Cranial Nerve Palsies in Spontaneous Carotid Artery Dissection
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J7ournal ofNeurology, Neurosurgery, and Psychiatry 1993;56:1191-1199 1 191 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.11.1191 on 1 November 1993. Downloaded from Cranial nerve palsies in spontaneous carotid artery dissection Matthias Sturzenegger, P Huber Abstract the reported "idiopathic" jugular foramen Two patients had isolated unilateral cra- (Vernet) and similar syndromes (Villaret, nial nerve palsies due to spontaneous Collet Siccard) in the older literature might internal carotid artery (ICA) dissection well have been caused by spontaneous- carotid without ischaemic cerebral involvement. dissection.5 In those times ICA dissection as a One had acute glossopharyngeal and cause of cranial nerve palsy was poorly vagal, the other isolated hypoglossal known, angiography quite risky, and ultra- nerve palsy. Reviewing all reported cases sound and MRI not available. Out of 31 con- of angiographically confirmed ICA dis- secutive patients with ICA dissection section in the literature, 36 additional diagnosed at our department within four cases with unequivocal ipsilateral cranial years, two presented with cranial nerve palsies nerve palsies were analysed. While an without cerebral involvement. isolated palsy ofthe IXth and Xth has not been reported previously, palsies of the XIIth nerve or the IXth to XIIth nerves Case reports were most frequently found. In these PATEENT 1 patients, lower cranial nerve palsies are A 42-year-old man had been in excellent probably the result of compression by an health. He had no vascular risk factors. After enlarging ICA due to mural haematoma. strenuous activity (cutting wood) in military Symptoms and signs indicative of carotid service he suddenly felt pain in the right dissection were concurrently present upper jaw which later extended to the ear and only in some reported cases. This raises the whole right side of his face. He suffered the question of unrecognised carotid dis- an episode of hazy vision on the right eye last- section as a cause of isolated cranial ing about five minutes. The following morn- nerve palsies. When the dissection occurs ing pain had almost disappeared, but he had in the subadventitial layer without rele- difficulty swallowing and could not drink vant narrowing of the arterial lumen and properly. His voice had a nasal sound. when an aneurysm is thrombosed, Otherwise he felt well. In the hospital, three angiography does not reliably yield the days after first symptoms had occurred, his diagnosis. Therefore, carotid dissection voice was severely hoarse, almost aphonic, might have been underestimated as a and nasal. On examination he had a drooping http://jnnp.bmj.com/ cause of isolated lower cranial nerve right palate arc. On phonation the palate as palsies before the advent of MRI. MRI well as the dorsal wall of the pharynx deviated demonstrates directly the extension of to the left. Sensation to touch of the right the wall haematoma in the axial and lon- posterior pharyngeal wall was reduced and gitudinal planes. Some arteriopathies the gag reflex could not be elicited on the such as fibromuscular dysplasia and tor- right side. No other signs were found. Chest tuosity make a vessel predisposed to dis- x-ray, ECG, and routine blood laboratory section. tests were normal. Initially brainstem infarc- on September 24, 2021 by guest. Protected copyright. tion was suspected and a posterior fossa CT ( Neurol Neurosurg Psychiatry 1993;56:1 191-1199) scan was performed which was normal. Department of Because of the right sided facial pain and the Neurology, University initial of of Berne, Inselspital, episode presumed right amaurosis CH-3010 Berne, The clinical presentation of spontaneous fugax, right ICA dissection was suspected. Switzerland internal carotid artery (ICA) dissection is Duplex sonographic examination of the M Sturzenegger highly variable. Sudden, intense unilateral carotid arteries performed the same day Department of pain in the neck and face, ipsilateral ocu- showed a patent carotid bifurcation, no ather- Neuroradiology, University of Berne, losympathetic paresis, and transient monocu- osclerotic changes, and symmetrical blood Inselspital, CH-3010 lar blindness (amaurosis fugax) associated flow velocities. Both common carotid arteries Berne, Switzerland with a contralateral transient ischaemic attack were also normal. Pulsed Doppler sono- P Huber or stroke form the typical presenting triad graphic examination of the upper cervical Correspondence to: M Sturzenegger, MD, suggesting extracranial carotid dissection.'23 segments of both ICAs disclosed a coiling of Department of Neurology, Lower cranial nerve palsies seem to be a rare both with adjacent antegrade and retrograde