ABSTRACT: In patients with hemifacial spasm (HFS), abnormal muscle

responses due to abnormal cross-transmission are observed in facial

muscles. However, the site in the facial nerve responsible for the cross-
transmission remains a matter of controversy. We have developed a model
in which by considering the electrophysiological parameters involved in pro-
ducing the abnormal muscle response, we can determine the site of the
abnormal cross-transmission within the facial nerve. This model was applied
to HFS patients with three different etiologies: idiopathic, post-Bells palsy,
and post-XII-VII anastomosis. Our data show that: in idiopathic HFS, the
cross-transmission may occur in the facial nerve at the level of the ponto-
cerebellar angle; in post-Bells palsy, it is inside the petrous bone; and in
XII-VII anastomosis, it must be in the extracranial part of the facial nerve.
The possible mechanisms for this cross talk are discussed in terms of
ephaptic transmission or of a central hyperexcitability in the facial motor
nucleus.
1998 John Wiley & Sons, Inc. Muscle Nerve 21: 10131018, 1998

ELECTROPHYSIOLOGICAL
DETERMINATION OF THE SITE
INVOLVED IN GENERATING
ABNORMAL MUSCLE RESPONSES IN
HEMIFACIAL SPASM
FREDERIC TANKERE, MD,1 THIERRY MAISONOBE, MD,2
GEORGES LAMAS, MD,1 JACQUES SOUDANT, MD,1 PIERRE BOUCHE, MD,2
2
EMMANUEL FOURNIER, MD, PhD, and JEAN CLAUDE WILLER, MD, DSc2

1
Department of Otorhinolaringology, Hopital Pitie-Salpetriere, Paris, France
2
Federation of Clinical Neurophysiology, Hopital Pitie-Salpetriere, 47, Bd. de
lHopital, 75013 Paris, France

Received 8 October 1997; accepted 10 January 1998

It is a classical feature of patients with hemifacial
spasm (HFS) that abnormal muscle responses can be
observed in facial muscles. For example, these ab-
normal responses can be elicited by electrical stimu-
lation of the marginal mandibular branch of the fa-
cial nerve (VIIth nerve) and recorded from the
orbicularis oculi muscles or conversely, by stimulat-
ing the temporozygomatic branch of the VIIth nerve
while recording from the mentalis muscles.2,16 Al-
though the precise physiological basis of these re-
sponses is not fully understood, it is obvious that they
are due in part to an abnormal cross-transmission
somewhere along the facial nerve pathway between
its nucleus and its terminal peripheral branches.
There is still some debate concerning the site at
which these abnormal responses are generated. At
Key words: hemifacial spasm; idiopathic; post Bells palsy; XII-VII anas-
tomosis; abnormal muscle response; abnormal cross-transmission
Correspondence to: Dr. Jean Claude Willer
CCC 0148-639X/98/081013-06
1998 John Wiley & Sons, Inc.
least two hypotheses can be proposed: a peripheral
nerve hypothesis and a central or nucleus hypoth-
esis. In the first, the abnormal responses are thought
to be due to ephaptic transmission at the site where
the nerve is injured,16,17 whereas the second hypoth-
esis states that the peripheral injury to the facial
nerve induces a functional reorganization of syn-
apses within the facial nucleus which is associated
with a general hyperexcitability of its whole moto-
neuronal pool. This in turn would result in abnor-
mal cross-transmission at the origin of the abnormal
response.8,9,1215
The present report is an attempt to show that it is
possible to localize quite accurately the site of the
abnormal cross-transmission which gives rise to the
abnormal response in patients with similar clinical
signs of hemifacial spasm but with three different
etiologies, namely: idiopathic HFS due to vascular
compression in the cerebellopontine angle, HFS fol-
lowing Bells palsy, and HFS in patients with XII-VII
anastomosis.

