Jugular Forman syndrome as described by Vernet

To the Editor,

We read with interest the case by Adjepong et al. of jugular foramen syndrome (JFS) caused by reactivation of varicella zoster (VZ) infection in the absence of a rash (zoster sin herpete). ¹ The frequency of zoster sin herpete involving the jugular foramen is presumably underestimated because clinicians frequently do not consider this diagnosis in the absence of a rash.^2,3 Lewis proposed that zoster sin herpete should be considered in cases where there is “unilateral paralysis of the soft palate, pharyngeal muscles, or vocal cords of obscure origin, especially when associated with otalgia or with an inflammatory reaction in, or around, the entrance to the larynx (p. 421).” ⁴ We reviewed the index case reported by Maurice Vernet (1887–1974) in 1917 to gain insight into the characteristic and etiology of this phenomenon. ⁵

Vernet reported on an adult male who presents with an enlarged palpable solitary lymph node in the carotid region behind the ascending portion of the maxilla and a 2.3 cm mildly exudative erosion with firm borders involving the upper lip. The erosion was present for 10 days, preceded by neck swelling, and followed by dysphonia and dysphagia. Vernet identified on examination paralysis of the right internal branch of the spinal accessory nerve (hemiparalysis of the veil of the larynx), paralysis of the right glossopharyngeal nerves (disorder of taste of the posterior aspect of the tongue, and paralysis of the superior constrictor of the pharynx) and paralysis of the right vagus nerve (disorders involving sensation of the veil, pharynx, larynx, and tachycardia). On laryngoscopy, he found that “the right arytenoid and right vocal cord remain stationary. They are excavated, red, and in phonation do not move synchronously with the other vocal cord (p. 79).” ⁵ Vernet recognized a complete and incomplete form of this syndrome depending upon whether the external branch of the spinal accessory nerve is involved. Because of the external branches anatomical relationship, most cases present in the incomplete form, sparing the sternocleidomastoid and trapezius muscle. ⁵

As to the cause of his symptoms:

In the preceding case, the evolution of this sensory disorder is in striking parallelism with the state of improvement of the lymph node, which appears to me to be the cause of compression at the posterior jugular foramen (p. 81). ⁵

Anatomically, the lymphatics of the upper lip drain into the deep (internal) jugular lymph nodes and Krause nodes in the jugular foramen. An aneurysm, neoplasm, trauma, internal jugular thrombosis, or an enlarged lymph node may compress the structures within the jugular foramen causing this syndrome. We are unaware of any case reporting the association between herpes simplex viral infection and JFS. Vernet postulated that the clinical symptoms were caused by compression of the cranial nerves in the jugular foramen by an enlarged lymph node. Herpes zoster and/or herpes simplex labialis may have accounted for this finding. Alternatively, the inflammatory reaction within the larynx, as described by Lewis, is consistent with zoster sin herpete and JFS. ⁴