ARTICLE IN PRESS
CLINICAL COMMUNICATION TO THE EDITOR

Varicella-Zoster Virus Vasculopathy
Leading to Multi-focal Stroke in an
Immunocompromised Patient
To the Editor:
A 51-year-old man presented to the dermatology clinic with
a 1-week history of diffuse vesicular eruption in his trunk,
back, and face (Figure 1). He had been recently hospitalized
for a flare of his underlying autoimmune hemolytic anemia
and was receiving therapy with mycophenolate mofetil and
high-dose prednisone of 40 mg.
Tzanck smear and varicella zoster virus polymerase
chain reaction (PCR) confirmed the diagnosis of disseminated cutaneous varicella zoster virus and the patient was
treated with intravenous acyclovir for 4 days, followed by
oral valacyclovir, for a total of 10 days of anti-viral therapy
with complete resolution of cutaneous varicella zoster
virus.
Over the following 2 months, he was hospitalized several times for generalized weakness. On his most recent
hospital admission, he presented with acute dysarthria and
left-sided hemiparesis. Thromboembolic workup with
transesophageal echocardiography and coagulation tests
were negative. Autoimmune workup was negative. Magnetic resonance imaging and computed tomography angiography of the brain demonstrated multiple bilateral basal
ganglia infarcts and irregular vascular stenoses concerning
for vasculitis (Figure 2). Lumbar puncture was performed
with opening pressure of 33 cm H2O, lymphocytic predominant pleocytosis, and positive cerebrospinal fluid varicella
zoster virus PCR.
The patient was diagnosed with varicella zoster virus
vasculopathy and was treated with oral prednisone 1 mg/kg
per day for 5 days and intravenous acyclovir 10 mg/kg
twice daily for 14 days. Upon completion of treatment, follow-up magnetic resonance imaging of the brain showed no
new strokes, and varicella zoster virus PCR of the cerebrospinal fluid was negative. The patient recovered partial
Funding: None.
Conflict of Interest: None.
Authorship: All authors had access to the data and a role in writing
this manuscript.
Written consent was obtained from the patient and his family to publish the case with the included images.
Requests for reprints should be addressed to Sae Jang, MD, Department of Internal Medicine, Mayo Clinic, Rochester, MN.
E-mail address: Jang.sae@mayo.edu

0002-9343/© 2019 Elsevier Inc. All rights reserved.

mobility of his left lower extremity by the time he was discharged from hospital. Given the patient’s severe course
and immunocompromised state, he was transitioned to oral
valacyclovir 1 gram twice daily, with infectious disease follow-up evaluation to determine treatment duration.
Infection is a rare cause of stroke. Among these infections, varicella zoster virus may cause ischemic stroke. In
retrospect, his symptoms of generalized weakness and confusion leading to his stroke may have been signs of vasculopathy.
Most varicella zoster virus vasculopathies develop
within 6 weeks after herpes zoster infection with a median
4-month interval for stroke.1 Both immunocompetent and
immunocompromised individuals may suffer from varicella
zoster virus central nervous system disease, but the latter
group tends to be more seriously affected.2 A prophylactic
dose of valacyclovir at 500 mg twice daily can be considered for long-term suppression in immunocompromised
patients.3
Sae K. Jang, MDa
Natalia E. Castillo Almeida, MDb
Randall Ambroz, MDc
Walter Wilson, MDb
a

Department of Internal Medicine,
Mayo Clinic, Rochester, Minn
b
Department of Infectious Disease,
Mayo Clinic, Rochester, Minn
c
Department of Radiology, Mayo
Clinic, Rochester, Minn

https://doi.org/10.1016/j.amjmed.2019.07.014

References
1. Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Varicella zoster virus
vasculopathies: diverse clinical manifestations, laboratory features,
pathogenesis, and treatment. Lancet Neurol 2009;8(8):731–40.
2. Kennedy PGE, Gershon AA. Clinical features of varicella-zoster virus
infection. Viruses 2018;10(11).
3. Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Varicella zoster virus
vasculopathies: diverse clinical manifestations, laboratory features,
pathogenesis, and treatment. Lancet Neurol 2009;8(8):731–40.

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ARTICLE IN PRESS

The American Journal of Medicine, Vol 000, No 000, && 2019

Figure 1 Numerous vesicles and bulla surrounded by an erythematous halo located on the trunk.

Figure 2 (A) Magnetic resonance imaging demonstrated diffusion weighted image
signal within the bilateral posterior lentiform nuclei and right posterior limb internal
capsule (black arrows). (B) Time-of-flight magnetic resonance angiography demonstrated alternating segments of vascular narrowing and dilation of the right middle cerebral artery (yellow arrow). Note the relatively normal appearance of the contralateral
left middle cerebral artery (blue arrow). (C) Computed tomography angiography of the
head and neck demonstrated irregular dilatation of the M2 branch of the right middle
cerebral artery (black arrow). Diffusion-weighted imaging overlay demonstrated
infarction within the right lentiform nuclei in the territory of the right lateral lenticulostriate arteries (yellow asterisk). Infarction within this area accounts for the presenting
symptoms of left-sided weakness.