Lower Cranial Nerve Palsies Due to Internal Carotid...
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1561 Lower Cranial Nerve Palsies Due to Internal Carotid Dissection Walter Waespe, MD, Jiirg Niesper, MD, Hans-Georg Imhof, MD, and Anton Valavanis, MD A 41-year-old man experienced intense headache and neck pain, bruits, and a complete unilateral cranial nerve palsy IX-XII (Collet-Sicard syndrome) after a trivial back trauma. Magnetic resonance imaging and angiography demonstrated features of bilateral internal carotid artery dissection with aneurysm formation at the base of the skull compressing the nerves at the level of the jugular foramen. Severe dysphagia persisted for 1 month but rapidly improved after occulsion of the carotid aneurysm with a detachable balloon. (Stroke 1988; 19:1561-1564) "S 1 pontaneous" dissection of the extra- cranial arteries is increasingly recog- nized as an important cause of acute headache, neck pain, and delayed focal cerebral ischemic symptoms in younger and middle-aged patients. 12 The most commonly affected vessel is the cervical internal carotid artery (ICA). Lower cranial nerve palsy as a complication of ICA dissec- tion is rare. 3 - 5 We report the clinical and neuroradio- logicfindingsand the management of a patient with a complete unilateral lower cranial nerve palsy IX-XII (Collet-Sicard syndrome) due to aneurysm formation of presumed ICA dissection at the base of the skull. Case Report A 41-year-old man was admitted for evaluation of hoarseness and dysphagia. His medical history was unremarkable. Eleven days before admission he was struck on his back by a small falling tree, not directly injuring his head or neck. The accident was deemed insignificant by the patient and was not reported spontaneously. The patient was well until 4 days later, when he experienced severe continu- ous left-sided headache and neck pain. Three days later (7 days after the trauma) the patient noted that his tongue deviated to the left when protruded. The following day he noticed a pulsatile sound in his right ear, hoarseness, and a progressive difficulty in swallowing, initially for food and later also for liquids, and a difficulty in turning his head to the right. From the Departments of Neurology (W.W., J.N.), Neuro- surgery (H-G.I.), and Neuroradiology (A.V.), University Hospi- tal, Zurich, Switzerland. Address for reprints: W. Waespe, MD, Department of Neu- rology, University Hospital, Frauenklinikstrasse 26, CH-8091 Zurich, Switzerland. Received May 9, 1988; accepted July 15, 1988. The general physical examination, ears, and neck were normal. No carotid bruits were heard, and carotid pulses were symmetric. Neurologic exami- nation revealed normal comprehension, memory, and speech. Pupils were symmetric, with normal light and near reactions. There was dysgeusia on the left side, a complete paralysis of the left-sided lower cranial nerves IX-XII with dysphagia for solids and liquids, regurgitation of liquid into the nose, displacement of the posterior wall of the pharynx toward the right side, and hemianesthesia. There was vocal cord paralysis with a dysphonic, hoarse voice. There was weakness of head turning to the right, with distinct atrophy of the left sterno- cleidomastoid muscle, and left-sided shoulder droop. His tongue had a shriveled appearance suggesting atrophy, and when protruded it was drawn toward the paralyzed side. His tongue's movements were slow, and it was believed that the force of the right hypoglossal muscle was also diminished. Respiration- dependent beat-to-beat variation of heart rate was present. The remainder of the neurologic examina- tion was normal. Extensive laboratory tests including investigation for collagen, vascular, and inflammatory disease showed no abnormalities. Cerebrospinal fluid exam- inations were normal on two occasions. Serologic tests for syphilis were negative. Clinically, we sus- pected a jugular foramen syndrome with an addi- tional paresis of the hypoglossal nerve on the left side, but a slight palsy of the right hypoglossal nerve could not be excluded. Results of Doppler ultrasonography of the carotid and vertebral arter- ies were entirely normal. Electromyographic find- ings (10 days after the development of clinical signs) revealed an almost complete paralysis of the left hypoglossal and sternocleidomastoid muscles, with only single motor unit potentials, but no denerva- by guest on April 19, 2018 http://stroke.ahajournals.org/ Downloaded from
Transcript of Lower Cranial Nerve Palsies Due to Internal Carotid...
