Short communication Acyclovir responsive brain stem disease after the Ramsay Hunt syndrome Shuching Hu a , Melanie Walker a , Todd Czartoski a , Alan Cheng b , Bagher Forghani c , Donald H. Gilden d , Gwenn A. Garden a, * a Department of Neurology, University of Washington, Box 356465, Seattle, WA 98195, USA b Department of Otolaryngology/Head and Neck Surgery, University of Washington, Seattle, WA, USA c Viral and Rickettsial Disease Laboratory, Division of Communicable Disease Control, California Department of Health Services, Richmond, CA, USA d Departments of Neurology and Microbiology, University of Colorado Health Sciences Center, Denver, CO, USA Received 30 May 2003; received in revised form 22 August 2003; accepted 25 August 2003 Abstract We report an immunocompetent patient with the Ramsay Hunt syndrome (RHS) followed days later by brainstem disease. Extensive virological studies proved that varicella zoster virus (VZV) was the causative agent. Treatment with intravenous acyclovir resulted in prompt resolution of all neurological deficits except peripheral facial palsy. This case demonstrates that after geniculate zoster, brainstem disease may develop even in an immunocompetent individual and effective antiviral therapy can be curative. D 2003 Elsevier B.V. All rights reserved. Keywords: Varicella; Ramsay-hunt syndrome; Polymerase chain reaction; Cerebellum; Brain stem 1. Introduction The Ramsay Hunt syndrome (RHS) is characterized by unilateral peripheral facial palsy, hearing loss and zoster oticus. RHS is a common cause of atraumatic peripheral facial paralysis and is now known to be caused by reacti- vation of varicella zoster virus (VZV) from the geniculate ganglion [1]. VZV migrates transaxonally and may enter the brainstem via cranial nerves or travel via sensory afferent fibers to posterior circulation vessels [2,3], suggesting two possible mechanisms by which brainstem disease may develop after RHS. While invasion of brain parenchyma and cerebral arteries by VZV is more common in immuno- compromised patients [4], VZV vasculopathy can also develop in immunocompetent individuals [5]. Here, we present clinical, radiological and virological proof of RHS complicated by brainstem disease due to VZV in an other- wise healthy middle-aged woman. 2. Case report A 49-year-old woman developed right-sided facial numbness and weakness with ipsilateral hearing loss. Three days later, she was treated with oral acyclovir, 400 mg, five times daily, and prednisone, 40 mg daily. Over the next 72 h, she developed right temporal headache, dizziness, in- creasing right peripheral facial weakness, unsteady gait, mild right arm dysesthesias and right-sided weakness and became confused. Vesicles were present in the right external ear, but no vesicles were observed on the palate. Neurolog- ical examination revealed right-sided ptosis, skew deviation in primary gaze, horizontal gaze-evoked and upgaze nys- tagmus, loss of sensation on the right face, right peripheral facial weakness and hearing loss, right arm dysmetria, a right hemiparesis, decreased sensation in the right arm, increased deep tendon reflexes, greater on the right, and a right extensor plantar response. There were 10,600 white blood cells per cubic millime- ter. She was HIV-negative, but serological analysis revealed antibodies to hepatitis B and C viruses; liver function tests were normal. Audiometry revealed 20-dB hearing loss on the right. Brain magnetic resonance imaging (MRI) showed small areas of gadolinium enhancement in the area of the superior cerebellar peduncles (Fig. 1). There were no MRI abnormalities observed on Flair or diffusion weighted sequences. Magnetic resonance angiography (MRA) and cervical spine MRI were normal. The cerebrospinal fluid (CSF) contained 71 nucleated cells, predominantly lympho- cytes; the CSF protein was 59 mg/dl. Polymerase chain reaction (PCR) analysis of the CSF revealed 6800 copies/ml 0022-510X/$ - see front matter D 2003 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2003.08.011 * Corresponding author. Tel.: +1-206-616-9402; fax: +1-206-685-8100. E-mail address: gagarden@u.washington.edu (G.A. Garden). www.elsevier.com/locate/jns Journal of the Neurological Sciences 217 (2004) 111 –113