Clinical,imaging and pathological features of primary angiitis of central nervous system / 临床神经病学杂志

ABSTRACT

Objective To study the clinical, imaging and pathological features of primary angiitis of central nervous system (PACNS). Methods The clinical data of 8 patients with PACNS (4 suspected and 4 definite) were retrospectively analysed.Results There were 5 males and 3 females in 8 patients with PACNS. Average age was 32 years old and average disease course was 11 months. The initial symptoms were headache in 5, extremital weakness in 2, and speech disorder in 1 patients. The major clinical manifestations were headache in all patients, extremital weakness and numbness in 5, speech disorder in 1, and seizure disorder in 2 patients. 7 patients were with slow-progressive disease courses, and 1 patients with a relapsing-remitting one. MRI showed abnormality in all patients. 6 cases had unilateral lesions and 2 had bilateral lesions. MRI showed ill-defined, irregular or atypical wedge-shaped foci in cortical and subcortical areas in 7 patients and strip-shaped foci with surrounding finger-like cerebral edema in the left frontal and parietal lobe and gyrus cinguli in 1 patient. All lesions enhanced with different degree on contrast-enhanced scan. Enhanced lesions along sulcus and gyrus were seen in 7 patients and mass-like irregular linear enhancement lesion in 1 patient. Diffusion-weighted imaging(DWI) showed hyper-signal and apparent diffusion coefficient(ADC) showed hypo-signal in 5 patients. MRA showed stiffness and lack of smooth in one or more cerebral arteries with multiple segmental narrowing and expanding, and rarity of arterial distal branches in 8 patients. Digital subtraction angiography (DSA) also showed multiple segmental narrowing and expanding in cerebral arteries, and rarity of arterial distal branches in 4 patients. Brain biopsy in 4 cases revealed angiitis with lymphocytic infiltration. 5 patients received treatment with single corticoid, and 3 with cyclophosphamide. All cases had good outcome. Conclusions The clinical manifestations of PACNS are various. The changes of imaging are obvious, MRI, MRA and DSA are helpful in the diagnosis of PACNS. The definite diagnosis relies on brain biopsy.