Clinical neurophysiology in the diagnosis of amyotrophic lateral sclerosis: The Lambert and the El Escorial criteria

  title={Clinical neurophysiology in the diagnosis of amyotrophic lateral sclerosis: The Lambert and the El Escorial criteria},
  author={Asa J. Wilbourn},
  journal={Journal of the Neurological Sciences},
  • A. Wilbourn
  • Published 1 October 1998
  • Psychology
  • Journal of the Neurological Sciences

An evaluation of neurophysiological criteria used in the diagnosis of motor neuron disease

Accepting EMG evidence of fasciculations as evidence of acute denervation increases the diagnostic certainty of MND, and the new criteria allow earlier diagnosis of M ND without increasing the false-positive rate.

Fasciculation potentials in amyotrophic lateral sclerosis and the diagnostic yield of the Awaji algorithm

The Awaji algorithm has restored the value of fasciculation potentials in the diagnosis of amyotrophic lateral sclerosis and the diagnostic yield of this algorithm is lower than that of the R‐EEC.

Electrophysiologic diagnostics of very early stages of ALS.

Complex fasciculations and their origin in amyotrophic lateral sclerosis and Kennedy's disease

Combined fasciculations are distinctive in ALS, and it is hypothesized that they are triggered by a supraspinal mechanism reflecting dysfunction of descending motor pathways.

Electrodiagnostic Evaluation of Motor Neuron Disorders

Nerve conduction studies (NCS) and needle electromyography (EMG) are essential in the evaluation of suspected ALS and are primarily used to exclude any potentially treatable motor neuropathy that may mimic ALS.

Variation in the neurophysiological examination of amyotrophic lateral sclerosis in Europe

The existence of two different examination approaches, one with quantitative EMG analyses and relatively few muscles studied, and one with more muscles studied using qualitative methods was clearly confirmed in the present study.

Sensitivity and specificity of the ‘Awaji’ electrodiagnostic criteria for amyotrophic lateral sclerosis: Retrospective comparison of the Awaji and revised El Escorial criteria for ALS

The new criteria for ALS do not result in a loss of specificity and can potentially improve the sensitivity by 16%.

Needle electromyography of the frontalis muscle in patients with amyotrophic lateral sclerosis

Examining the frontalis provides useful information in establishing the diagnosis of ALS by identifying clinically evident or subclinical abnormalities in the craniobulbar region.

EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives

Early diagnosis should be pursued and a number of investigations should be performed with high priority, and every effort should be made to maintain patient autonomy during the entire course of the disease.



Role of electromyography in amyotrophic lateral sclerosis

It is revealed that a large proportion of patients with a clinical diagnosis of ALS fail to have classical findings on initial electrodiagnostic studies, and several caveats of electrodiagnostics testing in these patients are revealed.

Amyotrophic lateral sclerosis : diagnosis and management for the clinician

Part 1: DIAGNOSIS 1. Epidemiology and Historical Perspective of ALS 2. Etiology and Pathogenesis of ALS: Biochemical, Genetic, and Other Theories 3. Definition of Terms, Classification, and

AAEE minimonograph #32: The electrophysiologic examination in patients with radiculopathies

The value and limitations of the various electrophysiologic procedures used in the diagnosis of radiculopathies are discussed, including motor and sensory nerve conduction studies, late responses, somatosensory evoked potentials, nerve root stimulation, and the needle electrode examination.


  • J. Simpson
  • Medicine
    Journal of neurology, neurosurgery, and psychiatry
  • 1956
Electromyographic diagnosis has hitherto depended on the demonstration of evidence of partial denervation confined to the muscles supplied by a particular motor nerve or one of its branches, but this method has certain practical disadvantages.


The increase of fasciculations produced by prostigmine and some of the observations reported on the effect of nerve root and peripheral nerve block cast doubt on the central origin of these involuntary movements.

Voluntary activation and fiber density of fasciculations in motor neuron disease

Differences in the peripheral microanatomy of a number of fasciculation units not activated voluntarily may underlie ectopic impulse generation in the terminal axonal arborization, endplate zone, or muscle fibers of these units.

The origin of fasciculations in motoneuron disease

The collision technique was used to determine the origin of distal fasciculations in 25 motor units from 9 patients with amyotrophic lateral sclerosis and 6 with other diseases involving motoneurons, and multiple sites of origin were found in 8 motor units.

Needle EMG of the tongue: motor unit action potential versus peak ratio analysis in limb and bulbar onset amyotrophic lateral sclerosis

Subclinical involvement of the tongue was found in 20% of the patients with limb onset amyotrophic lateral sclerosis and could be more accurately assessed with MUAP analysis than with automatic EMG.