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Invited Editorial Comment
Temporal lobe seizures, amnesia and autoantibodies – identifying a potentially reversible form of non-paraneoplastic limbic enc
Angela Vincent, Christian G Bien
Review article
Chromosomal disorders associated with epilepsy
Agatino Battaglia, Renzo Guerrini
with different seizure types, but there are a few aberrations featuring specific seizure and electroencephalographic (EEG) patterns. The analysis of electro-clinical patterns associated with chromosomal aberrations is a major tool in the identification of epilepsy susceptibility genes. Advances in molecular cytogenetic techniques will certainly increase the diagnostic yield, and an increasing number of individuals previously diagnosed as having "cryptogenic" epilepsy will turn out to have an underlying chromosomal aberration. We review the types of seizures, EEG findings, and their natural history in the chromosomal disorders that are consistently associated with epilepsy.
Original article
Ictal dysprosody and the role of the non-dominant frontal operculum
Alexandra Montavont, Geneviève Demarquay, Marc Guenot, Jean Isnard, François Mauguière, Philippe Ryvlin
information. The prosodic component of ictal speech has not yet been specifically studied in epileptic patients. We report the case of a patient with right mesial temporal lobe epilepsy who developed ictal, recurrent speech utterances associated with an altered prosody during both spontaneous seizures and a very localized stimulation-induced discharge of the non-dominant precentral operculum, a finding consistent with results of functional magnetic resonance imaging studies in healthy subjects. This should prompt future studies, with the aim of better evaluating the localizing value of ictal dysprosody in patients with drug resistant, partial epilepsy.[Published with videosequences]
Heart rate increase in otherwise subclinical seizures is different in temporal versus extratemporal seizure onset: support for
Sabine Weil, Stephan Arnold, Ilonka Eisensehr, Soheyl Noachtar
stress response. In addition, we aimed to evaluate the localizing significance of pure ictal tachycardia. We included 21 epilepsy patients, who showed an ictal EEG seizure pattern during 22, otherwise subclinical seizures. All patients underwent ictal video-EEG recordings to evaluate the possibility of resective epilepsy surgery. The changes in heart rate in these patients were investigated in order to determine their relationship to localization and duration of EEG seizure patterns. Ictal tachycardia was observed in 41% of the otherwise subclinical seizures (nine out of 22), and significantly more often in seizures arising from the temporal lobe than from extratemporal regions (62% versus 11%, p < 0.0018). The seizure duration as defined by EEG was significantly positively correlated with an increase of heart rate (p = 0.043). Ictal heart rate can increase as a result of epileptic activation of autonomic cortex, reflecting a temporal lobe autonomic influence. Thus, measurement of heart rate should be included in the evaluation of otherwise subclinical epileptic seizures, because of its localizing value.
Pilomotor seizures and status in non-paraneoplastic limbic encephalitis
Stephan Wieser, Anna Kelemen, Peter Barsi, Angela Vincent, Csaba Borbely, Gyorgy Rasonyi, Susanne Mueller, Klaus Hess, Heinz Gregor Wieser, Peter Halasz
performed video-EEG monitoring, structural MRI,
<sup>18</sup>F-FDG-PET,
<sup>1</sup>H-MRS, neuropsychological testing and antibody serology. Results. A 42-year-old male patient presented in an acute phase of non-paraneoplastic limbic encephalitis confirmed by MRI, with antibodies to voltage-gated potassium channels. His pilomotor status was pharmacoresistant to antiepileptic drugs, but responded to corticosteroid and azathioprine treatment. The MRI findings improved. The pilomotor seizures recurred when the immunosuppressive therapy was discontinued after 18 months. MRI at that time was consistent with hippocampal sclerosis. Complete seizure control was achieved after reintroduction of steroids. Conclusion. Pilomotor seizures were the predominant seizure type in this case of non-paraneoplastic limbic encephalitis. Immunosuppressive therapy may provide recovery including seizure control. However, long-term immunosuppression may be necessary to prevent relapse. Hippocampal sclerosis and chronic epilepsy might evolve as sequelae of limbic encephalitis.
Mortality in adult patients with epilepsy in Taiwan
Rong-Chi Chen, Yang-Chyuan Chang, Tony Hsiu-Hsi Chen, Hui-Min Wu, Horng-Huei Liou
and 31 December 1991, were prospectively enrolled and followed up until 31 December 2000. A total of 32 deaths were reported. Overall case-fatality rate was 12.2%. The age-adjusted standard mortality ratio (SMR) was calculated to compare the risk of death in patients with epilepsy to the general population. Patients with epilepsy had a 3.5-fold higher risk of death as compared with the general population (SMR: 3.47, 95% CI: 2.46-4.91). The Cox proportional hazards regression model was used to assess relevant clinical contributions to death. Patients with an age-at-onset ≥ 40 years had a 4-fold higher risk of death as compared with those with an earlier onset. The multivariate analysis revealed that age-at-onset between 40 and 59 years, tumor etiology, and being male increased the risk of death in epilepsy. One-third of the deaths in patients with age-at-onset between 40-59 years died of liver cirrhosis and hepatoma. Hepatitis B virus infection is endemic in Taiwan, and this is closely associated with liver cirrhosis and hepatoma. Whether anticonvulsants contributed to the hepatotoxicity that led to fatal liver disease in this group needs further investigation.
