Ramsay Hunt syndrome

Case contributed by Ashesh Ishwarlal Ranchod

Presentation

Acute onset of left facial paralysis, vertigo, nausea, vomiting, and a vesicular rash involving the tongue, hard palate, and left external auditory canal and pinna.

Patient Data

Age: 45 years
Gender: Female
mri

There is focal enhancement of the canalicular left facial-vestibulocochlear nerve complex in comparison to the right. There is an asymmetric enhancement of the left geniculate ganglion.

There is an avid enhancement of the left external auditory canal and external ear/ pinna.

There is an incidental left type III anterior inferior cerebellar artery( AICA) loop.

Contrast-enhanced MRI brain is otherwise normal.

Note: sagittal T1 has suboptimal contrast resolution due to a technical problem.

Annotated MRI

Annotated image

High-resolution axial T2 Fiesta demonstrates an incidental left type III AICA loop.

Zoomed and annotated images demonstrate the enhancement of the left facial-vestibulocochlear nerve complex within the internal auditory canal(fundal tuft sign).

There is avid and asymmetric left external auditory canal and pinna enhancement.

Photographs

Photo

Image 1: loss of ipsilateral(left) eye closure-lagophthalmos.

Image 2: varicella-zoster vesicular rash predominantly left tongue.

Image 3: developing varicella-zoster vesicular rash and erythema involving the left external ear (left triangular fossa, concha cymba, concha cavum, antitragus and intertragic notch).

Image 4: normal contralateral right external ear.

The patient kindly gave permission to photograph her clinical features for teaching purposes.

Case Discussion

This patient presented with the typical clinical features of a left-sided Ramsay Hunt syndrome. She had an acute onset of severe vertigo, central nausea, vomiting, headache, with paresthesia of her tongue, hard palate and left external ear. On examination she had a typical left Bell's palsy clinically with facial asymmetry, she was unable to close her left eye, and a vesicular rash was identified on her hard palate, left tongue, left external auditory canal and left external ear/ pinna.

Ramsay Hunt syndrome is also known as herpes zoster oticus or shingles of the facial nerve, and due to varicella-zoster reactivation in the geniculate ganglion.

MRI brain confirmed focal canalicular enhancement at the apex of the left internal auditory canal, likely representing a combined enhancement of the facial-vestibulocochlear nerve complex(known as the fundal tuft sign). There is also asymmetric and prominent enhancement of the left geniculate ganglion, especially in comparison to the asymptomatic right. These features would fit with a left Bell's palsy.

There is an avid noticeable enhancement of the left external auditory canal and external ear/ pinna, an unusual MRI finding and comment, however attributable to the clinical presentation of the varicella-zoster vesicular rash of the external auditory canal and pinna.

There is an incidental finding of type III anterior inferior cerebellar arterial loop extending significantly into the left internal auditory canal. There was no prior history of vertigo, tinnitus or any hemifacial spasm. Clinically the acute presentation was fully suspected due to the varicella-zoster reactivation and suggested likely involvement of the left vestibulocochlear nerves too.

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