14 Harpes.indd

Ramsay Hunt syndrome, also known as herpes zoster oticus is usually caused by reactivation of preexisting varicella zoster virus in the geniculate ganglion. Here we report a case of herpes zoster oticus masquerading as lateral pontomedullary syndrome occurring during the pre-eruptive phase of chicken pox. The case is being reported for its rare and unusual clinical presentation. Neurology Asia 2011; 16(3) : 259 – 261 Address correspondence to: Prof. Parvaiz A Shah, H. No: 35, Momina-bad, Hyderpora Bye-pass (East), Srinagar, Kashmir, J & K, India 190014. e-mail: [email protected] INTRODUCTION Ramsay Hunt syndrome was fi rst described in 1907 by James Ramsay Hunt in a patient who had otalgia associated with mucocutaneous rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3. The syndrome comprises of an acute infranuclear facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx. Various clinical features reported with this syndrome include herpes auricularis, herpes facialis and herpes occipito-collar with facial palsy. Cranial nerves VIII, IX, V, and VI are less commonly involved and when they do get involved, they are affected in descending order of frequency as listed above. The infection gives rise to vesiculation and ulceration of the external ear and ipsilateral anterior two thirds of the tongue and soft palate with ipsilateral infranuclear facial palsy and geniculate ganglionopathy. Varicella zoster virus (VZV) infection causes two distinct clinical syndromes. Primary infection, also known as varicella or chickenpox, is a common erythematous disease characterized by a highly contagious generalized vesicular rash in a centripetal pattern. After chickenpox, VZV remains latent in the neurons of cranial nerves and dorsal root ganglia. Subsequent reactivation of latent VZV can result in localized vesicular rash, known as herpes zoster. VZV infection or reactivation involving the geniculate ganglion of the facial nerve within the temporal bone is the main pathophysiological mechanism of Ramsay Hunt syndrome. Ramsay Hunt syndrome is a rare complication of latent VZV infection. VZV has been detected by polymerase chain reaction (PCR) in the tear fl uid of patients with Bell’s palsy. Ramsay Hunt syndrome is rare below the age of 6 years and accounts for 16% of all causes of unilateral facial palsies in children and 18% of facial palsies in adults.