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Invited Editorial Comment
The classification of status epilepticus
Simon Shorvon
Original article
A semiological classification of status epilepticus
Sabine Rona, Felix Rosenow, Stephan Arnold, Mar Carreño, Beate Diehl, Alois Ebner, Brita Fritsch, Hajo M Hamer, Hans Holthausen, Susanne Knake, Bernd Kruse, Soheyl Noachtar, Tom Pieper, Ingrid Tuxhorn, Hans O Lüders
often defy classification according to the current international classification scheme. The semiological seizure classification (SSC) has been in use in several epilepsy centers for more than a decade, and has proven to be a valid approach to the classification of epileptic seizures. Based on the detailed analysis of more than 100 episodes of SE documented with video-EEG recordings, the authors now present a proposal for a semiological classification of status epilepticus (SCSE). The SCSE reflects the assumption implied by all modern definitions of SE that "there are as many types of status as there are types of seizures" and relies on the same principles as the SSC, focusing on the main clinical manifestations and the evolution of the status episode. The clinical manifestations of SE are subdivided into semiological components and classified along three axes: the type of brain function predominantly compromised by the seizure activity, the body part involved, and the evolution over time. Each axis contains several subcategories, so that many different levels of accuracy are possible. The SCSE, just like the SSC, is meant to be part of a comprehensive epilepsy classification which classifies as independent variables (epileptogenic zone, ictal semiology, etiology, related medical conditions) the main features of the patient’s epilepsy, allowing for each variable maximum flexibility. [Published with videosequences]
Symptoms of narcolepsy in children misinterpreted as epilepsy
Stewart Macleod, Colin Ferrie, Sameer M Zuberi
have disastrous consequences. Incorrectly identifying an event as an epileptic seizure can lead to unnecessary investigations and instigation of inappropriate treatment regimes. We report five patients referred to regional Paediatric Neuroscience Centres for investigation of events initially suspected of being epileptic seizures. All five patients were subsequently diagnosed as having narcolepsy. Suspected diagnoses were absence epilepsy (four patients), generalized epilepsy with astatic seizures (two patients) and focal epileptic seizures (two patients). Diagnostic confusion arose because lack of responsiveness due to excessive sleepiness was mistaken for epileptic absences, and cataplexy was confused with a variety of seizure types. In each case, videotape recording of clinical events aided in making the diagnosis of cataplexy. At presentation, all five children had excessive daytime sleepiness with cataplexy. Following correct diagnosis and appropriate management, an improvement in symptoms was reported in all cases. Narcolepsy/cataplexy should be included in the differential diagnoses of paroxysmal disorders, particularly if there are associated sleep symptoms or behavioural difficulties. It is important to take a sleep history when evaluating any disorder of the central nervous system. [Published with videosequences]
Analysis of the characteristics of epilepsy in 37 patients with the molecular diagnosis of Angelman syndrome
Marta Galván-Manso, Jaume Campistol, Joan Conill, Francesc-Xavier Sanmartí
with severe mental retardation, characteristic physical appearance, behavioral traits, and severe, early-onset epilepsy. We retrospectively reviewed the medical histories of 37 patients, all with the molecular diagnosis of Angelman syndrome and at least three years of follow-up in our neurology department, for further information about their epilepsy: age of onset, type of seizures initially and during follow-up, EEG recordings, treatments and response. The molecular studies showed 87% deletions de novo, 8% uniparental, paternal disomy, and 5% imprinting defects. The median age at diagnosis was 6.5 years, with 20% having begun to manifest febrile seizures at an average age of 1.9 years. Nearly all (95%) presented with epilepsy, the majority under the age of three (76%). The most frequent seizure types were myoclonic, atonic, generalized tonic-clonic and atypical absences. At onset, two patients exhibited West syndrome. EEG recordings typical of Angelman syndrome were found in 68%. Normalization of EEG appeared in 12 patients after nine years. Control of epileptic seizures improved after the age of 8.5 years. The most effective treatments were valproic acid and clonazepam. We conclude that epilepsy was present in nearly all of our cases with Angelman syndrome, and that the EEG can be a useful diagnostic tool. On comparing the severity of epilepsy with the type of genetic alteration, we did not find any statistically significant correlations.