1561
Lower Cranial Nerve PalsiesDue to Internal Carotid Dissection
Walter Waespe, MD, Jiirg Niesper, MD, Hans-Georg Imhof, MD,
and Anton Valavanis, MD
A 41-year-old man experienced intense headache and neck pain, bruits, and a complete unilateralcranial nerve palsy IX-XII (Collet-Sicard syndrome) after a trivial back trauma. Magneticresonance imaging and angiography demonstrated features of bilateral internal carotid arterydissection with aneurysm formation at the base of the skull compressing the nerves at the level ofthe jugular foramen. Severe dysphagia persisted for 1 month but rapidly improved after occulsionof the carotid aneurysm with a detachable balloon. (Stroke 1988; 19:1561-1564)
"S1pontaneous" dissection of the extra-cranial arteries is increasingly recog-nized as an important cause of acute
headache, neck pain, and delayed focal cerebralischemic symptoms in younger and middle-agedpatients.12 The most commonly affected vessel isthe cervical internal carotid artery (ICA). Lowercranial nerve palsy as a complication of ICA dissec-tion is rare.3-5 We report the clinical and neuroradio-logic findings and the management of a patient with acomplete unilateral lower cranial nerve palsy IX-XII(Collet-Sicard syndrome) due to aneurysm formationof presumed ICA dissection at the base of the skull.
Case ReportA 41-year-old man was admitted for evaluation of
hoarseness and dysphagia. His medical history wasunremarkable. Eleven days before admission hewas struck on his back by a small falling tree, notdirectly injuring his head or neck. The accident wasdeemed insignificant by the patient and was notreported spontaneously. The patient was well until4 days later, when he experienced severe continu-ous left-sided headache and neck pain. Three dayslater (7 days after the trauma) the patient noted thathis tongue deviated to the left when protruded. Thefollowing day he noticed a pulsatile sound in hisright ear, hoarseness, and a progressive difficulty inswallowing, initially for food and later also forliquids, and a difficulty in turning his head to theright.
From the Departments of Neurology (W.W., J.N.), Neuro-surgery (H-G.I.), and Neuroradiology (A.V.), University Hospi-tal, Zurich, Switzerland.
Address for reprints: W. Waespe, MD, Department of Neu-rology, University Hospital, Frauenklinikstrasse 26, CH-8091Zurich, Switzerland.
Received May 9, 1988; accepted July 15, 1988.
The general physical examination, ears, and neckwere normal. No carotid bruits were heard, andcarotid pulses were symmetric. Neurologic exami-nation revealed normal comprehension, memory,and speech. Pupils were symmetric, with normallight and near reactions. There was dysgeusia onthe left side, a complete paralysis of the left-sidedlower cranial nerves IX-XII with dysphagia forsolids and liquids, regurgitation of liquid into thenose, displacement of the posterior wall of thepharynx toward the right side, and hemianesthesia.There was vocal cord paralysis with a dysphonic,hoarse voice. There was weakness of head turningto the right, with distinct atrophy of the left sterno-cleidomastoid muscle, and left-sided shoulder droop.His tongue had a shriveled appearance suggestingatrophy, and when protruded it was drawn towardthe paralyzed side. His tongue's movements wereslow, and it was believed that the force of the righthypoglossal muscle was also diminished. Respiration-dependent beat-to-beat variation of heart rate waspresent. The remainder of the neurologic examina-tion was normal.
Extensive laboratory tests including investigationfor collagen, vascular, and inflammatory diseaseshowed no abnormalities. Cerebrospinal fluid exam-inations were normal on two occasions. Serologictests for syphilis were negative. Clinically, we sus-pected a jugular foramen syndrome with an addi-tional paresis of the hypoglossal nerve on the leftside, but a slight palsy of the right hypoglossalnerve could not be excluded. Results of Dopplerultrasonography of the carotid and vertebral arter-ies were entirely normal. Electromyographic find-ings (10 days after the development of clinical signs)revealed an almost complete paralysis of the lefthypoglossal and sternocleidomastoid muscles, withonly single motor unit potentials, but no denerva-
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1562 Stroke Vol 19, No 12, December 1988
FIGURE 1. Magnetic resonanceimages of41-year-old man, axialsections through base of skull(A, C) and left-sided para-sagittal sections (B, D). R, right;L, left. T ,-weighted images; rep-etition time 500-650 msec, echotime 30 msec. A, B: Three weeksafter onset of nerve palsies.White arrows mark pathologicchanges. C, D: Control images9 months later.
tion potentials. The pattern of activation on theright side was within normal limits.
