Table 1 Inter-rater agreement, evaluated as a percentage agreement, and as a kappa value or ICC, for the 39 items common to the SRQ and CEF used in the statistical comparison; class sizes are provided and items are grouped by sections

Diagnosis

Age at first symptom

236

–

0.81 [0.76–0.85]

First symptom

255

0.52 [0.44–0.60]

–

Family history

266

0.79 [0.72–0.87]

–

Molecular diagnosis lab

253

0.94 [0.90–0.98]

–

Age at molecular diagnosis

236

–

0.97 [0.96–0.98]

Number of repeats

219

–

0.98 [0.97–0.98]

EcoRI fragment length

8

–

1.00 [ – ]*

Demographics

Marital status

270

0.89 [0.84–0.93]

–

Professional status

272

0.84 [0.79–0.89]

–

Height

271

–

0.99 [0.99–0.99]

Weight

266

–

0.99 [0.99–0.99]

Dominant side

268

0.84 [0.75–0.93]

–

Care

Scapula surgery

276

0.76 [0.61–0.92]

–

Use of cane

281

0.77 [0.67–0.86]

–

Use of walker

281

0.87 [0.77–0.96]

–

Use of manual wheelchair

281

0.63 [0.49–0.77]

–

Use of electric wheelchair

281

0.71 [0.58–0.83]

–

Respiratory assistance

19

1.00 [1.00–1.00]

–

Eye surgery

40

0.74 [0.54–0.95]

–

Hearing device

30

0.71 [0.46–0.97]

–

Muscle function

Brooke scale

254

–

0.66 [0.58–0.72]

Vignos scale

250

–

0.86 [0.82–0.89]

Visual Analog Pain Score (VAPS)

191

–

0.61 [0.51–0.69]

Muscular pain

270

0.65 [0.53–0.76]

–

Difficulties to sit up

263

0.49 [0.37–0.61]

–

Scapular winging

241

0.22 [0.06–0.38]

–

Ability to whistle

271

0.17 [0.10–0.25]

–

Difficulties in closing eyes

272

0.16 [0.10–0.21]

–

Heart

Palpitations

281

0.14 [0.04–0.23]

–

Syncope

281

0.14 [-0.04–0.32]

–

Vertigo

281

0.00 [0.00–0.00]

–

Retrosternal pain

281

0.02 [-0.05–0.08]

–

Respiratory

Exertional dyspnea

281

0.32 [0.21–0.43]

–

Dyspnea at rest

281

0.28 [0.06–0.49]

–

Orthopnea

281

0.24 [0.01–0.47]

–

Excessive daytime sleepiness

281

0.02 [-0.07–0.12]

–

Morning headaches

281

0.26 [0.10–0.42]

–

GI tract

Dysphagia

281

0.32 [0.16–0.47]

–

Pulmonary aspiration

281

0.41 [0.27–0.55]

–