Vernet's syndrome [Neurosurgery Wiki]

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====== 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
((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.
)).

===== Symptoms =====

Unilateral paralysis of the [[palate]], [[vocal cord]]s, [[sternocleidomastoid]], [[trapezius]], with
loss of taste in the posterior 1/3 [[tongue]], [[anesthesia]] of the [[soft palate]], [[larynx]] and [[pharynx]].
----

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
((Robbins KT, Fenton RS. Jugular foramen syndrome. J Otolaryngol. 1980
Dec;9(6):505-16. PubMed PMID: 7206037.
)).
----
The causes of Vernet syndrome are primary tumors such as [[Glomus jugulare tumor]]s (most frequently), [[meningioma]], [[vestibular schwannoma]], [[cerebellopontine angle metastases]], inflammation such as meningitis and malignant otitis externa, and sarcoidosis, Guillain-Barre syndrome
((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.)).

Trauma 
((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.
))
((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.
)).

[[Cholesteatoma]] (very rare)
((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.
)).

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

Varicella-zoster virus
((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.
)).

Giant cell arteritis 
((Jeret JS. Giant cell arteritis and Vernet's syndrome. Neurology. 1999
Feb;52(3):677. PubMed PMID: 10025824.))
((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.
)).

Internal jugular vein thrombosis
((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.
)).

After [[carotid endarterectomy]]
((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.
)).

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

Systemic erythematous lupus
((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.
)).

===== Case reports =====

A case of a giant cervical osteophyte resulting in compression of the jugular foramen. A 74-year-old man who presented with progressive dysphagia and dysarthria was found to have right-sided tongue deviation, left palatal droop, and hypophonia. His dysphagia had progressed to the point that he had lost 25 kg over a 4-month period, necessitating a gastrostomy to maintain adequate nutrition. He underwent extensive workup for his dysphagia with several normal radiographic studies. Ultimately, CT scanning and postcontrast MRI revealed a posterior osteophyte arising from the C1-2 joint space and projecting into the right jugular foramen. This resulted in a jugular foramen syndrome in addition to delayed filling of the patient's right internal jugular vein distal to the osteophyte. Although rare, a posterior cervical osteophyte should be considered in cases of jugular foramen syndrome.

===== References =====