Assessment of the upper motor neuron in amyotrophic lateral sclerosis

@article{Huynh2016AssessmentOT,
  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},
  year={2016},
  volume={127},
  pages={2643-2660}
}

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...

References

SHOWING 1-10 OF 228 REFERENCES

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.
...