Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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November 9, 2015
Ramsay Hunt Syndrome
- Background:
- First described by James Ramsay Hunt in 1907, and caused by reactivation of varicella zoster virus which lies latent in sensory root ganglion for years in patients who had chickenpox earlier
- It is a rare condition that accounts for 12% of all facial nerve paralyses, and it is more common in older patients, usually over 60 years. The incidence rates in men and women are quite similar.
- Clinical Presentation: Patients with Ramsay Hunt syndrome typically present with fever, malaise, tinnitus, vertigo, nystagmus, hearing loss, peripheral facial nerve palsy, and a painful vesicular rash with burning blisters around the ear and on the face, mouth, and sometimes even the tongue.
- Key Diagnostic Features:
- Laboratory serologic diagnostic tests show positive ELISA, IGM, and PCR for varicella herpes zoster virus.
- On MRI examination, we can depict abnormal enhancement of the canalicular segment (which should never enhance) or asymmetric enhancement of the geniculate ganglia labyrinthine, tympanic, and/or mastoid compared with the normal side.
- Vestibular and cochlear nerves can also show abnormal enhancement, as well as the membranous labyrinth structures.
- In some cases there is enhancement on the pontine colliculus facialis on the affected side.
- Volumetric FLAIR acquisitions can show high signal intensities on the involved nerves.
- DDx:
- Bell palsy
- Otites externa and interna
- Stroke
- Migraine headache
- Treatment: Antiviral agents (acyclovir, valacyclovir, famciclovir), corticosteroids, and pain management. Antibiotics are used for treating secondary bacterial infection.
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