Central motor conduction time reveals upper motor neuron involvement masked by lower motor neuron impairment in a significant portion of patients with amyotrophic lateral sclerosis

Tokimura, Ryo; Murakami, Takenobu; Ugawa, Yoshikazu

doi:10.1016/j.clinph.2020.05.021

Cited by 9 publications

(5 citation statements)

References 28 publications

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“…Prolonged CMCT of FDI was defined as 7.9 ms or longer. This was our in-house cut-off value, which is comparable with other studies ( Claus, 1990 , Tokimura et al, 2020 ). Prolonged CMCT of TA was 17.0 ms or longer ( Matsumoto et al, 2010 ).…”

Section: Methodssupporting

confidence: 90%

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Neurophysiological differentiation of upper motor neuron damage in neurodegenerative disorders

Shirota

Otsuka²,

Toda³

et al. 2022

Clinical Neurophysiology Practice

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Section: Methodssupporting

confidence: 90%

“…In ALS patients, abnormality of the CMCT testing has been established ( Civardi et al, 2020 , Groppa et al, 2012 , Ugawa et al, 1988 ). It can even “unmask” the UMN signs that were not readily apparent from clinical examination alone ( Tokimura et al, 2020 , Zoccolella et al, 2020 ).…”

Section: Discussionmentioning

confidence: 99%

Neurophysiological differentiation of upper motor neuron damage in neurodegenerative disorders

Shirota

Otsuka²,

Toda³

et al. 2022

Clinical Neurophysiology Practice

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“…CMCT prolongation in ALS can be explained by the degeneration of the fastest conducting corticospinal fibers. CMCT measurement has also been shown useful to detect UMN dysfunction in ALS patients without clinically predominant UMN signs but may have low sensitivity in the early stages of the disease (Eisen et al, 1996;Floyd et al, 2009;Huynh et al, 2018;Kohara et al, 1996;Mills, 2003;Tokimura et al, 2020), which is confirmed by this study showing that CMCT is rarely abnormal at the time of the patient's initial diagnostic visit.…”

Section: Discussionsupporting

confidence: 71%

Assessing the upper motor neuron in amyotrophic lateral sclerosis using the triple stimulation technique: A multicenter prospective study

Grapperon¹,

Verschueren²,

Jouve

et al. 2021

Clinical Neurophysiology

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h i g h l i g h t s-Triple stimulation technique can be used in routine clinical practice and in multicenter studies in ALS patients.-Assessment of corticospinal dysfunction at the first visit of the patient was improved by the use of triple stimulation technique compared to conventional TMS.-Triple stimulation technique results are correlated with the clinical upper motor neuron score and ALSFRS-R. a b s t r a c tObjective: To evaluate the relevance of transcranial magnetic stimulation (TMS) using triple stimulation technique (TST) to assess corticospinal function in amyotrophic lateral sclerosis (ALS) in a large-scale multicenter study. Methods: Six ALS centers performed TST and conventional TMS in upper limbs in 98 ALS patients during their first visit to the center. Clinical evaluation of patients included the revised ALS Functional Rating Scale (ALSFRS-R) and upper motor neuron (UMN) score. Results: TST amplitude ratio was decreased in 62% of patients whereas conventional TMS amplitude ratio was decreased in 25% of patients and central motor conduction time was increased in 16% of patients. TST amplitude ratio was correlated with ALSFRS-R and UMN score. TST amplitude ratio results were not different between the centers. Conclusions: TST is a TMS technique applicable in daily clinical practice in ALS centers for the detection of UMN dysfunction, more sensitive than conventional TMS and related to the clinical condition of the patients. Significance: This multicenter study shows that TST can be a routine clinical tool to evaluate UMN dysfunction at the diagnostic assessment of ALS patients.

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“…61,62 It has been reported that autopsied cases with PMA usually have pathological involvement of UMNs, similar to cases with classic ALS 50 ; UMN signs/symptoms and UMN involvement are not always concordant in ALS cases. This fact indicates the usefulness of established markers of corticospinal tract degeneration, such as diffusion tensor imaging 63 or central motor conduction time, 64,65 even in cases with PMA. The finding of interest in Case 4 was, however, minimal pathological involvement of the UMNs, and a subset of PMA cases may be associated with minimal changes in the UMNs.…”

Section: Discussionmentioning

confidence: 89%

Unexpected postmortem diagnoses in cases of clinically diagnosed amyotrophic lateral sclerosis

et al. 2021

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Amyotrophic lateral sclerosis (ALS) is a motor neuron disease that is clinically and pathologically characterized by impairment of the upper and lower motor neurons. The clinical diagnosis of ALS is not always straightforward because of the lack of specific biomarkers and clinical heterogeneity. This review presents the clinical and pathological findings of four autopsied cases that had been diagnosed with ALS before death. These cases had demonstrated definite and progressive motor neuron signs and symptoms, whereas postmortem assessment revealed miscellaneous disorders, including fungal infection, paraneoplastic syndrome, and amyloidosis. Importantly, nonmotor neuron signs and symptoms, including seizures, extra-pyramidal signs, ocular movement disorders, sensory disturbance, and dysautonomia, had also been documented during the disease course of the cases in the present study. The ALS-unlike symptoms were indicative of the "true" diagnosis in each case when those symptoms were isolated from motor neuron signs/ symptoms.

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Central motor conduction time reveals upper motor neuron involvement masked by lower motor neuron impairment in a significant portion of patients with amyotrophic lateral sclerosis

Cited by 9 publications

References 28 publications

Neurophysiological differentiation of upper motor neuron damage in neurodegenerative disorders

Neurophysiological differentiation of upper motor neuron damage in neurodegenerative disorders

Assessing the upper motor neuron in amyotrophic lateral sclerosis using the triple stimulation technique: A multicenter prospective study

Unexpected postmortem diagnoses in cases of clinically diagnosed amyotrophic lateral sclerosis

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