Generalized tonic-clonic status epilepticus in the elderly in China
Andrew CF Hui, Anita K Lam, Adrian Wong, Kai-Ming Chow, Eric LY Chan, Simon L Choi, Ka-Shing Wong
the etiology, response to treatment, outcome and predictors of mortality in a group of elderly patients with generalized tonic-clonic SE in Hong Kong, China. Factors for increased mortality were analyzed using a logistic regression model. Of the 80 acute admissions for SE from two large urban hospitals over a seven-year period, 1996-2002, the two leading causes were attributed to cerebral infarct (n=28, 35%) and cerebral haemorrhage (n=14, 17.5%). The mean age was 74.2 years (range 60-93 years). At six months from the onset of seizures, 26 patients (32.5%) had made a good recovery but another 28 (35%) had died. Results showed that mortality was associated with increasing age (OR 1.08, 95% CI 1.01-1.16) and SE due to an acute symptomatic disturbance (OR 4.90, 95% CI 1.17-13.67). SE is associated with significant morbidity and mortality in this age group.
Case reports
Leucopenia and thrombocytopenia possibly associated with lamotrigine use in a patient
Ali Ugur Ural, Ferit Avcu, Zeki Gokcil, Oral Nevruz, Turker Cetin
weeks after starting lamotrigine. Within weeks after lamotrigine was discontinued, all of the haematopoietic abnormalities had disappeared. To our knowledge, this is the first report of combined leucopenia and thrombocytopenia associated with lamotrigine treatment suggesting, in our patient, a causal reaction.
Periodic lateralized epileptiform discharges (PLEDs) as the sole electrographic correlate of a complex partial seizure
Gagandeep Singh, Mary-Anne Wright, Josemir W Sander, Matthew C Walker
with a left anterior temporal emphasis. The abnormality, which persisted throughout the whole recorded seizure, lends support to the claim that PLEDs can be an ictal phenomenon.
Video case reports
Ictal movement disorders and hypothalamic hamartoma
William Szurhaj, Christine Daems-Monpeurt, Haouaria Sediri, Alain Destée, Philippe Derambure
manifested themselves clinically as characteristic, paroxysmal movement disorders, such as choreic/ballistic movement. This type of phenomenon is rarely of epileptic origin: we thus suggest that the movement disorder observed here could be due to functional disorganization of the basal ganglia network by the epileptic discharge, causing loss of the inhibition of thalamic activity and thus allowing the occurrence of abnormal movements. [Published with videosequences]
Startle-induced seizures associated with infantile hemiplegia: implication of the supplementary motor area
Melinda A Nolan, Hiroshi Otsubo, Koji Iida, Berge A Minassian
support involvement of the supplementary motor area in the generation of this seizure type. We present the case of an 11-year-old girl with an uncommon form of startle-induced seizures, illustrated on video-EEG, against the background of left infantile hemiplegia associated with extensive right hemispheric porencephaly but preserved cognitive functioning. The epileptic focus appears to be in the dorsolateral frontal lobe, with seizure semiology involving the supplementary motor cortex. [Published with videosequences]
Wall-eyed bilateral internuclear ophtalmoplegia presenting as monocular alternating nystagmus: a non-epileptic phenomenon in a
Feriha Ozer, Aytul Mutlu, Hasan Meral, Tulay Kansu, Bedri Carsancaklı
axonal injury involving supratentorial and infratentorial structures, and the splenium of the corpus callosum. The monocular alternating nystagmus was thought to be independent of seizures and occurred as a result of diffuse axonal injury affecting the medial longitudinal fasciculus bilaterally. [Published with videosequences]
Correspondence
Neurocutaneous melanosis and epilepsy surgery
Frederick Andermann, P. Halász, A. Holló, G. Rásonyi, Dileep Nair, Armin Mohamed, Richard Burgess, Hans Lüders
Book review
Reflex epilepsies: progress in understanding
Paolo Tinuper
