Table 2 Long-term outcome of patients with hyperornithinaemia–hyperammonaemia–homocitrullinuria syndrome with the F188del mutation

Patient	Age, years	Motor status			Cognitive functions			Functional independence level*
Patient	Age, years	HR	Clonus	Spastic gait	Verbal IQ	Non-verbal IQ	Global IQ	Job	Daily activity	Housing
1†	40	+	+	NA	NA	NA	36	3	3	3
2	35	–	+	–	64	58	53	2	2	2
3	25	+	+	–	109	100	105	3	2	2
4	33	+	–	–	57	54	40	3	3	3
5‡	21	+	+	+	93	83	87	3	1	3
6	22	+	–	+	95	80	86	1	1	1
7‡	14	+	+	+	108	81	92
8‡	13	+	–	–	110	103	107
9	9	+	–	–	NA	NA	90
10	7	+	+	–	98	80	84
11‡	6	+	+	–	NA	NA	32
12	29	+	+	+	85	75	79	2	1	1
13	23	+	+	+	82	78	78	2	2	2
14	31	+	+	+	76	70	73	2	2	3
15	17	+	–	+	75	69	71
16	5	+	+	–	NA	NA	NA

HR, hyper-reflexia; NA, not applicable.
*For patients >18 years old: 1, autonomous; 2, supervised; 3, dependent (or unemployed). †Confined to a wheelchair. ‡Received botulinum toxin injections.