2006
DOI: 10.1002/mus.20519
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Cortical versus spinal dysfunction in amyotrophic lateral sclerosis

Abstract: Little is known about the possible link between cortical and spinal motor neuron dysfunction in amyotrophic lateral sclerosis (ALS). We correlated the characteristics of the responses to transcranial magnetic stimulation (TMS) with the electromechanical properties and firing pattern of single motor units (MUs) tested in nine ALS patients, three patients with Kennedy's disease, and 15 healthy subjects. In Kennedy's disease, 19 of 22 MUs were markedly enlarged with good electromechanical coupling and discharged … Show more

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Cited by 23 publications
(28 citation statements)
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References 65 publications
(107 reference statements)
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“…The other explanation, which we discussed in another recent study, may be the occurrence of spinal synaptic reorganization subsequent to motoneuron loss, leading to greater convergence of still intact corticomotoneuronal axons onto resilient motoneurons. 4 The second most frequently abnormal TMS parameter was the SP. Interestingly, in the patients with suspected or possible ALS, this was the most frequently abnormal parameter (Table 2), in agreement with our previous results.…”
Section: Discussionmentioning
confidence: 99%
“…The other explanation, which we discussed in another recent study, may be the occurrence of spinal synaptic reorganization subsequent to motoneuron loss, leading to greater convergence of still intact corticomotoneuronal axons onto resilient motoneurons. 4 The second most frequently abnormal TMS parameter was the SP. Interestingly, in the patients with suspected or possible ALS, this was the most frequently abnormal parameter (Table 2), in agreement with our previous results.…”
Section: Discussionmentioning
confidence: 99%
“…This may account for both the increase in excitatory and inhibitory responses, which decreased with time and disease progression. 3,4 The marked TMS-induced inhibitory effects on motor unit firing observed in this study may reflect a transient withdrawal of the motoneuron net excitatory drive induced by TMS (i.e., disfacilitation), as previously suggested. 6,11 This effect might involve cortical inhibitory processes recurrently activated by pyramidal collaterals, which are liable to effectively shut down the cortical contribution to the motoneuron excitatory drive.…”
Section: Discussionmentioning
confidence: 71%
“…In this study we showed that, in the early stages of ALS, inhibitory responses were increased, as is also the case for excitatory responses. 3 We propose that spinal reinnervation may occur in the earliest stage in patients in whom intact corticospinal axons lose some of their motoneuronal targets and branch to converge on surviving motoneurons. This may account for both the increase in excitatory and inhibitory responses, which decreased with time and disease progression.…”
Section: Discussionmentioning
confidence: 99%
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“…However, some of the few single motor unit studies performed in patients with ALS have not been covered in this review. The interested reader may find additional information in Schmied et al (1999), Attarian et al (2006Attarian et al ( , 2008.…”
mentioning
confidence: 99%