Differentiation between neurosarcoidosis and primary central nervous system vasculitis based on demographic, cerebrospinal and imaging features.

OBJECTIVES: Neurosarcoidosis (NS) and primary angiitis of central nervous system (PACNS) are inflammatory diseases affecting central nervous system, with overlapping clinical and pathological characteristics. Distinguishing these diseases is important given distinct therapeutic implications. In this study, we aimed to compare demographic, CSF and MRI characteristics between these two conditions. METHODS: All the clinical, CSF and laboratory characteristics at the time of presentation were retrieved from electronic medical records. Brain and/or spinal cord MRI performed near the time of presentation were blindly evaluated by two neuroradiologists. Data regarding involvement of pachy- and leptomeninges, basal meninges, cranial nerves, cerebral grey and white matter, and spinal cord were recorded for each patient. RESULTS: 78 patients with PACNS and 25 patients with NS were included in the study. Mean age of patients was 43.7 (±16.7) and 43.6 (±12.5) in PACNS and NS, respectively. African-American race was found to be associated with the diagnosis of NS rather than PACNS. Patients with PACNS had higher frequency of cerebral involvement, while patients with NS demonstrated more frequent spinal cord, basal meningeal and cranial nerve involvements. CONCLUSIONS: These findings suggest that MRI can be an efficient tool in distinguishing PACNS from NS. A follow-up study with a larger sample size would be required to validate our results.

Long-term outcomes of patients with primary angiitis of the central nervous system.

OBJECTIVES: Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the brain and spinal cord, which often presents with severe cognitive and functional deficits. Despite progress in diagnosis, little is still known about long-term outcomes. Our aim was to evaluate long-term functional capabilities, quality of life, and depression, and to determine the effect of treatment duration on patient outcomes. METHODS: We identified patients by ICD-9 codes for cerebral angiitis, and included them if they met two of the three following criteria: inflammatory cerebrospinal fluid (CSF), cerebral angiogram typical of vasculitis, or findings of vasculitis on pathologic examination of brain tissue. Disability was assessed by the Barthel Index, quality of life was assessed by EuroQol, and depression was assessed with Patient Health Questionnaire. RESULTS: Seventy-eight patients met the inclusion criteria, of which 27 responded to the questionnaire (34.6%). Mean follow-up of those who responded was 5.5 years (± 4.7). Nineteen of 27 patients (70.4%) had mild disability; meanwhile, 5 (18.5%) had severe disability. Fourteen of 27 patients (51.9%) had no mobility problem, 18 (66.7%) had no problems with self-care, 15 (55.6%) had no problems with usual activities, 14 (51.9%) had no pain, and 8 (29.6%) had no anxiety. Approximately 70% of patients had minimal or no depression. CONCLUSIONS: This is the longest reported follow-up of patients with PACNS described in the literature to date. Most patients had mild long-term disability and minimal to no depression, which may be reflective of treatment advances.