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Erschienen in: Journal of Neurology 9/2020

04.05.2020 | Original Communication

A nerve conduction study predicts the prognosis of sporadic amyotrophic lateral sclerosis

verfasst von: Eriko Imai, Tomohiko Nakamura, Naoki Atsuta, Masahiro Nakatochi, Masashi Suzuki, Yumiko Harada, Ryoichi Nakamura, Naoki Hayashi, Gen Sobue, Masahisa Katsuno

Erschienen in: Journal of Neurology | Ausgabe 9/2020

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Abstract

Objective

To clarify the relationship between nerve conduction study (NCS) and prognosis in patients with amyotrophic lateral sclerosis (ALS).

Methods

We included 190 patients with sporadic ALS. We used onset age, sex, onset site (bulbar vs. spinal), revised El Escorial criteria category (definite vs. others), and the King’s clinical systems, and the Milano–Torino (MiToS) functional staging systems, and decline rates of revised ALS functional rating scale (ALSFRS-R) as known prognostic factors. An NCS was performed on the median, ulnar, tibial, and sural nerves. The endpoint was death or the introduction of tracheostomy positive-pressure ventilation. Multivariate analysis for each NCS variable, known prognostic factors was performed using Cox stepwise proportional hazards analysis. Univariate analysis was performed for NCS variables that showed a significant association with prognosis in multivariate analysis. Survival was analyzed with a Kaplan–Meier curve and log-rank test.

Results

The Cox model identified the compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes of the median nerve as prognostic factors. In the log-rank test, patients with higher median nerve CMAP amplitude had a significantly better prognosis than those with lower amplitude, regardless of age. And prognosis was better in the group with lower median nerve SNAP amplitude only in patients younger than the 25th percentile (~ 57 years).

