Table 4.

Age at onset of clinical seizures and rates of seizures, abnormal EEG, and survival in large studies of epilepsy in Menkes disease

Subject numbera	Friedman et al. (1978) (n=8)	White et al. (1993) (n=9)	Bahi-Buisson et al. (2006) (n=12)	Current study (n=24)
Age at onset of clinical seizures (weeks)
1	No seizures	N.A.	12	No seizures
2	10	10	11	No seizures
3	9	15	10	No seizures
4	9	10	11	No seizures
5	11	12	20	No seizures
6	10	24	22	No seizures
7	14	14	10	No seizures
8	24	52	11	No seizures
9		No seizures	32	No seizures
10		32	11	No seizures
11			21	No seizures
12			12	No seizures
13–15, 18, 19, 21–24				No seizures
16				16
17				14
20				31
Mean±SD age at 1st seizure (weeks)	12.4±5.4	21.1±14.6	14 (SD not given)	20.3±9.3
Range (weeks)	9–24	10–52	10–32	14–31
Percentage with seizures	87.5%	88.9%	100%	12.5%b
Percentage with EEG abnormalities	87.5%	88.9%	100%	45.8%c
Percent survival	37.5%	33.3%	8.3%	62.5%d
Early copper treatment	No	No	No	Yes

N.A. Not applicable

^aSubject number refers to patients listed in Friedman et al. (1978), White et al. (1993), and in Table 1 of this paper. Mean (n=12) and range for age at onset of seizures were available from Bahi-Buisson et al. (2006), and we estimated individual ages (weeks) from Fig. 4 of that paper. For White et al., age at onset seizures and survival were not included in the original paper and are provided here from our records on these patients

^bP=3.8×10⁻⁹

^cP=5.0×10⁻⁴

^dP=5.0×10⁻³

P values represent comparison of the present cohort (n=24) with three prior studies combined (n=29)