Naz Yeni

Surgical outcome of patients with mesial temporal lobe epilepsy related to hippocampal sclerosis

Seizure outcome in mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE‐HS) that was evaluated according to a noninvasive protocol was assessed in 165 patients and reported using both Engels and ILAE classifications. The mean postoperative follow‐up was 5.0 ± 2.7 years. At the end of first year, 77.1% of patients were in Engel‐I, and 52.7% were in ILAE‐I. Antiepileptic drugs (AEDs) were discontinued in 41 patients (42.7%), all remained seizure‐free for >2 years that could be accepted as “cure.” Thirty‐six patients had recurrences, 19 had running‐down phenomena. Anterior temporal lobectomy (ATL) was performed in 27 patients with a better outcome when compared to patients operated by selective anterior hippocampectomy. Clinical risk factors for better and worse outcome, which show some similarity in different reports, seem to veil the main reason, which is the accurate delineation of epileptogenic zone considering the presence of different subgroups and underlying developmental pathologies.

Orgasm-induced Seizures: A Study of Six Patients

Summary:  Purpose: Reflex seizures are known as the epileptic seizures triggered by some specific stimuli in sensitive patients. They are often classified according to the stimuli that trigger them rather than by the type of the seizure. Epileptic seizures induced by sexual orgasm are very rare in the literature.

Lack of association between IL-1β/α gene polymorphisms and temporal lobe epilepsy with hippocampal sclerosis

Summary Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is one of the most common medically intractable epilepsy syndromes and the pathogenesis of HS remains highly obscure. Recent studies demonstrated controversial results about the relationship between interleukin (IL) gene polymorphism and epilepsy in different ethnic groups. This correlation was investigated in Turkish patients with MTLE-HS. The allele distribution of IL-1α and IL-1β in 47 patients of Turkish ancestry was determined and compared with 99 ethnically matched control subjects. Analysis of genotype frequencies between patients and controls showed no statistically significant difference ( p >0.05). Our data suggest that IL-1α and IL-1β gene polymorphisms do not act as a strong susceptibility factor for MTLE-HS in individuals of Turkish ancestry.

Memory in Patients with Drug-responsive Mesial Temporal Lobe Epilepsy and Hippocampal Sclerosis

Summary:  Purpose: Mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE‐HS) is the most common of the antiepileptic drug (AED)‐resistant seizure syndromes that are remediable mostly with surgery, although a small group of patients have benign prognosis with fewer seizures. Material‐specific memory impairment is an important feature in these patients and may be related to both the structural abnormality and the frequent seizures. In this study, we investigated the relation between memory deficit and HS by taking seizure frequency into account.

Low-grade temporal gliomas: Surgical strategy and long-term seizure outcome

Low-grade gliomas (LGGs) are generally located in temporal lobe and cause medically-intractable seizure so that surgical treatment becomes inevitable. This study includes a retrospective analysis of our patients with temporal LGGs retrieved from our epilepsy surgery data base and tries to present appropriate surgical approach and long-term seizure and anti-epileptic drug (AED) outcomes. Fifty-three patients including children and adults underwent surgery on temporal lobe LGGs and 35 patients were reached to report seizure and AED outcomes. On the non-dominant temporal lobe, anterior temporal resection with hippocampectomy whether mesial structure are involved or not is the appropriate approach. On the dominant temporal lobe mesial structures should be respected. However, total resection of the tumor should be the goal of surgery. Mean follow-up period was 8.3 years and favorable seizure outcome was found to be 91.4%. Surgery decreased AED usage and mean number of AED significantly decreased. Children also benefited from surgery as adults. Surgical treatment of tumor-related epilepsy from temporal lobe controls seizures, and total removal should be the main goal of surgery as neuropsychological testing permit.

Interobserver variability of seizure semiology between two neurologist and caregivers

PURPOSE We aimed to compare the extent of inter-observer variability in the description of seizure semiology between both neurologists and caregivers. METHOD We prospectively investigated 93 consecutive patients monitored over the past 5 years in our video-EEG unit. The videotaped seizures of the patients were reviewed independently by two neurologists who were blind to the clinical data. The questionnaires were completed by neurologists and caregivers. Interobserver rate of agreement between neurologists and caregivers was analyzed by using the kappa analysis and intraclass correlation coefficients. RESULTS There was excellent agreement for questions regarding whether the patients eyes remained open, laterality of head deviation, arm movements, and ictal period. On the other hand, interobserver rate of agreement was fair to moderate for the laterality of hand automatisms, the presence of nose-wiping, and oral clonic jerks. CONCLUSION Besides variability in interobserver agreement among clinicians, the variability or concordance between physicians and caregivers are also of great importance, especially in case of epilepsy, where the accurate description of the attacks is the major determinant of an accurate diagnosis.

