Stroke related to herpes simplex virus-associated vasculitis of cerebral arteries

Natalia Nazarova MDa, Kirill Fedorov MDb, Svetlana Gebert MDa, Pavel Novikov MDb, Prof Sergey Moiseev MDb

a First Clinical Hospital, Moscow,
b Russia Clinic of Nephrology, Internal and Occupational Disease, First Moscow State Medical University, Moscow, Russia

Objective

To our knowledge herpes simplex virus (HSV) is an extremely rare cause of cerebral vasculitis.

Methods

Description of the clinical, radiological and laboratory findings.

Results

A 34-year-old female with a history of recurrent headache, smoking, oral contraception and prior spontaneous abortion presented to ICU in June 2013 with a severe headache, vomiting, depressed consciousness and left sided focal deficit. Brain CT showed two ischemic lesions in subcortical nucleus of the right hemisphere. MRI revealed multiple cortical and subcortical strokes in the frontal, temporal, occipital and hippocampal regions of the right hemisphere. Digital subtraction angiography (DSA) on admission showed no stenosis of the cerebral arteries. WBC count was increased, serum protein C level was slightly reduced. Cerebrospinal fluid (CSF) was normal. Extended examination excluded SLE and antiphospholipid syndrome. MTHFR, PAI 1 and ACE genes showed heterozygote mutations. The patient was treated with LMWH for 14 days, dexamethasone 16 mg for 7 days, aspirin and statin. She recovered well within two weeks to a mild hemiparesis. In July 2013 noncontrast brain MRA showed absence of the blood flow in the right anterior and middle cerebral arteries which was confirmed by digital subtraction angiography. Brain and neck MRI revealed thickening and contrast enhancement of the right internal carotid and the proximal part of the  middle cerebral arteries walls confirming CNS vasculitis. There was four-fold increase in plasma anti-HSV IgM while CSF PCR was positive for HSV DNA. Intravenous acyclovir 20 mg/kg was started. Within two weeks HSV was eliminated from CSF but MRA showed no improvement. Oral prednisolone 30 mg daily and rivaroxaban 20 mg were administered. In August 2013 a CTA and MRA imaging revealed complete cerebral arteries recanalization with a slight residual extra- and intracerebral arteries narrowing. Prednisolone was tapered off. In September 2014 she was symptom free.

Discussion

There are only few reports of cerebral vasculitis related to HSV infection. Occlusion of the intracranial arteries was not evident at presentation but developed later, apparently due to the untreated HSV infection. Acyclovir administration leaded to elimination of HSV from CSF but recanalization of the cerebral arteries was achieved after steroid treatment only.
Conclusion. HSV should be considered as a potential etiology of the CNS vasculitis. MRI showing the inflammation of the vessel wall in conjunction with CSF PCR for viral DNA should be performed for the evaluation of unexplained stroke.