Demystifying Neurosarcoidosis and Informing Prognosis

Abstract

Using internally developed risk models to assess heterogeneity in treatment effects in clinical trials.

Clinicopathology conference: 41‐year‐old woman with chronic relapsing meningitis

“…Neurosarcoidosis is an autoimmune non‐necrotizing granulomatous condition that can present with both systemic and neurological disease . Neurosarcoidosis commonly causes a relapsing chronic meningitis, and chronic lumbar arachnoiditis has been described .…”

“…Paradoxical worsening of TBM is an immunemediated response that can present up to a year after effective treatment, with 4% of patients developing spinal arachnoiditis. 38 Neurosarcoidosis commonly causes a relapsing chronic meningitis, and chronic lumbar arachnoiditis has been described. Most cases have negative cryptococcal cultures in CSF.…”

“…37 Neurosarcoidosis is an autoimmune non-necrotizing granulomatous condition that can present with both systemic and neurological disease. 38 Neurosarcoidosis commonly causes a relapsing chronic meningitis, and chronic lumbar arachnoiditis has been described. 39,40 Neurological symptoms can be the initial presentation of neurosarcoidosis in 50% of patients, with 85% ultimately developing systemic disease but with a significant percentage continuing to have isolated CNS involvement.…”

A 46-Year-Old Presenting With Inferior Mononuclear Visual Field Defect as the Sole Manifestation of Neurosarcoidosis

“…NS presents in 5% of patients who have sarcoidosis [3]. In addition, establishing a diagnosis of NS has been difficult because until recently there were no established criteria and getting a nerve tissue biopsy was tough [4]. However, in 2018, the Neuro-sarcoidosis Consortium Consensus Group established some baseline criteria that clinicians can use to help establish a diagnosis of NS [5].…”

A young man with numbness in arms and legs

“… 25 Retrospective analyses suggest that mycophenolate mofetil may be less effective than methotrexate (at least for preventing “relapse”), but such studies risk confounding by indication. 25 , 26 In retrospective analyses, infliximab, a TNFα inhibitor, is associated with favorable outcomes, including some cases refractory to other therapies. 8 …”