Journal of neurology
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Journal of neurology · Jul 2017
Observational StudyRelevance of early cervical cord volume loss in the disease evolution of clinically isolated syndrome and early multiple sclerosis: a 2-year follow-up study.
Upper cervical cord area (UCCA) atrophy is a prognostic marker for clinical progression in longstanding multiple sclerosis (MS). The objectives of the study were to quantify UCCA atrophy and evaluate its impact in clinically isolated syndrome (CIS) and relapsing-remitting MS (RRMS); to compare converting CIS patients with stable CIS, and to study changes of UCCA and brain white matter (WM) and grey matter (GM) at 2-year follow-up. 110 therapy-naive patients including 53 CIS [6 ± 6 months after symptom onset (SO)] and 57 early RRMS (SO: 12 ± 9 months) underwent sagittal 3D-T1w brain MR (3T). ⋯ RRMS (78.1 ± 8.7 mm2, p = 0.011) and converting CIS (77.3 ± 8.0 mm2, p = 0.046) presented with baseline UCCA atrophy, when compared with controls (82.7 ± 5.2 mm2), but not stable CIS (82.6 ± 7.4 mm2, p = 0.998). Baseline WM was reduced in RRMS (509.3 ± 25.7 ml vs.
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Journal of neurology · Jul 2017
Assessment of the factorial validity and reliability of the ALSFRS-R: a revision of its measurement model.
The amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) is a widely used primary outcome measure in amyotrophic lateral sclerosis (ALS) clinical practice and clinical trials. ALSFRS-R items cannot, however, validly be summed to obtain a total score, but constitute domain scores reflecting a profile of disease severity. Currently, there are different measurement models for estimating domain scores. ⋯ Correlation between factors ranged from weak to modest, confirming that the ALSFRS-R constitutes a profile of four clinically relevant domain scores rather than a total score that expresses disease severity. The internal consistency of the four domain scores was satisfactory. Our revision of the measurement model may allow for a more adequate estimation of disease severity and disease progression in epidemiological studies and clinical trials.