Prediction of prognosis of ALS: Importance of active denervation findings of the cervical-upper limb area and trunk area

Nakatani, Eiji; Watanabe, Yasuhiro; Fukushima, Masanori; Nakashima, Kenichiro; Kannagi, Mari; Kanatani, Yasuhiro; Mizushima, Hiroshi

doi:10.5582/irdr.2015.01043

Cited by 11 publications

(9 citation statements)

References 26 publications

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“…Subjects were then divided into categories, according to the presence of 1) none or one region 2) two regions, 3) three regions showing signs of UMN and/or LMN degeneration [3,9]. Given that denervation activity has been previously described as an EMG marker associated with poor prognosis in ALS [21,22], we chose to investigate the correlations, if any, between CSF biomarker changes and a denervation score (DS).…”

Section: Case Classificationmentioning

confidence: 99%

Diagnostic-prognostic value and electrophysiological correlates of CSF biomarkers of neurodegeneration and neuroinflammation in amyotrophic lateral sclerosis

Abu‐Rumeileh

Vacchiano²,

Zenesini

et al. 2020

J Neurol

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Neurofilament light chain protein (NfL) is currently the most accurate cerebrospinal fluid (CSF) biomarker in amyotrophic lateral sclerosis (ALS) in terms of both diagnostic and prognostic values, but the mechanism underlying its increase is still a matter of debate. Similarly, emerging CSF biomarkers of neurodegeneration and neuroinflammation showed promising results, although further studies are needed to clarify their clinical and pathophysiological roles. In the present study we compared the diagnostic accuracy of CSF NfL, phosphorylated (p)-tau/total (t)-tau ratio, chitinase-3like protein 1 (YKL-40) and chitotriosidase 1 (CHIT1), in healthy controls (n = 43) and subjects with ALS (n = 80) or ALS mimics (n=46). In ALS cases, we also investigated the association between biomarker levels and clinical variables, the extent of upper motor neuron (UMN) and lower motor neuron (LMN) degeneration, and denervation activity through electromyography (EMG). ALS patients showed higher levels of CSF NfL, YKL-40, CHIT1, and lower values of p-tau/ttau ratio compared to both controls and ALS mimics. Among all biomarkers, NfL yielded the highest diagnostic performance (> 90% sensitivity and specificity) and was the best predictor of disease progression rate and survival in ALS. NfL levels showed a higher correlation with the extent of LMN involvement, whereas YKL-40 levels increased together with the number of areas showing both UMN and LMN damage. EMG denervation activity did not correlate with any CSF biomarker change. These findings confirm the highest value of NfL among currently available CSF biomarkers for the diagnostic and prognostic assessment of ALS and contribute to the understanding of the pathophysiological and electrophysiological correlates of biomarker changes.

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Section: Case Classificationmentioning

confidence: 99%

Diagnostic-prognostic value and electrophysiological correlates of CSF biomarkers of neurodegeneration and neuroinflammation in amyotrophic lateral sclerosis

Abu‐Rumeileh

Vacchiano²,

Zenesini

et al. 2020

J Neurol

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“…Another important prognostic factor was cervical weakness, with a relative survival risk of 128%. Other studies [20,21] agree that cervical weakness is a factor associated with poor prognosis and lower survival.…”

Section: Discussionmentioning

confidence: 86%

A Predictive Model for Prognosis in Motor Neuron Disease

Moura¹,

Casulari²,

Novaes³

2016

J Neurol Disord

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Objectives: There have been attempts to establish biomarkers for motor neuron disease, without success. The study aim to seek possible markers to be used in the clinical routine evaluation, to optimize timing for palliative interventions.Methods: A cohort study evaluated clinical, respiratory and neurophysiological variables every 3-4 months across 20 months in 101 patients with motor neuron disease using riluzole. Primary endpoint was death or tracheostomy. The most significant parameters in cox regression analysis created a predictive model.Results: There were 58 men and 43 women with a mean age of 57.2 ± 11.7 years. 77 patients (76.2%) had spinal onset and 24 (23.8%) had bulbar onset. The mean survival time was 43.5 ± 5.7 months ). The variables related to worse prognosis were: age > 65 years (HR=2.50, CI 95% (1.23-5.08); involvement of a second site in less than six months (HR=2.02, CI 95% (1.04 -3.94); supine Forced Vital Capacity <63% (HR=2.78, CI 95% 1.03-7.48), neck weakness (HR=2.28, CI 95% (1.03-5.05) and presence of pyramidal syndrome (HR= 2.36, CI 95% (1.05-5.33). Conclusion:It was created a five-factors set that predicts evolution to death or tracheostomy within one year.

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“…To our knowledge, there has been no study examining the state of NF1 progression using a registry of patients who submitted claims for medical expense subsidies. However, there have been studies regarding other rare diseases, such as amyotrophic lateral sclerosis [21] and spinocerebellar ataxia [1], using a registry of patients who submitted claims to receive medical expense subsidies in Japan. The registration system enables researchers involved in the MHLW research system for intractable rare diseases to utilize the anonymized national registry of individuals with these diseases.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of neurofibromatosis type 1 progression using a nationwide registry of patients who submitted claims for medical expense subsidies in Japan between 2008 and 2012

Yamauchi

Suka

Nishigori

et al. 2019

Orphanet J Rare Dis

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Background No study to date has followed disease progression in patients with neurofibromatosis type 1 (NF1), including the incidence of various manifestations, using a national registry. Here we examined the state of NF1 progression using a nationwide registry of patients who submitted claims to receive medical expense subsidies for NF1 in Japan over a five-year period. A total of 342 eligible patients (194 females and 148 males) with NF1 who newly submitted claims for medical expense subsidies in Japan in 2008 were followed until 2012. Results More than half of the patients were classified as Stage 5 in 2008. Of the eligible patients, 205 (60%) submitted claims to renew the subsidies between 2009 and 2012. During the study period, NF1 stage progressed in 30 patients, yielding an overall stage progression rate of 19% and progression incidence rate per 100 person-years of 12.2. Both stage progression rate and progression incidence rate were the highest in the 0–19 year age group at the time of registration and, as compared to other age groups, progression of neurological and bone manifestations was more prevalent in this age group. Conclusions The progression of neurological and bone manifestations was more prevalent in the 0–19 year age group compared to other age groups. The registry we used in the present study is useful for understanding the characteristics of patients with uncommon conditions, such as NF1. Our findings also highlight the feasibility of conducting quality research using registries of patients with rare diseases, such as NF1, that were not designed specifically for scientific research. Electronic supplementary material The online version of this article (10.1186/s13023-019-1148-8) contains supplementary material, which is available to authorized users.

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Prediction of prognosis of ALS: Importance of active denervation findings of the cervical-upper limb area and trunk area

Cited by 11 publications

References 26 publications

Diagnostic-prognostic value and electrophysiological correlates of CSF biomarkers of neurodegeneration and neuroinflammation in amyotrophic lateral sclerosis

Diagnostic-prognostic value and electrophysiological correlates of CSF biomarkers of neurodegeneration and neuroinflammation in amyotrophic lateral sclerosis

A Predictive Model for Prognosis in Motor Neuron Disease

Evaluation of neurofibromatosis type 1 progression using a nationwide registry of patients who submitted claims for medical expense subsidies in Japan between 2008 and 2012

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