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Epileptic Disorders

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Typical semiology of benign childhood epilepsy with centrotemporal spikes (BCECTS) Volume 2, supplement 4, Supplément 1, Décembre 2000

Epidemiology

BCECTS has an incidence rate of 5.6-21/100 000 in the age group of 0-15 years and a prevalence rate in all epilepsies of 8-23%. There is a male preponderance (1.4-1.5) [2-4].

Semiology

Due to the mainly nocturnal and unpredictable occurrence of the seizures, with low frequency, the number of ictal recordings is limited, the semiology is mostly recounted by patients and witnesses: partial (lateralised), oro-(hemi-) facial and/or oropharyngeal tonic and/or tonic-clonic and/or clonic muscle contractions with speech arrest and hypersalivation are the main motor (and vegetative) symptoms, which manifest e.g. at the tongue, mouth, chin, cheek. The involvement of the (epi-) glottis and pharynx (> 50%) produces guttural sounds of various bizarre types, resembling vocalisations of different animals. These may be accompanied by contractions of the respiratory (in combination with "closed glottis"?) and abdominal muscles (vomiting like contractions). Postictal Todd paralysis is observed in 10% of the cases [5].

The efforts of the child to explain the situation produce additional sounds. Speech arrest occurs in > 40% of seizures with dys - or anarthria. In stimulation tests, speech arrest was demonstrated to be elicitable as a positive as well as a negative motor effect from the right and the left hemisphere or as a result of interference with the language process elicited only from the dominant hemisphere [3]. In some patients, speech problems persist as a postictal phenomenon minutes after the end of other seizure symptoms.

Often (~60%) consciousness is completely preserved in Rolandic seizures, in about 13% it is preserved only initially and in the rest not preserved at all with no recall of ictal events in the latter. Children get out of bed and run to their parents.

Somatosensory symptoms may consist of paresthesia in parts of and/or in the whole oropharyngeal /oro-facial region, e.g. tongue, (inner) cheek, gum, teeth, lips. These sensations may have all possible sensory qualities, e.g. pins and needles, prickling, freezing. There are single reports on cases with paroxysmal tooth ache as sensory manifestation of benign partial epilepsy. Some report on feelings of suffocation/strangulation. There might be also expressions of fear (probably a psycho-reactive and not a genuine ictal symptom). The frequency of partial seizures is reported to vary between 11 and 20% [3].

The ictal symptoms may spread to the ipsilateral upper extremity and to the legs, and then generalise secondarily: GTCS are a frequent seizure type: secondary GTCS are present in one to two thirds of children with (mostly nocturnal) rolandic epilepsy (occurring in 24-87% of cases with centrotemporal sharp waves; summarised by Lüders et al. [3]). There are no reports on primary GTCS in rolandic epilepsy.

Duration of seizures is from few seconds (30-60 sec) to few minutes (less than 2-3 min). In general, nocturnal seizures are longer than diurnal, but in few instances seizures last up to more than one hour, and it is difficult to term these typical seizures - probably such cases belong to the group of atypical benign partial epilepsies.

Precipitation of seizures and their circadian distribution: seizures occur mostly while the child is asleep (51-80%) or drowsy, e.g. before going to sleep or after awakening. In about 15% seizures occur both during sleep and on awaking and in 10-20% only on awaking.

Frequency of seizures: in 10-20%, only one witnessed seizure may occur, 60-70% may have 2-10 fits, but in the other 10-20% seizures may be more frequent and difficult to treat. Clusters of seizures and even status epilepticus may occur in one night or within a short period of time [6, 7]. Few experience clusters or single seizures after various intervals of suspected remission.

Associated Neurology: children with BCECTS have migraine and neuropsychological problems more often than the general population (see the contributions of F. Andermann, T. Deonna and G. Carlsson in this volume). There is also an increased incidence of febrile convulsions, neonatal difficulties, including seizures and head injuries in these children [8].

Course of children with rolandic seizures: age at onset of seizures peaks between 5 and 9 years of age, the age of the last seizures between 8 and 12 years. There is a close to 100% remission rate at 16 years of age [2].