TABLE 3.
Pattern of changes in neurological condition at 5th year, stratified by age at first visit (0–35 months vs over 35 months).
| Overall | 0–35 months | >35 months | |
|---|---|---|---|
| N (%) | 55 | 37 | 18 |
| Deterioration by | |||
| One class | 11 (20.0) | 8 (21.6) | 3 (16.7) |
| Two classes | 14 (25.5) | 11 (29.7) | 3 (16.7) |
| Three classes | 13 (23.6) | 11 (29.7) | 2 (11.1) |
| Four classes | 1 (1.8) | 1 (2.7) | — |
| Improvement | |||
| One class | 1 (1.8) | 1 (2.7) | — |
| Stability | 15 (27.3) | 5 (13.5) | 10 (55.6) |
| Autism spectrum disorder | |||
| Deterioration | 1 (1.8) | 1 (2.7) | — |
| Improvement | — | — | — |
| Stability | 54 (98.2) | 36 (97.3) | 18 (100.0) |
| Behavior | |||
| Oppositional defiant disorder | |||
| Deterioration | 7 (12.7) | 4 (10.8) | 3 (16.7) |
| Improvement | 2 (3.6) | — | 2 (11.1) |
| Stability | 46 (83.6) | 33 (89.2) | 13 (72.2) |
| Attention deficit | |||
| Deterioration | 21 (38.2) | 16 (43.2) | 5 (27.8) |
| Improvement | 2 (3.6) | 2 (5.4) | — |
| Stability | 32 (58.2) | 19 (51.4) | 13 (72.2) |
| Autism spectrum symptoms | |||
| Deterioration | 7 (12.7) | 5 (13.5) | 2 (11.1) |
| Improvement | — | — | — |
| Stability | 48 (87.3) | 32 (86.5) | 16 (88.9) |
| Obsessive compulsive disorder | |||
| Deterioration | 1 (1.8) | — | 1 (5.6) |
| Improvement | 1 (1.8) | — | 1 (5.6) |
| Stability | 53 (96.4) | 37 (100.0) | 16 (88.9) |
| Neurological examination | |||
| Normal | |||
| Deterioration | 26 (47.3) | 21 (56.8) | 5 (27.8) |
| Improvement | 2 (3.6) | 2 (5.4) | ‐ |
| Stability | 27 (49.1) | 14 (37.8) | 13 (72.2) |
| Ataxia | |||
| Deterioration | 22 (40.0) | 20 (54.1) | 2 (11.1) |
| Improvement | 2 (3.6) | 2 (5.4) | — |
| Stability | 31 (56.4) | 15 (40.5) | 16 (88.9) |
| Pyramidal signs | |||
| Deterioration | 2 (3.6) | 2 (5.4) | — |
| Improvement | 1 (1.8) | 1 (2.7) | — |
| Stability | 52 (94.5) | 34 (91.9) | 18 (100.0) |
| Extrapyramidal signs | |||
| Deterioration | 1 (1.8) | — | 1 (5.6) |
| Improvement | 1 (1.8) | 1 (2.7) | — |
| Stability | 53 (96.4) | 36 (97.3) | 17 (94.4) |
| Action myoclonus | |||
| Deterioration | 7 (12.7) | 6 (16.2) | 1 (5.6) |
| Improvement | 2 (3.6) | 1 (2.7) | 1 (5.6) |
| Stability | 46 (83.6) | 30 (81.1) | 16 (88.9) |
| Gait abnormality | |||
| Deterioration | 14 (25.5) | 13 (35.1) | 1 (5.6) |
| Improvement | 3 (5.5) | 2 (5.4) | 1 (5.6) |
| Stability | 38 (69.1) | 22 (59.5) | 16 (88.9) |
| Neuropsychological assessment | |||
| N (%) | 22 | 15 | 7 |
| Language | |||
| Deterioration | 7 (31.8) | 5 (33.3) | 2 (28.6) |
| Improvement | 2 (9.1) | 1 (6.7) | 1 (14.3) |
| Stability | 10 (45.5) | 7 (46.7) | 3 (42.9) |
| Not assessable | 3 (13.6) | 2 (13.3) | 1 (14.3) |
| Intellectual disability | |||
| N (%) | 15 | 11 | 4 |
| Deterioration by | |||
| One class | 1 (6.7) | 1 (9.1) | — |
| Two classes | 4 (26.7) | 3 (27.3) | 1 (25.0) |
| Three classes | 2 (13.3) | 2 (18.2) | — |
| Four classes | 1 (6.7) | 1 (9.1) | — |
| Five classes | 1 (6.7) | 1 (9.1) | — |
| Improvement | |||
| One class | 1 (6.7) | 1 (9.1) | — |
| Stability | 2 (16.7) | — | 2 (50.0) |
| Not assessable | 3 (20.0) | 2 (18.2) | 1 (25.0) |