Background. Primary angiitis of the central nervous system is a rare disease with heterogeneous clinical pictures, often difficult to distinguish in clinical presentation from other inflammatory diseases of CNS, such as multiple sclerosis. The identification of specific clinical and paraclinical patterns is necessary to reach a definite diagnosis of this often unresolved, puzzling disease. Case report. A 39-year-old male developed, in a period of 18 months, subcontinue aspecific symptoms characterized by headache, nausea and vomiting, mild fever, and fatigue. At first evaluation the clinical neurological examination was normal; brain MRI showed a diffuse abnormality of infratentorial and supratentorial white matter on T2 scans with multiple, punctate, contrast-enhancing areas after gadolinium injection. The CSF examination revealed 26 cells/mmc, 105 mg/dl proteins. At cytology small and reactive lymphocytes were found. A complete screening for immunological and/or infective diseases was negative. After steroid treatment, the patient reported an improvement in his symptoms and even MRI and CSF findings showed a dramatic improvement in the pathological findings. After steroid wash-out MRI and CSF showed a disease reactivation with findings similar to those found at first examination, compatible with an aspecific inflammatory disease of CNS. A brain MRI scan after injection of a triple dose of gadolinium showed a diffuse perivascular enhancement in the white matter of the entire brain, in spite of a negative cerebral angiography. A diagnostic leptomeningeal and parenchimal biopsy showed a mild inflammatory infiltrate and increased capillary vascularization. The immunohistochemistry analysis revealed a damage of the BBB documented by abnormalities in adhesion molecules, espressed with an increased proteolytic activity in the CSF and in the serum for the 92-kDa gelatinase (MMP-9). These findings could explain the triple dose MRI data above mentioned. The patient started a long-term prednisone treatment with a persistent clinical improvement. Repetitive brain MRI with triple dose of gadolinium revealed a persistent and progressive reduction of the brain white matter inflammatory features, close to normality. Conclusions. This report supports usefulness of a non invasive diagnostic examination such as brain MRI with triple dose of gadolinium in detecting specific BBB abnormalities, although only the leptomeningeal biopsy can lead to a definite diagnosis of primary angiitis of CNS.
|Issue number||4 SUPPL.|
|Publication status||Published - 2000|
ASJC Scopus subject areas
- Clinical Neurology