Facial Palsy in Cerebral Venous Thrombosis

Transcranial Stimulation and Pathophysiological Considerations

J. Straub, MD; M.R. Magistris, MD; J. Delavelle, MD; T. Landis, MD
BackgroundCranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear,
and data from electrophysiological examinations in such patients are missing.
Case DescriptionWe report the case of a 17-year-old woman with familial protein S deficiency who was admitted with
extensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, and
MR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsy
occurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magnetic
stimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealed
conduction block proximal to the facial canal.
ConclusionsFacial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. We
suggest that elevated venous transmural pressure in the nerves satellite vein, which belongs to the affected drainage
territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular
system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial
resistance. (Stroke. 2000;31:1766-1769.)
Key Words: cerebral thrombosis cranial nerves neural conduction protein S deficiency
transcranial magnetic stimulation

he clinical picture of cerebral venous thrombosis (CVT)

is variable, and a wide spectrum of neurological symptoms has been described.15 Cranial nerve palsy in CVT is
rare and is often thought to result from cavernous sinus
thrombosis or elevated intracranial pressure.2,4 8 In the early
literature, several cranial nerve syndromes in CVT have been
identified and attributed to extension of thrombosis into
contiguous venous tributaries,2 presumably leading to direct
pressure palsy of the nerves lying in proximity to the clot. It
is only recently that Kuehnen et al9 have drawn new attention
to cranial nerve syndromes in thrombosis of the transverse
and sigmoid sinus and suggested that local stasis in the
cranial nerve veins draining into the transverse sinus might
cause temporary nerve dysfunction.9 This case report of
isolated facial palsy in CVT related to protein S deficiency
refines their hypothesis, as electrophysiological examination
has permitted more insight into the pathogenesis of facial
palsy in CVT.

Case Report
We describe a 17-year-old woman with familial protein S
deficiency who began taking oral contraceptives in August
1997. Four months later, on December 25, she developed a
severe headache, with photophobia and phonophobia, nausea,
and neck stiffness, and was hospitalized 2 days later. Cere-

brospinal fluid analysis was normal, but the initial pressure

was not measured. After transient improvement, the headache
worsened despite analgesic treatment, episodes of vomiting
occurred, and bilateral papilledema was noted. Cerebral MRI
showed extensive thrombosis in the territory of the left
internal and proximal right internal cerebral veins, the vein of
Galen, and the straight, superior longitudinal, left transverse,
and sigmoid sinuses. High-dose intravenous heparin and
coumarin were initiated, and oral contraceptives were discontinued. The patient was transferred to our hospital on January
6, with intense nuchal pain and rigidity, papilledema with
only minimal reduction of visual acuity, and hyperreflexia.
On January 8 she developed right peripheral facial palsy with
lagophthalmus and dry eye without keratitis, diminished
taste, and hyperacusis of the right ear. The palsy was mild and
more marked on the upper part of the face (frontalis and
orbicularis oculi muscles). Bedside otorhinolaryngological
examination ruled out otitis and did not reveal eighth nerve
palsy; audiometry was not performed. Repeat MRI showed
persistence of thrombosis in the above-mentioned sinuses,
particularly segmental occlusion of the right transverse sinus
(Figure 1). There was no contrast enhancement of the facial
nerve and no temporal bone abnormality (Figure 2). On day
4 after palsy onset, electrophysiological examination was
performed on a Viking IV electromyography apparatus

Received February 8, 2000; final revision received March 30, 2000; accepted March 30, 2000.
From the Departments of Neurology (J.S., M.R.M., T.L.), and Radiology (J.D.), University Hospital of Geneva, Geneva, Switzerland.
Correspondence to Dr J. Straub, c/o Dr M.R. Magistris, Clinique de Neurologie, Hopital Cantonal Universitaire de Gene`ve, CH-1211 Gene`ve 14, rue
Micheli-du-Crest 24, Switzerland. E-mail judith.straub@hcuge.ch
2000 American Heart Association, Inc.
Stroke is available at http://www.strokeaha.org