Hemifacial Spasm MUSCLE & NERVE August 1998 1013

 For this purpose, we developed a theoretical
model based on electrophysiological findings, in
which we considered the parameters involved in de-
termining the latency of the abnormal response (see
below). Applied to the three types of patients, our
data suggest that the abnormal response is probably
due to ephaptic transmission at the site of the nerve
injury at least in HFS following Bells palsy and HFS
in patients with XII-VII anastomosis.
PATIENTS AND METHODS
The electrophysiological studies were carried out on
three groups (A, B, C) of 5 patients. All of the pa-
tients were complaining of spontaneous clonic and
tonic contractions of facial muscle localized to one
side of the face and associated with synkinesiae of
variable intensity and topography, for at least 68
months. A group of 5 normal subjects (members of
the medical staff, 4 men, 1 woman, 2945 years, me-
dian age: 38) was also included in this study to obtain
control values for the motor conduction velocity of
the different branches of the facial nerve.
Group A was composed of 3 men and 2 women,
in the age range 4563 years (median age: 52), who
had idiopathic HFS due to vascular compression of
the facial nerve at its root entry zone (as demon-
strated by magnetic resonance imaging).
Group B was composed of 3 women and 2 men,
in the age range 2855 years (median age: 39), who
had developed HFS following Bells palsy. These pa-
tients had recovered partially and progressively over
45 months.
Group C was composed of 5 women in the age
range 4269 years (median age: 55), who had under-
gone a peripheral hypoglossofacial (XII-VII) anasto-
mosis as restorative surgery following removal of a
part of the facial nerve. These patients each had an
extensive cerebellopontine neuroma of the acoustic
nerve which reached the facial nerve, necessitating
the removal of part of this nerve during the surgical
ablation of the neuroma. While the facial palsy im-
proved progressively in the 810 months following
surgery, all 5 patients exhibited clinical signs of HFS
1215 months after the anastomosis. In these cases,
massive contractions of all the facial muscles were
also observed during spontaneous or voluntary swal-
lowing.
Both the patients and the normal control sub-
jects gave informed consent for this work, which was
approved by a local committee. During the electro-
physiological studies, all the subjects were comfort-
ably reclined in a bed with a headrest in order to
ensure a good general muscular relaxation.
1014 Hemifacial Spasm
Electrophysiological Methods. Stimulation and Re-
cordings. The facial nerve was stimulated with a pair
of surface electrodes (1 cm apart) applied to the skin
overlying the nerve after the skin had been cleaned
with a mixture of ether and acetone. The stimulus
was a rectangular single shock of 0.2-ms duration
delivered at a rate of 0.5 Hz from a constant-current
stimulator. In all the subjects, the stimulus intensity
was adjusted to be clearly supramaximal.
Recordings were made from the orbicularis oculi
and mentalis muscles using surface electrodes (1 cm
apart) placed on the cleaned and degreased skin
over the muscle.
General Experimental Procedure. In general, the
nerves were stimulated only when no spontaneous
electrical activity was detected in the facial muscles.
This was done to avoid any artifactual response
