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February 05, 2019; 92 (6) Article

Amyotrophic lateral sclerosis diagnostic index

Toward a personalized diagnosis of ALS

Nimeshan Geevasinga, James Howells, Parvathi Menon, View ORCID ProfileMehdi van den Bos, Kazumoto Shibuya, José Manuel Matamala, Susanna B. Park, Karen Byth, Matthew C. Kiernan, Steve Vucic
First published January 11, 2019, DOI: https://doi.org/10.1212/WNL.0000000000006876
Nimeshan Geevasinga
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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James Howells
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Parvathi Menon
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Mehdi van den Bos
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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  • ORCID record for Mehdi van den Bos
Kazumoto Shibuya
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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José Manuel Matamala
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Susanna B. Park
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Karen Byth
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Matthew C. Kiernan
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Steve Vucic
From Westmead Clinical School (N.G., P.M., M.v.d.B., S.V.), Brain and Mind Center (J.H., K.S., J.M.M., S.B.P., M.C.K.), and NHMRC Clinical Trials Centre (K.B.), University of Sydney; and Westmead Hospital (K.B.), Research and Education Network, Sydney, Australia.
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Citation
Amyotrophic lateral sclerosis diagnostic index
Nimeshan Geevasinga, James Howells, Parvathi Menon, Mehdi van den Bos, Kazumoto Shibuya, José Manuel Matamala, Susanna B. Park, Karen Byth, Matthew C. Kiernan, Steve Vucic
Neurology Feb 2019, 92 (6) e536-e547; DOI: 10.1212/WNL.0000000000006876

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Abstract

Objective The aim of the study was to assess the utility of a novel amyotrophic lateral sclerosis (ALS) diagnostic index (ALSDI).

Methods A prospective multicenter study was undertaken on patients presenting with suspected ALS. The reference standard (Awaji criteria) was applied to all patients at recruitment. Patients were randomly assigned to a training (75%) and a test (25%) cohort. The ALSDI was developed in the training cohort and its diagnostic utility was subsequently assessed in the test cohort.

Results A total of 407 patients were recruited, with 305 patients subsequently diagnosed with ALS and 102 with a non-ALS mimicking disorder. The ALSDI reliably differentiated ALS from neuromuscular disorders in the training cohort (area under the curve 0.92, 95% confidence interval 0.89–0.95), with ALSDI ≥4 exhibiting 81.6% sensitivity, 89.6% specificity, and 83.5% diagnostic accuracy. The ALSDI diagnostic utility was confirmed in the test cohort (area under the curve 0.90, 95% confidence interval 0.84–0.97), with ALSDI ≥4 exhibiting 83.3% sensitivity, 84% specificity, and 83.5% diagnostic accuracy. In addition, the diagnostic utility of the ALSDI was confirmed in patients who were Awaji negative at recruitment and in those exhibiting a predominantly lower motor neuron phenotype.

Conclusion The ALSDI reliably differentiates ALS from mimicking disorders at an early stage in the disease process.

Classification of evidence This study provides Class I evidence that for patients with suspected ALS, the ALSDI distinguished ALS from neuromuscular mimicking disorders.

Glossary

ALS=
amyotrophic lateral sclerosis;
ALSDI=
amyotrophic lateral sclerosis diagnostic index;
ALSFRS-R=
Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised;
AUC=
area under the curve;
CI=
confidence interval;
CMAP=
compound muscle action potential;
CSP=
cortical silent period;
ICF=
intracortical facilitation;
LMN=
lower motor neuron;
MEP=
motor evoked potential;
MRC=
Medical Research Council;
OR=
odds ratio;
SICI=
short-interval intracortical inhibition;
TMS=
transcranial magnetic stimulation;
UMN=
upper motor neuron

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Editorial page 255

  • Class of Evidence: NPub.org/coe

  • Received April 3, 2018.
  • Accepted in final form September 28, 2018.
  • © 2019 American Academy of Neurology
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