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Table 1 MRC results for nusinersen treated and untreated cohorts

From: Motor function in type 2 and 3 SMA patients treated with Nusinersen: a critical review and meta-analysis

Source

SMA TYPE

MRC baseline (mean + SD or mean (range))

Time from infusion/FU timing

N

MRC change

Nusinersen-treated cohorts

De Wel

3 and 4

36.9 + 10.3

14 months

15

Mean: 2.53, 99% CI 0.18–4.88

Walter

3

113.95 + 22.91

10 months

16

Mean: 4.55

Mosche-Lilie

2 and 3

Not reported

12 months

10

Mean %: + 2.13%

Untreated cohorts

Wjngaarde

2A

Not reported

12 months

68

Annual slope: − 0.73, 95% CI − 1.00; − 0.45

Wjngaarde

2B

Not reported

12 months

50

Annual slope: − 0.65, 95% CI − 0.89; − 0.40

Wjngaarde

3A

Not reported

12 months

63

Annual slope: − 0.84, 95% CI − 1.07; − 0.61

Wjngaarde

3B

Not reported

12 months

40

Annual slope: − 0.83, 95% CI − 1.13; − 0.54

Carter GT

2

Mean 2.3 + 0.6*

10 years

18

Decade slope − 0.24 per muscle

Piepers 2008

3B and 4

Mean 294 + 12

Mean 30 months (19–36 months)

9

Mean change: 0

Wadman

1C, 2, 3, 4

Not reported

12 months

180

Annual slope: − 1

Werlauff

2

29% (9–41)

Median 17 years (12–20 years)

21

Annual slope: − 0.22 (Upper limb), CI − 0.39; − 0.02

Otto 2020

2 and 3

142.7 + 41.6

Mean 13.1 months (368–442 days)

9

Mean change: − 1.1

  1. Key to table: *average grade for all muscles groups combined