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]
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]
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.
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.