Primary
vasculitis (
angiitis) of the central nervous system (
PACNS) is a
rare disease targeting the vessels of the brain, spinal cord and leptomeninges without systemic involvement. The etiology is not clear enough. The authors review clinical, laboratory and radiological features of
PACNS. Clinical manifestations are variable and depend on the caliber of affected vessels. The main clinical manifestations of small sized vessel
vasculitis include
encephalopathy (
cognitive disorders, epileptic
seizures),
headache and
transient cerebral ischemia. The main clinical presentation of
vasculitis of medium/large cerebral arteries is
ischemic strokes, which usually develop in different vascular territories. CSF findings in the majority of patients show modest lymphocytic
pleocytosis, elevated
protein level and occasionally the presence of
oligoclonal bands. MRI data are not specific and include
infarcts, hyperintensity (FLAIR) and sometimes
tumor-like lesions. The gold standard for the verification of
PACNS affected small-sized arteries is brain and leptomeningeal biopsy. Cerebral angiography allows the verification of
vasculitis of medium and large cerebral arteries revealing segmental narrowings (beading). High resolution black blood MRI before and after contrast injection may visualize intracranial vessel wall contrast enhancement - the sign of
inflammation in intracranial arteries. Treatment of
PACNS includes
corticosteroids and
cyclophosphamide. In the case of patient intolerance,
rituximab and blockers of
tumor necrosis factor may be used.