Differential diagnosis of central nervous system vasculopathies: two case reports

Introduction: Arteriopathies usually manifest as a transient focal cerebral angiopathy and are the main cause of arterial ischemic stroke (AIS) in children. In preschool children postvaricella angiopathy is common. Inflammatory etiologies need to be ruled out by imaging and laboratory investigations including cerebrospinal fluid (CSF) analysis. The diagnostic work up should comprise vasculitis mimics and primary central nervous system vasculitis.

Case 1: A 5-year old girl with history of chickenpox and recent headaches presents with recurrent transient hemiparesis. MRI reveals stenosis of the distal left carotid artery and proximal middle and anterior cerebral artery, vessel wall contrast enhancement and multiple small lacunar AISs. CSF shows mild pleocytosis, VZV-DNA and VZV-IgG antibodies are positive. Therapy with acyclovir, steroids, and salicylic acid is initiated. The stenosis improves and the girl remains asymptomatic.

Case 2: A 4-year old boy with history of mood disorder and chickenpox develops two transient episodes of gait disorder, dysphasia, and facial palsy. MRI shows acute AIS of the right putamen and stenosis of the right middle cerebral artery (M2). There is mild CSF pleocytosis. VZV-DNA in CSF and serum Borrelia burgdorferi antibodies are negative. He is treated with acyclovir, steroids, and salicylic acid. Following steroid withdrawal 4 months after initial presentation his mood disorder worsens and he develops chorea. He responds to resumption of steroid treatment. Angiography shows stenosis of the right carotid artery, occlusion of the proximal anterior cerebral artery, and vessel wall contrast enhancement of both middle cerebral arteries without new ischemic lesions. Progressive vasculitis of the central nervous system is diagnosed and an additional therapy with cyclophosphamide is started.

Conclusion: Vascular imaging in AIS is essential and comprises angiography (usually MRA) and studies of the arterial wall. VZV vasculopathy is usually monophasic. Involvement of further arteries in the course of the disease warrants reevaluation and usually intensification of immunosuppressive therapy.