which might occur during a spasm.
As illustrated in Figure 1 (experimental setup),
the facial nerve or the XII-VII crossover was stimu-
lated at the stylomastoid site (S1) just before the
facial nerve branches. The cathode was placed dis-
tally in all cases. This stimulation elicited a proxi-
mal direct motor response in both muscles from
which recordings were being made. In order to
study the conduction velocity of the motor fibers of
the zygomatic and mandibular branches of the
nerve, a second stimulus was applied more distally
on each branch, termed as S2 and S3, respectively,
as shown in Figure 1. The distances between S1 and
S2 and between S1 and S3 were then measured at
an accuracy of 1 mm. The latency of each response
was measured to an accuracy of 0.1 ms, while
the amplitude (peak to peak) had an accuracy of
0.001 mV.
Finally, an abnormal response was recorded in
the orbicularis oculi following stimulation at the dis-
tal site (S3) of the mandibular branch using a pre-
viously described procedure.16
In that case, if we follow the course of action
potentials along the implicated nerve pathway, we
can assume that the latency of this abnormal re-
sponse (Lab.) will be the sum of the following:
Antidromic conduction in the mandibular branch of
the facial nerve:
Conduction time from S3 to S1, which can be
expressed as Dt2.
Proximal conduction time (Dtx) from S1 to
the site of the cross-transmission.
MUSCLE & NERVE August 1998
FIGURE 1. Design of the general experimental setup for stimulation and recording of all the parameters involved in the abnormal muscle
response. Recordings from the orbicularis oculi muscle: S1, stimulation of the facial nerve at the stylomastoid site; S2, stimulation of the
temporozygomatic branch of the facial nerve; S3, stimulation of the marginal mandibular branch of the facial nerve. S1S2 stimuli elicited
similar direct motor responses. The time delay (Dt1) was measured along with the conduction distance (d1) to allow the conduction
velocity of this branch of the facial nerve to be determined (CV1). The S3 stimulus elicited an abnormal muscle response in the orbicularis
oculi muscle, the latency of which was measured. Recordings from the mentalis muscles: S1, stimulation of the facial nerve at the
stylomastoid site; S3, stimulation of the marginal mandibular branch of the facial nerve. S1S3 stimuli elicited similar direct motor
responses. The time delay (Dt2) was measured along with the conduction distance (d2) to allow the conduction velocity of this branch of
the facial nerve to be determined (CV2). Hand-drawn simulated responses are shown for both recording situations to clarify what was
done, measured, and calculated. Lab. (in ms) is the total latency of the abnormal response; d1 (in mm) is the distance between S1 and
S2; d2 (in mm) is the distance between S1 and S3; dx (in mm) is the unknown distance of the site of the abnormal transmission from the
S1 point; CV1 (in mm/ms or m/s) is the conduction velocity of the motor fibers running into the mandibular branch; CV2 (in mm/ms or m/s)
is the conduction velocity of the motor fibers running into the zygomatic branch; dL (in ms) is the distal latency of the response in the
orbicularis oculi muscle elicited by the distal (S2) stimulus. The formula expressed in the lower part of the figure shows the way to calculate
the distance dx to the site of the abnormal transmission.

