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Table 2 The result of examination on patients in the conference

From: Phenotypic diversity in an international Cure VCP Disease registry

  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 participationa 55.2 ± 9.9
Forced vital capacity, % predictedb (range) 82 ± 25 (48–146)
ALS-CBSb,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-FRSb,d (range) 29.0 ± 7.0 (9–39)
Timed up and go testb,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
  1. aMean ± SD, years
  2. bMean ± SD
  3. 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
  4. 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
  5. 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
  6. fAmong the nine patients, five patients always used a cane, while four patients only sometimes used it
  7. gAmong the eleven patients, two patients always used a walker, while nine patients only sometimes used it
  8. hAmong the five patients, one patient always used a wheelchair, while four patients only sometimes used it