Ramsay Hunt syndrome type 2 (RHS; herpes zoster oticus) is a rare and specific type of shingles which affects the geniculate ganglion of the facial nerve. After the original chickenpox infection, the varicella zoster virus remains dormant in the nerve cells and is reactivated causing the syndrome. In less than 1% of cases of shingles the facial nerve is affected.
Justin Bieber shared this photo on Instagram when announcing that he had Ramsey Hunt syndrome
The syndrome gained some public notice in June 2022 when the entertainer, Justin Bieber cancelled concerts and announced that he had Ramsay Hunt syndrome. It was notable as this did raise a lot of awareness about the condition.
Clinically there is an inability to move many of the facial muscles on one side, intense pain in the ear on that side, loss of taste on the tongue, dry eyes and mouth, and a vesicular rash in the ear canal and on the tongue. Sometimes there is also tinnitus and vertigo. There is a wide variability in the presenting symptoms.
Differential diagnosis: Bells Palsy; stroke (CVA); acoustic neuroma; trigeminal neuralgia.
Initial treatment is usually with a corticosteroid (eg prednisone) and an antiviral drug (eg acyclovir, valacyclovir, famciclovir). The later the treatment is started, the more likely there is some permanent disability.
Up to 70% recover most functionality. Common long term affects include a permanent paralysis of the facial muscles; neuropathic pain and post-herpetic neuralgia; weakness in the affected facial muscles; hearing loss; difficulty closing the eyelid
There are other types of Ramsay Hunt syndrome which are not related to each other other than they were first described by the neurologist James Ramsay Hunt (1872–1937):
Ramsay Hunt syndrome type 1 (Ramsay Hunt cerebellar syndrome) – this is a rare form of cerebellar degeneration that manifests with epilepsy, progressive ataxia, tremors, and dementia; first described in 1921.
Ramsay Hunt syndrome type 2 (herpes zoster oticus) – this is the reactivation of herpes zoster discussed above; first described in 1907.
Ramsay Hunt syndrome type 3 (Hunt’s disease; artisan’s palsyis) – this is an occupation related neuropathy of the deep palmar branch of the ulnar nerve.
Commentary:
- When Justin Bieber announced that he had Ramsay Hunt syndrome, the anti-vaccination cult widely shared memes and comments that it was caused by the COVID-19 vaccinations. When asked if that was actually the case, then what caused Ramsay Hunt syndrome before the vaccinations were available, they could not offer an answer.
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