Assessment of the upper motor neuron in amyotrophic lateral sclerosis

  title={Assessment of the upper motor neuron in amyotrophic lateral sclerosis},
  author={William Huynh and Neil G. Simon and Julian Grosskreutz and Martin R. Turner and Steve Vucic and Matthew C. Kiernan},
  journal={Clinical Neurophysiology},

Upper motor neuron assessment and early diagnosis in ALS

Early diagnosis of ALS is challenging, and may be made more so by the fact that progressive LMN weakness can be caused by so many other pure motor disorders (e.g., motor radiculopathy, multifocal motor neuropathy, myasthenia gravis, and the inflammatory myopathies).

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.

Methods of Detecting Lesions of Upper Motor Neuron in Amyotrophic Lateral Sclerosis using Transcranial Magnetic Stimulation

Navated TMS is considered as a promising technology for identifying upper motor neuron involvement in patients with ALS because it allows assessing the excitability of motor cortex, corticospinal tract function, and mapping of cortical representation of the muscles.

Functional Biomarkers for Amyotrophic Lateral Sclerosis

An overview of recently developed novel non-invasive electrophysiological techniques that may serve as biomarkers to assess UMN and LMN dysfunction in ALS patients is provided.

Neurophysiological features of primary lateral sclerosis

Trans transcranial magnetic stimulation is sensitive to identify cortical dysfunction in PLS and might have potential for monitoring UMN function in longitudinal studies and in clinical trials and the findings of TMS need to be interpreted in the context of the clinical presentation and phenotype.

Primary lateral sclerosis: consensus diagnostic criteria

An updated consensus diagnostic criteria for primary lateral sclerosis is presented with the aim of reducing diagnostic delay, optimising therapeutic trial design and catalysing the development of disease-modifying therapy.

Upper motor neuron burden measurement in motor neuron diseases: Does one scale fit all?

  • Suma Babu
  • Psychology, Medicine
    Muscle & nerve
  • 2020
The study provided evidence that the summated score of the reflexes and spasticity subdomains of the PUMN had good interrater reliability between two blinded raters, providing evidence of its clinical relevance in assessing ALS in patients.

Finding Markers in Amyotrophic Lateral Sclerosis Diagnosis

Electrophysiological and Image techniques like markers in ALS diagnosis and correlate these results with MRI methods show abnormalities in motor and not motor structures of brain in ALS patients.

The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis

There is a need to develop a universally accepted and validated cognitive assessment tool to be administered in a multidisciplinary clinic that is efficient and sensitive, as well as being integrated into the design and analysis of future ALS drug trials.



Amyotrophic lateral sclerosis: Objective upper motor neuron markers

Progress has been made over the past two decades towards establishing valid diagnostic and natural history markers of upper motor neuron involvement in ALS.

Testing upper motor neuron function in amyotrophic lateral sclerosis: the most difficult task of neurophysiology.

No method to investigate upper motor neuron function has proved useful and applicable as a measure of efficacy in clinical trials, despite some enthusiasm for the threshold tracking transcranial magnetic stimulation as a marker of early diagnosis.

Neuroimaging as a New Diagnostic Modality in Amyotrophic Lateral Sclerosis

The potential diagnostic value of advanced neuroim imaging techniques that are increasingly being used to study the brain in ALS are reviewed and the role of neuroimaging in clinical therapeutic trials is addressed.

Diagnostic utility of cortical excitability studies in amyotrophic lateral sclerosis

The diagnostic utility of a threshold tracking transcranial magnetic stimulation technique as an aid to the research‐based Awaji criteria in establishing an earlier diagnosis of ALS is assessed.

Why are upper motor neuron signs difficult to elicit in amyotrophic lateral sclerosis?

  • M. Swash
  • Psychology, Biology
    Journal of Neurology, Neurosurgery & Psychiatry
  • 2012
The different mechanisms underlying the clinical phenomenology of the functional motor defect in amyotrophic lateral sclerosis, that lead to difficulty in detecting classical upper motor neuron signs, are discussed.

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.

Utility of transcranial magnetic stimulation in delineating amyotrophic lateral sclerosis pathophysiology.

Electrophysiologic evaluation of upper motor neuron involvement in amyotrophic lateral sclerosis

  • V. DroryI. KováchG. B. Groozman
  • Medicine, Biology
    Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
  • 2001
BACKGROUND: The demonstration of upper motor neuron (UMN) dysfunction in patients with amyotrophic lateral sclerosis (ALS) with predominantly lower motor neuron (LMN) signs is clinically sometimes

Clinical diagnosis and management of amyotrophic lateral sclerosis

Optimal treatment is based on symptom management and preservation of quality of life, provided in a multidisciplinary setting, and Riluzole remains the only effective drug, and extends the average survival of patients by 3–6 months.

Uncovering amyotrophic lateral sclerosis phenotypes: Clinical features and long-term follow-up of upper motor neuron-dominant ALS

  • M. SabatelliM. Zollino A. Conte
  • Psychology
    Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
  • 2011
The UMN-D phenotype was a strong independent predictor of long survival and showed significant differences in age of onset, sex ratio, pattern of spreading and prognosis with respect to other ALS forms, most probably reflecting biological differences.