Relapsing Polychondritis With Encephalitis

Abstract

Central nervous system manifestations are rare in patients with relapsing polychondritis. We report a case of encephalitis associated with relapsing polychondritis. The patient presented with bilateral auricular chondritis first and then tracheal chondritis, and encephalitis developed. The symptoms and signs of encephalitis were felt mostly likely to be the result of vasculitis and improved dramatically after pulse corticosteroid therapy. We believe that early recognition of central nervous system involvement in relapsing polychondritis is extremely important. High-dose corticosteroids should be given to suspected cases as early as possible.

Relapsing Polychondritis in the Department of Defense Population and Review of the Literature

“…The pathogenesis of neurologic involvement is thought to be secondary to vasculitis. This has been corroborated on autopsy studies demonstrating diffuse vasculitis of small arteries and arterioles (29). MRI of the brain also supports vasculitis as an etiology, typically showing areas of multifocal gray and white matter enhancement (29).…”

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

“…Although CNS involvement due to vasculitis is rare, RP has been reported to be associated with aseptic meningitis, meningoencephalitis, encephalitis, and ischemic stroke (3). To our knowledge, there have been 18 cases of RP-associated meningoencephalitis and encephalitis demonstrated clinically and on neuroimaging in the English literature (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). The clinical features of the previously reported cases and the present case are summarized in Table. Among these cases, the age of onset was between 29 and 73 years (mean, 55 years).…”

Meningoencephalitis or meningitis in relapsing polychondritis: Four case reports and a literature review

“…Some patients presenting with limbic encephalitis or dementia showed lesions in the temporal lobes on MRI. [14][15][16]18,20,23,24 Massry et al 10 reported that MRI in RP may be characterized by bilateral hypertensities in the basal ganglia. Addition of gadolinium enhanced smaller adjacent areas, an area in the left posterior thalamus, and a wedge-shaped area in the cortical grey matter.…”

“…Seventeen patients, including our Patients 1, 2 and 4, showed pleocytosis with a predominance of mononuclear cells, including lymphocytes and monocytes in the CSF. 6,7,9,10,[14][15][16]19,21,23,24,26 Six patients showed pleocytosis with a predominance of PMN in the CSF. 7,8,12,13,20,25 At first onset of meningoencephalitis, two patients showed pleocytosis with a predominance of mononuclear cells in the CSF, but the recurrences had a predominance of PMN 11,17 Our Patient 3 showed pleocytosis with a predominance of PMN in the CSF at first but later a predominance of mononuclear cells (Table 1).…”