Ictal kissing: occurrence and etiology.

Ictal kissing is a rare semiological manifestation in patients with epilepsy. We tried to estimate its occurrence and characterize the underlying etiology. We retrospectively reviewed all video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004-2015) for the occurrence of the term kiss . We then searched the electronic database PubMed on September 21, 2016 using the following search terms in the English language: ictal OR seizure OR epilepsy AND kiss OR kissing . Relevant original studies were included. During the study period, 5133 patients were investigated in our epilepsy monitoring unit. One patient (0.02%) had one episode of documented ictal kissing. He had drug-resistant right mesial temporal lobe epilepsy. In total, five studies met the criteria for inclusion in the review. These studies described nine patients with drug-resistant focal epilepsy and ictal kissing behavior. Six out of ten patients with ictal kissing had hippocampal sclerosis. We add to the literature on ictal kissing by providing additional information on its frequency and etiology. The most common underlying etiology for ictal kissing is hippocampal sclerosis. However, this semiological ictal phenomenon is not pathognomonic for any etiology or localization

Ictal pain: occurrence, clinical features, and underlying etiologies.

PURPOSE: We analyzed a series of patients with ictal pain to estimate its occurrence and characterize the underlying etiologies. METHODS: We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004-2015) for the occurrence of the term pain in the text body. All the extracted reports were reviewed, and patients with at least one documented episode of ictal pain in the epilepsy monitoring unit (EMU) were included in the study. RESULTS: During the study period, 5133 patients were investigated in our EMU. Forty-six patients (0.9%) had at least one documented episode of ictal pain. Twenty-four patients (0.5%) had psychogenic nonepileptic seizures (PNES), 10 patients (0.2%) had epilepsy, 11 patients (0.2%) had migraine, and one woman had a cardiac problem. Pain location was in the upper or lower extremities (with or without other locations) in 80% of the patients with epilepsy, 33% of the patients with PNES (p=0.01), and none of the patients with migraine. CONCLUSION: Ictal pain is a rare finding among patients evaluated in EMUs. Psychogenic nonepileptic seizures are the most common cause, but ictal pain is not specific for this diagnosis. Location of the ictal pain in a limb may help differentiate an epileptic cause from others

Auras in patients with temporal lobe epilepsy and mesial temporal sclerosis.

We investigated auras in patients with drug-resistant temporal lobe epilepsy (TLE) and mesial temporal sclerosis (MTS). We also investigated the clinical differences between patients with MTS and abdominal auras and those with MTS and non-mesial temporal auras. All patients with drug-resistant TLE and unilateral MTS who underwent epilepsy surgery at Jefferson Comprehensive Epilepsy Center from 1986 through 2014 were evaluated. Patients with good postoperative seizure outcome were investigated. One hundred forty-nine patients (71 males and 78 females) were studied. Thirty-one patients (20.8%) reported no auras, while 29 patients (19.5%) reported abdominal aura, and 30 patients (20.1%) reported non-mesial temporal auras; 16 patients (10.7%) had sensory auras, 11 patients (7.4%) had auditory auras, and five patients (3.4%) reported visual auras. A history of preoperative tonic-clonic seizures was strongly associated with non-mesial temporal auras (odds ratio 3.8; 95% CI: 1.15-12.98; p=0.02). About one-fifth of patients who had MTS in their MRI and responded well to surgery reported auras that are historically associated with non-mesial temporal structures. However, the presence of presumed non-mesial temporal auras in a patient with MTS may herald a more widespread epileptogenic zone

Ictal crying

PURPOSE: The purpose of this study was to describe a series of patients with ictal crying to estimate its occurrence and characterize the clinical features and the underlying etiology. METHODS: We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004-2015) for the occurrence of the terms cry or sob or weep in the text body. All the extracted reports were reviewed, and patients with at least one episode of documented ictal crying at the epilepsy monitoring unit (EMU) were included in the study. RESULTS: During the study period, 5133 patients were investigated at our EMU. Thirty-two patients (0.6%) had at least one documented seizure accompanied by crying. Twenty-seven patients (26 women and one man) had psychogenic nonepileptic seizures (PNES), and five patients (0.1%) had epilepsy. Among patients with epileptic ictal crying, four patients had focal epilepsy (two had definite, and two had probable frontal lobe epilepsy), while one patient had Lennox-Gastaut syndrome. CONCLUSION: Ictal crying is a rare finding among patients evaluated at the EMUs. The most common underlying etiology for ictal crying is PNES. However, ictal crying is not a specific sign for PNES. Epileptic ictal crying is often a rare type of partial seizure in patients with focal epilepsy. Dacrystic seizures do not provide clinical value in predicting localization of the epileptogenic zone

Historical Risk Factors Associated with Seizure Outcome After Surgery for Drug-Resistant Mesial Temporal Lobe Epilepsy.

