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Original article
Temporal lobe epilepsy: neuropathological and clinical correlations in 243 surgically treated patients
Laura Tassi, Alessandra Meroni, Francesco Deleo, Flavio Villani, Roberto Mai, Giorgio Lo Russo, Nadia Colombo, Giuliano Avanzini, Chiara Falcone, Manuela Bramerio, Alberto Citterio, Rita Garbelli, Roberto Spreafico
of follow-up, and specimens sufficiently preserved to allow a precise evaluation of both lateral neocortex and hippocampus. The frequency of different types of lesion and hippocampal sclerosis (HS), isolated or associated with neocortical lesions, risk factors and surgical outcomes in relation to neuropathological findings were evaluated. We found tumours in 33% of patients, malformations of cortical development (MCD) in 45%, isolated HS in 14%, no lesion in 5% and less common lesions in 3%. HS was present in 8% of tumour cases and 70% of MCD. Statistical analysis of antecedents was significantly associated only with febrile seizures (FS). In MCD patients with no history of FS, a strong association between HS and duration of epilepsy was revealed. A Class I outcome was identified in 87% of cases with tumours and 79% in cases with MCD. In 93 patients the antiepileptic drug therapy was withdrawn. Our findings show that MCD, which is significantly associated with HS, is the most common lesion in TLE and support the concept that an optimal outcome is obtained when mesial and neocortical structures are removed. FS are particularly relevant in patients with focal cortical dysplasia and HS.
Electroencephalographic changes in pyridoxine-dependant epilepsy: new observations
Georges Naasan, Mohamad Yabroudi, Amal Rahi, Mohamad A Mikati
patients with PDE. Materials and methods. EEG tracings from four patients fulfilling the clinical criteria for PDE were reviewed. Relative to the time of treatment with pyridoxine, EEG recordings were available before treatment in two patients (at ages four and 10 months), immediately after treatment in two patients and during long-term follow-up with treatment in all four patients. Results. Pre-pyridoxine interictal EEG findings included: diffuse slowing, bilateral independent multifocal epileptiform discharges, generalized bursts of polyspike slow waves and focal or generalized sharp waves. In addition, the EEG was often asymmetrical and included: generalized semi-rhythmic sharp and slow waves, a burst suppression pattern and continuous generalized spike and slow waves. In one patient, who was followed subsequently, a decrease in multifocal spikes and sharp waves and permanent cessation of clinical seizures, within 10 minutes of concurrent reduction of spikes in the pre-existing generalized spike slow wave pattern, was observed immediately after pyridoxine treatment. However, despite the clinical response in this patient we observed persistent generalized burst suppression for four days, and fluctuation of the EEG with diffuse slowing on day four and transient exacerbation of discharges with continuous spike slow waves on day 22. This was followed by intermittent sharp waves at eight and 20 months, mild slowing at 31 months and normal EEG at 43 months. Long-term EEG findings in the other three patients receiving pyridoxine ranged between normal and intermittent multifocal sharp waves. Conclusion. Our data confirm previous observations and provide the following new findings: (1) the presence of burst suppression pattern after cessation of seizures can occur for up to five days after initiation of pyridoxine and should not exclude the diagnosis of PDE, (2) possible fluctuation and even transient worsening of electrographic discharges were observed for up to three weeks after initiation of pyridoxine and (3) the abnormal EEG can persist for up to 43 months before normalizing (range 1-43 months) and in other cases in which it continues to be abnormal it may still improve after increasing the dose of pyridoxine.
