Abstract

BackgroundUp to 50% of motor neuron disease (MND) patients show neuropsychological deficits which negatively affect prognosis and care. However, disability-related logistical issues and uneven geographical coverage of healthcare services may prevent MND patients from accessing neuropsychological evaluations. This study thus aimed to standardize for the Italian population the ALS Cognitive Behavioral Screen-Phone Version (ALS-CBS™-PhV), an MND-specific, telephone-based screening for frontotemporal dysfunction.MethodsThe cognitive section of the ALS-CBS™-PhV, the Italian telephone-based Mini-Mental State Examination (Itel-MMSE), and the Telephone Interview for Cognitive Status (TICS) was administered to 359 healthy individuals (143 males, 216 females; age, 52.7 ± 15.8; education, 13.1 ± 4.4). Norms were derived through equivalent scores. Validity, factorial structure, reliability, diagnostic accuracy, and item difficulty and discrimination were examined. Statistical equivalence between the telephone-based and in-person versions was tested.ResultsALS-CBS™-PhV measures were predicted by age and education. The ALS-CBS™-PhV reflected a mono-component structure, converged with Itel-MMSE and TICS scores (rs = .23–.51) and was equivalent to its in-person format (t = .37; p = .72). Good internal (Cronbach’s α = .61), test–retest (ICC = .69), and inter-rater (ICC = .96) reliability was detected. High accuracy was found when tested against both the Itel-MMSE and the TICS (AUC = .82–89). Backward digit span items were the most discriminative.DiscussionThe ALS-CBS™-PhV is a statistically solid screening test for frontotemporal disorders featuring MND. Its standardization allows for (1) improvements in tele-healthcare for MND patients, (2) epidemiological applications, and (3) effective assessments in decentralized clinical trials. The ALS-CBS™-PhV can be also suitable for assessing bedridden and visually impaired patients with motor disorders.

Highlights

  • Since motor neuron diseases (MNDs) and frontotemporal (FT) degenerations are pathophysiologically related [6], neuropsychological deficits within the FT spectrum occur in up to 50% of MND patients [34].Nadia Bolognini and Ildebrando Appollonio contributed to this work.Extended author information available on the last page of the articleEarly detection of FT involvement in MND is crucial due to its unfavorable impact on patients’ prognosis and management [24]

  • The ALS-CBSTM accounts for motor disabilities and targets FT functions

  • Three hundred fifty-nine healthy individuals were recruited from different regions of Italy

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Summary

Introduction

Since motor neuron diseases (MNDs) and frontotemporal (FT) degenerations are pathophysiologically related [6], neuropsychological deficits within the FT spectrum occur in up to 50% of MND patients [34].Nadia Bolognini and Ildebrando Appollonio contributed to this work.Extended author information available on the last page of the articleEarly detection of FT involvement in MND is crucial due to its unfavorable impact on patients’ prognosis and management [24]. Neuropsychological screening in MND patients has entered the customary clinical practice [40], with an ad hoc nosographic system [34] and the development of MND-specific diagnostic tools [33]. Among the latter, the ALS Cognitive Behavioral Screen (ALS-CBSTM) [39] represents a guideline-recommended [34], I-level test. This study aimed to standardize for the Italian population the ALS Cognitive Behavioral Screen-Phone Version (ALS-CBSTM-PhV), an MND-specific, telephone-based screening for frontotemporal dysfunction. The ALS-CBSTM-PhV reflected a mono-component structure, converged with Itel-MMSE and TICS scores (rs = .23–.51) and was equivalent to its in-person format (t = .37; p = .72). The ALS-CBSTM-PhV can be suitable for assessing bedridden and visually impaired patients with motor disorders

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