OBJECTIVE: To investigate the possible influence of risk factors on seizure outcome after surgery for drug-resistant temporal lobe epilepsy (TLE) and mesial temporal sclerosis (MTS). METHODS: This retrospective study recruited patients with drug-resistant MTS-TLE who underwent epilepsy surgery at Jefferson Comprehensive Epilepsy Center and were followed for a minimum of 1 year. Patients had been prospectively registered in a database from 1986 through 2014. After surgery outcome was classified into 2 groups: seizure-free or relapsed. The possible risk factors influencing long-term outcome after surgery were investigated. RESULTS: A total of 275 patients with MTS-TLE were studied. Two thirds of the patients had Engel\u27s class 1 outcome and 48.4% of the patients had sustained seizure freedom, with no seizures since surgery. Patients with a history of tonic-clonic seizures in the year preceding surgery were more likely to experience seizure recurrence (odds ratio, 2.4; 95% confidence interval 1.19-4.80; P = 0.01). Gender, race, family history of epilepsy, history of febrile seizure, history of status epilepticus, duration of disease before surgery, intelligence quotient, and seizure frequency were not predictors of outcome. CONCLUSIONS: Many patients with drug-resistant MTS-TLE respond favorably to surgery. It is critical to distinguish among different types and etiologies of TLE when predicting outcome after surgery

Mesial temporal lobe epilepsy with childhood febrile seizure.

OBJECTIVES: To evaluate the demographic and clinical manifestations of patients with mesial temporal sclerosis and temporal lobe epilepsy (MTS-TLE) with childhood febrile seizure (FS) and establishing the potential differences as compared to those without FS. We also investigated the surgery outcome in these two groups of patients. MATERIALS AND METHODS: In this retrospective study, all patients with a clinical diagnosis of drug-resistant TLE due to mesial temporal sclerosis, who underwent epilepsy surgery at Jefferson Comprehensive Epilepsy Center, were recruited. Patients were prospectively registered in a database from 1986 through 2014. Postsurgical outcome was classified into two groups; seizure-free or relapsed. Clinical manifestations and outcome were compared between patients with MTS-TLE with FS and those without FS. RESULTS: Two hundred and sixty-two patients were eligible for this study. One hundred and seventy patients (64.9%) did not have FS in their childhood, while 92 patients (35.1%) reported experiencing FS in their childhood. Demographic and clinical characteristics of these two groups of patients were not different. Postoperative seizure outcome was not statistically different between these two groups of patients (P = 0.19). CONCLUSIONS: When MTS is the pathological substrate of TLE, clinical manifestations and response to surgical treatment of patients are very similar in patients with history of febrile seizure in their childhood compared to those without such an experience. In other words, when the subgroup of patients with MTS-TLE and drug-resistant seizures is examined history of childhood febrile seizure loses its value as a distinguishing factor in characteristics or predictive factor for surgery outcome

Hemi-ESES associated with agenesis of the corpus callosum and normal cognition.

Corpus callosum plays the important role in bilateral synchronous expression of focal discharges of ESES. Sparing dominant hemisphere form continuous spike and slow waves during sleep accounts for normal cognitive scores. Early detection and treatment of ESES have a great impact on cognitive and language scores and final prognosis

Efficacy and tolerability of adjunctive brivaracetam in patients with prior antiepileptic drug exposure: A post-hoc study.

Brivaracetam (BRV), a selective, high-affinity ligand for synaptic vesicle protein 2A, is a new antiepileptic drug (AED) for adjunctive treatment of focal (partial-onset) seizures in adults with epilepsy. This post-hoc analysis was conducted to explore the efficacy of adjunctive BRV in patients with prior levetiracetam (LEV) exposure and whether changes in efficacy were related to the similar mechanism of action of these two drugs. Data were pooled from three Phase III studies (NCT00490035; NCT00464269; NCT01261325) of adults with focal seizures taking 1-2 AEDs who received placebo or BRV 50-200mg/day without titration over a 12-week treatment period. Patients taking concomitant LEV at enrollment were excluded from this analysis. Patients were categorized by their status of prior exposure to LEV, carbamazepine (CBZ), topiramate (TPM), or lamotrigine (LTG), to investigate any consistent trend towards reduced response in AED-exposed subgroups compared to AED-naïve subgroups, regardless of the mechanism of action. Study completion rates, percent reduction from baseline in focal seizure frequency over placebo, ≥50% responder rates, and tolerability were evaluated for each subgroup. A total of 1160 patients were investigated. Study completion rates were similar in the AED-exposed subgroups and AED-naïve subgroups. In subgroups with (531 patients) or without (629 patients) prior LEV exposure, ≥50% responder rates for each dose of BRV compared with placebo were generally higher among the LEV-naïve subgroups than the previously LEV-exposed subgroups. LEV-exposed subgroups receiving BRV doses ≥50mg/day showed greater ≥50% responder rates than those receiving placebo. Similar results were observed for CBZ, TPM, and LTG. Previous treatment failure with commonly prescribed AEDs (LEV, CBZ, TPM, or LTG) is associated with a reduced response to BRV irrespective of the mechanism of action. Hence, this post-hoc analysis indicates that previous treatment failure with LEV does not preclude the use of BRV in patients with epilepsy

