A Diagnostic Dilemma: Is it CNS Vasculitis?

A case study from Rula Hajj-Ali, MD

Imaging of vessels in CNS vasculitis

A 56-year old presented with manic symptoms and encephalopathy. Following work-ups for infection and malignancy, which included a brain biopsy, she was diagnosed and treated for primary angiitis of the central nervous system (PACNS) at an outside hospital. However, she had a transient response and then became progressively worse and was transferred to our hospital.


Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

In this video, vasculitis specialist Rula Hajj-Ali, MD, presents the complex case that seemed refractive to treatment. Is it really central nervous system vasculitis (CNS-V)?

A systemic approach to diagnosis in CNS vasculitis

Arriving at a diagnosis of CNS-V is fraught with challenges. Clinical presentation can be quite variable, and there is no classic presentation. Further complicating matters, the condition has several mimics, brain tissue is inaccessible and there is no disease-specific test. However, advances in neuroimaging and next-generation sequencing — along with the involvement of a multidisciplinary clinical team — have added formidably to our knowledge of CNS-V.

Regardless of the scenario, we take a systematic approach to the work-up of any patient suspected to have CNS-V. This approach includes a general history and physical exam, with a thorough review of symptoms associated with systemic autoimmune disease (e.g., fever, rashes, sinus disease, sicca, joint pains, cough, peripheral neuropathy, oro-genital ulcers, inflammatory eye disease, deep venous thrombosis or recurrent miscarriages). Clinicians should look for infectious and/or malignant conditions that might be associated with many of these nonspecific symptoms, especially fever, malaise, joint pains and weight loss. Other important aspects when interviewing patients include eliciting travel history, work hazards, chronic exposure to recreational drugs and family history of neurologic events; this information can unravel rare conditions.


Related Articles

patient consult
Key Elements for Optimizing Care in People with Vasculitis

Evidence-based therapies, monitoring, prevention and more

Case: When Central Nervous System Vasculitis Is Suspected

Holistic approach is necessary to ensure a correct diagnosis

Keys for Recognizing Drug-Induced Vasculitis

Knowing the affected organs and vessels can help in identifying cause

pulmonary artery stenosis
Takayasu Arteritis Case Study: Pulmonary Embolism or Something Else?

When to consider the possibility of pulmonary artery involvement

A Challenging Case of Giant Cell Arteritis

When GCA initially presents without cranial symptoms

Case Report: Long-Term Monitoring a Must for ANCA-Associated Vasculitis

Relapses are frequent even with long periods of remission

A series of brain MRI images used to help diagnose CNS vasculitis
Neurologic Involvement in ANCA-Associated Vasculitis

Raising awareness of a common manifestation