Neurophysiological indices in amyotrophic lateral sclerosis correlate with functional outcome measures, staging and disease progression

תקציר:

OBJECTIVE: This study examined neurophysiological (NI), split-hand (SI) and split-leg (SLI) index in patients with amyotrophic lateral sclerosis (ALS), and their correlation with functional status, disease duration, staging and survival. METHODS: Eighty-two patients underwent nerve conduction study to analyze NI, SI and SLI. Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), disease progression rate ($Δ$FS), Milano-Torino (MiToS) and King's staging systems, Forced Vital Capacity (FVC), and survival data were collected. RESULTS: Both NI and SI indices were significantly associated with ALSFRS-R, MiToS, King's and FVC. Slow progressor patients ($Δ$FS < 0.5) reported a significantly higher NI and SI values compared to both normal (0.5 $łeq$ $Δ$FS < 1.00) and fast progressors ($Δ$FS $\geq$ 1.0). After dichotomizing patients in slow progressors ($Δ$FS < 0.5) and not-slow progressors ($Δ$FS $\geq$ 0.5), a combination of SI index and disease duration revealed to be the best prediction model to discriminate patients in accordance with their disease progression (c-index: 0.92), leading to a new prognostic index: the 'Split-Hand prognostic index' (SHpi). CONCLUSION: SI and NI are correlated with functional status and FVC. SHpi index could represent an useful tool to discriminate patients in accordance with their disease progression. SIGNIFICANCE: These data provide novel evidence of neurophysiological indices as promising biomarkers in ALS.