A Case of Ramsay Hunt Syndrome with Cranial Polyneuropathy
A 66-year-old female was referred to our clinic with left facial palsy, diplopia, and vertigo six days after the onset of left ear pain and headache. The patient underwent a severe emotional stress due to her sister's death at the time of onset of left ear pain. The clinical manifestations developed gradually over a few days. Physical examinations revealed vesicular eruptions with crusts on left auricle, left facial palsy, left abducens nerve palsy, and mild right beating spontaneous nystagmus. The degree of FNP was House-Brackmann (HB) grade V (). Diplopia was present in the primary position and was more prominent on attempted abduction of the left eye (). Audiogram showed sensorineural hearing loss on her left ear (). Vestibular function test was performed on the fourth hospital day when the patient no longer complained of dizziness, and videonystagmography showed no evidence of spontaneous nystagmus. The size and response to light were normal on both pupils. Except for cranial nerves VI, VII, and VIII, examination for other cranial nerves revealed intact function. Magnetic resonance imaging showed no sign of tumor, infection or other demyelinating diseases of the brain. Serology results for VZV IgG antibodies were positive, but negative for VZV IgM antibodies. The patient had no associated immunocompromised condition including immunosuppressant drug use or immunodeficiency syndrome. A diagnosis of RHS with CP was made based on the patient's characteristic presentation, and from a clinical point of view cranial nerves VI, VII, and VIII were found to be involved. Systemic administration of steroid and antiviral agents was started. Sixty mg of prednisolone was initially given to the patient and tapered orally for 14 days, and 3000 mg of acyclovir was administered intravenously for 7 days. No immediate or delayed side effects were observed during and after the treatment. Among symptoms, dizziness improved most rapidly within several days. During 3 weeks from the start of treatment a gradual improvement of the facial weakness and diplopia was observed. Four months after the treatment, FNP recovered to HB grade I and the abducens nerve palsy completely improved without diplopia ().