Update: Vernet’s syndrome

Jugular foramen syndrome, or Vernet’s syndrome is characterized by the paresis of 9th–11th (with or without 12th) cranial nerves together.

In contrast to the majority of classic brainstem syndromes, the interpretation of Schmidt’s syndrome (ipsilateral palsy of the IX, X, XI, and XII cranial nerves with contralateral hemiparesis) and Vernet’s syndrome (ipsilateral palsy of the IX, X, and XI nerves with contralateral hemiparesis) is controversial. They are sometimes addressed as crossed brainstem syndromes but also as syndromes due to multiple cranial nerve lesions without contralateral hemiparesis. In this study, the historic descriptions and recent publications about Schmidt’s and Vernet’s syndromes were reviewed and critically analysed. We conclude that historic descriptions and later publications describe exclusively patients with extracerebral lesions of multiple cranial nerves. “Central” syndromes of Schmidt and Vernet caused by brainstem lesion appear not to exist. An extremely extensive lesion explaining these hypothetical unilateral brainstem syndromes is theoretically possible but, however, was apparently never observed in any of the known unilateral brainstem diseases 1).

Symptoms

Symptoms of this syndrome are consequences of this paresis. As such, in an affected patient, you may find:

dysphonia/hoarseness

soft palate dropping

deviation of the uvula towards the normal side

dysphagia

loss of sensory function from the posterior 1/3 of the tongue

decrease in the parotid gland secretion

loss of gag reflex

sternocleidomastoid and trapezius muscles paresis.

Etiology

A variety of neoplasms, vascular insults, infections, and trauma have been reported to cause JFS 2).

The causes of Vernet syndrome are primary tumors such as Glomus jugulare tumors (most frequently), meningioma, vestibular schwannoma, cerebellopontine angle metastases, inflammation such as meningitis and malignant otitis externa, and sarcoidosis, Guillain-Barre syndrome 3).

Trauma 4) 5).

Cholesteatoma (very rare) 6).

Obstruction of the jugular foramen due to bone diseases 7).

Varicella-zoster virus 8).

Giant cell arteritis 9) 10).

Internal jugular vein thrombosis 11).

After carotid endarterectomy 12).

Large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media 13).

Systemic erythematous lupus 14).

1) Krasnianski M, Neudecker S, Zierz S. [The Schmidt and Vernet classical syndrome. Alternating brain stem syndromes that do not exist?]. Nervenarzt. 2003 Dec;74(12):1150-4. Review. German. PubMed PMID: 14647918.
2) Robbins KT, Fenton RS. Jugular foramen syndrome. J Otolaryngol. 1980 Dec;9(6):505-16. PubMed PMID: 7206037.
3) Ha SW, Kim JK, Kang SJ, Kim MJ, Yoo BG, Kim KS, et al. A case of Vernet’s syndrome caused by non-specific focal inflammation of the neck. J Korean Soc Clin Neurophysiol. 2007;9:81–84.
4) , 5) Kim HS, Ko K. Penetrating trauma of the posterior fossa resulting in Vernet’s syndrome and internuclear ophthalmoplegia. J Trauma. 1996 Apr;40(4):647-9. PubMed PMID: 8614050.
6) Erol FS, Kaplan M, Kavakli A, Ozveren MF. Jugular foramen syndrome caused by choleastatoma. Clin Neurol Neurosurg. 2005 Jun;107(4):342-6. PubMed PMID: 15885397.
7) Erol FS, Kaplan M, Kavakli A, Ozveren MF.Jugular foramen syndrome caused by choleastatoma. Clin Neurol Neurosurg. 2005 Jun;107(4):342-6.
8) Jo YR, Chung CW, Lee JS, Park HJ. Vernet syndrome by varicella-zoster virus. Ann Rehabil Med. 2013 Jun;37(3):449-52. doi: 10.5535/arm.2013.37.3.449. PubMed PMID: 23869347; PubMed Central PMCID: PMC3713306.
9) Jeret JS. Giant cell arteritis and Vernet’s syndrome. Neurology. 1999 Feb;52(3):677. PubMed PMID: 10025824.
10) Cherin P, De Gennes C, Bletry O, Lamas A, Launay M, Dubs A, Godeau P. Ischemic Vernet’s syndrome in giant cell arteritis: first two cases. Am J Med. 1992 Sep;93(3):349-52. PubMed PMID: 1524092.
11) Shima K, Iwasa K, Yoshita M, Yamada M. Vernet’s syndrome induced by internal jugular vein thrombosis. J Neurol Neurosurg Psychiatry. 2016 Nov;87(11):1252-1253. doi: 10.1136/jnnp-2015-311665. PubMed PMID: 26354943.
12) Tamaki T, Node Y, Saitoum N, Saigusa H, Yamazaki M, Morita A. Vernet’s syndrome after carotid endarterectomy. Perspect Vasc Surg Endovasc Ther. 2013 Dec;25(3-4):65-8. doi: 10.1177/1531003514525476. PubMed PMID: 24625858.
13) Amano M, Ishikawa E, Kujiraoka Y, Watanabe S, Ashizawa K, Oguni E, Saito A, Nakai Y, Ikeda H, Abe T, Uekusa Y, Matsumura A. Vernet’s syndrome caused by large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media–case report. Neurol Med Chir (Tokyo). 2010 Jan;50(1):45-8. PubMed PMID: 20098025.
14) Leache Pueyo JJ, Campos del Alamo MA, Gil Paraíso P, Ortiz García A. [Vernet’s syndrome as an early manifestation of systemic erythematous lupus]. An Otorrinolaringol Ibero Am. 1997;24(2):135-41. Spanish. PubMed PMID: 9199109.

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