic spasms. Behavioral change was frequent, and hypermotor seizures were not seen. In five patients, the motor features were contralateral to the epileptic focus, including two children with asymmetric epileptic spasms. Secondarily generalized tonic-clonic seizures (SGTCSs) were not recorded, but had been reported in the history of two patients. Complex motor automatisms were not seen, whereas oral automatism appeared in three children. CONCLUSIONS: Motor features are common in young children with FLE, as reported in adults. The characteristics, however, differ. Epileptic spasms and subtle behavioral change were frequent. Hypermotor seizures and complex motor automatisms were not seen, and SGTCSs were unusual. Our results suggest that FLE in young children shows age-related features differing from those of adults.

Adult↗
PubMed2000

EEG predicts surgical outcome in lesional frontal lobe epilepsy.

BACKGROUND: Because of the relatively poor results of frontal lobe epilepsy (FLE) surgery, identification of prognostic factors for surgical outcome is of great importance. METHODS: To identify predictive factors for FLE surgery, we analyzed the data of 61 patients (mean age at surgery 19.2) who had undergone presurgical evaluation and resective surgery in the frontal lobe. Postoperative follow-up ranged from 0.5 to 5 years (mean 1.78). Fifty-nine patients had MRI-detectable lesions. Histopathologic examination showed dysplasia (57.4%), tumor (16.4%), or other lesions (26.2%). Thirty postoperatively seizure-free patients were compared with 31 non-seizure-free patients with respect to clinical history, seizure semiology, EEG and neuroimaging data, resected area, and postoperative data including histopathology. RESULTS: Three preoperative and two postoperative variables were related to poor outcome: generalized epileptiform discharges, generalized slowing, use of intracranial electrodes, incomplete resection detected by MRI, and postoperative epileptiform discharges. The only preoperative factor associated with seizure-free outcome was the absence of generalized EEG signs. Multivariate analysis showed that only the absence of generalized EEG signs predicts the outcome independently. Moreover, the occurrence of a somatosensory aura, secondarily generalized seizures, and negative MRI was identified as additional independent risk factors for poor surgical results. CONCLUSIONS: The absence of generalized EEG signs is the most predictive variable for a seizure-free outcome in FLE surgery. Furthermore, nonlesional MRI, somatosensory aura, and secondarily generalized seizures are risk factors for poor surgical results.

Adolescent↗
PubMed2000

Hippocampal malrotation with normal corpus callosum: a new entity?

Among 527 MRI examinations of patients with a suspicion of epilepsy in 5 years, we found 32 cases of hippocampal malrotation (HIMAL). The characteristic features are: incomplete inversion of the hippocampus with and abnormally round shape; unilateral involvement of the whole hippocampus; normal signal intensity and size; blurred internal structure; an abnormal angle of collateral sulcus; abnormal position and size of the fornix; normal size of the temporal lobe; enlargement and particular configuration of the temporal horn, typical of corpus callosum agenesis; and a normal corpus callosum. In 7 cases (22%) HIMAL occurred together with developmental disorders. It was predominantly seen in men. The clinical features were varied. Based on some MRI features, the presence of developmental disorders, the male predominance, the frequently positive family history, and a review of the literature, we think HIMAL may be the consequence of a mild hemisphere developmental disorder. It is probably not the basic cause of epilepsy in such varied clinical setting, but may be a sign of a developmental disorder and can help in selecting patients for more meticulous investigation. It also may give some new understanding of brain development.

Adult↗
PubMed2000

Are ictal vocalisations related to the lateralisation of frontal lobe epilepsy?

The purpose was to analyse whether non-speech vocalisations in seizures originating in the frontal lobe do have lateralising value. Patients were included who had undergone presurgical evaluation with ictal video-EEG monitoring at the Epilepsy Centre, had had resective epilepsy surgery involving the frontal lobe, and who had remained seizure free>1 year postoperatively. Twenty seven patients aged 1-42 years (mean 18) met the inclusion criteria. Age at epilepsy onset ranged from 1 month to 41 years (mean 7.1 years). All selected patients had a unilateral MRI detected lesion within the frontal lobe. Fifteen patients had right sided, 12 patients had left sided epileptogenic zones. Seizures recorded during EEG-video monitoring were re-evaluated to identify the occurrence of ictal vocalisations. Pure ictal vocalisations were distinguished from ictal sound productions due to motor or vegetative seizure activity (for example, cloni or respiratory sounds). Pure ictal vocalisation occurred in 11 patients of whom nine had a left frontal epileptogenic zone (p<0.01). It is concluded that ictal vocalisation could be an additional lateralising sign in frontal lobe epilepsy. The results suggest that not only speech, but vocalisation at a subverbal level also shows a left hemispheric dominance in humans.

Adolescent↗
PubMed1997

Unstable insertion in the 5' flanking region of the cystatin B gene is the most common mutation in progressive myoclonus epilepsy type 1, EPM1.

Progressive myoclonus epilepsy type 1 (EPM1, also known as Unverricht-Lundborg disease) is an autosomal recessive disorder characterized by progressively worsening myoclonic jerks, frequent generalized tonic-clonic seizures, and a slowly progressive decline in cognition. Recently, two mutations in the cystatin B gene (also known as stefin B, STFB) mapping to 21q22.3 have been implicated in the EPM1 phenotype: a G-->C substitution in the last nucleotide of intron 1 that was predicted to cause a splicing defect in one family, and a C-->T substitution that would change an Arg codon (CGA) to a stop codon (TGA) at amino acid position 68, resulting in a truncated cystatin B protein in two other families. A fourth family showed undetectable amounts of STFB mRNA by northern blot analysis in an affected individual. We present haplotype and mutational analyses of our collection of 20 unrelated EPM1 patients and families from different ethnic groups. We identify four different mutations, the most common of which consists of an unstable approximately 600-900 bp insertion which is resistant to PCR amplification. This insertion maps to a 12-bp polymorphic tandem repeat located in the 5' flanking region of the STFB gene, in the region of the promoter. The size of the insertion varies between different EPM1 chromosomes sharing a common haplotype and a common origin, suggesting some level of meiotic instability over the course of many generations. This dynamic mutation, which appears distinct from conventional trinucleotide repeat expansions, may arise via a novel mechanism related to the instability of tandemly repeated sequences.

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