Computed tomography of the posterior fossa andthe craniocervical region was unremarkable, withnormal brain structures and normal hypoglossalcanals and jugular foramina on both sides. Magneticresonance imaging (MRI) with axial and parasagittalscanning planes (Figure 1, A and B) revealedincreased signal intensity in the wall of both ICAsjust at the base of the skull at the entrance of thevessels into the canal of the petrous bone. Thesepathologic changes were circular on the left sidewith narrowing of the lumen and irregular on theright side. The white matter of the brain was nor-mal, with no foci suggestive of intracerebral isch-emia. Four-vessel digital subtraction angiographydemonstrated narrowing and dilatation of the pre-petrous segment of both ICAs with aneurysm for-mation (Figure 2). This pathologic change is stronglysuggestive of bilateral carotid dissection.1-6 Alsocharacteristic for the diagnosis is the abrupt recon-stitution of the lumen near the carotid canal. Theleft posterior cerebral artery was filled from the leftcarotid artery. On the right side a persistent trigem-inal artery was visualized, with retrograde filling ofboth ICAs via the persistent trigeminal artery on
compression of the left carotid artery. The vertebralarteries were unremarkable.
The patient was unable to swallow, and nutritionhad to be administered by an enteric tube. After 1month of persistent dysphagia, the left-sided symp-tomatic aneurysm was obliterated with a detachableballoon after an extracranial-intracranial (EC-IC)bypass was performed. Seven days after surgery,the patient could swallow liquid without nasal regur-gitation and nasogastric feedings could be stopped.Ten days after surgery, he started to swallow foodand his articulation improved. Fourteen days aftersurgery he could gargle again, and in the followingdays nerve paralysis further improved; only thedysgeusia persisted unchanged.
The pulsatile bruit in his right ear subsided 1month after release from the hospital. Eighteenmonths after surgery, the patient felt normal; histongue was still atrophic on the left side but devi-ated only slightly to the left when protruded. Inparallel with his clinical improvement, the electro-physiological parameters in the electromyogramwere also improved. The left hypoglossal muscleshowed signs of chronic neurogenic denervation,with signs of reinnervation. In control MRI 9 monthsafter surgery (Figure 1, C and D), no increased
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Waespe et al Carotid Dissection 1563
B
FIGURE 2. Digital subtraction angiography of right (A) and left (B) internal carotid artery.
signal intensity could be detected in the wall of theright ICA, and probably only the anterolateral wallof the left ICA still showed some pathologic signalincrease.
DiscussionWe describe a patient with a complete unilateral
IX-XII nerve palsy (Collet-Sicard syndrome) thatdeveloped rapidly after a trivial back trauma. Var-ious syndromes of multiple lower cranial nervepalsies have been described in combination withlesions near the jugular foramen.7 Causes of Collet-Sicard syndrome are numerous: vascular lesions ofthe jugular vein and the carotid artery below theskull base, inflammatory lesions, fractures of theskull base, and various tumors such as neurinomas,metastases, lymphomas, and, most importantly,jugular glomus tumors.7-10
The glossopharyngeal, vagus, and accessorynerves leave the base of the skull through thejugular foramen (pars nervosa) anteromedial to thejugular vein; the hypoglossal nerve normally passesthrough its own canal. These nerves join in therestrostyloid space, which also harbors the jugularvein, the carotid artery, the sympathetic chain, andthe lymphatics. The accessory nerve turns laterallyimmediately after leaving the foramen and crossesthe jugular vein posteriorly. We assume that theaneurysm formation of the dissecting ICA com-
pressed the nerves in the retrostyloid space on theleft side. Rapid recovery after occlusion of thepseudoaneurysm rather excludes ischemic segmen-tal infarction of these nerves.10
The presence of a carotid artery pathology in thecervical segment was suggested in our patient byMRI. The angiographic findings with narrowing andaneurysm formation at the base of the skull aretypical for "spontaneous" dissection of the ICA, adiagnosis also suggested by the fairly typical his-tory, with severe headache and neck pain, and asubjective bruit following a trivial trauma.1-311 Aneu-rysm formation in the upper cervical carotid arteryis the most specific finding of extracranial carotiddissection.5 Among the extracranial cerebral arter-ies, the ICA is the artery where a "spontaneous"dissection typically occurs, beginning 2-3 cm distalto the bifurcation and extending to the base of theskull but rarely extending into the intrapetrosalsegment of the vessel. The most common manifes-tations of ICA dissection are unilateral headache,focal cerebral ischemic symptoms, and oculosym-pathetic paresis and bruits.11 Except for dysgeusia,lower cranial nerve palsies are fairly rare in carotiddissection.3-510 Hart and Easton" report an inci-dence of hypoglossal nerve palsy of 5%.
