Facial Palsy in Cerebral Venous ThrombosisTranscranial Stimulation and Pathophysiological Considerations

J. Straub, MD; M.R. Magistris, MD; J. Delavelle, MD; T. Landis, MD

Background—Cranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear,and data from electrophysiological examinations in such patients are missing.

Case Description—We report the case of a 17-year-old woman with familial protein S deficiency who was admitted withextensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, andMR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsyoccurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magneticstimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealedconduction block proximal to the facial canal.

Conclusions—Facial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. Wesuggest that elevated venous transmural pressure in the nerve’s satellite vein, which belongs to the affected drainageterritory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascularsystem of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitialresistance.(Stroke. 2000;31:1766-1769.)

Key Words: cerebral thrombosisn cranial nervesn neural conductionn protein S deficiencyn transcranial magnetic stimulation

The clinical picture of cerebral venous thrombosis (CVT)is variable, and a wide spectrum of neurological symp-

toms has been described.1–5 Cranial nerve palsy in CVT israre and is often thought to result from cavernous sinusthrombosis or elevated intracranial pressure.2,4–8 In the earlyliterature, several cranial nerve syndromes in CVT have beenidentified and attributed to extension of thrombosis intocontiguous venous tributaries,2 presumably leading to directpressure palsy of the nerves lying in proximity to the clot. Itis only recently that Kuehnen et al9 have drawn new attentionto cranial nerve syndromes in thrombosis of the transverseand sigmoid sinus and suggested that local stasis in thecranial nerve veins draining into the transverse sinus mightcause temporary nerve dysfunction.9 This case report ofisolated facial palsy in CVT related to protein S deficiencyrefines their hypothesis, as electrophysiological examinationhas permitted more insight into the pathogenesis of facialpalsy in CVT.

Case ReportWe describe a 17-year-old woman with familial protein Sdeficiency who began taking oral contraceptives in August1997. Four months later, on December 25, she developed asevere headache, with photophobia and phonophobia, nausea,and neck stiffness, and was hospitalized 2 days later. Cere-

brospinal fluid analysis was normal, but the initial pressurewas not measured. After transient improvement, the headacheworsened despite analgesic treatment, episodes of vomitingoccurred, and bilateral papilledema was noted. Cerebral MRIshowed extensive thrombosis in the territory of the leftinternal and proximal right internal cerebral veins, the vein ofGalen, and the straight, superior longitudinal, left transverse,and sigmoid sinuses. High-dose intravenous heparin andcoumarin were initiated, and oral contraceptives were discon-tinued. The patient was transferred to our hospital on January6, with intense nuchal pain and rigidity, papilledema withonly minimal reduction of visual acuity, and hyperreflexia.On January 8 she developed right peripheral facial palsy withlagophthalmus and dry eye without keratitis, diminishedtaste, and hyperacusis of the right ear. The palsy was mild andmore marked on the upper part of the face (frontalis andorbicularis oculi muscles). Bedside otorhinolaryngologicalexamination ruled out otitis and did not reveal eighth nervepalsy; audiometry was not performed. Repeat MRI showedpersistence of thrombosis in the above-mentioned sinuses,particularly segmental occlusion of the right transverse sinus(Figure 1). There was no contrast enhancement of the facialnerve and no temporal bone abnormality (Figure 2). On day4 after palsy onset, electrophysiological examination wasperformed 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 Geneve, CH-1211 Geneve 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

1766 by guest on March 23, 2015http://stroke.ahajournals.org/Downloaded from

(Nicolet), with recordings from mentalis and nasalis musclesusing surface electrodes.10,11For both muscles, supramaximalelectrical stimulation (ElStim) was performed in the fossastylomastoidea, and magnetic stimulation with a MagStim200 stimulator (Magstim Company Ltd) was applied to theipsilateral mastoidal region (MagStim), permitting excitationof the facial nerve at its entrance into the acoustico-facialmeatus.12 For the nasalis muscle, stimulation was also appliedto the face-associated motor cortex (CxStim). The ElStim andMagStim evoked responses were within normal range on both

mentalis and nasalis muscles, whereas the CxStim evokedresponse (from nasalis muscle) was markedly reduced inamplitude compared with the unaffected side (Figure 3).These findings are suggestive of a partial conduction block ofthe facial nerve, proximal to its entrance into the facial canal;normal response to MagStim speaks against a coincidentalidiopathic facial palsy or pressure palsy within the canal.10,11

The blink reflex R1 and R2 responses (recorded fromorbicularis oculi muscles with stimuli applied to the supraor-bital nerve) showed normal latencies but were reduced inamplitude on the palsy side.