Orthodromic conduction in the zygomatic branch of
the facial nerve:
Proximal conduction time (Dtx8) from the site
of the cross-transmission to S1.
Conduction time from S1 to S2, which can be
expressed as Dt1.
Distal latency (dL) from S2 to the orbicularis
oculi muscle.
This can be expressed as:
Lab. = Dt2 + Dtx + Dtx8 + Dt1 + dL
Since the values of Dtx and Dtx8 depend on the con-
duction velocities (CV2 and CV1) of the motor fibers
in the mandibular and zygomatic branch, respec-
tively, but relate to the same distance (dx), it is pos-
sible to express these two parameters as follows:
Dtx = dx/CV2
Dtx8 = dx/CV1
Thus, eq. (a) can be expressed as:
Lab. = Dt2 + dx/CV2 + dx/CV1 + Dt1 + dL (b)
Since in eq. (b) all values except dx are known from
(a) our measurements, it is possible to calculate dx as

Hemifacial Spasm MUSCLE & NERVE August 1998 1015

the distance from S1 (stylomastoid site) up to the
intra- or extracranial point where the abnormal or
ephaptic transmission is occurring.
If this model is correct, we can assume that this
distance will be the longest one in patients from
group A, since the compression is known to be at the
cerebellopontine angle; it should be a little shorter
in patients from group B, since the nerve is pre-
sumed to be injured within the petrous bone; finally
it should be shortest of all in patients with a XII-VII
anastomosis (group C). In this last group, since the
nerve injury is probably at the level of the suture, the
distance from the S1 point to the visible scar on the
skin was also measured.
RESULTS
On the normal side of the HFS patients and in the
normal subjects, the amplitudes of the direct motor
responses elicited by proximal and distal stimulation
of the facial nerve were 1.1 0.6 mV for the orbicu-
laris oculi and 4.3 0.8 mV (mean SD) for the
mentalis muscle. The conduction velocities of the
motor fibers were 45 2.7 m/s and 47 3.1 m/s for
the zygomatic and the mandibular branches of the
facial nerve, respectively.
On the normal side of the HFS patients and in
the normal subjects, we never observed any ephap-
ticlike nor F-like responses in the facial muscles
following facial nerve stimulation.
By contrast, on the affected side of all the HFS
patients, it was possible to record an abnormal re-
sponse from the orbicularis oculi muscles following
distal stimulation of the mandibular branch of the
facial nerve or of the XII-VII crossover, depending
on the group of patients under study. This is illus-
FIGURE 2. Individual examples from a representative patient from each group. (A) Patient with idiopathic HFS. (B) Patient with post-Bells
palsy HFS. (C) Patient with post XII-VII anastomosis HFS. In the three cases, recordings were made from orbicularis oculi muscles
following stimulation of the facial nerve at: the stylomastoid site (S1) just before the facial nerve branches; the temporozygomatic branch
(S2); and the marginal mandibular branch (S3). Each trace represents the average of 10 successive responses.
1016 Hemifacial Spasm
trated in Figure 2, with individual examples from
each group of patients. In these examples, as pre-
dicted by our model, we were able to localize the site
responsible for the abnormal response at 11.0 cm,
9.3 cm, and 2.1 cm up from the stylomastoid site
(S1) in group A, group B, and group C patients,
respectively. The pooled data (mean and SD) for the
parameters under study are summarized in Table 1.
These values show no significant difference in the
distances S1S2 or S1S3 from one group to an-
other. By contrast, the conduction velocity, which
was already in the lower range of normal values in
group A patients, was significantly lower in the two
other groups (B and C) of HFS patients, as deter-
mined with paired t-tests: t = 3.91, P < 0.005, n = 8;
and t = 4.51, P < 0.005, n = 8 for group B and group
C, respectively.
Finally, as can be seen from the right-hand col-
umn in Table 1, the distance of the abnormal cross-
transmission from the stylomastoid site up the facial
nerve or the trunk of the XII-VII crossover was long-
est in group A patients (109 1.46 mm), while it was
92.96 2.35 mm in group B and 19.75 1.19 mm in
group C. In this last group, the distance from the
stylomastoid site to the scar behind the neck, where
the surgical procedure of XII-VII anastomosis had
been carried out, was measured visually as 22 5
mm, which is in good accord with that found from
our theoretical model.
DISCUSSION
The present study clearly show that in HFS patients,
the site responsible for the abnormal response elic-
ited in the orbicularis oculi muscles by stimulating
the marginal mandibular branch of the facial nerve
MUSCLE & NERVE August 1998
 Table 1. Numerical values (mean and SD) of the overall pooled data of the parameters which allowed the determination of the site
of the abnormal cross-transmission (last column) for the three groups of patients.
LE (ms) d1 (mm) d2 (mm)
Idiopathic HFS
Mean 10.98 81.25 79.75
SD 0.13 10.97 4.27
Post-Bells palsy HFS
Mean 12.63 79.00 79.75
SD 0.98 10.23 3.40
XII-VII HFS
Mean 9.05 79.50 81.50
SD 0.90 4.65 2.89
d1, distance S1S2; d2, distance S1S3.
