Perivasculitic Panencephalitis with Relapsing Polychondritis: An Autopsy Case Report and Review of Previous Cases

Niwa, Atsushi; Okamoto, Yoko; Kondo, Takayuki; Nabatame, Hidehiko; Takahashi, Ryōsuke; Tomimoto, Hidekazu

doi:10.2169/internalmedicine.53.1381

Cited by 6 publications

(5 citation statements)

References 15 publications

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“…Meningitis and enephalitis in RP can show various symptoms like cognitive impairment, psychomotor abnormality, confusion, and disorientation ( 8 ). Characteristic MRI pattern has not been established but multifocal hyper-intensity lesions, especially in basal ganglia and periventricular and subcortical white matter are the most common findings ( 7 9 10 ).…”

Section: Discussionmentioning

confidence: 99%

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

Jeon

2016

J Korean Med Sci

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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.

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Section: Discussionmentioning

confidence: 99%

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

Jeon

2016

J Korean Med Sci

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“…Subclinical brain MRI abnormality has not been reported in RP. Autopsy studies of RP patients with symptomatic CNS involvement have shown meningoencephalitis, vasculitis, or encephalitis with perivascular inflammatory cells . Anti‐neutral glycosphingolipid antibodies are found specific in RP patients with encephalitis and may be related to the pathophysiology, which was absent in our patient.…”

Section: Discussionmentioning

confidence: 46%

“…Autopsy studies of RP patients with symptomatic CNS involvement have shown meningoencephalitis, vasculitis, or encephalitis with perivascular inflammatory cells. 6 Anti-neutral glycosphingolipid antibodies are found specific in RP patients with encephalitis and may be related to the pathophysiology, 5,7 which was absent in our patient. The reason why the MRI abnormality in our patient caused no or very mild CNS symptoms are unknown, but the FLAIRhigh lesions may have largely been vasogenic edema associated with perivascular inflammation, which is consistent with contrast enhancement restricted to the perivascular space, increased ADC in the acute phase of lesion enlargement, and the improvement of FLAIR-high lesions without atrophy.…”

Section: Discussionmentioning

confidence: 50%

Severe visual impairment and subclinical encephalitis preceding clinical signs of chondritis in relapsing polychondritis

Miyano

Kurihara

Orimo

et al. 2018

Neurology & Clinical Neurosc

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A 71‐year‐old woman with a 6‐month history of relapsing bilateral anterior scleritis presented with severe right visual impairment due to posterior scleritis. Despite radiological signs of encephalitis, the patient and her family members noticed no cognitive decline. The patient subsequently developed slight auricular pain without any visual changes such as redness or swelling, which, however, showed increased uptake of 18F‐fluorodeoxyglucose on positron emission tomography. Auricular cartilage biopsy revealed perichondrial inflammation suggesting relapsing polychondritis. Steroid therapy improved her symptoms and radiological findings. This case illustrates that asymptomatic brain inflammatory lesions can precede clinical signs of chondritis in relapsing polychondritis, and that auricular cartilage biopsy should be considered even with mild auricular pain without apparent clinical findings of inflammation.

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“…[29][30][31] Moreover, an increasing body of evidence has revealed pathological presentations of vasculitis and inflammatory changes-such as T-cell infiltration, panencephalitis, and granuloma formation-in RP patients. [32][33][34][35][36] In our study, the incidence of ear involvement was higher in RP patients with CNS involvement compared with RP patients without CNS involvement. The involvement of the eyes and heart was correlated with RP patients with CNS involvement, after the factors sex and the admission age were controlled.…”

Section: Discussionmentioning

confidence: 86%

Comparison of relapsing polychondritis patients with and without central nervous system involvement: A retrospective study of 181 patients

Cao

Zhu

Li³

et al. 2021

Int J Immunopathol Pharmacol

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The relapsing polychondritis (RP) patients with central nervous system (CNS) involvement were rare. We aimed to determine the clinical characteristics of RP patients with CNS involvement. The clinical data of 181 RP patients, hospitalized at Peking Union Medical College Hospital between December 2005 and February 2019, were collected. The patients were categorized into two subgroups: 25 RP patients with CNS involvement, and 156 RP patients without CNS involvement. The involvement of the ear was more frequent in RP patients with CNS involvement, compared with those of RP patients without CNS involvement ( P < 0.01). After controlling sex and the admission age, logistic regression analysis revealed hypertension (odds ratio = 4.308, P = 0.006) and involvement of eye (odds ratio = 5.158, P = 0.001) and heart (odds ratio = 3.216, P = 0.025) were correlated with RP patients with CNS involvement, respectively. In addition, pulmonary infection (odds ratio = 0.170, P = 0.020), tracheal involvement (odds ratio = 0.073, P < 0.01), and involvement of laryngeal (odds ratio = 0.034, P = 0.001), costochondral joint (odds ratio = 0.311, P = 0.013), sternoclavicular joint (odds ratio = 0.163, P = 0.017) and manubriosternal joint (odds ratio = 0.171, P = 0.021) were associated with RP patients without CNS involvement, respectively. In contrast to RP patients without CNS involvement, the incidence of ear involvement was higher in RP patients with CNS involvement. After controlling the potential confounding factor sex and the admission age, hypertension and involvement of eye and heart were related with RP patients with CNS involvement, respectively.

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Perivasculitic Panencephalitis with Relapsing Polychondritis: An Autopsy Case Report and Review of Previous Cases

Cited by 6 publications

References 15 publications

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

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

Severe visual impairment and subclinical encephalitis preceding clinical signs of chondritis in relapsing polychondritis

Comparison of relapsing polychondritis patients with and without central nervous system involvement: A retrospective study of 181 patients

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