Figure 1. Sagittal (A) and coronal (B) cerebral MR venographyshowing thrombosis in the territory of the left internal and proxi-mal right internal cerebral veins, the vein of Galen (A), andstraight and superior longitudinal sinuses, as well as the lefttransverse (B) and the junction of right transverse and sigmoidsinuses (B arrow).

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

Figure 3. Electrophysiological data. Recordings from nasalismuscle, 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 (Mag-Stim) and over the contralateral motor cortex (CxStim). On day 4(from facial palsy onset), latencies are normal; amplitudes arewithin normal range (although slightly reduced on the palsyside); MagStim evokes a normal response, thus discarding acoincidental idiopathic facial palsy or a pressure palsy within thecanal; CxStim-to-ElStim amplitude ratio is decreased on thepalsy side (14%) compared with the healthy side (43%) sug-gesting proximal conduction block. On day 18, palsy has sub-sided and ElStim and MagStim are unchanged, whereas latencyto CxStim is delayed and responses seem dispersed; CxStim-to-ElStim amplitude ratio is still decreased (18%).

Straub et al Facial Palsy in Cerebral Venous Thrombosis 1767

by guest on March 23, 2015http://stroke.ahajournals.org/Downloaded from

Under anticoagulant and analgesic treatment the patientrecovered slowly, and MR venography on January 15 showedpartial recanalization of the right transverse and most of theother sinuses (Figure 4). The patient was discharged the nextday and had completely recovered from facial palsy 10 dayslater. Because the first electrophysiological examination tookplace early and therefore could not completely rule out thesubsequent occurrence of wallerian degeneration, a secondrecording was performed. On day 18, when facial palsy hadsubsided, the ElStim and MagStim evoked responses re-mained unchanged. The CxStim response was delayed anddispersed; amplitude was still reduced.

DiscussionIn our patient, the chronology of events documented by repeatMRI (Figure 4), which showed occlusion and later recanali-zation of the right transverse sinus concomitant with onsetand recovery from facial palsy, led us to believe that the palsywas a direct consequence of the right transverse sinusocclusion. CT of the petrous bones was normal, and MRIshowed no abnormal gadolinium enhancement of the nerve,which may be seen in facial palsy of other origin.13 Patientswith phenotypic protein S deficiency may develop multiplethromboembolic complications, including CVT14,15; how-ever, there is no mention of increased incidence of nervepalsies in such patients.

Although involvement of all cranial nerves has beendescribed in CVT,2,3,5,9,16facial palsy in CVT is very rare andlittle is known about its pathophysiology. The cranial nervesyndromes identified in the earlier literature have beenthought to be caused by pressure palsy, either directly by thethrombotic clot lying in proximity to the nerves5 or by raisedintracranial pressure.7 Another explanation for cranial nervepalsy in CVT was given by Kuehnen et al,9 who reported 5cases of cranial nerve palsy as the only feature of isolatedtransverse sinus thrombosis. The authors hypothesized thatthe palsy was due to reversible compromised oxygen orglucose consumption caused by impaired drainage of thecranial nerve veins into the transverse sinus, but no electro-physiologic examination was done to support this hypothesis.In our patient, ElStim and MagStim performed on day 4 afterfacial palsy onset showed normal responses, whereas bothCxStim and blink reflex disclosed markedly decreased am-plitudes on the affected side. This indicated a normal con-duction within the facial canal and thus discarded a coinci-dental idiopathic facial palsy10,11 or a pressure palsy withinthe canal, as might have been suspected from the earlierliterature5 and knowledge about the ultrastructure of thenerve.17–19Clinically, the rapid recovery of the nerve functionwas suggestive of a palsy due to neurapraxia; diminishedtaste, hyperacusis, and dry eye indicated a suprageniculateblocking. This was confirmed by the electrophysiologicalfindings, consistent with a conduction block of the nerveproximal to the site of excitation that is proximal to itsintracanalicular segment.12