University of Beme, Inselspital, CH-3010 Berne, finding, with the hypoglossal nerve being the flow in the high cervical portion. However, Switzerland. most frequently affected (5%)4: However, the blood flow velocities on the right side Received 2 November 1992 when ICA dissection occurs without CNS were twice as high as on the left side in both in revised form 31 December 1992 involvement, it is not readily recognised as flow directions. This finding supported the Accepted 14 January 1993 the cause of a cranial nerve palsy. Some of diagnosis of an ICA dissection and anticoagu- 1192 Sturzenegger, Huber J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.11.1191 on 1 November 1993. Downloaded from lation with heparin sodium was started. The yielded the explanation. A large ICA mural following day, a right common carotid arteri- haematoma was demonstrated as a hyperin- ogram was performed which also revealed the tense signal on the sagittal and axial sections ICA tortuosity. There were irregularities of of the Ti- and T2-weighted and proton den- the vessel wall about 3 cm distal of the bifur- sity spin echo sequences (fig 2). It extended cation and extending cranially beyond the from beneath the coiling up to the petrosal coiling. Equivocal slight stenosis on the carotid canal. In the axial plane, a large sub- anteroposterior projections was visible (fig 1). adventitial extension of the haematoma was In addition, a pouch with trapped contrast demonstrated with expansion of the vessel media showed up on the late lateral views wall in the carotid space. This led to displace- (fig 1 a2 and 1 a3) which is a quite distinct ment of the parapharyngeal space (fig 2 bl sign of dissection. These findings confirmed and 2 b2) where the glossopharyngeal nerve the suspected diagnosis of ICA dissection. crosses the ICA medially leading from the They could, however, not explain the glosso- jugular foramen to the root of the tongue. In pharyngeal and vagal nerve palsy. this region the Xth cranial nerve is also in MR images of the upper cervical region close contact with the ICA (fig 3). Cranial performed eight days after the first symptoms MRI was normal. After one week of heparin treatment, oral anticoagulation with a coumarin was started. No visual or TIA-like symptoms occurred. Six weeks after admission, hoarseness and nasal voice were still present, but swallowing was possible again. Pharyngeal sensation was normal on both sides, no palate droop was found any more and pharyngeal wall shift on phonation was only minimal. Doppler sonog- raphy still showed a higher blood flow veloc- ity on the right side. A second MRI scan eight weeks after admission showed complete resolution of the mural haematoma. Anti- coagulants were continued and after a total of 11 weeks all symptoms and signs had cleared. A persistent slight asymmetry of blood flow velocities at Doppler examination was consid- ered to be due to possible slight residual stenosis. Coumarins were stopped and aspirin started. PATENT 2 A 45-year-old man had untreated mild arter- ial hypertension for several years. During the past two years he suffered from intermittent ::.; ... ..:. slight nuchal pain. Two days before admis- http://jnnp.bmj.com/ zg. sion nuchal pain intensified but did not ....X. .... r r F change its well known quality. There was no }w special event and no trauma. On the day of .:.: .. ::...... admission he noticed difficulty with articula- .: '? :. :?:::. .. j. *:.:::. tion, a heavy tongue, and problems to eat. *; .,: ... ... ........ Slightly slurred speech was noted by his wife. *:". :: Brainstem infarction was suspected and a CT :;::: ....: .t..:L:: scan performed which was normal. On on September 24, 2021 by guest. Protected copyright. :. e ^ ;. :.: .v. .... .. ... ::: :^ .: .: :. admission he clearly stated that he could not _ ::: .: ::; ... .. .::::.. :::: :.x; .... : ::: ... properly move the food with his tongue, while :::: .:: ..: oral sensibility, chewing, and swallowing were .. ..,: .: .. ...'.5 -..: h.r . not disturbed. Examination showed moderate left hypoglossal palsy with tongue deviation to 0: :: ::.*: the left when thrusting it out and deviation to :.-. Z..: the right when phonating. No other signs were found. Chest and cervical spime x ray, electrocardiogram, and routine blood labora- tory tests were normal. * X : .... : : : ::: Doppler and Duplex sonographic examina- . - ': : .. : . :. tion of the cervical carotid and vertebral arteries and transcranial Doppler sonography Figure 1* ;' . .g:B(Patient . .\.: ............1):.Right common carotid arteriogram (al to a3 on the day of admission were normal. Repeat sequential lateral views, bI and b2 sequential anteroposterior projections). Lateral views demonstrate only minimal wall irregularities (arrows) without recognisable stenosis.