Assessment of upper and lower motor neurons in Kennedy's disease: implications for corticomotoneuronal PSTH studies.

Abstract:

:We used peristimulus time histograms (PSTHs) to estimate characteristics of the composite excitatory postsynaptic potentials (EPSPs) generated at the anterior horn cell by a descending cortical volley induced by subthreshold transcranial magnetic stimuli in 11 normal subjects, 9 patients with DNA-confirmed Kennedy's disease, and 10 patients with amyotrophic lateral sclerosis (ALS). In Kennedy's disease the mean of the estimated EPSP amplitude (3.5 +/- 1.6 mV), as well as the means of duration (4.1 +/- 1.3 ms) and onset latency (20.4 +/- 2.7 ms) of the primary peak of 35 different motor units were not significantly different from normal subjects, in whom corresponding values of 49 motor units measured 2.9 +/- 1.6 mV (EPSP amplitude), 3.5 +/- 1.4 ms (duration) and 19.3 +/- 2.8 ms (onset latency). In 48 motor units of patients with ALS the mean of the estimated EPSP amplitude, duration, and latency measured 2.7 +/- 1.8 mV, 7.8 +/- 5.7 ms, and 24.0 +/- 6.5 ms, respectively, differing significantly from normal subjects and patients with Kennedy's disease. The normal cortically induced EPSP in Kennedy's disease is very different from the range of EPSP abnormalities in ALS, indicating that lower motor neuron disease per se does not result in an abnormal cortically induced EPSP derived from PSTHs. The normal findings in Kennedy's disease support our previous suggestions that the EPSP abnormalities in ALS are supraspinal in origin.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Weber M,Eisen A

doi

10.1002/(sici)1097-4598(199903)22:3<299::aid-mus2>

subject

Has Abstract

pub_date

1999-03-01 00:00:00

pages

299-306

issue

3

eissn

0148-639X

issn

1097-4598

pii

10.1002/(SICI)1097-4598(199903)22:3<299::AID-MUS2>

journal_volume

22

pub_type

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