SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature

Abstract

Hypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation. The link between systemic lupus erythematosus (SLE) and hypertrophic pachymeningitis (HP) is extremely rare, with only six other cases reported in the literature. We, however, report the first case of SLE pachymeningitis presenting with multiple cranial nerve palsies. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction. Prompt therapy prevents long-term neurological sequelae.

Neurologic Manifestations of Rheumatologic Disorders

“…50 Similar to the meningitis seen in Sjögren syndrome, it is described primarily in case reports. 51,52 It can be focal or diffuse and can present as a leptomeningitis or pachymeningitis. Given that many patients with SLE receive long-term immunotherapy and particularly because terminal complement inhibition is used in some patients with SLE, it is imperative to carefully rule out an infectious cause of meningitis (eg, meningococcal infection seen as a complication of terminal complement inhibitors) in patients with SLE presenting with this clinical syndrome.…”

“…Meningitis occurs in less than 1% of patients with SLE 50 . Similar to the meningitis seen in Sjögren syndrome, it is described primarily in case reports 51,52 . It can be focal or diffuse and can present as a leptomeningitis or pachymeningitis.…”

A clinical approach to hypertrophic pachymeningitis

“…The clinical presentation is acute, with stroke-like events and seizures 31,32,33 . There are reported cases of systemic lupus erythematosus, giant-cell arteritis, relapsing polychondritis, Behçet syndrome, and Sjögren syndrome presenting with HP 34,35,36,37,38 .…”