Multiple ischemic strokes in a case of Transient Cerebral Arteriopathy (TCA) following chickenpox

In children arterial ischemic strokes (AIS) are often caused by infections. VZV can lead to unilateral intracranial vasculopathies with consecutive ischemic strokes. We report on an 11 years old girl who had chickenpox 7 years before onset of neurological symptoms.

The girl presented in our clinic with acute temporal located headaches, a rightsided hemi- and facial nerve paresis and a motoric aphasia. She had a history of chronic migraine-like headaches over 2 years. Her mother and brother suffered from migraine with neurological deficits. After admission she improved rapidly under symptomatic therapy. Later performed cranial MRI and MR-Angiography (MRA) revealed acute and older ischemic lesions and a stenosis of the left MCA, highly suspicious of a cerebral vasculitis. We found no cardiac abnormalities, hypercoagulability or systemic inflammatory disease. Familiar hemiplegic migraine could be excluded by genetic tests. Lumbar punction showed a lymphocytosis and a positive VZV-PCR. The Antibody-Specificity-Index (ASI) confirmed an intrathecal IgG-synthesis underpinning our hypothesis of a subacute persistent Varicella-infection. Under antiviral therapy and anticoagulation with heparin initially and ASS later the arteriopathy improved rapidly. MRI and MRA following 4 months after onset of the symptoms showed a nearly complete normalization of the MCA stenosis. Up to now the child has no neurological sequelae, but shows mild cognitive symptoms such as an attention deficit. Extremely remarkablein this case is the latency between the VZV infection and the occurance of an acute ischemic stroke on the ground of a post-varicella arteriopathy 7 years later.