In our patient, transmural thrombosis of the distal trans-verse sinus, which involves the drainage site of the superiorpetrosal sinus and thus affects the drainage territory of thelateral pontine vein (via the superior petrosal vein),20–22couldhave raised the intraluminal pressure in the nerve’s satellitevein. Impaired venous drainage of the nerve root’s satellitevein is transmitted to the intrinsic vascular system of thenerve, which consists of segmentally arranged vessels, vari-able in number and size, that connect with the larger adjacentvessels of the extrinsic system. The postcapillary collectingvenules that run in the intraneural septa of the nerve have alumen lined with endothelial cells connected with tightjunctions which represent the venous blood-brain barri-er.18,20,22 A rise in venous intraluminal pressure in theextrinsic system can increase the venous transmural pressurein these intraneurial venules and lead to venous blood-brainbarrier dysfunction, with leakage of fluids and ions into theendoneurial space.22 Thus, an impairment of the saltatorycurrent flow, with reversible slowing of conduction or evenconduction block,23 could account for the neurapraxia prox-imal to the facial canal observed in our patient. In thefallopian canal, the drainage of the nerve is assured by awidely connected venous network that communicates withthe blood vessels of the entire temporal bone. Main vesselsempty into the lateral, superior, or inferior petrosal sinuses,the pterygoid plexus of veins, and the middle meningealveins17,24and thus provided efficient drainage of the intracan-alicular nerve portion. Because the satellite veins of the morecaudal cranial nerves drain via the inferior petrosal vein andpetrosal sinus distal to the occlusion site into the sigmoid

Figure 4. Enlargement of arrow in Figure 1B showing transmu-ral thrombosis at the junction of right transverse and sigmoidsinus (above) and control MR venography showing its recanali-zation (below).

1768 Stroke July 2000

by guest on March 23, 2015http://stroke.ahajournals.org/Downloaded from

sinus or directly into the jugular vein,21 they also remainedunaffected. We suggest that some of the cranial nervesyndromes reported earlier might have been a consequence ofsimilar electrophysiological changes rather than of directcompression or reversible compromised oxygen and glucoseconsumption.

AcknowledgmentsSupported in part by Swiss National Science Foundation Grant31–53748.98. The authors gratefully acknowledge the editorialassistance of Margaret Asomaning Delteil.

References1. Ameri A, Bousser MG. Cerebral venous thrombosis.Neurol Clin. 1992;

10:87–111.2. Lefkowitz D. Cortical thrombophlebitis and sinovenous disease. In:

Vinken PJ, Bruyn GW, Klawans HL, Toole JF, eds.Handbook of ClinicalNeurology, Vol 54.Amsterdam, Netherlands: Elsevier Science Pub-lishers; 1989:395–423.

3. Bousser MG, Chiras J, Bories J, Castaigne P. Cerebral venous throm-bosis: a review of 38 cases.Stroke. 1985;16:199–213.

4. Kalbag RM, Woolf AL. Thrombosis and thrombophlebitis of cerebralveins and dural sinuses. In: Vinken PJ, Bruyn GW, eds.Handbook ofClinical Neurology, Vol 12.Amsterdam, Netherlands: North-HollandPublishing Company; 1972:422–446.

5. Kalbag RM. Cerebral venous thrombosis. In: Kapp JP, Schmidek HH,eds. The Cerebral Venous System and Its Disorders.Orlando, Fla: Grune& Stratton; 1984:505–536.

6. Symonds CP. Intracranial thrombophlebitis: otolaryngology lecture.AnnRoy Coll Surg Engl. 1952;10:347–356.

7. Chopra JS., Banerjee AK. Primary intracranial sinovenous occlusions inyouth and pregnancy. In: Vinken PJ, Bruyn GW, Klawans HL, Toole JF,eds.Handbook of Clinical Neurology, Vol 54.Amsterdam, Netherlands:Elsevier Science Publishers; 1989:425–452.

8. Bansal BC., Gupta RR., Prakash C. Stroke during pregnancy and puer-perium in young females below the age of 40 years as a result of cerebralvenous/venous sinus thrombosis.Jpn Heart J. 1980;21:171–183.

9. Kuehnen J, Schwartz A, Neff W, Hennerici M. Cranial nerve syndromein thrombosis of the transverse/sigmoid sinuses.Brain. 1998;121:381–388.

