Quantitating progression in ALS

Carvalho, Mamede de; Scotto, Manuel G.; Lopes, Arminda; Swash, Michael

doi:10.1212/01.wnl.0000162036.76024.ab

Cited by 56 publications

(54 citation statements)

References 10 publications

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“…A Keypoint machine (Dantec, Skovlunde, Denmark) was used for the neurophysiological studies. The temperature of the Abbreviations: ADM, abductor digiti minimi muscle; ALS, amyotrophic lateral sclerosis; ALS-FRS, amyotrophic laterals sclerosis functional rating scale; CSP, cortical silent period; CMAP, compound muscle action potential; EMG, electromyogram; FVC, forced vital capacity; LMN, lower motor neuron; MEP, motor evoked potential; MUNE, motor unit number estimation; MRC, Medical Research Council (UK); NI, Neurophysiological Index; UMN, upper motor neuron Correspondence to: M. de Carvalho; e-mail: mamedemg@mail.telepac.pt investigated limb was kept at 32 C. Motor unit number estimation (MUNE), 2,3 the Neurophysiological Index (NI), 3 and transcranial magnetic stimulation (TMS) 6 were evaluated at each test session. For MUNE measurements we used the incremental technique.…”

Section: Methodsmentioning

confidence: 99%

“…For MUNE measurements we used the incremental technique. 3 The NI is derived from conventional neurophysiological measurements, including M-wave amplitude, distal motor latency, and F-wave frequency in the ulnar territory using the formula: (M-wave amplitude/distal motor latency) Â (Fwave responses per 20 stimuli). 3 Both right and left ulnar nerve-ADM nerve-muscle systems were studied in each subject.…”

Section: Methodsmentioning

confidence: 99%

“…1 Many such tests have been suggested, and several are known to be sufficiently sensitive to measure change in ALS. [1][2][3] We studied LMN and UMN function in the same group of patients over a 6-month period. Longitudinal studies of this kind have not previously been reported, but they are essential to test the association between UMN and LMN degeneration, a key feature of ALS.…”

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confidence: 99%

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Sensitivity of electrophysiological tests for upper and lower motor neuron dysfunction in ALS: A six‐month longitudinal study

Carvalho

Swash

2009

Muscle and Nerve

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By following a group of amyotrophic lateral sclerosis (ALS) patients longitudinally using lower motor neuron (LMN) and upper motor neuron (UMN) markers of dysfunction it may be possible to better understand the functional relationships between these motor systems in this disease. We used neurophysiological techniques to follow UMN and LMN dysfunction in a group of 28 patients with ALS, in comparison with the ALS functional rating scale (ALS-FRS) score and the forced vital capacity (FVC). We used motor unit number estimation (MUNE), compound muscle action potential (CMAP) amplitude, and the Neurophysiological Index (NI) to quantify the LMN disorder, and transcranial motor stimulation to study cortical motor threshold, motor-evoked response amplitude, central motor conduction time, and cortical silent period (CSP). The patients were studied shortly after diagnosis and then 6 months later, using both abductor digiti minimi muscles (ADM); ADM strength was initially >MRC 3 (Medical Research Council, UK). The NI and MUNE changed more than any other variable. CSP increased by about 30%, a change more marked than the slight increase observed in the cortical motor threshold (9%). The normal increase of CSP after acute muscle fatigue was preserved during disease progression. The CSP increase correlated with the MUNE rate of decay but not to the NI reduction, perhaps because NI includes F-wave frequency in itscalculation. There was no definite correlation between UMN and LMNdysfunction or progression, but there was a link between CSP and LMN changes in ALS. The CSP may be a useful variable in following UMN dysfunction in clinical practice and in clinical trials.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

mentioning

confidence: 99%

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Sensitivity of electrophysiological tests for upper and lower motor neuron dysfunction in ALS: A six‐month longitudinal study

Carvalho

Swash

2009

Muscle and Nerve

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“…6 In that ALS population, the decline in strength of individual muscles correlated well with the decline in MUNE counts. Questions remain, however, as to whether it is valid to extrapolate from a small number of muscles to the overall neuromuscular function of an individual.…”

Section: Discussionmentioning

confidence: 75%

“…Other problems with MUNE include the technical difficulty of performing such studies and the variability of the data obtained. Only one study has shown an association between MUNE and strength of individual muscles, 6 and an association of MUNE with overall neuromuscular function has not been demonstrated.…”

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confidence: 99%

Motor unit number estimates correlate with strength in polio survivors

2006

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Motor unit number estimation (MUNE) has been proposed as an outcome measure in clinical trials for the motor neuron diseases. One major criticism of MUNE is that it may not represent a clinically meaningful endpoint. We prospectively studied a cohort of polio survivors over a period of 15 years with respect to MUNE and strength. We identified a significant association between thenar MUNE and arm strength, extensor digitorum brevis MUNE and leg strength, and the summated MUNE and global strength of the polio survivors. These findings confirm the clinical relevance of MUNE as an outcome measure in the motor neuron diseases and provide further validation for its use in clinical trial research.

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