Tonic seizures in subacute sclerosing panencephalitis: a video-illustration of two cases
Veysi Demirbilek, Gulcin Benbir, Ozlem Cokar, Destina Yalcin, Hicran Bulut, Aysin Dervent
report two cases, documented by video-EEG that during the course of the disease also presented with tonic seizures. The differential diagnosis of non-epileptic paroxysmal events might prove to be a problem.[Published with videosequences]
Atypical absences and recurrent absence status in an adult with Angelman syndrome due to the UBE3A mutation
Alberto J Espay, Danielle M Andrade, Richard A Wennberg, Anthony E Lang
15q11-13. We report the case of a 29-year-old, mentally retarded man with unusual electroencephalographic changes during periods of atypical absence status epilepticus, a previously unreported manifestation of the usually milder, drug-responsive epilepsy associated with Angelman syndrome due to the UBE3A mutation.[Published with video sequences]
Clinical experience with levetiracetam in idiopathic generalized epilepsy according to different syndrome subtypes
Carlo Di Bonaventura, Jinane Fattouch, Francesco Mari, Gabriella Egeo, Anna Elisabetta Vaudano, Massimiliano Prencipe, Mario Manfredi, Anna Teresa Giallonardo
16 women) affected by idiopathic generalized epilepsy were followed for at least 6 months following the introduction of levetiracetam. Patients were categorized according to syndrome subtype: juvenile myoclonic epilepsy (8), juvenile absence epilepsy (5), childhood absence epilepsy (4), and eyelid myoclonia with absences (2). Eleven patients received levetiracetam as monotherapy, eight as add-on therapy. Effectiveness was demonstrated in 18 patients: 13 became seizure-free (five juvenile myoclonic epilepsy, five juvenile absence epilepsy, three childhood absence epilepsy), and five achieved significant reductions in seizure frequency (three juvenile myoclonic epilepsy, one childhood absence epilepsy, one eyelid myoclonia with absences). Only one patient experienced no change in seizure frequency (eyelid myoclonia with absences). Clinical improvement was accompanied by EEG abnormality suppression or reduction. Levetiracetam was well tolerated; no patient reported side-effects.
Topiramate: a prospective study on the relationship between concentration, dosage and adverse events in epileptic patients on c
Walter Fröscher, Katja R Schier, Markus Hoffmann, Andreas Meyer, Theodor W May, Bernhard Rambeck, Johannes Rösche
study in patients with poorly controlled epilepsy treated with TPM, predominantly in combination with other antiepileptic drugs. The goal of the study was to investigate the relationship between the occurrence of adverse events due to TPM and its serum concentration or dosage, respectively. Methods. The relationship between the occurrence of adverse events and TPM serum concentration or dosage, respectively, was examined in a group of 42 young adult and adult patients with poorly controlled epilepsy. Within 22 months, all patients treated with TPM had been included in the study. The 8 adverse events occurring most frequently (difference ≥ 10%) in TPM-treated patients in 5, double-blind, placebo-controlled, parallel group studies, were checked regularly. This side effect profile has been presented by Reife et al. (1995a). Other possible or probable adverse events were also documented. Results. The difference in TPM serum concentrations and TPM dosages (mg/kg) for patients without an adverse event, and patients with a given adverse event was statistically significant for “abnormal thinking, impaired concentration, weight loss, dizziness, speech problems, somnolence, ataxia, increased seizure frequency and paresthesia”. To avoid adverse events, we recommend an initial “maintenance serum concentration” of below 4 μg/mL. As regards the TPM dosage, our results suggest initial maintenance dosages of 100 TPM or lower, 1.5 mg/kg or lower, respectively. These conclusions are limited by the relatively small number of patients.
Case report
A case of systemic lupus erythematosus with complex partial status epilepticus
Masayasu Tsuji, Hisashi Tanaka, Masahito Yamakawa, Ryuichi Sagawa, Hideki Azuma
initially considered to be psychotic in nature, due to SLE. However, the EEG demonstrated the epileptic etiology of her symptoms. In patients with lupus presenting with prolonged episodes of confusion and behavioural disturbance, an EEG should be performed, whenever possible during the episode, even in the absence of a previous history of epilepsy.
Conference report
Nonconvulsive status epilepticus: Epilepsy Research Foundation Workshop Reports
Matthew Walker, Helen Cross, Shelagh Smith, Camilla Young, Jean Aicardi, Richard Appleton, Sarah Aylett, Frank Besag, Hannah Cock, Robert DeLorenzo, Franck Drislane, John Duncan, Colin Ferrie, Denson Fujikawa, William Gray, Peter Kaplan, Micheal Koutroumanidis, Mary O’Regan, Perrine Plouin, Josemir Sander, Rod Scott, Simon Shorvon, David Treiman, Claude Wasterlain, Udo Wieshmann
(a charitable organization), to discuss and debate the definition, diagnosis and treatment of nonconvulsive status epilepticus. We felt that such a meeting would be useful, as nonconvulsive status epilepticus is a subject that provokes strong reactions, perhaps largely due to the relative lack of evidence and the surfeit of opinion.
The meeting was arranged such that there were formal talks followed by a discussion led by one of the attendees. We present here the extended abstracts of the main talks with the points raised by the discussants. Despite disagreements on certain issues there was much in the way of consensus. First, it was agreed that nonconvulsive status epilepticus is a term that covers a range of disparate conditions with varying prognoses and treatments. The agreed definition was thus suitably vague, «Nonconvulsive status epilepticus is a term used to denote a range of conditions in which electrographic seizure activity is prolonged and results in nonconvulsive clinical symptoms». Secondly, it was agreed that even within a specific condition (e.g. complex partial status epilepticus), the prognosis and treatment depends upon the context in which the condition occurs (e.g. in the critically ill, in coma, in the «walking wounded» and in people with prior epilepsy). Perhaps, most importantly it was agreed that we lacked good clinical data, and the challenge was to design good studies for a condition that is underrecognised and often difficult to diagnose.
