Gonzalo wrote:
>
> I would like opinions on how to approach an infant born with
> orbicularis oris atrophy. This particular case is now three months
> old and the deviation of the corner of the mouth remains unchanged.
>
> I doubt the possibility I am dealing with facial palsy as the
> findings are totally localized in the area defined. Eyelids, tongue,
> etc. appear to move normally.
>
> Any suggested workup and differential diagnoses is appreciated.
>
> Needless to say the birth history is totally benign.
> Gonzalo Mantilla Jr., MD.
> Oficina del Decano
> USFQ Colegio de Ciencias de la Salud
>
Maybe the following can be of some help:
Title
Morphology of denervated human facial muscles.
Author
Schwarting_S; Schröder_M; Stennert_E; Goebel_HH
Source
ORL J Otorhinolaryngol Relat Spec, 1984, 46:5, 248-56
Abstract
17 biopsies of denervated facial muscles, the zygomatic, the
orbicularis oris and the levator labii
muscles, showed atrophic myofibers in most cases. There was loss of
fiber typability when applying
the NADH, the MAG and the alkaline ATPase reactions. The acid
ATPase preparations allowed
differentiation of myofibers into type I and type II without
subtypes. Contrary to normal facial
muscles that are richly endowed with motor endplates, no
neuromuscular junctions were observed in
denervated muscle fibers except one example which might have been
obtained by false sampling from
the marginal area of denervation or might be the result of partial
reinnervation due to sprouting axons
from the neighborhood. There was no correlation between the degree
of muscle fiber atrophy and the
duration of the paralysis. However, fibrosis corresponded to length
of denervation. The presence of
highly atrophic muscle fibers even 36 years after denervation
indicates that the final aim of facial nerve
surgery, namely the reinnervation of denervated facial musculature
may still be achievable. However,
endomysial and perimysial fibrosis may have a considerable impact
on the final outcome of such facial
nerve surgery. Unsatisfactory correlation between morphological and
clinical as well as
electromyographical findings in denervated facial muscles requires
individual morphological study of
each biopsy to assess the probable outcome of reconstructive facial
nerve surgery. It therefore
appears reasonable even in long-standing facial paralysis, to
biopsy denervated facial muscles before
or during surgical reanastomosation of the facial nerve. This study
provides hints that morphological
examination of denervated facial muscles may supplement clinical,
electrophysiological, and possibly
biochemical diagnostic findings.
Language of Publication
English
Unique Identifier
85013697
Order full text for this document
Title
Facial action myoclonus in patients with olivopontocerebellar
atrophy.
Author
Lou_JS; Valls-Solé_J; Toro_C; Hallett_M
Address
Human Motor Control Section, National Institute of Neurological
Disorders and Stroke, National
Institutes of Health, Bethesda, Maryland 20892.
Source
Mov Disord, 1994 Mar, 9:2, 223-6
Abstract
We studied four patients with familial olivopontocerebellar atrophy
(OPCA) who had abnormal
twitching of the cheeks and perioral muscles induced by facial
movements. With the muscles at rest,
electromyographic (EMG) recordings of the orbicularis oris and
risorius muscles revealed myokymic
discharges in the absence of visible movements. With voluntary
contraction, the EMG showed
synchronous discharges in the orbicularis oris and risorius muscles
ipsilaterally associated with visible
twitching. The duration of the EMG bursts was 10 to 75 ms with a
frequency of 8 to 25 Hz, which
suggested that the abnormal twitching was most consistent with a
myoclonic disorder. Because it was
induced by activation of the facial muscles, this movement disorder
represents a form of action
myoclonus.
Title
Brainstem reflexes in patients with olivopontocerebellar atrophy.
Author
Valls-Solé_J; Lou_JS; Hallett_M
Address
Human Motor Control Section, National Institute of Neurological
Disorders and Stroke, National
Institutes of Health, Bethesda, Maryland.
Source
Muscle Nerve, 1994 Dec, 17:12, 1439-48
Abstract
In 4 patients with familial olivopontocerebellar atrophy (OPCA) we
have recently described an
abnormal movement of facial muscles characterized by rhythmic
muscle twitching during voluntary
activation (facial action myoclonus). In the present article, we
present the results of a
neurophysiological study of brainstem reflexes in those 4 patients,
in 4 other patients with OPCA but
without facial action myoclonus, in 3 patients with pure cerebellar
cortical atrophy, and in 6 normal
volunteers used as control subjects. All patients had similar
clinical features, but only the patients with
facial action myoclonus and only one of the other patients with
OPCA had brainstem atrophy
detected on magnetic resonance imaging. Electrophysiological
abnormalities were found in all patients
with facial action myoclonus and consisted of myokymia in perioral
muscles at rest, spread of
spontaneous and reflex blinking to the orbicularis oris, and
enhanced long-latency facial reflex
responses to stimuli applied to the facial or trigeminal nerve.
Other relevant electrophysiological
abnormalities were the absence of jaw jerk in 2 patients, the
absence of an R1 response of the blink
reflex in 1 patient, and a markedly reduced compound muscle action
potential of the facial nerve in
another patient. Comparable electrophysiological abnormalities were
found in only 1 of the patients
with OPCA but without facial action myoclonus, and in none of the
patients with pure cerebellar
cortical atrophy. Facial action myoclonus is a clinical
manifestation of a global brainstem functional
derangement that may characterize a subgroup of patients with OPCA
or constitute a distinctive step
in the natural evolution of some forms of the disease.
Language of Publication
English
Unique Identifier
95059191