The management of patients with carotid dissec-tion is less than clear. Several medical and surgicalprocedures, including removal of intramural hema-
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1564 Stroke Vol 19, No 12, December 1988
toma, distal thrombectomy, ligation of the distalextracranial carotid artery combined with an EC-IC bypass, and anticoagulation and antiplateletagents,"-13 have been suggested. Recent experi-ences suggest a remarkably favorable outcomeirrespective of therapeutic interventions.111 Mostremarkably, dissections rarely recur, and follow-up angiographic studies have shown reconstitutionof normal lumina.514-16 About two thirds of thedissecting aneurysms were found to decrease insize or to resolve completely.1 Bradac and co-workers described a patient with an incompleteunilateral IX-XII nerve palsy that was due toaneurysmal ICA dissection and that spontane-ously resolved. With such a favorable naturalhistory, it is difficult to be certain whether thecombined surgical-neuroradiologic intervention wasbeneficial in our patient. However, our decisionwas made after 1 month of debilitating, complete,unilateral lower cranial nerve palsy with dyspha-gia and no signs of any recovery. The patient'srapid postoperative recovery was remarkable. It ispossible that resolution of the nerve palsies wouldhave occurred spontaneously later, as many dis-secting aneurysms tend to decrease in size. Spon-taneous resolution of the dissection on the oppo-site, probably asymptomatic, side is suggested bythe unremarkable MRI repeated 9 months after theinitial diagnosis. Presently, no general recommen-dations can be made for the management of patientswith symptomatic dissecting ICA aneurysms.
References1. Mokri B, Sundt TM, Houser OW, Piepgras DG: Spontane-
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2. Caplan LR, Zarins CK, Hemmati M: Spontaneous dissec-tion of the extracranial vertebral arteries. Stroke 1985;16:1030-1038
3. Davis JM, Zimmermann RA: Injury of the carotid andvertebral arteries. Neuroradiology 1983;25:55-69
4. Bradac GB, Kaernbach A, Bolk-Weischedel D, Finck GA:Spontaneous dissecting aneurysm of cervical cerebral arter-ies. Neuroradiology 1981 ;21:149-154
5. Fisher CM, Ojemann RG, Roberson GH: Spontaneous dis-section of cervicocerebral arteries. Can J Neurol Sci 1978;5:9-19
6. Fisher CM: The headache and pain of spontaneous carotiddissection. Headache 1982;22:60-65
7. Roger J, Bille J, Vigouroux RA: Multiple cranial nervepalsies, in Vinken PJ, Bruyn GW (eds): Handbook of Clin-ical Neurology. 1969, vol 2, pp 86-106
8. Kramer W: Glomus jugulare tumours, in Vinken PJ, BruynGW (eds): Handbook of Clinical Neurology. 1975, vol 18, pp435-455
9. Spector GJ, Gado M, Ciralsky R, Ogura JH, Maisel RH:Neurologic implications of glomus tumours in the head andneck. Laryngoscope 1975;85:1387-1395
10. Havelius U, Hindfelt B, Brismar J, Cronqvist S: Carotidfibromuscular dysplasia and paresis of lower cranial nerves(Collet-Sicard syndrome). J Neurosurg l982;56:850-853
11. Hart RG, Easton DJ: Dissections of cervical and cerebralarteries, in Barnett HJM (ed): Cerebrovascular disease.Neurol Clin 1983;!: 155-182
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14. Gee W, Kaupp HA, McDonald KM, Lin FZ, Curry JL:Spontaneous dissection of internal carotid arteries: Sponta-neous resolution documented by serial ocular pneumoplethys-mography and angiography. Arch Surg 1980;l 15:944-949
15. Houser OW, Mokri B, Sundt TM, Baker HL, Reese DF:Spontaneous cervical cephalic arterial dissection and itsresiduum: Angiographic spectrum. AJNR !984;5:27-34
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KEY WORDS • carotid artery diseases • dissection
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W Waespe, J Niesper, H G Imhof and A ValavanisLower cranial nerve palsies due to internal carotid dissection.
Print ISSN: 0039-2499. Online ISSN: 1524-4628 Copyright © 1988 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.19.12.1561
1988;19:1561-1564Stroke.
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