gms | German Medical Science

Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx. VZV (varicella zoster virus) infection/reactivation involving the geniculate ganglion of CN VII within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome. The primary infection is usually acquired early in life and remains latent until it is reactivated later, where symptoms emerge, usually in association with an immune deficiency or suppression.

Ramsay Hunt syndrome presenting with multiple cranial neuropathies is rare. Possible mechanisms for the RHS–related polyneuropathy include formation of an anatomical chain from the adjacent nerve ganglia; a direct perineural spread of the virus along anastomotic pathways, or from a vasculitis in which the virus spreads through the small branches of the infected carotid artery, middle meningeal artery, and ascending pharyngeal artery that supply blood to cranial nerves V, VII, IX, X, XI, and XII.

The aim of this paper is to present two clinical cases of Ramsay Hunt syndrome with atypical unilateral multicranial nerve involvement – V, VII, VIII, IX, X and XI CN.

The methods used included a full neurological examination, ENT examination, CBC, basic metabolic panel, CSF analysis, serological tests, EMG, EEG, CT and MRI brain imaging.

Both patients were treated successfully by a 14-day course of 5 x 800 mg p.o. acylcovir plus low-dosed corticosteroids.

In conclusion, a diagnosis of RHS should be considered in patients with unilateral multiple cranial nerve palsies, as early antiviral and steroid treatment

significantly improves the prognosis.

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