“Prominent ear sign” on diffusion-weighted magnetic resonance imaging in relapsing polychondritis

Abstract

Relapsing polychondritis is a rare autoimmune disease characterized by recurrent inflammation of cartilage in multiple sites of the body, including the auricles. Central nervous system involvement appears rare. We encountered a case of relapsing polychondritis with encephalitis that could be diagnosed by the unique appearance of the auricle with signal hyperintensity on diffusion-weighted magnetic resonance imaging.

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

“…11,13,17 A patient with encephalitis lethargica with RP showed diffuse hyperintense lesions bilaterally in the basal ganglia on T2WI and fluid attenuated inversion recovery (FLAIR) images. 22 All of our patients had abnormal signals in the affected auricle on MRI and CT scans, which is similar to the ''prominent ear sign'' described by Kuwabara et al 21 A biopsy or autopsy of the brain was performed in seven of the 24 previously reported patients summarised in Table 3. 6,7,11,13,16,18,23 The aetiology of central nervous system (CNS) involvement in RP patients is still unknown but seems to originate from autoimmunity.…”

“…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).…”

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).…”

Imaging of connective tissue diseases of the head and neck

“…1 Recurrent inflammation of the larynx can result in permanent laryngomalacia or stenosis of the larynx with inspiratory dyspnea or obstructive sleep apnea syndrome. [33][34][35][36] The association of relapsing polychondritis with hematological malignancies, particularly with myelodysplastic syndrome, has been described. 6 External auditory canal calcification should suggest the diagnosis of relapsing polychondritis.…”

“…Diffusion-weighted MR imaging shows the characteristic finding of auricular signal hyperintensity, known as ‘prominent ear sign’. 34 Destruction of the nasal cartilage with nasal cartilage collapse in patients with relapsing polychondritis results in a classic ‘saddle’ deformity of the nasal bridge. 35 CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures 33 (Figure 7).…”