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. 2021 Feb 11;92(11):1244–1246. doi: 10.1136/jnnp-2020-324532

Table 1.

HINE-2 evolution of the patients according to SMN2 copy number and age of nusinersen initiation

Characteristics Total
(n=40)
One copy of SMN2
(n=1)
Two copies of SMN2
(n=25)
Three copies of SMN2
(n=14)
Sex, M:F, n 18:22 1:0 10:15 7:7
Age at symptom onset 3.0 (0.0–6.0) 0.0 2.0 (0.0–6.0) 4.5 (1.0–6.0)
Age at nusinersen initiation 20.0 (0.35–294.0) 2.0 19.0 (0.35–140.0) 25.3 (4.0–294.0)
Disease duration before nusinersen 17.5 (0.0–291.5) 2.0 16.0 (0.35–140.0) 17.5 (0.0–291.5)
HINE-2 score
M0 0.0 (0.0–4.0), n=37 0.0, n=1 0.0 (0.0–2.0), n=24 2.0 (0.0–4.0), n=12
M6 2.5 (0.0–17.0), n=34 4.0, n=1 1.0 (0.0–11.0), n=22 4.0 (1.0–17.0), n=11
M10 4.0 (0.0–24.0), n=32 5.0, n=1 2.5 (0.0–20.0), n=20 4.0 (1.0–24.0), n=11
Time effect p value <0.001 / <0.001 0.002
Delta M6M0 2.0 (0.0–16.0), p<0.001 4.0 1.0 (0.0–11.0), p=0.002 2.0 (1.0–16.0), p=0.019
Delta M10M0 3.0 (0.0–20.0), p<0.001 5.0 2.5 (0.0–20.0), p=0.003 3.0 (1.0–20.0), p=0.010
HINE-2 score (age ≤7 months old at onset of treatment)
M0 0.0 (0.0–4.0), n=13
M6 6.0 (2.0–16.0), n=11
M10 11.0 (3.0–24.0), n=11
Time effect p value <0.001
Delta M6M0 6.0 (2.0–12.0), p<0.001
Delta M10M0 9.0 (3.0–20.0), p<0.001
HINE-2 score (age ≤2 years old at onset of treatment)
M0 0.0 (0.0–4.0), n=22
M6 5.0 (0.0–17.0), n=19
M10 9.0 (0.0–24.0), n=18
Time effect p value <0.001
Delta M6M0 4.0 (0.0–16.0), p<0.001
Delta M10M0 7.5 (0.0–20.0), p<0.001
HINE-2 score (age >2 years old at onset of treatment)
M0 0.0 (0.0–2.0), n=15
M6 1.0 (0.0–4.0), n=15
M10 0.5 (0.0–7.0), n=14
Time effect p value 0.002
Delta M6M0 1.0 (0.0–2.0), p=0.003
Delta M10M0 0.5 (0.0–5.0), p=0.013

* Data are listed as median (range) in months.

‡ One-way repeated measures ANOVA.

§ Paired t-test.

¶ Multiple regression demonstrated that there was significant difference in the changes of the HINE-2 scores, delta M6M0 and delta M10M0, between the two groups with two and three SMN2 copies with the standard coefficients β of 0.263 (p=0.003).

† Three patients were not included in the HINE-2 M0 analysis, due to the following reasons: one patient did not have baseline HINE-2 performed; two patients did not have HINE-2 performed.

HINE-2, Hammersmith Infant Neurologic Examination Part 2 motor milestones score; M0, before treatment; M6, 6 months of treatment; M10, 10 months of treatment; SMN, survival motor neuron.