UCONN Emergency Medicine Interest Group

EM Images #9

In IMAGES on December 4, 2011 at 7:00 AM

Syndicated from the UCONN EM Residency website; credit to Dr. Suozzi for images and text.

Just a short hint for this case: this is from a patient that Dr. Suozzi saw in Uganda.

What is the diagnosis?

James Ramsay Hunt Syndrome II.

From whonamed it.com: Unilateral herpes zoster infection of the geniculate ganglion (respectively n. Intermedius) with lesions of the external ear and oral mucosa. The symptoms include facial paralysis, severe pain in the ear with a bloody serous discharge due to vesicles on the tympanic membrane. The face is paralysed on the affected side, and there is loss of sense of taste in the anterior two-thirds of the tongue on the affected side. Other features include decreased salivation, hoarseness, tinnitus, decreased lacrimation, hearing disorder, and vertigo. Rare in children but seen commonly in the elderly population, with equal distribution between men and women. It is not contagious.

It is a common complication of shingles. Shingles is an infection caused by the varicella-zoster virus, which is the virus that causes chickenpox. Shingles occurs in people who have had chickenpox and represents a reactivation of the dormant varicella-zoster virus. Ramsay Hunt syndrome II is caused by the spread of the varicella-zoster virus to facial nerves.

Ramsay Hunt in 1907 first described this combination of symptoms as a syndrome. He separated between 4 forms: 1) herpes zoster without neuralgic signs; 2) herpes zoster oticus with facial paresis; 3) herpes zoster oticus with facial paresis and hearing symptoms 4) Ramsay Hunt syndrome – auricular herpes zoster syndrome.

NB:  Zoster Vaccine can protect against Ramsay Hunt Syndome:
Licensed zoster vaccine is a lyophilized preparation of a live, attenuated strain of VZV, the same strain used in the varicella vaccines. However, its minimum potency is at least 14-times the potency of single-antigen varicella vaccine. In a large clinical trial, zoster vaccine was partially efficacious at preventing zoster. It also was partially efficacious at reducing the severity and duration of pain and at preventing PHN among those developing zoster. (beginning of monograph) (<http://www.cdc.gov/mmwr/preview/mmwrhtml/rr57e0515a1.htm&gt;)

Biography of James Ramsay Hunt: From wikipedia AND whonamedit.com (same bio, which is interesting, to say the least): James Ramsay Hunt (born 1872 in Philadelphia; died July 22, 1937 in Katonah, New York) was an American neurologist. He graduated M.D. from the University of Pennsylvania School of Medicine in 1893. He then studied in Paris, Vienna, and Berlin and returned to practise neurology in New York, working at Cornell University Medical School from 1900 – 1910 with Charles Loomis Dana. He did major research on the anatomy and disorders of the corpus striatum and the extrapyramidal system. He was consulting physician at several New York hospitals and was appointed professor of neurology at Columbia University College of Physicians and Surgeons, New York, in 1924.


References:

1. http://en.wikipedia.org/wiki/James_Ramsay_Hunt

2. http://www.whonamedit.com/doctor.cfm/2009.html

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