Effect of methylphenidate on the quality of life in children with epilepsy and attention deficit hyperactivity disorder:
Hanik K Yoo, Subin Park, Hee-Ryung Wang, Joong Sun Lee, Kunwoo Kim, Kyoung-Won Paik, Mi Sun Yum, Tae-Sung Ko
of children with epilepsy and ADHD. Twenty-five subjects (aged 10.1 ± 3.0 years) with ADHD and epilepsy were recruited at an outpatient clinic in Seoul, Korea. We used the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE), ADHD rating scale (ARS) and clinical global impression (CGI) in this study. Osmotic-controlled release oral delivery system (OROS) methylphenidate, 1.0 ± 0.4 mg/kg/day, was administered for 55.2 ± 7.5 days. The QOL subscales including physical restriction (p = 0.005), self-esteem (p = 0.002), memory (p < 0.001), language (p = 0.005), other cognition (p < 0.001), social interaction (p = 0.002), behaviour (p < 0.001), general health (p = 0.002) and QOL (p < 0.001) were significantly increased and the ARS (p < 0.001) and CGI-Severity of illness scores (p < 0.001) were significantly reduced after medication. Although 60% of subjects had experienced adverse effects, most were tolerable and only two subjects withdrew from the study owing to unbearable adverse effects (anorexia and insomnia). Two subjects had seizure attacks during the study period without having to discontinue the trial drug. Despite limitations related to the small sample size and the open design of the present pilot study, our results suggest that OROS methylphenidate may be well tolerated and effective in reducing ADHD symptoms and improving QOL in this patient population.
Myoclonic status epilepticus in juvenile myoclonic epilepsy
Julia Larch, Iris Unterberger, Gerhard Bauer, Johannes Reichsoellner, Giorgi Kuchukhidze, Eugen Trinka
precipitating factors and clinical course by studying patients with JME from a large outpatient epilepsy clinic. Methods. We retrospectively screened all patients with JME treated at the Department of Neurology, Medical University of Innsbruck, Austria between 1970 and 2007 for a history of MSE. We analyzed age, sex, age at seizure onset, seizure types, EEG, MRI/CT findings and response to antiepileptic drugs. Results. Seven patients (five women, two men; median age at time of MSE 31 years; range 17-73) with MSE out of a total of 247 patients with JME were identified. The median follow-up time was seven years (range 0-35), the incidence was 3.2/1,000 patient years. Median duration of epilepsy before MSE was 26 years (range 10-58). We identified three subtypes: 1) MSE with myoclonic seizures only in two patients, 2) MSE with generalized tonic clonic seizures in three, and 3) generalized tonic clonic seizures with myoclonic absence status in two patients. All patients responded promptly to benzodiazepines. One patient had repeated episodes of MSE. Precipitating events were identified in all but one patient. Drug withdrawal was identified in four patients, one of whom had additional sleep deprivation and alcohol intake. Two patients received inappropriate treatment (carbamazepine, phenytoin). Conclusions. MSE is a rare event in JME. Precipitating factors are commonly identified and for such cases the treatment response and outcome are excellent, in contrast to other cases with unknown causes.
Clinical commentary
Convulsive syncope: a condition to be differentiated from epilepsy
Cigdem Ozkara, Baris Metin, Serdar Kucukoglu
the episodes, head/eye deviations, automatisms and dystonic movements, suggesting an epileptic seizure, were observed. The EEG revealed diffuse slow waves when the patient lost consciousness and complete cessation of the cerebral activity occurred when the dystonic movements started. On ECG recordings, bradycardia, followed by complete asystolia lasting for 40 seconds, was observed. We conclude that the differential diagnosis of epilepsy and syncope can be quite misleading and clinical features may not always be reliable. In cases where diagnosis is uncertain, circulatory and cardiac causes should always be kept in mind and video-EEG with simultaneous cardiac recordings are mandatory for accuracy of diagnosis. [Published with video sequences]
Obstructive sleep apnea syndrome and nocturnal epilepsy with tonic seizures
Piotr Bialasiewicz, Dariusz Nowak
The prolonged apneic periods with tonic muscle contracture lasting minutes and occurring exclusively at night with ensuing confusional state posed diagnostic difficulties because of the negative EEG at the beginning of the workup and the absence of other epilepsy symptoms (i.e. clonic phase, tongue biting, enuresis, seizures while awake). Numerous apneas on polysomnography led to the diagnosis of sleep apnea syndrome. No effect of continuous positive airways pressure (CPAP) treatment on frequency of the nocturnal tonic epileptic fits and the repetitive character of the clinical presentation combined with the typical pathologic changes on subsequent EEGs permitted to suggest the epileptic nature of the paroxysmal events. Episodes stopped following administration of clonazepam. However, spontaneous, coincidental remission of seizures cannot be excluded since the patient remained seizure free even after discontinuation of the drug.