Conclusions

CMAP and SNAP amplitudes of the median nerve are considered to be independent prognostic factors of sporadic ALS.
Literatur
1.
Zurück zum Zitat Atsuta N, Watanabe H, Ito M et al (2011) Development of a telephone survey system for patients with amyotrophic lateral sclerosis using the ALSFRS-R (Japanese version) and application of this system in a longitudinal multicenter study. Shinkei Kenkyu No Shinpo 63:491–496PubMed Atsuta N, Watanabe H, Ito M et al (2011) Development of a telephone survey system for patients with amyotrophic lateral sclerosis using the ALSFRS-R (Japanese version) and application of this system in a longitudinal multicenter study. Shinkei Kenkyu No Shinpo 63:491–496PubMed
2.
Zurück zum Zitat Watanabe H, Atsuta N, Hirakawa A et al (2016) A rapid functional decline type of amyotrophic lateral sclerosis is linked to low expression of TTN. J Neurol Neurosurg Psychiatry 87:851–858PubMed Watanabe H, Atsuta N, Hirakawa A et al (2016) A rapid functional decline type of amyotrophic lateral sclerosis is linked to low expression of TTN. J Neurol Neurosurg Psychiatry 87:851–858PubMed
3.
Zurück zum Zitat Chiò A, Mora G, Leone M et al (2002) Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology 59:99–103PubMed Chiò A, Mora G, Leone M et al (2002) Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology 59:99–103PubMed
4.
Zurück zum Zitat Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman OM (2000) Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population-based study. Arch Neurol 57:1171–1176PubMed Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman OM (2000) Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population-based study. Arch Neurol 57:1171–1176PubMed
5.
Zurück zum Zitat del Aguila MA, Longstreth WT Jr, McGuire V, Koepsell TD, van Belle G (2003) Prognosis in amyotrophic lateral sclerosis: a population-based study. Neurology 60:813–819PubMed del Aguila MA, Longstreth WT Jr, McGuire V, Koepsell TD, van Belle G (2003) Prognosis in amyotrophic lateral sclerosis: a population-based study. Neurology 60:813–819PubMed
6.
Zurück zum Zitat Millul A, Beghi E, Logroscino G, Micheli A, Vitelli E, Zardi A (2005) Survival of patients with amyotrophic lateral sclerosis in a population-based registry. Neuroepidemiology 25:114–119PubMed Millul A, Beghi E, Logroscino G, Micheli A, Vitelli E, Zardi A (2005) Survival of patients with amyotrophic lateral sclerosis in a population-based registry. Neuroepidemiology 25:114–119PubMed
7.
Zurück zum Zitat Chio A, Logroscino G, Hardiman O et al (2009) Prognostic factors in ALS: a critical review. Amyotrophic Lateral Scler 10:310–323 Chio A, Logroscino G, Hardiman O et al (2009) Prognostic factors in ALS: a critical review. Amyotrophic Lateral Scler 10:310–323
8.
Zurück zum Zitat Ning P, Yang B, Li S et al (2019) Systematic review of the prognostic role of body mass index in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 20:356–367PubMed Ning P, Yang B, Li S et al (2019) Systematic review of the prognostic role of body mass index in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 20:356–367PubMed
9.
Zurück zum Zitat Kimura F, Fujimura C, Ishida S et al (2006) Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology 66:265–267PubMed Kimura F, Fujimura C, Ishida S et al (2006) Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology 66:265–267PubMed
10.
Zurück zum Zitat Kollewe K, Mauss U, Krampfl K, Petri S, Dengler R, Mohammadi B (2008) ALSFRS-R score and its ratio: a useful predictor for ALS-progression. J Neurol Sci 275:69–73PubMed Kollewe K, Mauss U, Krampfl K, Petri S, Dengler R, Mohammadi B (2008) ALSFRS-R score and its ratio: a useful predictor for ALS-progression. J Neurol Sci 275:69–73PubMed
11.
Zurück zum Zitat Lanznaster D, Bejan-Angoulvant T, Patin F et al (2019) Plasma creatinine and amyotrophic lateral sclerosis prognosis: a systematic review and meta-analysis. Amyotroph Lateral Scler Frontotemporal Degener 20:199–206PubMed Lanznaster D, Bejan-Angoulvant T, Patin F et al (2019) Plasma creatinine and amyotrophic lateral sclerosis prognosis: a systematic review and meta-analysis. Amyotroph Lateral Scler Frontotemporal Degener 20:199–206PubMed
12.
Zurück zum Zitat Ikenaka K, Atsuta N, Maeda Y et al (2019) Increase of arginine dimethylation correlates with the progression and prognosis of ALS. Neurology 92:e1868–e1877PubMed Ikenaka K, Atsuta N, Maeda Y et al (2019) Increase of arginine dimethylation correlates with the progression and prognosis of ALS. Neurology 92:e1868–e1877PubMed
13.
Zurück zum Zitat Roche JC, Rojas-Garcia R, Scott KM et al (2012) A proposed staging system for amyotrophic lateral sclerosis. Brain 135:847–852PubMedPubMedCentral Roche JC, Rojas-Garcia R, Scott KM et al (2012) A proposed staging system for amyotrophic lateral sclerosis. Brain 135:847–852PubMedPubMedCentral
14.
Zurück zum Zitat Chiò A, Hammond ER, Mora G et al (2015) Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 86:38–44PubMed Chiò A, Hammond ER, Mora G et al (2015) Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 86:38–44PubMed
15.
