Centrotemporal spikes probably are the most common specific epileptiform abnormalities found in classic Landau-Kleffner syndrome (LKS) and in children with autistic spectrum disorders with and without regression.22 Although Rolandic epilepsy usually is a benign, age-dependent form of epilepsy, extreme cases have been described.47,49,,51,60–63 These patients have had prolonged deficits, including:
Deonna et al.48 have suggested that an oromotor deficit may be the initial symptom in Rolandic epilepsy, preceding the appearance of overt seizures. This pattern parallels the appearance of verbal auditory agnosia in classic LKS prior to the onset of seizures. Deonna speculates that the variations in clinical symptoms between classic LKS and its variants are related to the main site, local extension, and bilaterality of the epileptic foci, rather than to a basic difference in pathophysiology. The patients reported with centrotemporal spikes and classic LKS do not appear to have greater expressive deficits, however. Deonna48 hypothesizes that oromotor difficulties interfere either with simple voluntary oromotor functions or with complex movements including speech production, depending on the location and spread of the epileptic focus around the perisylvian region. Thus, nonlinguistic deficits such as intermittent drooling, oromotor apraxia, or dysfluency, as well as linguistic deficits involving phonologic production, all can occur.
The most severe deficit produced in this context is a complete anterior operculum syndrome. The reported rapidity of onset, duration, progression, and recovery of the deficit varies widely. Such differences could reflect the degree of epileptic activity. As with LKS, the paroxysmal EEG activity (which usually is bilateral) and the clinical deficit sometimes run in parallel, and rapid improvement with antiepileptic medication occurs in some cases.
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.
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