Posthemiplegic focal limb dystonia: a report of two cases.

Posthemiplegic focal limb or hemidystonias are rare movement disorders usually due to vascular lesions of the contralateral basal ganglia. The pathogenesis of posthemiplegic dystonia is unknown and its management is usually difficult. In this paper, we report two patients who suffered from a single limb dystonia and hemidystonia, respectively. In the latter patient, hemidystonia developed due to an ischaemic cerebrovascular accident 2 or 3 months after the recovery of hemiplegia. Computed tomography and magnetic resonance imaging scans showed evidence of contralateral putamen and thalamus infarcts.

Four novel and two recurrent NHLRC1 (EPM2B) and EPM2A gene mutations leading to Lafora disease in six Turkish families

Lafora disease (LD) is a type of autosomal recessive, progressive myoclonus epilepsy resulting mostly from mutations in the EPM2A and NHLRC1 genes. Mutational analysis in both genes was initiated with the aim of establishing LD DNA diagnosis in Turkey. Four novel NHLRC1 (p.G131X, p.P69S and p.D82H) and EPM2A (p.V7A) and two recurrent NHLRC1 (p.D146N) and EPM2A (p.R241X) mutations were identified in six families. The delineation of causative mutations in patients provided early disease diagnosis for other family members and contributed to the knowledge of LD pathogenesis.

The role of sleep electroencephalography in patients with new onset epilepsy

PURPOSE An increased propensity for seizures is associated with different stages of the sleep-wake cycle. In this study, we prospectively analyzed patients with new-onset epilepsy and investigated the clinical correlates of the yield obtained from sleep electroencephalography (EEG) recordings in patients with a normal wakefulness EEG. METHODS All patients admitted to our epilepsy unit due to unprovoked epileptic seizures and not yet treated with antiepileptic drugs were recruited consecutively for the last three years. All had a routine EEG at wakefulness (WEEG), and those with no epileptiform activity had a video-EEG recording during sleep (SEEG). RESULTS We investigated a total of 241 patients; 129 patients (53.5%) had both wakefulness and sleep EEG recordings. The patients with abnormal WEEG were older than those with normal WEEG (p = 0.005). Abnormal WEEG was detected in only 31.2% of patients with focal seizures, but in 77.3% of patients with generalized seizures (p < 0.001). WEEG was abnormal in 44.0% of patients with diurnal seizures, but in 27.5% of nocturnal seizures (p = 0.007). Abnormal WEEG was present in 75.5% of patients with a presumed genetic origin and in 59.3% of patients with structural etiology (p < 0.001). Sleep EEG detected an abnormality in 41.8% of patients with normal WEEG; of these, 82.8% were focal abnormalities. In contrast, the majority of abnormalities detected in WEEG were generalized (55.8%, p < 0.001). CONCLUSION Our results showed a greater likelihood of abnormal WEEG in older patients and in those with generalized epilepsy, diurnally precipitating seizures, and epilepsy of presumed genetic origin.

Startle Response in Progressive Myoclonic Epilepsy.

Cortical reflex myoclonus is a typical feature of progressive myoclonic epilepsy (PME) in which it is accompanied by other types of mostly drug-resistant seizures and progressive neurological signs. Although PME is characterized by cortical hyperexcitability, studies have demonstrated atrophy and degenerative changes in the brainstem in various types of PME. Thus, we have questioned whether any stimuli may trigger a hyperactive response of brainstem reticular formation in PME and investigated the startle reflex in individuals with PME. We recorded the auditory startle response (ASR) and the startle response to somatosensory inputs (SSS) in patients with PME, and compared the results with healthy volunteers and patients with other types of drug-resistant epilepsy. All patients were using antiepileptic drugs (AEDs), 12 were on multiple AEDs. The probability of ASR was significantly lower and mean onset latency was longer in patients with PME compared with other groups. SSS responses over all muscles were low in both the PME and drug-resistant epilepsy groups; however, the differences were not statistically significant. The presence of a response over the biceps brachii muscle was zero in the PME group and showed a borderline difference compared with the other groups. Decreased probability and prolonged latencies of ASR in PME indicate inhibition of reflex circuit. A trend for decreased responses of SSS suggests hypoactive SSS in both PME and other epilepsy groups. Hypoactive ASR in PME and hypoactive SSS in both PME and other epilepsies may be attributed to the degeneration of pontine reticular nuclei in PME and functional inhibition by AEDs in both disorders.