10. Glocker FX, Magistris MR, Rosler KM, Hess CW. Magnetic transcranialand electrical stylomastoidal stimulation of the facial motor pathways inBell’s palsy: time course and relevance of electrophysiological parame-ters.Electroencephalogr Clin Neurophysiol. 1994;93:113–120.

11. Rosler KM, Magistris MR, Glocker FX, Kohler A, Deuschl G, Hess CW.Electrophysiological characteristics of lesions in facial palsies of differentetiologies: a study using electrical and magnetic stimulation techniques.Electroencephalogr Clin Neurophysiol. 1995;97:355–368.

12. Rosler KM, Hess CW, Schmid UD. Investigation of facial motorpathways by electrical and magnetic stimulation: sites and mechanisms ofexcitation.J Neurol Neurosurg Psychiatry. 1989;52:1149–1156.

13. Brandle P, Satoretti-Schefer S, Bohmer A, Wichmann W, Fisch U. Cor-relation of MRI, clinical, and electroneuronographic findings in acutefacial nerve palsy.Am J Otol. 1996;17:154–161.

14. Bostroem H, Merten F, Asseuer T, Bewermeyer P. Cerebral thrombo-phlebitis, sural phlebitis, embolies pulmonaires et deficits en proteine S.Rev Neurol. 1996;152:755–758.

15. Borgel D, Gradille S, Aiach M. Protein S deficiency.Thromb Haemost.1997;78:351–356.

16. Smith BH. Infections of the dura and its venous sinuses. In: Baker AB,Baker LH, eds.Clinical Neurology, Vol 2.Philadelphia, Pa: Harper andRow; 1984:1–34.

17. Blunt MJ. The blood supply of the facial nerve.J Anat. 1954;88:520–526.18. Bagger-Sjo¨back D, Graham MD, Thomander L. The intratemporal

vascular supply of the facial nerve: a light and electron microscopic study.In: Graham MD, House WF.Disorders of the Facial Nerve.New York,NY: Raven Press; 1982:17–31.

19. Sunderland S, Cossar D. F. The structure of the facial nerve.Anat Rec.1953;116:147–165.

20. Capra NF, Anderson KV. Anatomy of the cerebral venous system. In:Kapp JP, Schmidek HH, eds.The Cerebral Venous System and its Dis-orders.Orlando, Fla: Grune & Stratton; 1984:1–36.

21. Lazorthes G, Gouaze A, Salomon G. Les veines de l’encephale. In:Lazorthes G, Gouaze A, Salomon G.Vascularisation et circulation del’encephale.Paris, France: Masson; 1976:236–263.

22. Auer LM, MacKenzie ET. Physiology of the cerebral venous system. In:Kapp JP, Schmidek HH, eds.The Cerebral Venous System and its Dis-orders.Orlando, Fla: Grune & Stratton; 1984:169–227.

23. Gates GA, Mikiten TM. Idiopathic facial paralyses (Bell’s palsies). In:Graham MD, House WF.Disorders of the Facial Nerve.New York, NY:Raven Press; 1982:279–285.

24. Schuknecht HF. Anatomy. In: Schuknecht HF.Pathology of the Ear.Philadelphia, Pa: Lea & Febiger; 1993:31–74.

Straub et al Facial Palsy in Cerebral Venous Thrombosis 1769

by guest on March 23, 2015http://stroke.ahajournals.org/Downloaded from

J. Straub, M. R. Magistris, J. Delavelle and T. LandisPathophysiological Considerations

Facial Palsy in Cerebral Venous Thrombosis: Transcranial Stimulation and

Print ISSN: 0039-2499. Online ISSN: 1524-4628 Copyright © 2000 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Stroke doi: 10.1161/01.STR.31.7.1766

2000;31:1766-1769Stroke. 

http://stroke.ahajournals.org/content/31/7/1766World Wide Web at:

The online version of this article, along with updated information and services, is located on the

  http://stroke.ahajournals.org//subscriptions/

is online at: Stroke Information about subscribing to Subscriptions: 

http://www.lww.com/reprints Information about reprints can be found online at: Reprints:

  document. Permissions and Rights Question and Answer process is available in the

Request Permissions in the middle column of the Web page under Services. Further information about thisOnce the online version of the published article for which permission is being requested is located, click

can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office.Strokein Requests for permissions to reproduce figures, tables, or portions of articles originally publishedPermissions:

by guest on March 23, 2015http://stroke.ahajournals.org/Downloaded from