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Tapia syndrome caused by a vertebral artery dissection.


Tapia syndrome is rare presentation in otorhinolaryngology, and a very limited number of reports of such cases has been published in the literature. This syndrome is characterized by unilateral paralysis of the Xth and XIIth cranial nerves. It is classified as either peripheral or central, depending on its presentation. We describe a case of peripheral Tapia syndrome secondary to a vertebral artery dissection.

Case report

A 63-year-old man complained of a 3-week history of hoarseness and associated right-sided neck pain. His symptoms arose following a period of vigorous coughing. He was otherwise healthy.

Examination confirmed the patient's hoarseness. No aphasia or dysarthria was present. His tongue deviated to the right, and his right vocal fold was fixed in the intermediate position. The rest of his cranial nerve functions were normal.

To exclude jugular foramen syndrome and dissection of the internal carotid artery, magnetic resonance imaging (MRI) of the head and neck was obtained. The MRI detected a narrowing of the right vertebral artery at the level of the first and second vertebrae over a distance of 1 cm (figure). No ischemic lesion was noted in the area of the right vertebral artery.

The patient was administered prophylactic anticoagulation with clopidogrel at 75 mg/day for 6 weeks. Speech therapy was also recommended. At follow-up, however, he showed only a partial improvement.


In 1906, Spanish otorhinolaryngologist Antonio Garcia Tapia became the first to describe a lesion of the vagus and hypoglossal nerves with contralateral hemiplegia. (1) Tapia's original patient was a matador who had sustained a wound to the neck from a bull's horn. Most later reports described Tapia syndrome as a peripheral disease of the Xth and XIIth cranial nerves.

Central Tapia syndrome, which is extremely rare, is characterized by hemiparesis, often with hemihypoesthesia contralateral to the cranial nerve palsies. In Tapia's original report, the cause of the hemiplegia was not stated. (1) Two possible causes are embolisms in a traumatized carotid artery and dissection of a vertebral artery with subsequent brainstem infarction.

To the best of our knowledge, only 3 cases of medial or mediolateral medullary infarction and the classic clinical features of central Tapia syndrome have been described during the past century; 2 of these patients had brainstem infarctions, and 1 had meningovascular syphilis. (2) The peripheral variant of Tapia syndrome can occur (1) following trauma or endotracheal intubation, (2) in association with a tumor of the parotid gland, or (3) iatrogenically as a result of surgery. (l,3,4) Congenital (5) and idiopathic (6) cases have also been described.

Our patient's peripheral Tapia syndrome was caused by a vertebral artery dissection, which usually occurs as the result of an expanding hematoma in the vessel wall following rupture of the intimal layer of the vessel. A vertebral artery dissection can occur spontaneously or as a result of trauma. Different types of vertebral artery dissection can result in arterial stenosis, occlusion, or pseudoaneurysm. Some vertebral artery dissections have induced a brainstem infarction, and some have been asymptomatic. One particular type--a subadventitial dissection--tends to cause dissecting aneurysms of the vertebral artery, which may compress adjacent neurologic structures. (7)


An understanding of the different anatomic features in this area may explain the development of Tapia syndrome in our patient. The vagus nerve leaves the skull base through the jugular foramen and descends with the accessory nerve. Just before the vagus nerve leaves the cranium, it exhibits an enlargement known as the superior vagal ganglion. After exiting the jugular foramen, the vagus nerve forms another swelling, called the inferior vagal (nodose) ganglion. Then it descends through the neck within the carotid sheath. Meanwhile, the hypoglossal nerve runs medial to the IXth, Xth, and XIth cranial nerves after its exit from the cranium. The hypoglossal nerve enters the paravertebral spatium and descends to the anterior triangle of the neck. It lies close to the posterior surface of the inferior vagal ganglion. Also at this level are some anastomotic branches and some connective tissue bonding between the Xth and XIIth cranial nerves. The hypoglossal nerve then crosses the vagus nerve and continues its route medially until it reaches the tongue.

Tapia syndrome is an extremely localized lesion where the vagus and hypoglossal nerves cross. (8) In view of the close anatomic relationship of these two nerves to the vertebral artery, we can assume that the Tapia syndrome in our patient was caused by compression from the vertebral artery dissection.

In the management of a vertebral artery dissection, it is recommended that MRI and magnetic resonance angiography (MRA) be performed as soon as possible. A marked improvement has been observed as early as 1 week after a dissection has occurred. The best time to obtain a control angiogram is open to debate because the natural history of vertebral artery dissections remains poorly documented; however, some authors have suggested that it could be obtained around the third month after diagnosis. For angiographic follow-up, MRA is sufficient to demonstrate the normalization of a vessel's diameter and configuration. Cross-sectional MRIs with fat-suppressed T1 and T2 weighting are also helpful for assessing normalization of the vessel wall.

Anticoagulants and antiplatelet agents have been used in patients with vertebral artery dissection to prevent secondarythrombotic and embolic complications. (9) Studies have indicated that anticoagulants are not harmful in this condition, although no conclusions can be drawn from comparisons of nonrandomized treatment groups. The long-term outcome of patients with an extracranial vertebral dissection appears to be favorable. (10)


(1.) Tapia AG. Un nouveau syndrome; quelques cas d'hemiplegie du larynx et de la langue avec ou sans paralysie du sterno-cleido-mastoidien et du trapeze. Arch Int Laryngol Otol Rhinol 1906;22:780-5.

(2.) Krasnianski M, Neudecker S, Schluter A, et al. Central Tapia's syndrome ("matador's disease") caused by metastatic hemangiosarcoma. Neurology 2003;61 (6):868-9.

(3.) Haerer AF. DeJong's The Neurologic Examination. 5th ed. Philadelphia: Lippincott-Raven; 1992.

(4.) Ruhrmann G. Congenital right-sided vagus and hypoglossal nerve paralysis (Tapia syndrome) as the cause of congenital stridor [in German]. Z Kinderheilkd 1963;88:22-6.

(5.) Milhan Guevara J, Royo Lopez J, Pascual Millan LF, et al. Idiopathic associated paralysis of the Xth and XIIth cranial nerves [in Spanish]. An Otorrinolaringol Ibero Am 1993;20(1):61-4.

(6.) Yavuzer R, Basterzi Y, Ozkose Z, et al. Tapia's syndrome following septorhinoplasty. Aesthetic Plast Surg 2004;28(4):208-11.

(7.) Caplan LR. Vertebrobasilar occlusive disease. In: Barnett H, ed. Stroke: Pathophysiology, Diagnosis, and Management. Vol. 1. London: Churchill Livingstone; 1986:549-619.

(8.) Gelmers HJ. Tapia's syndrome after thoracotomy. Arch Otolaryngol 1983;109(9):622-3.

(9.) Hart RG, Easton JD. Dissections of cervical and cerebral arteries. Neurol Clin 1983;1(1):155-82.

(10.) Mas JL, Bousser MG, Hasboun D, Laplane D. Extracranial vertebral artery dissections: A review of 13 cases. Stroke 1987;18(6): 1037-47.

Mutlaq Al-Sihan Jr., MD; Martin Schumacher, MD; Erwin Lohle, MD

From the Department of Otorhinolaryngology, Freiburg University Hospital, Baden-Wurttemberg, Germany.

Corresponding author: Dr. Mutlaq Al-Sihan, Denglerstr. 78, 53173 Bonn, Germany. Email:
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Article Details
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Author:Sihan, Mutlaq Al-, Jr.; Schumacher, Martin; Lohle, Erwin
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:4EUGE
Date:Jul 1, 2011
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