Amyotrophic lateral sclerosis: moving towards a new classification system

  title={Amyotrophic lateral sclerosis: moving towards a new classification system},
  author={Ammar Al-Chalabi and Orla Hardiman and Matthew C. Kiernan and Adriano Chi{\`o} and Benjamin Rix-Brooks and Leonard H. Berg},
  journal={The Lancet Neurology},

Amyotrophic lateral sclerosis: a clinical review

Different aspects of ALS are discussed, including epidemiology, aetiology, pathogenesis, clinical features, differential diagnosis, investigations, treatment and future prospects.

Amyotrophic lateral sclerosis: disease state overview.

  • D. Hulisz
  • Psychology, Medicine
    The American journal of managed care
  • 2018
The best practices for the management of ALS include an interdisciplinary approach aimed at addressing the physical and psychological needs and desires of patients and their families and caregivers.

Amyotrophic lateral sclerosis: a complex syndrome that needs an integrated research approach

The complexity of the disease is commented on, some recent findings are suggested, and how amyotrophic lateral sclerosis research might be reoriented to foster the advance in the diagnostic and therapeutic questions are suggested.

Lower and upper motor neuron involvement and their impact on disease prognosis in amyotrophic lateral sclerosis

This review focuses on the current approaches in amyotrophic lateral sclerosis classification and staging system based on clinical examination and additional instrumental methods, highlighting the role of upper and lower motor neuron involvement in different phenotypes of the disease.

Diagnostics of Amyotrophic Lateral Sclerosis: Up to Date

The typical form of ALS with upper and lower motoneuron involvement and progressive bulbar paralysis with bulbar muscle involvement is observed and survival may be prolonged based on prompt diagnosis, ALS subtype and proper management with supportive treatment.

Focus on the heterogeneity of amyotrophic lateral sclerosis

A promising area of progress in modeling the heterogeneity of human ALS is represented by the use of human induced pluripotent stem cell (iPSCs)-derived motor neurons, and although the translational value of iPSCs remains unclear, this model is attractive in the perspective of replicating theogeneity of sporadic ALS.

Amyotrophic lateral sclerosis

Two possible disease-modifying therapies that can slow disease progression are available for ALS, but patient management is largely mediated by symptomatic therapies, such as the use of muscle relaxants for spasticity and speech therapy for dysarthria.

Clinical Controversies in Amyotrophic Lateral Sclerosis

The deposition of TAR DNA-binding protein 43 is also a relevant finding given the overlap with frontotemporal dysfunction and is proposed for consideration of future diagnostic paradigms.

Language impairment in motor neuron disease phenotypes different from classical amyotrophic lateral sclerosis: a review

Patients with different-from-classical-ALS MND phenotypes display language deficits similar to those of classical ALS patients (as far as both prevalence and type are concerned) and thus could be validly included in the MND-FTLD continuum at a neuropsychological level.



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.

El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis

  • B. BrooksRobert G. MillerM. SwashT. Munsat
  • Medicine, Psychology
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2000
The criteria described below represent the result of a three-day workshop, convened at Airlie Conference Center, Warrenton, Virginia on 2–4 April, 1998 by the World Federation of Neurology Research Committee on Motor Neuron Diseases, and are placed on the WFN ALS website.

Biomarkers in amyotrophic lateral sclerosis

The genetics and neuropathology of amyotrophic lateral sclerosis

The genetic architecture of ALS is explored, some of the genes implicated in pathogenesis are highlighted, and their phenotypic range and overlap with other diseases are described.

Analysis of terms used for the diagnosis and classification of amyotrophic lateral sclerosis and motor neuron disease

A five-question online survey with ALS specialists explored the range of descriptors and how they are used, finding that the most frequently used labels were ‘ALS’, ‘PLS’ and ‘familial’.

Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study

The epidemiological findings suggest that ALS phenotypes carry distinctive and easily distinguishable clinical and prognostic characteristics, strongly related to a complex interplay between gender and age.

ALS motor phenotype heterogeneity, focality, and spread

Heterogeneity of motor phenotypes suggests motor neuron degeneration in ALS is in actuality a very orderly and actively propagating process and that fundamental molecular mechanisms may be uniform and their chief properties deduced.

Natural history of young-adult amyotrophic lateral sclerosis

The findings show that young-adult amyotrophic lateral sclerosis with the predominant upper motor neuron phenotype represents a distinctive clinical variant characterized by a unique clinical pattern, longer survival, and male prevalence.

Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

The only clinical feature that distinguishes recognized hereditary from apparently sporadic ALS is a lower mean age of onset in the former, and all the clinical features reported in hereditary cases have also been observed in sporadic cases.

Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up.

The findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS, and which clinical features at onset and during follow-up could help differentiate between PLS and ALS.