Crouch Andrew E., Hohman Marc H., Moody Minhee P., Andaloro Claudio
Arrowhead Regional Medical Center
Uniformed Services University/Madigan Army Medical Center
Ramsay Hunt syndrome, also known as herpes zoster oticus or geniculate ganglion herpes zoster, is a late complication of varicella-zoster virus (VZV) infection, resulting in inflammation of the geniculate ganglion of cranial nerve VII. The syndrome is named after James Ramsay Hunt (1872-1937), an American neurologist and Army officer in World War I who described three different syndromes, the most famous of which is the second, which is discussed herein as "Ramsay Hunt syndrome." Early stages of VZV infection cause fever and diffuse vesicular rash, a condition that is commonly referred to as chickenpox. After the initial infection, the virus will often remain dormant in the body. Subsequent reactivation of the virus causes a "zoster" or "herpes zoster" phenomenon. This syndrome consists of pain and a vesicular rash along the involved nerve's distribution, typically corresponding to a single dermatome. The distribution and associated symptoms depend on the nerve involved. Less than 1% of zoster cases involve the facial nerve and result in Ramsay Hunt syndrome. Although the classic triad of Ramsay Hunt syndrome is ipsilateral facial paralysis, otalgia, and a vesicular rash, there is significant variability in clinical presentation, with some patients demonstrating facial paralysis before the rash or, sometimes, no rash at all. In the latter, the patient's chief complaints are severe ear pain and facial weakness; this variant is known as zoster sine herpete and can be very difficult to clinically distinguish from Bell's palsy. Zoster sine herpete has been reported to comprise up to 30% of Ramsay Hunt cases. If a rash is present, it may be frankly vesicular or maculopapular, and can involve the affected side of the face, scalp, palate, and tongue. Additional symptoms that may be reported include a change in taste sensation, dry eye, tearing, hyperacusis, nasal obstruction, and dysarthria. Hearing loss, tinnitus, and vertigo can be seen with involvement of the vestibulocochlear nerve, and hoarseness or aspiration may indicate involvement of the vagus nerve.
拉姆齐·亨特综合征，也称为耳带状疱疹或膝状神经节带状疱疹，是水痘-带状疱疹病毒（VZV）感染的晚期并发症，导致颅神经VII的膝状神经节发炎。该综合征以詹姆斯·拉姆齐·亨特（1872 - 1937）命名，他是一位美国神经学家，也是第一次世界大战中的军官，他描述了三种不同的综合征，其中最著名的是第二种，本文将其作为“拉姆齐·亨特综合征”进行讨论。VZV感染的早期阶段会引起发热和弥漫性水疱疹，这种情况通常被称为水痘。初次感染后，病毒通常会在体内保持潜伏状态。病毒随后的重新激活会导致“带状疱疹”现象。该综合征包括沿受累神经分布的疼痛和水疱疹，通常对应于单个皮节。分布和相关症状取决于受累神经。不到1%的带状疱疹病例累及面神经并导致拉姆齐·亨特综合征。虽然拉姆齐·亨特综合征的典型三联征是同侧面部麻痹、耳痛和水疱疹，但临床表现存在很大差异，一些患者在出疹前出现面部麻痹，有时根本没有皮疹。在后者中，患者的主要症状是严重耳痛和面部无力；这种变体被称为无疹性带状疱疹，在临床上很难与贝尔面瘫区分开来。据报道，无疹性带状疱疹占拉姆齐·亨特病例的30%。如果出现皮疹，可能是明显的水疱疹或斑丘疹，可累及面部、头皮、腭和舌的患侧。可能报告的其他症状包括味觉改变、干眼、流泪、听觉过敏、鼻塞和构音障碍。前庭蜗神经受累时可出现听力损失、耳鸣和眩晕，声音嘶哑或误吸可能表明迷走神经受累。