Zurück zum Zitat Tramacere I, Della Bella E, Chiò A et al (2015) The MITOS system predicts long-term survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 86:1180–1185PubMed Tramacere I, Della Bella E, Chiò A et al (2015) The MITOS system predicts long-term survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 86:1180–1185PubMed
16.
Zurück zum Zitat Pinto S, de Carvalho M (2017) Comparison of slow and forced vital capacities on ability to predict survival in ALS. Amyotroph Lateral Scler and Frontotemporal Degener 18:528–533 Pinto S, de Carvalho M (2017) Comparison of slow and forced vital capacities on ability to predict survival in ALS. Amyotroph Lateral Scler and Frontotemporal Degener 18:528–533
17.
Zurück zum Zitat Watanabe H, Atsuta N, Nakamura R et al (2015) Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener 16:230–236PubMed Watanabe H, Atsuta N, Nakamura R et al (2015) Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener 16:230–236PubMed
18.
Zurück zum Zitat Hama T, Hirayama M, Hara T et al (2012) Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials. Muscle Nerve 45:169–174PubMed Hama T, Hirayama M, Hara T et al (2012) Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials. Muscle Nerve 45:169–174PubMed
19.
Zurück zum Zitat Ohashi Y, Tashiro K, Itoyama Y et al (2001) Study of functional rating scale for amyotrophic lateral sclerosis: revised ALSFRS (ALSFRS-R) Japanese version. No To Shinkei 53:346–355PubMed Ohashi Y, Tashiro K, Itoyama Y et al (2001) Study of functional rating scale for amyotrophic lateral sclerosis: revised ALSFRS (ALSFRS-R) Japanese version. No To Shinkei 53:346–355PubMed
20.
Zurück zum Zitat Kimura J (2001) Electrodiagnosis in diseases of nerve and muscle: principles and practice, 3rd edn. Oxford University Press, New York, pp 130–177 Kimura J (2001) Electrodiagnosis in diseases of nerve and muscle: principles and practice, 3rd edn. Oxford University Press, New York, pp 130–177
21.
Zurück zum Zitat Koike H, Mori K, Misu K et al (2001) Painful alcoholic polyneuropathy with predominant small-fiber loss and normal thiamine status. Neurology 56:1727–1732PubMed Koike H, Mori K, Misu K et al (2001) Painful alcoholic polyneuropathy with predominant small-fiber loss and normal thiamine status. Neurology 56:1727–1732PubMed
22.
Zurück zum Zitat Suzuki K, Katsuno M, Banno H et al (2008) CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA. Brain 131:229–239PubMed Suzuki K, Katsuno M, Banno H et al (2008) CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA. Brain 131:229–239PubMed
23.
Zurück zum Zitat American Association of Electrodiagnostic Medicine, American Academy of Neurology, and American Academy of Physical Medicine and Rehabilitation (2002) Practice parameter for electrodiagnostic studies in carpal tunnel syndrome: summary statement. Muscle Nerve 25:918–922 American Association of Electrodiagnostic Medicine, American Academy of Neurology, and American Academy of Physical Medicine and Rehabilitation (2002) Practice parameter for electrodiagnostic studies in carpal tunnel syndrome: summary statement. Muscle Nerve 25:918–922
24.
Zurück zum Zitat Kano O, Iwamoto K, Ito H et al (2013) Limb-onset amyotrophic lateral sclerosis patients visiting orthopedist show a longer time-to-diagnosis since symptom onset. BMC Neurol 13:19PubMedPubMedCentral Kano O, Iwamoto K, Ito H et al (2013) Limb-onset amyotrophic lateral sclerosis patients visiting orthopedist show a longer time-to-diagnosis since symptom onset. BMC Neurol 13:19PubMedPubMedCentral
25.
Zurück zum Zitat Kihira T, Yoshida S, Okamoto K et al (2008) Survival rate of patients with amyotrophic lateral sclerosis in Wakayama Prefecture, Japan, 1966 to 2005. J Neurol Sci 268:95–101PubMed Kihira T, Yoshida S, Okamoto K et al (2008) Survival rate of patients with amyotrophic lateral sclerosis in Wakayama Prefecture, Japan, 1966 to 2005. J Neurol Sci 268:95–101PubMed
26.
Zurück zum Zitat Shin JO (1993) Clinical electromyography: nerve conduction studies, 3rd edn. Williams and Wilkins, Baltimore, pp 582–600 Shin JO (1993) Clinical electromyography: nerve conduction studies, 3rd edn. Williams and Wilkins, Baltimore, pp 582–600
27.
Zurück zum Zitat Bradley WG, Good P, Rasool CG, Adelman LS (1983) Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis. Ann Neurol 14:267–277PubMed Bradley WG, Good P, Rasool CG, Adelman LS (1983) Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis. Ann Neurol 14:267–277PubMed
28.
Zurück zum Zitat Kawamura Y, Dyck PJ, Shimono M, Okazaki H, Tateishi J, Doi H (1981) Morphometric comparison of the vulnerability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 40:667–675PubMed Kawamura Y, Dyck PJ, Shimono M, Okazaki H, Tateishi J, Doi H (1981) Morphometric comparison of the vulnerability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 40:667–675PubMed
29.
Zurück zum Zitat Wilbourn AJ (2000) The “split hand syndrome”. Muscle Nerve 23:138PubMed Wilbourn AJ (2000) The “split hand syndrome”. Muscle Nerve 23:138PubMed
30.
Zurück zum Zitat Kuwabara S, Sonoo M, Komori T et al (2008) Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity. Muscle Nerve 37:426–430PubMed Kuwabara S, Sonoo M, Komori T et al (2008) Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity. Muscle Nerve 37:426–430PubMed
