Abstract

A 62-year-old lady presented with thunderclap onset, severe, bi-occipital headache with associated motion sensitivity and vomiting on a background of gradually worsening, constant bi-temporal throbbing pain over a two year period. CT brain demonstrated acute superior frontocortical subarachnoid blood but CT venogram, angiogram and brain MRI were all normal. Peripheral autoimmune and inflammatory serology was unremarkable, as were initial results from CSF analysis. Cerebral vasculitis was suspected and the patient underwent formal catheter angiography. This found multifocal irregularity of the intracranial medium and small sized vessels in addition to both superficial temporal arteries, the latter revealing intimal thickening and elastic lamina disruption on biopsy. Despite no previous shingles, viral polymerase chain reaction on CSF returned positive for Varicella zoster DNA prompting commencement of intravenous acyclovir and methylprednisolone. Within 24 hours, she developed expressive dysphasia and left-sided hemiparesis. A second CT brain showed fresh haemorrhage and low densities within the cerebellar hemispheres with MRI now revealing a flurry of cortical and subcortical infarctions bilaterally particularly within occipital and parietal lobes. Although no new lesions have occurred, she continues to experience visuoperceptual difficulties including simultagnosia. This case highlights several manifestations of intracranial Varicella vasculopathy in the absence of previous or current shingles.