Paroxysmal episodes, “re-build up” phenomenon and moyamoya disease
Nomazulu Dlamini, Sushma Goyal, Jozef Jarosz, Timothy Hampton, Ata Siddiqui, Elaine Hughes
The electroencephalographic finding characteristically seen after hyperventilation in about 50% of children with cerebrovascular disease includes gradual frequency decrease and activation of amplitude of slow waves which appear after the disappearance or attenuation of ordinary build up. This is termed the “re-build up” phenomenon, which is rarely seen and therefore may be under-recognized. We present video telemetry during a transient ischaemic event of a child subsequently diagnosed with moyamoya-like syndrome. We highlight the potential for misdiagnosis of organic non-epileptic events. Hyperventilation during EEG should be avoided in children with known moyamoya disease. [Published with video sequences]
Acetaminophen toxicity with concomitant use of carbamazepine
Glen Jickling, Angela Heino, S Nizam Ahmed
even at conventional therapeutic doses. We report a case of a 34-year-old man on carbamazepine for complex partial seizures who developed acute liver and renal failure on less than 2.5 grams a day of acetaminophen. This raises caution that patients on carbamazepine should avoid chronic use of acetaminophen, and if required use at lower doses with vigilant monitoring for signs of liver damage.
Hypothalamic hamartoma causing gelastic seizures treated with stereotactic radiofrequency thermocoagulation
Wei Wang, Weimin Wang, Xiaofei Guo, Yanjun Zeng, Xiaodan Jiang
man presented with intractable gelastic seizures and focal seizures refractory to medical treatment. Magnetic resonance imaging showed a 6 mm × 6 mm × 7 mm sessile intraventricular HH. Under local anesthesia, four intra-hamartoma lesions were made via stereotactic radiofrequency thermocoagulation using a depth electrode for recording and stimulation. Results. Transient central hyperthermia, hypertension, and tachycardia were observed during the coagulation procedure. Intra-hamartoma spikes and slow waves were identified on depth electrode recordings. No gelastic seizure was induced by deep stimulation. The patient was seizure-free during the 12-month follow up and no permanent surgical complications occurred. Conclusion. Stereotactic radiofrequency thermocoagulation may be an effective and safe treatment option in selected cases of hypothalamic hamartoma with gelastic seizures.
Zonisamide in West syndrome: an open label study
Mi-Sun Yum, Tae-Sung Ko
of infantile spasms, there are still limitations of their use. We determined the efficacy and tolerability of high-dose zonisamide in patients with recent-onset infantile spasms. Methods. Seventeen patients with infantile spasms, who were admitted to our hospital between October 2005 and November 2007, were eligible for enrolment within two months of diagnosis. Zonisamide was administered at a starting dose of 2-8 mg/kg/day, increasing by 2-5 mg/kg/day every three to four days until the seizures disappeared or the dose reached 30 mg/kg/day. Complete response was defined as clinical cessation of infantile spasms over 28 consecutive days and disappearance of hypsarrhythmia by EEG analysis. Results. Of the 17 treated patients (nine who received initial monotherapy and eight add-on therapy), five of 12 (42.0%) cryptogenic patients and two of five (40.0%) symptomatic patients showed complete disappearance of spasms. The maximum daily dose was 10–28 mg/kg, and the effective daily dose was 10-22 mg/kg. Mean time period before disappearance of spasms and hypsarrhythmia was eight days. Seizure recurrence was observed in three of the seven patients who showed complete disappearance of spasms. Adverse effects included irritability in four patients and poor oral intake in two patients. Conclusion. Although further study of zonisamide is warranted, high-dose zonisamide can be effective and safe in some infants with newly diagnosed West syndrome.
Congresses and meetings
Articles with video-sequences published in 2009
