Aetiology
Ramsay Hunt syndrome is caused by reactivation of latent varicella zoster virus (VZV), which is the same virus that causes chickenpox and shingles, and spreads to the facial nerve. VZV may remain dormant for decades in the geniculate ganglion (cranial nerve VII) or sensory ganglion of the face (cranial nerve V) following primary infection.[7] As adaptive immunity wanes, especially in times of physiological stress or immunocompromised state, the virus can be reactivated in the geniculate ganglion, or the trigeminal ganglion, causing facial paralysis, facial pain, and vesicular lesions in a dermatomal pattern.[6] Although it has been reported that people without prior VZV vaccination or known infection have been diagnosed with Ramsay Hunt syndrome, this is uncommon (0.5% to 1.0% of patients with herpes zoster reported no prior vaccination or known infection in one small population-based study of people aged <20 years).[8]
Pathophysiology
The pathophysiology underlying Ramsay Hunt syndrome is believed to be due to reactivation of VZV following earlier primary infection or VZV vaccination leading to inflammation and compression of the facial nerve, especially in the labyrinthine segment. This results in loss of axonal transmission and decreased nerve signal to the muscles of facial expression and sudden-onset facial palsy.[2][9] During primary VZV infection, the virus is carried down the axons to the areas of the skin innervated by the affected ganglion, causing local inflammation.
[Figure caption and citation for the preceding image starts]: Functional anatomy of the facial nerve. Proximally, the four cranial nerve nuclei involved in facial nerve functions are shown at the pontomedullary junction: the motor nucleus of VII, the nucleus of the solitary tract, the superior salivatory nucleus, and the spinal nucleus of V. Special visceral efferent motor fibres from the motor nucleus of VII (solid red line) exit the brainstem and travel through the internal acoustic meatus to enter the bony facial canal and exit through the stylomastoid foramen to supply facial muscles. In Ramsay Hunt syndrome, these fibres are affected as they pass through the geniculate ganglion, disrupting motor functions of the seventh cranial nerve. The solitary tract receives special visceral afferent taste fibres (solid blue line) emanating from the anterior two thirds of the tongue. These fibres travel with the chorda tympani through the petrotympanic fissure (not shown). The cell bodies of these special visceral afferent fibres are in the geniculate ganglion which is the site of varicella zoster virus (VZV) reactivation when vesicles erupt on the tongue. The fibres reach the brainstem via the nervus intermedius and can be affected by local inflammation as they pass the geniculate ganglion. Special visceral efferent parasympathetic fibres (thin dotted red line) to the lacrimal and salivary glands emanate from the superior salivatory nucleus, travel in the nervus intermedius, and branch at the geniculate ganglion into the greater petrosal and chorda tympani nerves. Decreased lacrimation may result from involvement of these fibres as they branch at the level of the geniculate ganglion. Special visceral efferent sympathetic fibres (thick dotted red line) emanate from the carotid plexus on the internal carotid artery and join the greater petrosal nerve as these structures pass through the foramen lacerum (not shown). The sympathetic fibres parallel the parasympathetic fibres as they supply the same areas. The spinal nucleus of V receives general somatic afferent fibres from the geniculate zone of the ear via the chorda tympani. Cell bodies of these neurons are located in the geniculate ganglia and are the site of VZV reactivation in classic Ramsay Hunt syndrome causing vesicular eruptions in geniculate zonesSweeney CJ et al. J Neurol Neurosurg Psychiatry. 2001 Aug;71(2):149-54; used with permission [Citation ends].
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