Table 3 Response to treatment in COLQ-CMS patients
From: COLQ-Congenital myasthenic syndrome in an Iranian cohort: the clinical and genetics spectrum
Tx | P1.1 | P1.2 | P2 | P3 | P4 | P5.1 | P5.2 | P6 | P7.1 | P7.2 | P8 | P9.1 | P9.2 | P10 | P11 | P12 | P13 | P14 | P15 | P16 | P17 | P18 | P19 | P20 | P21 | P22 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Pyridostigmine | NT | NT | W | NT | NT | NC | NC | NC | NT | NT | W | NC | NC | W | W | W | NT | W | W | W | W | W | W | NC | NC | NT |
Ephedrine | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | |||||||||||
ΔMGADL for Ephedrine Tx | 1 | 2 | 1 | 2 | 1 | 1 | 1 | 3 | 2 | 3 | 2 | 4 | 2 | 5 | 2 | |||||||||||
Salbutamol Response | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | + | ||||||||
ΔMGADL For Salbutamol Tx | 3 | 1 | 1 | 2 | 1 | 4 | 2 | 2 | 2 | 2 | 3 | 2 | 3 | 3 | 2 | 2 | 2 | 2 |