Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center

Abstract

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.

Keywords: Dementia; Limbic Encephalitis; Meningoencephalitis; Relapsing Polychondritis.

Fig. 1

Brain MRI and MR angiography. T2 weighted image showed edema of brain cortex and some high signals (arrows) in parenchyma of bilateral cerebellum (A) and basal ganglia (B). MR angiography showed near complete occlusion (arrow heads) of left distal internal carotid artery, left proximal middle cerebral artery, and left anterior cerebral artery (C).

Fig. 2

Electroencephalography (EEG). There were frequent brief rhythmic spikes and slow wave complexes on EEG.

Fig. 3

PET-CT scan of brain. PET-CT scan showed (A) symmetric decreased FDG uptake in both temporal lobe and both cerebellum, and asymmetric decreased FDG uptake in right basal ganglion in axial view, and (B) decreased FDG uptake in posterior cingulate gyrus and precuneus area in sagittal image.