Medical technology assessment. Electrodiagnosis in motor neuron diseases and amyotrophic lateral sclerosis.
In motor neuron diseases/amyotrophic lateral sclerosis (MND-ALS), electrodiagnostic techniques are essential in supporting the diagnosis and excluding other conditions that clinically resemble MND-ALS. Electrodiagnostic techniques can also monitor disease progression and provide prognostic information. Electromyography has an important role in the diagnosis of MND-ALS, but some drawbacks should be borne in mind. Although internationally accepted electrophysiological criteria have been defined to support MND-ALS diagnosis, differences in different laboratories can raise unexpected difficulties in application of diagnosis criteria. Much work needs to be done to increase standardisation of the electrodiagnosis of MND-ALS in order to improve quality. Differential diagnosis with motor axonal neuropathies may be particularly difficult. EMG is an essential tool for the early diagnosis of MND-ALS, which increases potential benefit of therapeutic interventions. A wide discussion among neurophysiologists from different schools could create a sound consensus on early diagnosis of MND-ALS.
de Carvalho
M
M
Department of Neurology, EMG Laboratory of Centro de Estudos Egas Moniz, Hospital de Santa-Maria, av, Prof. Egas Moniz, 1600 Lisbon, Portugal. mamedemg@mail.telepac.pt
Johnsen
B
B
Fuglsang-Frederiksen
A
A
D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004576:Electromyography / Q000379:methods / Q000592:standards; D006801:Humans; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology; D009046:Motor Neurons / Q000502:physiology; D009121:Muscle Denervation; D009416:Nerve Regeneration; D009468:Neuromuscular Diseases / Q000175:diagnosis; D011379:Prognosis; D011786:Quality Control