Cerebellar infarction caused by primary central nervous system angiitis of childhood: case report

Cerebellar infarction of childhood is rare, and is difficult in pathological diagnosis. We describe a case of radiographically diagnosed primary central nervous system angiitis of childhood.

A 7-year-old boy experienced dizziness and headache persisting for 7 days. Diffusion-weighted images of magnetic resonance (MR) showed high signals in the left cerebellar hemisphere and vermis. The MR angiogram (MRA) findings were normal. A conventional angiogram demonstrated severe stenoses and occlusions at distal portion of left posterior inferior cerebellar artery, and irregularity in the wall of the cervical portion of the left vertebral artery (VA). Although he recovered without any neurologic deficits, an angiogram 3 months after admission showed occlusion at the cervical portion of left VA and filling of the distal VA with collateral arteries from the deep cervical artery. He was doing well, with no additional changes demonstrated on MRA, 12 months after the onset.

Although MRA can detect abnormality within the proximal intracranial vessels, angiography is essential, especially in cases with distal stenoses. Repeated angiography in primary central nervous system angiitis of childhood is necessary at least 3 months after the onset, even if the patient has no symptom.