Table 2.

Results of standardized test (CMS-ST) in 17/21 patients compared to neurological assessment and reported symptoms by patients and their parents.

Clinical symptom	CMS-ST (n = 17)	Clinical assessment and self-reported (n = 17)
Ocular symptoms	Ptosis: 15/17 patients, Double vision: 13/17 patients	Ptosis: 13/17 patients 9/17 double vision reported
Bulbar symptoms	10/17 patients	6/17 patients reported chewing problems
Respiratory function	6/14 abnormal	5/17 patients reported respiratory problems
Cervical weakness	15/17 patients	Not stated
Proximal weakness	10/17 patients	5/17 patients
Distal weakness	11/17 patients	Not stated
Impaired muscular endurance	8/17 Patienten	13/17 impaired muscular endurance reported

Pulmonary function was performed in 14/17 patients; one patient was intellectually impaired and not capable to perform the test (total: n = 17).