The diagnostic conundrum of primary angiitis of the central nervous system: a case report

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare inflammatory vasculitis that may present with nonspecific symptoms and requires a histological diagnosis. Here, we report a case of PACNS that presented atypically, and also responded poorly to immunosuppressive treatment. We discuss the steps necessary for confirming the diagnosis, and the on-going management during this process. This case highlights the diagnostic conundrum offered by non-specific inflammatory conditions of the CNS.A 15-year-old girl initially presented with migrainous episodes. Some months later she was admitted with headache and vomiting, which developed into an encephalitic illness with associated seizures. Her medical history was only notable for bilateral serous otitis media at the age of three. On admission, vital signs were normal and there was no evidence of meningism. Examination revealed brisk reflexes, increased tone, and sustained ankle clonus bilaterally in addition to extensor plantar responses. Power and sensation were normal, but she displayed mild left sided finger-nose and heel-shin dysmetria and was unable to tandem walk. The remainder of the examination was unremarkable.MRI showed diffuse hyperintense lesions, the CSF was acellular with a mildly elevated protein (530 mg/l), and an infection and autoimmune screen were negative. A cerebral angiogram had also been normal and peripheral vasculitis markers including ANA and ANCA were all negative.

Primary Angiitis of the Central Nervous System - Clinical Approaches, Challenges and Controversies

“…61 Persistent or recurrent headaches, new neurological or systemic manifestations, persistent inflammation evidenced from blood and CSF analysis, and new CNS lesions shown by MRI or vascular imaging may reflect lack of disease control or complications of immunosuppressive therapy (e.g., CNS infections) and should prompt re-investigation and adjustment of treatment. In refractory or relapsing PACNS, new attempts to induce remission often combine high-dose corticosteroids with one or more of cytotoxic agents, plasmapheresis, intravenous immunoglobulins, 67 anti-CD20 monoclonal antibodies (rituximab), 68 and TNF-α blockers (infliximab, ethanercept). 64…”

“…Despite therapy, which sometimes can be aggressive and multimodal, 25,68 PACNS remains fatal in approximately one-sixth of patients. 14 In survivors, neurological status tends to improve over time, more than 90 % being left with no-to-moderate disability.…”