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Facial Palsy in Cerebral Venous Thrombosis

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Figure 2. Axial T1-weighted gadolinium MRI shows no abnormal contrast enhancement of the facial nerve in its intracranial
segment (above) or in the fallopian canal (below).

mentalis and nasalis muscles, whereas the CxStim evoked

response (from nasalis muscle) was markedly reduced in
amplitude compared with the unaffected side (Figure 3).
These findings are suggestive of a partial conduction block of
the facial nerve, proximal to its entrance into the facial canal;
normal response to MagStim speaks against a coincidental
idiopathic facial palsy or pressure palsy within the canal.10,11
The blink reflex R1 and R2 responses (recorded from
orbicularis oculi muscles with stimuli applied to the supraorbital nerve) showed normal latencies but were reduced in
amplitude on the palsy side.

Figure 1. Sagittal (A) and coronal (B) cerebral MR venography

showing thrombosis in the territory of the left internal and proximal right internal cerebral veins, the vein of Galen (A), and
straight and superior longitudinal sinuses, as well as the left
transverse (B) and the junction of right transverse and sigmoid
sinuses (B arrow).

(Nicolet), with recordings from mentalis and nasalis muscles

using surface electrodes.10,11 For both muscles, supramaximal
electrical stimulation (ElStim) was performed in the fossa
stylomastoidea, and magnetic stimulation with a MagStim
200 stimulator (Magstim Company Ltd) was applied to the
ipsilateral mastoidal region (MagStim), permitting excitation
of the facial nerve at its entrance into the acoustico-facial
meatus.12 For the nasalis muscle, stimulation was also applied
to the face-associated motor cortex (CxStim). The ElStim and
MagStim evoked responses were within normal range on both

Figure 3. Electrophysiological data. Recordings from nasalis

muscle, on the palsy side (RIGHT) and control healthy side
(LEFT). Electrical stimuli are applied at the fossa stylomastoidea
(ElStim); magnetic transcranial stimuli are on the mastoid (MagStim) and over the contralateral motor cortex (CxStim). On day 4
(from facial palsy onset), latencies are normal; amplitudes are
within normal range (although slightly reduced on the palsy
side); MagStim evokes a normal response, thus discarding a
coincidental idiopathic facial palsy or a pressure palsy within the
canal; CxStim-to-ElStim amplitude ratio is decreased on the
palsy side (14%) compared with the healthy side (43%) suggesting proximal conduction block. On day 18, palsy has subsided and ElStim and MagStim are unchanged, whereas latency
to CxStim is delayed and responses seem dispersed; CxStimto-ElStim amplitude ratio is still decreased (18%).

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Stroke

July 2000

Figure 4. Enlargement of arrow in Figure 1B showing transmural thrombosis at the junction of right transverse and sigmoid
sinus (above) and control MR venography showing its recanalization (below).

Under anticoagulant and analgesic treatment the patient

recovered slowly, and MR venography on January 15 showed
partial recanalization of the right transverse and most of the
other sinuses (Figure 4). The patient was discharged the next
day and had completely recovered from facial palsy 10 days
later. Because the first electrophysiological examination took
place early and therefore could not completely rule out the
subsequent occurrence of wallerian degeneration, a second
recording was performed. On day 18, when facial palsy had
subsided, the ElStim and MagStim evoked responses remained unchanged. The CxStim response was delayed and
dispersed; amplitude was still reduced.

Discussion
In our patient, the chronology of events documented by repeat
MRI (Figure 4), which showed occlusion and later recanalization of the right transverse sinus concomitant with onset
and recovery from facial palsy, led us to believe that the palsy
was a direct consequence of the right transverse sinus
occlusion. CT of the petrous bones was normal, and MRI
showed no abnormal gadolinium enhancement of the nerve,
which may be seen in facial palsy of other origin.13 Patients
with phenotypic protein S deficiency may develop multiple
thromboembolic complications, including CVT14,15; however, there is no mention of increased incidence of nerve
palsies in such patients.

