TABLE 1

Individual Characteristics of Patients

Patient no.SexAge (y)Age at onset (y)Duration of epilepsy (y)Duration of status epilepticus (d)MRI at time of PETNeuropsychologic findings at time of PETIctal EEG during status epilepticusMain seizure signs during FIRES chronic stageIctal EEG during FIRES chronic stageInterictal spikes during FIRES chronic stage
1F9.84.35.58Bilateral HH, left HAFIQ, 61; VIQ, 64; PIQ, 67 (WISC-III); standardized score, PPVT-R = 83; reduced word fluency; severe memory deficit (RBMT = 8/22); behavior disorders; slowness and perseverationsRight DHE, left cloniaRight hemisphereLeft temporal
2F10.44.55.98NormalMental deficiency; IQ < 50 (Terman Merill test); aphasia and auditory agnosia; attention problems and hyperactivity; aggressive behaviorRight temporofrontalStaring, fear, crying, mouth deviation (G)Right temporofrontalLeft hemisphere
3M9.34.64.76NormalReduced receptive language; antisocial behavior, aggressionStaring, eyelid and arm cloniaLeft and right temporofrontalLeft temporofrontal
4M6.24.71.560Bilateral HH, bilateral HAUntestable; loss of speech; major behavior disordersLeft and right temporalStaring, agitation, right cloniaLeft and right temporooccipitalLeft and right temporooccipital
5M8.86.22.617NormalMental deficiency; IQ < 45 (WPPSI-III); language disorders; anxiety/depressionLeft occipitalGeneralized tonic–clonicLeft and right frontalRight temporal
6M7.96.8215NormalVIQ, 97; PIQ, 77; attention deficit hyperactivityLeft DHE, left limb clonia, 4 limbs tonicRight temporalLeft temporal
7F9.36.92.456Bilateral HH, bilateral HAReduced mental ability; FIQ, 51; VIQ, 60; PIQ, 53; standardized score, PPVT-R = 79; reduced speech production; severe memory deficit (RBMT = 3/22); attention problems and hyperactivity; thought problemsStaring, left mouth deviation, eyelid cloniaLeft and right temporalLeft and right temporal
8M12.98.64.321Generalized cerebral atrophyReduced mental ability; FIQ, 54; VIQ, 60; PIQ 68; standardized score, PPVT-R = 78; speech fluency disorder; memory deficit (RBMT = 14/22); anxiety/depression; slowness and perseverationsStaring, left mouth and arm clonia, eyelid clonia (G)Left and right frontalLeft and right frontal
  • DHE = deviation of head and eyes; FIQ = full-scale IQ; (G) = tonic–clonic generalization ; HA = hippocampal atrophy; HH = hippocampal hypersignal; PIQ = performance IQ; PPVT-R = Peabody Picture Vocabulary Test-Revised; RBMT = Rivermead Behavior Memory Test; VIQ = verbal IQ; WISC-III = Wechsler Intelligence Scale for Children; WPPSI = Wechsler Preschool and Primary Scale of Intelligence.