Changes in electrophysiological findings of spinal muscular atrophy type I after the administration of nusinersen and onasemnogene abeparvovec: two case reports

Table 1 CHOP-INTEND score, motor development, and CMAP amplitudes of the median and tibial nerves in two patients

Patient 1		2 months old (before nusinersen started)	1 year old (6 months after OA)	2 years old (18 months after OA)
CHOP-INTEND score		18	46	58
motor ability			head control sitting	rolling over
CMAP	median nerve	0.60 mV (-3.4 SD)	1.60 mV (-2.2 SD)	2.61 mV (-2.7 SD)
CMAP	tibial nerve	0.60 mV (-9.9 SD)	4.97 mV (-2.0 SD)	7.33 mV (-1.2 SD)
Patient 2		6 months old (before nusinersen started)	1 year old (5 months after OA)	2 years old (17 months after OA)
CHOP-INTEND score		15	43	49
motor ability				no head control rolling to side
CMAP	median nerve	0.26 mV (-2.3 SD)	2.31 mV (-1.8 SD)	2.56 mV (-2.7 SD)
CMAP	tibial nerve	1.11 mV (-3.2 SD)	1.21 mV (-3.3 SD)	3.86 mV (-2.3 SD)

Patient 1 received nusinersen four times since she was 2 months old, and OA was administered at 6 months old.
Patient 2 received nusinersen two times since she was 6 months old, and OA was administered at 7 months old.
SD values are calculated from the mean and SD values of healthy children at each age [13].
CHOP-INTEND, Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders; CMAP, compound muscle action potential; OA, onasemnogene abeparvovec

ISSN: 1471-2377