Although involvement of all cranial nerves has been

described in CVT,2,3,5,9,16 facial palsy in CVT is very rare and
little is known about its pathophysiology. The cranial nerve
syndromes identified in the earlier literature have been
thought to be caused by pressure palsy, either directly by the
thrombotic clot lying in proximity to the nerves5 or by raised
intracranial pressure.7 Another explanation for cranial nerve
palsy in CVT was given by Kuehnen et al,9 who reported 5
cases of cranial nerve palsy as the only feature of isolated
transverse sinus thrombosis. The authors hypothesized that
the palsy was due to reversible compromised oxygen or
glucose consumption caused by impaired drainage of the
cranial nerve veins into the transverse sinus, but no electrophysiologic examination was done to support this hypothesis.
In our patient, ElStim and MagStim performed on day 4 after
facial palsy onset showed normal responses, whereas both
CxStim and blink reflex disclosed markedly decreased amplitudes on the affected side. This indicated a normal conduction within the facial canal and thus discarded a coincidental idiopathic facial palsy10,11 or a pressure palsy within
the canal, as might have been suspected from the earlier
literature5 and knowledge about the ultrastructure of the
nerve.1719 Clinically, the rapid recovery of the nerve function
was suggestive of a palsy due to neurapraxia; diminished
taste, hyperacusis, and dry eye indicated a suprageniculate
blocking. This was confirmed by the electrophysiological
findings, consistent with a conduction block of the nerve
proximal to the site of excitation that is proximal to its
intracanalicular segment.12
In our patient, transmural thrombosis of the distal transverse sinus, which involves the drainage site of the superior
petrosal sinus and thus affects the drainage territory of the
lateral pontine vein (via the superior petrosal vein),20 22 could
have raised the intraluminal pressure in the nerves satellite
vein. Impaired venous drainage of the nerve roots satellite
vein is transmitted to the intrinsic vascular system of the
nerve, which consists of segmentally arranged vessels, variable in number and size, that connect with the larger adjacent
vessels of the extrinsic system. The postcapillary collecting
venules that run in the intraneural septa of the nerve have a
lumen lined with endothelial cells connected with tight
junctions which represent the venous blood-brain barrier.18,20,22 A rise in venous intraluminal pressure in the
extrinsic system can increase the venous transmural pressure
in these intraneurial venules and lead to venous blood-brain
barrier dysfunction, with leakage of fluids and ions into the
endoneurial space.22 Thus, an impairment of the saltatory
current flow, with reversible slowing of conduction or even
conduction block,23 could account for the neurapraxia proximal to the facial canal observed in our patient. In the
fallopian canal, the drainage of the nerve is assured by a
widely connected venous network that communicates with
the blood vessels of the entire temporal bone. Main vessels
empty into the lateral, superior, or inferior petrosal sinuses,
the pterygoid plexus of veins, and the middle meningeal
veins17,24 and thus provided efficient drainage of the intracanalicular nerve portion. Because the satellite veins of the more
caudal cranial nerves drain via the inferior petrosal vein and
petrosal sinus distal to the occlusion site into the sigmoid

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Straub et al
sinus or directly into the jugular vein,21 they also remained
unaffected. We suggest that some of the cranial nerve
syndromes reported earlier might have been a consequence of
similar electrophysiological changes rather than of direct
compression or reversible compromised oxygen and glucose
consumption.

Acknowledgments
Supported in part by Swiss National Science Foundation Grant
3153748.98. The authors gratefully acknowledge the editorial
assistance of Margaret Asomaning Delteil.

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Facial Palsy in Cerebral Venous Thrombosis: Transcranial Stimulation and

Pathophysiological Considerations
J. Straub, M. R. Magistris, J. Delavelle and T. Landis
Stroke. 2000;31:1766-1769
doi: 10.1161/01.STR.31.7.1766
Stroke is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
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