Brain, Vol 120, Issue 10 1877-1883, Copyright © 1997 by Oxford University Press
J Valls-Sole, F Valldeoriola, E Tolosa and MJ Marti
Spontaneous and voluntary eyelid motility is often abnormal in patients
with progressive supranuclear palsy. In contrast, their eyelid reflex
responses are relatively preserved, and only those generated by an acoustic
startle have been found absent or severely reduced. We hypothesized that,
because of their relevant brainstem pathology, patients with progressive
supranuclear palsy might have other brainstem reflex abnormalities which,
on detection, could help with their neurophysiological characterization. In
this study, we examined facial reflex responses in 14 patients with
progressive supranuclear palsy, 12 patients with multisystem atrophy, 10
patients with Parkinson's disease, six patients with corticobasal
ganglionic degeneration, 11 patients with various non-parkinsonian
neurological illnesses and 10 normal subjects. EMG activity was
simultaneously recorded from the orbicularis oculi and mentalis muscles
following electrical stimulation of the median nerve at the wrist. Mentalis
responses were obtained in two normal subjects and in all patients except
one with Parkinson's disease, one with progressive supranuclear palsy and
one with corticobasal ganglionic degeneration; there were no differences
between groups of subjects regarding latency or peak amplitude. Orbicularis
oculi responses were always present in control subjects and patients who
exhibited mentalis responses, with the significant exception of patients
with progressive supranuclear palsy, in whom only the response of mentalis
was obtained. Blink-reflex responses to supraorbital nerve electrical
stimuli were present at a normal latency and amplitude in all patients. An
abnormally enhanced blink-reflex excitability recovery curve to paired
stimuli was found in a similar percentage of patients with progressive
supranuclear palsy, multisystem atrophy and Parkinson's disease, but in
only two patients with corticobasal ganglionic degeneration. Patients with
progressive supranuclear palsy have a functional involvement of circuits
mediating orbicularis oculi responses to median nerve electrical stimuli,
that is a distinctive feature with respect to other parkinsonian syndromes.
ARTICLES
Distinctive abnormalities of facial reflexes in patients with progressive supranuclear palsy
Unitat d'EMG, Hospital Clinic, Barcelona, Spain.
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