An Atypical Presentation of Varicella Zoster (VZV) Vasculopathy

Abstract

A 65-year-old female patient with history of hypertension presented with a two-day history of generalized
weakness, incoordination of bilateral limb movements,
and severe headaches for a week. She noted the day
prior to the onset of weakness that she had to take her
antihypertensive for an abnormally elevated blood pressure, and she awoke with weakness the next morning.
On admission the patient was nontoxic appearing and all
vital signs were within normal limits. Her mental status
and cranial nerve examination were normal. She was
noted to have 4/5 strength on the right upper and lower
extremities with diminished patchy sensation on the
right and without cerebellar abnormality or rash. Head
and neck computerized tomography angiography was
positive for diffuse narrowing in bilateral anterior and
posterior circulations [Figure 1(A) and (B)]. Her magnetic resonance imaging of the brain was positive for
bilateral watershed infarcts [see Figure 1(C) and (D)]. At
that point, reversible cerebral vasoconstriction syndrome
(RCVS) was our working diagnosis given the new onset
severe headache and diffuse severe vasospasm. Cerebrospinal fluid (CSF) studies suggestive of bacterial/fungal
meningitis with 7050 of red cells (nontraumatic), 630 of
white cells (73% neutrophil dominant), glucose 19
mg/dl, and protein 260 mg/dl but only the Varicella zoster virus (VZV) polymerase chain reaction was positive.
The patient was started on acyclovir with improvement
of her symptoms. At three months follow-up, the patient
remained asymptomatic and MRA head showed interval
improvement of the vasculopathy. A nontoxic appearance with subtle or no signs of infection might be falsely
reassuring for patients presenting with CNS vasculopathy, and RCVS can be diagnosed prematurely because
true diagnosis of RCVS requires resolution of symptoms
on interval/repeat vessel imaging 1–3 months later and