Prognostic factors for the course of functional status of patients with ALS: a systematic review

  title={Prognostic factors for the course of functional status of patients with ALS: a systematic review},
  author={Huub Creemers and Hepke F. Grupstra and Frans Nollet and Leonard H. van den Berg and Anita Beelen},
  journal={Journal of Neurology},
The progressive course of amyotrophic lateral sclerosis (ALS) results in an ever-changing spectrum of the care needs of patients with ALS. Knowledge of prognostic factors for the functional course of ALS may enhance clinical prediction and improve the timing of appropriate interventions. Our objective was to systematically review the evidence regarding prognostic factors for the rate of functional decline of patients with ALS, assessed with versions of the ALS Functional Rating Scale (ALSFRS… 

Prognostic value of clinical and electrodiagnostic parameters at time of diagnosis in patients with amyotrophic lateral sclerosis

Clinical and electrodiagnostic data at time of diagnosis provide additional prognostic information compared to other known prognostic factors, and diagnostic category according to Awaji and the extensiveness of LMN involvement contain the most additional value.

Cardiovascular diseases may play a negative role in the prognosis of ALS

A large, multicenter study demonstrated that the presence of dementia, hypertension, heart diseases, chronic obstructive pulmonary disease, haematological and psychiatric diseases were independent prognostic factors of survival in ALS.

Factors predicting survival in ALS: a multicenter Italian study

The authors' large, multicenter study demonstrated the role of some clinical and demographic factors on ALS survival, and showed some interesting differences between referral centers’ patients and the general ALS population.

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

It is confirmed that ALSFRS-R decline is not homogeneous among ALS patients and during the disease, and factors influencing this trend may not match with those affecting survival.

Milano–Torino Staging and Long-Term Survival in Chinese Patients with Amyotrophic Lateral Sclerosis

MITOS progression during the early disease course could serve as a prognostic marker of long-term survival and may have utility in clinical trials.

Serum C-Reactive Protein as a Prognostic Biomarker in Amyotrophic Lateral Sclerosis

It is suggested that patients with ALS and elevated serumCRP levels progress more rapidly than do those with lower CRP levels and that this elevation may reflect a neuroinflammatory state potentially responsive to the immune regulators such as NP001.

The frontotemporal syndrome of ALS is associated with poor survival

There is an association between the frontotemporal syndrome and poor survival in ALS, which remains present after initiation of NIV.

Metabolic Alteration and Amyotrophic Lateral Sclerosis Outcome: A Systematic Review

To provide a reliable record, a public policy in which all research and clinical centers might assess the parameters discussed herein is suggested is suggested to provide a comprehensible database to facilitate multicentered collaboration, validation, and clinical translation.

Unraveling the Complexity of Amyotrophic Lateral Sclerosis Survival Prediction

The dimensionality and fluctuating predictors of ALS survival must be considered when developing predictive models for clinical trial development or in-clinic usage and is needed for population and personalized forecasting of survival.



Prognostic factors in ALS: A critical review

A systematic review of factors related to survival in ALS revealed the rate of symptom progression was revealed to be an independent prognostic factor, and these findings have relevant implications for the design of future trials.

A role for functional classification in the early identification of prognostic factors in ALS

  • Amy C RauchwayK. Kaiboriboon G. Hayat
  • Psychology, Medicine
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2007
The results, which showed a median of four months duration to initial electrodiagnostics for Group I (ALSFRS scores below 26) versus 10 months (range 1–24 months) for Group II (p = 0.02), suggest this measure is a marker of disease progression in ALS.

Analysis of factors that modify susceptibility and rate of progression in amyotrophic lateral sclerosis (ALS)

  • M. Muddasir QureshiD. Hayden M. Cudkowicz
  • Medicine, Psychology
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2006
D disease progression was faster in the ALS subjects having bulbar onset and a shorter time period between onset of symptoms and diagnosis, and significant risk factors for ALS included reported exposure to lead and pesticides.


A retrospective multivariate analysis of 713 patients with ALS over a 20 year period from the South-East England Amyotrophic Lateral Sclerosis population register found it is possible to correctly classify patients into prognostic categories using clinical data easily available at time of diagnosis.

Can selection of rapidly progressing patients shorten clinical trials in amyotrophic lateral sclerosis?

This strategy of selecting patients with rapidly progressing ALS for inclusion in exploratory, short phase II clinical trials offers substantial savings in costs and time, and could accelerate the process of testing potentially useful drugs for the treatment of ALS.

ALSFRS and appel ALS scores: Discordance with disease progression

Both scales showed significant change with disease progression, but ALSFRS consistently underestimated disease severity defined by AALS (P < 0.001).

A proposed staging system for amyotrophic lateral sclerosis

Staging criteria for amyotrophic lateral sclerosis is proposed to provide a universal and objective measure of disease progression with benefits for patient care, resource allocation, research classifications and clinical trial design and validation of this staging system will require further studies.

Medications and laboratory parameters as prognostic factors in amyotrophic lateral sclerosis

It is concluded that aspirin or NSAID use may shorten survival in ALS, while calcium use may prolong survival, and a need to further explore the role of neuroinflammation in the pathogenesis of ALS.

Evaluation of the Quality of Prognosis Studies in Systematic Reviews

A review of reviews to describe methods used to assess the quality of prognosis studies and to describe how well current practices assess potential biases is to develop recommendations to guide future quality appraisal.