Ramsay-Hunt Syndrome – updated

Thanks to Adam Tabbaa and Megan Lockwood for presenting a 40 y/o healthy man who presented with multiple cranial neuropathies and ear vesicles concerning for Ramsey Hunt Syndrome
What is Ramsey Hunt syndrome?
  • classically, Facial nerve paralysis + Zoster oticus (zoster lesions in the ear) +/- hearing loss
  • Pathophysiologically – Zoster reactivation in the geniculate ganglion.
This case hinged upon identifying the patient’s cranial neuropathies and trying to identify an atomic location whether those cranial nerves ran together. So here’s a quick refresher on the facial nerve and the geniculate ganglion.
facial nerve 2
  • The facial nerve exits the brain stem, courses through the temporal bone, then forms a knee-shaped ganglion at its exit

facial nerve 1.png

  • As this less detailed image shows. The facial nerve runs *extremely close* to the ear when exits the temporal bone. So when VZV reactivates in the geniculate ganglion, it travels transaxonally through the nerves to skin around the ear, causing vesicles there.
    • Most VZV reactivation in the face is in the trigeminal nerve, which is why we are so accustomed to seeing zoster in a V1, V2, or V3 distribution. Turns out it can affect whatever nerve it feels like.
  • When the facial nerve runs next to the ear, it also runs in close quarters with the vestibulocochlear nerve. So Ramsey-Hunt can also include deafness or vestibular symptoms and for this reason is considered an ENT emergency.
  • Ramsey Hunt is usually managed with acyclovir + steroids. A cochrane review showed no evidence of benefit for acyclovir but the overall evidence quality was poor.
  • Ramsey Hunt, a peripheral nerve manifestation of zoster, should be distinguished from CNS zoster which can be devastating. Zoster encephalitis, if it affects the brain stem, can also pick off specific cranial nerves. Because our patient also had deficits in non-adjacent cranial nerves (IX and X), we suspected brainstem disease.
Multiple Cranial Neuropathies
  • So interesting! So serious. More pearls about them here: https://ucsfmed.wordpress.com/2016/10/28/zsfg-neuro-report-multiple-cranial-neuropathies-spotlight-on-the-cavernous-sinus/
Sources
Kleinschmidt-DeMasters BK1, Gilden DH. Varicella-Zoster virus infections of the nervous system: clinical and pathologic correlates. Arch Pathol Lab Med. 2001 Jun;125(6):770-80.
Whitley RJ1.A 70-year-old woman with shingles: review of herpes zoster. JAMA. 2009 Jul 1;302(1):73-80. doi: 10.1001/jama.2009.822. Epub 2009 Jun 2.
Rebecca Miller-Kuhlmann, MD. My very favorite neurologist who texted me many pictures of cranial nerves.
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