Table 2.

Data on initial evaluation and treatment, EEG evolution and brain MRI

	Term. Clinical examination at birth	Treatment during the first month	EEG evolution (age)	Brain MRI (age)
Patient 1	Full term. Hypotonia. No eye contact. BW: 3000 g HC: 35 cm	PHB VGB	1 m: Continuous with rare posterior spikes and fast rhythms	Day 7: Normal, absence of any signal abnormality. 2 y: Discrete global brain atrophy, thin corpus callosum
			6 m: slow background, rare focal spikes.
Patient 2	34 GW Fetal distress, apnea, movements disorder BW: 2,040 g HC: 30 cm	MDZ, PHB, B6, TPM, VGB	0-7 m: Suppression-burst >7 m: Hypsarythmia	D13: absence of any signal abnormality. 3 m: Absence of signal abnormality
Patient 3	Full term Failure. to thrive. Feeding difficulties BW: 3,770 g HC: 37.5 cm	PHB, B6, PHT, VGB, TPM, CLB.	1-6 w: Asynchronous SB. 2-8 m: bilateral Bursts of central spikes 1-6 y: Bursts of rhythmic generalized spikes at 3 Hz	Day 3: T1 bilateral hypersignal of pallida, tegmentum, locus niger, hippocampi. Abnormal ADC in these regions. 2 y: T1 hypersignal of the same structures and diffuse T1 hypersignal of the white matter. Brain atrophy
Patient 4	Full term. Normal BW: 3,580 g HC: 37 cm	PHB, PHT, VPA	0-2 m: Suppression-burst. 2–12 m: hypsarythmia. >12 m: Frequent multifocal spikes	Day 7: Normal CT scan 2 y: Thin Corpus Callosum, absence of signal abnormality
Patient 5	Full term. Normal BW: 3,240 g HC: 34 cm	PHB, PHT, B6, VGB, VPA	0-2 m: Suppression-burst. 2–6 m: continuous, slow background, multifocal spikes. >6 m: Rare spikes in temporal and occipital lobes	Day 10: no signal abnormality.
Patient 6	Full term. Normal BW: 2,790 g HC: 34,5 cm	ND	0-1 m: Suppression-burst. <1 m: Multifocal spikes, slow background	1 m: Normal
Patient 7	Full term. Normal BW: 3,180 g, HC: 36 cm	PHB, B6, PHT	0-1 m: Suppression-burst. 1–7 m: Continuous EEG, multifocal spikes 7–24 m: Hypsarythmic pattern. >24 m: Frequent spikes and spike wave in frontal regions	Day 4: Normal, absence of any signal abnormality
Patient 8	ND	PHB, PHT	0-2 m: Bursts of multifocal spikes, periods of flatness. >2 m: Multifocal spikes, poor organization	1 y: No structural or signal abnormality.
Patient 9	At term. Hypotonia. No eye contact. BW: 3,450 g HC: 35 cm	PB, CZP, VGB	0-2 m: Suppression-burst 2–6 m: Slow background, rare generalized spike waves. 6–12 m: Hypsarythmic pattern. >12 m: Rare asynchronous frontal and temporal spikes	Day 10: Normal, absence of any signal abnormality
Patient 10	Full term. No eye contact BW: 3,120 g HC: 33 cm	PB, PHT, TPM, VGB, B6,	0-2 m: discontinuous EEG. >2 m: continuous, slow EEG with rare generalized spikes	Day 5: T1: symmetrical hypersignal of the pallida, caudate nuclei and hippocampi T2: bilateral hypersignal of the parietal occipital white matter
Patient 11	Full term. Hypotonia, hyporeactivity, failure to feed	PHB, PHT, VPA.	0-1 m: Left or right spikes on a moderately abnormal background. >1 m: Occipital or temporal spikes with left prominence with progressive migration on the central temporal region	1 m: normal
Patient 12	Full term. Normal BW and HC	VGB, CBZ	0-2 m: Suppression-burst. 2–6 m: General slowing of the traces, no spike. 6 m-2 y: Rare spikes in the right central region, Normal background. >2 y: normal traces.	Day 7: T2 hyperintensity of the basal ganglia 2 y: Normal 3y: Normal
Patient 13	Full term. Fetal distress. BW, HC: ND	ND	ND	1 m: No structural abormality, no signal change
Patient 14	Full term. BW 3,750 g. Poor eye contact, trunk hypotonia with bouts of hypertonia	PHB, VGB, CBZ;	0-4 m: Asymmetrical suppression-burst 4-10 m: Left occipital spikes and slow waves 10 m-3 y: Normal background activity + posterior theta waves, No spike 3 y: Intermittent slow background, no spike >8 y: Normal	3 m: Normal
Patient 15	Oligoamnios Born at 30 Weeks (GA) BW: 1580 g HC: 29 cm	PHB, VPA	0-1 m: Suppression-burst >1 m: continuous traces (normal)	2 y: normal
Patient 16	Full term Global hypotonia, weak cry	CLN, PHT	0-1 m: absence of physiologic features, slow waves, spikes, brief flattening. 1–6 m: improvement of background activity, some generalized flattening episodes, left occipital slow waves. 6 m – 6 y: slow. background activity, rare spikes. 27 y: bilateral temporal slow waves. 30 y: normal background activity, bilateral fronto-temporal bursts of slow waves, photic stimulation-evoked slow spikes	17 y: slight T2 and FLAIR hyperintensity of thalami.

GW gestational week, HC Head circumference, m month, Y year, PHB Phenobarbital, PHT phenytoine, VGB vigabatrin, TPM topiramate, CLN clonazepam, VPA Sodium Valproate, CBZ carbamazepine, CLB clobazam.