Neuropsychological Deterioration

The majority of patients have normal neuropsychological and motor functions prior to the onset of ESES, as exemplified by 18 of the 29 patients reported by Tassinari et al. (1992b), 23 of the 31 cases described by Morikawa et al. (1989), and 30 of the 43 patients discussed by Dalla Bernardina et al. (1989). The remaining patients have abnormal development prior to the occurrence of ESES.

All cases exhibit further, and often severe, decrease in function during the stage of ESES. The disturbances include a marked impairment of intelligence quotient (IQ), deterioration of language, temporo-spatial disorientation, behavioral changes (reduced attention span, hyperkinesis, aggressiveness, and difficulty in contact), and, rarely, psychotic states (Tassinari et al., 1985, 1992b). IQ was thoroughly investigated in 59 patients assembled during the Venice Colloquium and analytic interpretation of test and single subtest results was carried out (Mira et al., 1995). The data showed that ESES is associated with a disruption of all cognitive functions, but the impairment is sometimes greater in the field of logical-structural intelligence and sometimes in the field of infrastructural intelligence, possibly in relation to a previously different intellectual organization.

Despite the absence of a global decline of cognitive functions, the pattern of psychomotor derangement may differ from patient to patient, and this seems to depend on the predominant localization of the SW discharges. A deterioration of language out of proportion to other abilities has been reported in some cases, showing the predominance of the paroxysmal abnormalities over one or both temporal regions (Patry et al., 1971; Billard et al., 1982; Tassinari et al., 1982, 1985; De Marco, 1988). On the other hand, mental and behavioral deterioration evoking a frontal lobe syndrome has been described in children exhibiting interictal frontal foci or clear-cut anterior predominance of the discharges (Billard et al., 1982; Roulet Perez et al., 1993). Neuropsychological impairment occurs in almost all cases of ESES syndrome, being usually coincidental with the detection of ESES and representing one of the crucial signs of the syndrome.

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