31.
Zurück zum Zitat Eisen A, Kuwabara S (2012) The split hand syndrome in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 83:399–403PubMed Eisen A, Kuwabara S (2012) The split hand syndrome in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 83:399–403PubMed
32.
Zurück zum Zitat Behse F (1978) Sensory action potentials and biopsy of the sural nerve in neuropathy. Brain 101:473–493PubMed Behse F (1978) Sensory action potentials and biopsy of the sural nerve in neuropathy. Brain 101:473–493PubMed
33.
Zurück zum Zitat Hammad M, Silva A, Glass J, Sladky JT, Benatar M (2007) Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology 69:2236–2242PubMed Hammad M, Silva A, Glass J, Sladky JT, Benatar M (2007) Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology 69:2236–2242PubMed
34.
Zurück zum Zitat Pugdahl K, Fuglsang-Frederiksen A, de Carvalho M et al (2007) Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study. J Neurol Neurosurg Psychiatry 78:746–749PubMedPubMedCentral Pugdahl K, Fuglsang-Frederiksen A, de Carvalho M et al (2007) Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study. J Neurol Neurosurg Psychiatry 78:746–749PubMedPubMedCentral
35.
Zurück zum Zitat Isak B, Tankisi H, Johnsen B et al (2016) Involvement of distal sensory nerves in amyotrophic lateral sclerosis. Muscle Nerve 54:1086–1092PubMed Isak B, Tankisi H, Johnsen B et al (2016) Involvement of distal sensory nerves in amyotrophic lateral sclerosis. Muscle Nerve 54:1086–1092PubMed
36.
Zurück zum Zitat Truini A, Biasiotta A, Onesti E et al (2015) Small-fibre neuropathy related to bulbar and spinal-onset in patients with ALS. J Neurol 262:1014–1018PubMed Truini A, Biasiotta A, Onesti E et al (2015) Small-fibre neuropathy related to bulbar and spinal-onset in patients with ALS. J Neurol 262:1014–1018PubMed
37.
Zurück zum Zitat Dalla Bella E, Lombardi R, Porretta-Serapiglia C et al (2016) Amyotrophic lateral sclerosis causes small fiber pathology. Eur J Neurol 23:416–420PubMed Dalla Bella E, Lombardi R, Porretta-Serapiglia C et al (2016) Amyotrophic lateral sclerosis causes small fiber pathology. Eur J Neurol 23:416–420PubMed
38.
Zurück zum Zitat Taylor PK (1993) CMAP dispersion, amplitude decay, and area decay in a normal population. Muscle Nerve 16:1181–1187PubMed Taylor PK (1993) CMAP dispersion, amplitude decay, and area decay in a normal population. Muscle Nerve 16:1181–1187PubMed
39.
Zurück zum Zitat Martínez-Silva ML, Imhoff-Manuel RD, Sharma A et al (2018) Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS. Elife 7:e30955PubMedPubMedCentral Martínez-Silva ML, Imhoff-Manuel RD, Sharma A et al (2018) Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS. Elife 7:e30955PubMedPubMedCentral
40.
Zurück zum Zitat Kanai K, Shibuya K, Sato Y et al (2012) Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 83:734–738PubMed Kanai K, Shibuya K, Sato Y et al (2012) Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 83:734–738PubMed
41.
Zurück zum Zitat Sangari S, Iglesias C, El Mendili MM et al (2016) Impairment of sensory-motor integration at spinal level in amyotrophic lateral sclerosis. Clin Neurophysiol 127:1968–1977PubMed Sangari S, Iglesias C, El Mendili MM et al (2016) Impairment of sensory-motor integration at spinal level in amyotrophic lateral sclerosis. Clin Neurophysiol 127:1968–1977PubMed
42.
Zurück zum Zitat Kieran D, Hafezparast M, Bohnert S et al (2005) A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J Cell Biol 169:561–567PubMedPubMedCentral Kieran D, Hafezparast M, Bohnert S et al (2005) A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J Cell Biol 169:561–567PubMedPubMedCentral
43.
Zurück zum Zitat Ilieva HS, Yamanaka K, Malkmus S et al (2008) Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci USA 105:12599–12604PubMed Ilieva HS, Yamanaka K, Malkmus S et al (2008) Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci USA 105:12599–12604PubMed
44.
Zurück zum Zitat Nakamura R, Sone J, Atsuta N et al (2016) Next-generation sequencing of 28 ALS-related genes in a Japanese ALS cohort. Neurobiol Aging 39:219.e1–8 Nakamura R, Sone J, Atsuta N et al (2016) Next-generation sequencing of 28 ALS-related genes in a Japanese ALS cohort. Neurobiol Aging 39:219.e1–8
45.
Zurück zum Zitat Swash M, de Carvalho M (2019) The 'neurophysiological index' predicts survival in amyotrophic lateral sclerosis. Clin Neurophysiol 130:1684–1685PubMed Swash M, de Carvalho M (2019) The 'neurophysiological index' predicts survival in amyotrophic lateral sclerosis. Clin Neurophysiol 130:1684–1685PubMed
Metadaten
Titel
A nerve conduction study predicts the prognosis of sporadic amyotrophic lateral sclerosis
verfasst von
Eriko Imai
Tomohiko Nakamura
Naoki Atsuta
Masahiro Nakatochi
Masashi Suzuki
Yumiko Harada
Ryoichi Nakamura
Naoki Hayashi
Gen Sobue
Masahisa Katsuno
Publikationsdatum
04.05.2020
Verlag
Springer Berlin Heidelberg
Erschienen in
Journal of Neurology / Ausgabe 9/2020
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-020-09858-5

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