E4501. Central Nervous System Manifestations and Neuroimaging of Pediatric Rheumatologic Diseases
  1. Wesley Chan; Albert Einstein College of Medicine
  2. Jessica Perfetto; Montefiore Medical Center
  3. Judah Burns; Montefiore Medical Center
Pediatric rheumatologic diseases can present with symptoms and complications that pose a diagnostic challenge. When patients present with isolated neurologic symptoms, neuroimaging is an essential diagnostic tool. Primary CNS involvement is characterized by isolated involvement of the brain and spinal cord, a diagnostic challenge in the absence of systemic symptoms that point to a rheumatic diagnosis. Secondary CNS inflammation may be triggered due to a systemic process such as vasculitis, malignancy, or infection, and may present with nonspecific imaging findings that overlap with other more common conditions. Although the pathophysiology of many rheumatologic diseases is unclear, a strong clinical picture and awareness of common neuroimaging findings in these conditions can aid radiologists in formulating a comprehensive differential diagnosis.

Educational Goals / Teaching Points
This guided educational exhibit demonstrates common CNS manifestations and radiologic findings of pediatric rheumatologic diseases using challenging patient cases. We will review the CNS manifestations of pediatric rheumatologic diseases with focus on the nature and distribution of lesions to create a cohesive display of these diseases, highlighting specific features, where applicable.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
CNS manifestations of rheumatologic disease include cerebrovascular disease, demyelinating disease, hemorrhage, intraparenchymal calcifications, cranial nerve lesions, pachymeningitis, and leptomeningeal disease. Specific conditions highlighted include systemic lupus erythematosus (SLE), primary angiitis of the CNS (PACNS), Behçet’s disease, sarcoidosis, systemic vasculitides, deficiency of adenosine deaminase 2 (DADA2), IgG4-related disease, hemophagocytic lymphohistiocytosis (HLH), and Cogan syndrome. Pathologic processes will be grouped by clinical symptoms, neuroradiologic findings, and anatomic location in order to guide a differential diagnosis.

This presentation describes neuroradiologic findings of pediatric rheumatologic diseases using cases and literature review to improve understanding and diagnosis among neuroradiologists and pediatric rheumatologists.