Determine whether duration of treatment and maintenance therapy lead to clinical improvement or remission. Determine if imaging and biological data can measure treatment response.

Varicella Zoster Virus (VZV) vasculopathy secondary to virus reactivation is a rare entity with no clear guidelines on treatment and follow-up.

We retrospectively reviewed the charts of 17 consecutive patients with VZV induced CNS vasculopathy, from January 2010 until September 2018, focusing on notes from office visits, re-admissions and repeated imaging.

The average follow-up duration was 3.4 years (s=2.11). All 17 patients were diagnosed with VZV vasculopathy of the CNS based on angiographic findings in the setting of CNS infection with VZV (Positive VZV IgG or VZV PCR in the CSF). Fifteen patients had ischemic strokes and the remaining two had meningo-encephalitis. All 17 patients were treated with IV Acyclovir with an average treatment duration of 29.2 days (s=13.13). Clinical resolution and remission were associated with starting maintenance treatment after therapeutic Acyclovir (p=0.027 and p=0.046 respectively) but not with a prolonged duration of treatment (3 weeks and more). All three patients who had subsequent strokes were not on maintenance therapy. Improvement and resolution vessel wall enhancement seen on high resolution MRI vessel wall imaging were not correlated to clinical improvement or remission. Improved stenosis only occurred in 2 out of the 11 patients on repeat angiography (18%). Flow velocity improved in 83% (5 out of 6) of patients on serial TCDs, 4 out of whom (80%) improved clinically and had no recurrence. Persistent CSF pleocytosis and elevated protein level were not clinically significant. VZV IgG was found in 3 patients (38%), two out of whom had clinical recurrence of symptoms.

Maintenance treatment with low dose oral acyclovir is associated with clinical improvement and remission. Persistent VZV IgG in CSF could be associated with risk of recurrence.