Teaching NeuroImage: Seizures as the Initial Symptom of Relapsing Polychondritis

Xu, Yingying; Meng, Yujie; Wang, Ping; Sun, Lingling; Xu, Shiqi

doi:10.1212/wnl.0000000000013144

Cited by 1 publication

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“…In our case, focal seizures were the initial neurological symptom, which appeared 1 week earlier than the other symptoms. Seizures have also been reported as an initial symptom of RP in a limited number of cases ( 31 ). Although seizures are more common in other connective tissue diseases, such as systemic lupus erythematosus, RP should also be included in the differential diagnosis of new-onset seizures, especially those with suggestive signs such as cauliflower ear and/or red eyes.…”

Section: Discussionmentioning

confidence: 99%

Relapsing polychondritis-associated meningoencephalitis initially presenting as seizure: a case report and literature review

Zhang,

Shi,

Zhang

et al. 2023

Front. Neurol.

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Background and purposeRelapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis.Case presentationA 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient's condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.ConclusionRP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.

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Section: Discussionmentioning

confidence: 99%