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

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).…”

“…In addition, obvious risk factors were not noted in the previously reported cases or our current case of RP-associated meningoencephalitis and encephalitis. The etiology of these CNS complications in cases of RP therefore remains unknown, although the disease appears to originate from autoimmunity (16). Autoantibodies to glutamate receptor GluRε2 and neutral glycosphingolipids are detectable in the CSF and sera obtained from some patients with limbic encephalitis associated with RP (13,20).…”

Relapsing polychondritis and lymphocytic meningitis with varied neurological symptoms

“…2,5 Seronegative polyarthritis and systemic compromise of other organs may also occur (including ocular inflammation, audiovestibular impairment, vasculitis, skin involvement, valvular insufficiency and neurological symptoms) due to compromise of proteoglycan-rich tissues. 6,7 Around 30% of the cases are associated with concurrent autoimmune disease, systemic vasculitis and myelodysplastic syndrome. 5,8 Diagnosis is made on clinical grounds, occasionally with pathology disclosing inflammatory compromise of affected cartilaginous tissue.…”

“…5,8 Diagnosis is made on clinical grounds, occasionally with pathology disclosing inflammatory compromise of affected cartilaginous tissue. 5,7 Currently, the diagnosis is made on the basis of demonstration of either chondritis in two of three sites (auricular, nasal, laryngotracheal); or one of these sites and two additional features, including ocular inflammation, audiovestibular damage, or sero-negative inflammatory arthritis. 1,9 There are no specific laboratory findings.…”

“…1,9 There are no specific laboratory findings. 5,7 Neurological symptoms occur in a minority of cases (3%) and may range from compromise of cranial nerves to a more overt presentation with cerebellar compromise, seizures or other focal findings suggestive of cortical compromise. These are vasculitic in nature.…”