Aseptic meningitis in relapsing polychondritis: a case report and literature review

Abstract

Aseptic meningitis is an extremely rare neurologic complication of relapsing polychondritis (RP). We reported a case of a 58-year-old Chinese female with intractable headache, puffy ears, pleocytosis, and cranial magnetic resonance imaging (MRI) showing thickened and enhanced meninges. She was finally diagnosed of aseptic meningitis due to RP after full exclusion of infectious causes. She gradually developed neurosensory hearing loss, vertigo, and saddle nose while glucocorticosteroid therapy and combined cyclophosphamide could not control her headache. Ultimately, cyclosporin A was tried showing a good response. Only 18 previous cases were found in the literature and the clinical manifestation, cerebrospinal fluid (CSF) characteristics, imaging features, and therapy considerations of RP-related aseptic meningitis were summarized by reviewing the literature. Aseptic meningitis due to RP is a rare condition of undetermined pathoetiology. Its diagnosis is primarily based on clinical manifestations combined with CSF and MRI examinations plus adequate exclusion of possible infections. Corticosteroid is the basic therapy but choice of protocol should be individualized.

Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy

“…Neurologic presentation of meningoencephalitis with RP varied between cases; reported presentations were hearing loss, unsteadiness, personality changes, generalized tonic-clonic seizure, impaired cognitive function, impaired visual acuity, confusion, memory loss, unstable gait, ataxia, hydrocephalus, delirium, cerebral infarction, coordination problems, distraction, word finding difficulty, emotional lability, abducens nerve palsy, anxiety, insomnia, memory loss, deafness, gait change, urinary incontinence, expressive and receptive aphasia, dullness, acalculia, and papilledema [ 6 ].…”

“…10 of 19 cases improved on glucocorticoids and 5 out of those 10 showed a relapse of disease. Cyclophosphamide was added to glucocorticoids in 3 cases, one case died [ 7 ], one improved with no relapse [ 8 , 9 ], and the most recent case had to add cyclosporin A to relieve symptoms, even though the patient relapsed [ 6 ]. Azathioprine was added to glucocorticoids in 4 cases all of which showed improvement, but three of those cases relapsed [ 6 ].…”

Elucidation of predictors of disease progression in patients with relapsing polychondritis at the onset: potential impact on patient monitoring

“…It was reported that CNS involvement occurred in 8–12% of RP patients [ 2 , 4 , 13 , 16 ] and the mortality rates were high [ 13 , 18 , 22 , 23 ]. When we reviewed the literature [ 18 , 22 – 42 ], 14 of 50 RP patients (28%) with meningitis/encephalitis developed neurological symptoms preceding any detectable chondritis (Table 4 ). At the last follow-up, 82 and 18% of the patients with CNS involvement were allocated to AL and BL subgroups, respectively (Table 4 ), consistent with our finding of close relationship between auricular involvement and CNS involvement [ 8 ].…”

“… a A review of the literature was conducted on RP meningitis/encephalitis using PubMed [ 18 , 22 – 42 ] b Time interval (months) between the onset of meningitis/encephalitis and that of chondritis c Follow-up duration of patients (months) d Age, time interval, and follow-up duration were expressed as mean ± standard error of the mean e NA, Not applicable …”

“…a A review of the literature was conducted on RP meningitis/encephalitis using PubMed [ 18 , 22 – 42 ]…”