or the XII-VII crossover, varies accordingly to the
etiology of the HFS. Furthermore, the distance of
site of the abnormal cross-transmission from the sty-
lomastoid site up the facial nerve or to the XII-VII
crossover point was found to be in line with our
theoretical model. Indeed, as could be expected, the
site most distant from the S1 level was found in pa-
tients with idiopathic HFS, whereas the closest (ex-
tracranial) one occurred in patients with XII-VII
anastomoses. In the patients with post-Bells palsy,
the distance suggested that the site of the cross-
transmission may be within the petrous bone. Our
electrophysiological data are in agreement the ana-
tomical findings; the total length of the facial nerve
involved in our measurements, from the pontocer-
ebellar angle to the point of the facial nerve where
the S1 stimulation was performed (i.e., just before
the facial nerve divides into its several peripheral
branches), has been estimated at 1012 cm.4,5,11
According to our present data, the hypothesis of
ephaptic transmission at the level of the nerve injury
appears to be the most likely in patients from groups
B and C, i.e., with HFS post-Bells palsy and post-XII-
VII anastomosis. Nevertheless, we are aware that the
position of the site given by our calculations involves
some approximations, e.g., ideally, we should in-
clude a time lapse for the ephaptic transmission. Al-
though this is immeasurable in our noninvasive ex-
perimental conditions, it is possible that this
parameter can be neglected without significantly af-
fecting the results, since ephaptic transmission is due
to electrotonic conduction through a reduced extra-
cellular space (23 nm). Such conduction is ex-
tremely rapidly (in terms of microseconds), with any
delay due to the membrane capacitance of the nerve
fibers or cells.1,7
The peripheral mechanisms of such ephaptic
transmission are supported by the following obser-
vations:
In a model of nerve section and neuroma forma-
Hemifacial Spasm
dL (ms) CV1 (m/s) CV2 (m/s) dx (mm)
1.95 42.50 41.75 109.49
0.13 2.38 1.26 1.46
2.93 35.50 35.75 92.96
0.54 2.08 1.71 2.35
3.10 34.75 33.50 19.75
0.16 4.43 4.04 1.19
tion in the rat, an accumulation of Na+ channel pro-
teins at the injured sites has been observed.3 This
shows that a nerve injury can trigger changes in ax-
olemnal Na+ channel distribution, which could ac-
count for ephaptic transmission.3 Such a phenom-
enon is likely to occur in HFS, since local
demyelination induced by the nerve injury permits
ectopic insertion of Na+ channels and thus makes
the nerve fibers hyperexcitable at this level. More-
over, it has been shown that the increase in axolem-
nal Na+ channel density, without any other change
in the membrane properties, can shift a neuron into
a state of hyperresponsiveness.10 In our present
work, it is possible to think that a similar mechanism
occurs at the site of the injured nerve, i.e., an accu-
mulation of Na+ channels which could by itself ac-
count for both ephaptic transmission and ectopic
neural discharges.
At this stage of the discussion, we can propose
that at least in HFS patients of groups B and C, the
abnormal response recorded in the orbicularis oculi
muscles after stimulation of the mandibular branch
of the facial nerve is effectively due to ephaptic trans-
mission at sites which have been clearly defined and
located by our model.
This could also be the case in patients with idio-
pathic HFS (group A); however, an alternative
proposition must be considered since: (1) the loca-
tion of the site of cross-transmission from the S1
stimulation site up the facial nerve pathway was
found to be 1011 cm; and (2) the facial nucleus is
very close to the facial root entry zone. Thus, there is
the possibility that in this case, the abnormal re-
sponse could also be due to a central hyperexcitabil-
ity of the whole facial motoneuronal pool. This latter
proposition is commonly termed as the nucleus
hypothesis. This hypothesis is supported by the ob-
servations that ectopic and abnormal peripheral
neural activity induce increased excitability in the
facial motor nucleus by creating a sustained anti-
MUSCLE & NERVE August 1998 1017
dromic activation of the neurons6,14,15,19 and by the
demonstration of a central hyperexcitability of facial
motoneurons in patients with idiopathic HFS.18,20
In conclusion, our study shows quite clearly that
the site of the cross-transmission causing the abnor-
mal response is located peripherally, through an
ephaptic mechanism in patients with post-Bells
palsy HFS (group B) and in patients with XII-VII
anastomosis HFS (group C). By contrast, we are un-
able to come to a definite conclusion in the case of
group A patients, and there remains an open debate
between peripheral and central hypotheses. As in
many controversies, it is probable that both these
mechanisms may be involved, although further stud-
ies must be undertaken to assess this idea.
We thank Mr. J.P. Bardon and Mr. M. Chastanet for technical
assistance and Dr. S.W. Cadden for English corrections.
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