Phenotypic diversity in an international Cure VCP Disease registry

Table 2 The result of examination on patients in the conference

	Total participants (n = 22)
Participant sex (M:F)	10:12
Age at onset (n = 19)^a	43.4 ± 8.9
Age at diagnosis (n = 17)^a	49 ± 7.9
Age at participation^a	55.2 ± 9.9
Forced vital capacity, % predicted^b (range)	82 ± 25 (48–146)
ALS-CBS^b,c (range)	17.1 ± 1.6 (14–20)
Facial weakness	11 (50%)
Weakness of eye closure	7 (32%)
Weakness of lip closure	9 (41%)
Scapular winging	12 (55%)
Bilateral	5 (23%)
Unilateral	7 (32%)
IBM-FRS^b,d (range)	29.0 ± 7.0 (9–39)
Timed up and go test^b,e (n = 17, s, range)	12.2 ± 6.0 (5.0–27.6)
Walking aid	14 (64%)
Cane	9 (41%)^f
Walker	11(50%)^g
Wheelchair	5 (23%)^h

^aMean ± SD, years
^bMean ± SD
^cAmyotrophic Lateral Sclerosis Cognitive Behavioral Screen (ALS-CBS) is a screening tool for frontal lobe dysfunction. We used the cognitive section of ALS-CBS whose total score is 20
^dInclusion Body Myositis Functional Rating Scale (IBM-FRS) consists of 10 questions about a patient’s ability to perform daily activities, such as walking, dressing, and handling utensils. Each question was answered using a number 0–4
^eThe Timed Up and Go test is a test for quantifying functional mobility and assessing the fall risk, in which the ability to rise from a seated chair position, walk 3 m, turn, walk back, and sit down is timed
^fAmong the nine patients, five patients always used a cane, while four patients only sometimes used it
^gAmong the eleven patients, two patients always used a walker, while nine patients only sometimes used it
^hAmong the five patients, one patient always used a wheelchair, while four patients only sometimes used it

ISSN: 1750-1172