West Syndrome in South Iran: Electro-Clinical Manifestations

How to Cite This Article: Asadi-Pooya AA, Sharifzade M. West Syndrome in South Iran: Electro-Clinical Manifestations. Iran J Child Neurol. 2013 Summer; 7(3): 40-44.ObjectiveWe aimed to determine the clinical and electroencephalographic (EEG) characteristics of the patients with West syndrome (WS) in south Iran.Materials & MethodsIn this retrospective study, all patients with a clinical diagnosis of WS were recruited in the outpatient epilepsy clinic at Shiraz University of Medical Sciences between September 2008 and May 2012. Age, gender, age at seizure onset, seizure type(s), epilepsy risk factors, EEG and imaging studies of all patients were registered routinely.ResultsDuring the study period, 2500 patients with epilepsy were registered at our epilepsy clinic. Thirty-two patients (1.3%) were diagnosed to have WS. Age of onset (mean ± standard deviation) was 4.99 ± 3.06 months. Sixteen patients were male and 16 were female. Nine (28.1%) were reported to have two or more seizure types and 23 (71.8%) had one seizure type (epileptic spasms). At referral, no developmental delay was detected in two patients and in the rest, a mild to severe delay was noted.Electroencephalography showed typical hypsarrhythmia in 59.4% of our patients and modified hypsarrhythmia or atypical presentations were seen in 40.6%. Two patients had pyridoxine (B6)-dependent seizures, confirmed by oral B6 trial.ConclusionVariants of the classical triad of WS including other seizure types, atypical EEG findings, and normal psychomotor function at the beginning could be observed in some patients. Rarely, treatable genetic disorders (e.g., pyridoxine-dependent seizures) should be considered in those in whom no other diagnosis is evident. References1. Blume WT, Lüders HO, Mizrahi E, Tassinari C, van Emde Boas W, Engel J Jr. Glossary of descriptive terminology for ictal semiology: report of the ILAE task force on classification and terminology. Epilepsia. 2001 Sep;42(9):1212-8.2. Carmant L. Infantile spasms: West syndrome. Arch Neurol. 2002 Feb;59(2):317-8.3. Hrachovy RA. West’s syndrome (infantile spasms).Clinical description and diagnosis. Adv Exp Med Biol.2002;497:33-50. Review.4. Riikonen R, Donner M. Incidence and aetiology of infantile spasms from 1960 to 1976: a population study in Finland. Dev Med Child Neurol. 1979 Jun;21(3):333-43.5. Dulac O. What is West syndrome? Brain Dev. 2001 Nov;23(7):447-52. Review. 6. Wong V. West syndrome-The University of Hong Kong experience (1970-2000). Brain Dev. 2001 Nov;23(7):609-15.7. Kalra V, Gulati S, Pandey RM, Menon S. West syndrome and other infantile epileptic encephalopathies -Indian hospital experience. Brain Dev. 2001 Nov; 23(7):593-602. Corrected and republished in: Brain Dev. 2002 Mar;24(2):130-9.8. Young C; Taiwan Child Neurology Society. National survey of West syndrome in Taiwan. Brain Dev. 2001 Nov;23(7):570-4.9. Asadi-Pooya AA, Mintzer S, Sperling MR. Nutritional supplements, foods, and epilepsy: is there a relationship?Epilepsia. 2008 Nov;49(11):1819-27.10. Mikati MA, Trevathan E, Krishnamoorthy KS, Lombroso CT. Pyridoxine - dependent epilepsy: EEG investigations and long-term follow-up. Electroencephalogr Clin Neurophysiol. 1991 Mar;78(3):215-21.11. Nabbout R, Soufflet C, Plouin P, Dulac O. Pyridoxine dependent epilepsy: a suggestive electroclinical pattern. Arch Dis Child Fetal Neonatal Ed. 1999 Sep;81(2):F125-9.12. Gospe SM Jr. Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment. Curr Opin Neurol. 2006 Apr;19(2):148-53. Review.13.Riikonen R. The latest on infantile spasms. Curr Opin Neurol. 2005 Apr;18(2):91-5. Review

Ictal verbal help-seeking: Occurrence and the underlying etiology.

PURPOSE: Ictal verbal help-seeking has never been systematically studied before. In this study, we evaluated a series of patients with ictal verbal help-seeking to characterize its frequency and underlying etiology. METHODS: We retrospectively reviewed all the long-term video-EEG reports from Jefferson Comprehensive Epilepsy Center over a 12-year period (2004-2015) for the occurrence of the term help in the text body. All the extracted reports were reviewed and patients with at least one episode of documented ictal verbal help-seeking in epilepsy monitoring unit (EMU) were studied. For each patient, the data were reviewed from the electronic medical records, EMU report, and neuroimaging records. RESULTS: During the study period, 5133 patients were investigated in our EMU. Twelve patients (0.23%) had at least one episode of documented ictal verbal help-seeking. Nine patients (six women and three men) had epilepsy and three patients (two women and one man) had psychogenic nonepileptic seizures (PNES). Seven out of nine patients with epilepsy had temporal lobe epilepsy; six patients had right temporal lobe epilepsy. CONCLUSION: Ictal verbal help-seeking is a rare finding among patients evaluated in epilepsy monitoring units. Ictal verbal help-seeking may suggest that seizures arise in or propagate to the right temporal lobe