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Letter to the Editor: Central Nervous System Involvement in Relapsing Polychondritis, a Rare and Difficult Diagnosis: a Case Report

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Julien Le Marec
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Stéphanie Jobard
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Adrien Bigot
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Benjamin Terrier at Université Paris Cité
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pISSN 1011-8934
eISSN 1598-6357
https://doi.org/10.3346/ jkms.2017.32.6.1048 • J Korean Med Sci 2017; 32: 1048-1049
Letter to the Editor:
Central Nervous System Involvement
in Relapsing Polychondritis, a Rare
and Dicult Diagnosis: a Case Report
Julien Le Marec,1 Stéphanie Jobard,1 Adrien Bigot,1
Benjamin Terrier,2 Jean-Philippe Cottier,3 Frédéric Bastides,4
and Elisabeth Diot1
1CHRU de Tours, Service de Médecine Interne, Université François-Rabelais de
Tours, Tours, France; 2CHU Cochin, Service de Médecine Interne, Université René
Descartes-Paris V, Paris, France; 3CHRU de Tours Service de Radiologie, Université
François-Rabelais de Tours, Tours, France; 4CHRU de Tours, Service de Médecine
Interne et Maladies Infectieuses, Université François-Rabelais de Tours, Tours,
France
To the Editor:
We read with interest the article by Chan Hong Jeon in the No-
vember 2016 issue of the Journal of Korean Medical Science re-
garding 3 cases of central nervous system involvement in relaps-
ing polychondritis (RP) (1). Neurological involvement is indeed
a rare but serious condition in RP, and raises diculties in diag-
nosis.
We report another case which illustrates the most important
dierential diagnosis to rule out in the presence of neurological
involvement in patient with RP. A 34-years-old Turkish woman,
with bilateral auricular, nasal chondritis and laryngeal involve-
ment, was diagnosed with RP, according to McAdam’s criteria
in February 2013 (2). She was initially treated by corticosteroids
and intravenous cyclophosphamide (6 pulses), followed by my-
cophenolate mofetil (2 g/day). Subcutaneous methotrexate was
initiated in June 2014 because of recurrent exacerbations. In
October 2014, she presented with fever at 40°C and high eleva-
tion of inammation biomarkers without bacteriological docu-
mentation, leading to the withdrawal of methotrexate. Seven
days later, appeared headaches, neck stiness and urine reten-
tion. e cerebrospinal uid (CSF) showed aseptic lymphocytic
meningitis (protein 1.19 g/L, glucose 2.20 mM, lactate 2.24 mM,
lymphocytes 150/µL). Complete microbiological investigations
in the blood and CSF, including quantiferon, herpes simplex,
cytomegalovirus (CMV), Epstein-Barr virus (EBV), toxoplasma,
borrelia, search for malignant cells, and lymphocytic count were
negative. Implication of methotrexate was initially suspected,
though recently stopped. An empirical treatment by doxycy-
cline was initiated. Her condition worsened with the appear-
ance of dizziness and rhombencephalitis with diplopia and bi-
lateral palsy of the abducens nerve. Brain magnetic resonance
imaging (MRI) was performed and showed an aspect of lepto-
meningitis surrounding the brainstem and the occipital lobes
(Fig. 1). In the presence of severe rhombencephalitis in a Turk-
ish woman, in France for 4 years, tuberculous meningoenceph-
alitis was suspected and a quadritherapy was started without
waiting for the results of CSF mycobacterial culture. Despite
this treatment, her neurological condition worsened, fever and
biological inammation persisted and inammatory joint pain
reappeared 2 weeks later. Behçet disease and mouth and geni-
tal ulcers with inamed cartilage (MAGIC) syndrome were hy-
pothesized but ruled out. Neurological involvement of RP with
meningo-encephalitis was at that moment presumed, inflix-
imab was initiated in association with methotrexate, with clinic
and biological remission within 2 weeks. Control of the CSF u-
id at one month was normal. To date, the disease is perfectly
controlled.
is observation, even if the neurological manifestations were
not present at diagnosis of RP as in the 2 cases reported in the
article, shows the diculties of dierential diagnosis of menin-
goencephalitis in patients with RP. It demonstrates the impor-
CORRESPONDENCE
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Fig. 1. Axial FLAIR image. Hyperintensity covering the pial surface of the brainstem
(arrows).
FLAIR = uid attenuated inversion recovery.
Marec JL, et al. • Central Nervous System Involvement in Relapsing Polychondritis
http://jkms.org 1049
https://doi.org/10.3346/jkms.2017.32.6.1048
tance to rule out at rst all infectious causes (viral, bacterial, fun-
gal, and parasitic) and eventual drugs adverse events. In our
clinical case, Methotrexate toxicity, although rarely described
in its sub cutaneous form was initially presumed responsible
but rapidly eliminated (3). After elimination of other infectious
causes, in this immunodepression context tuberculosis was the
most likely hypothesis to raise in the presence of a lymphocytic
meningo-encephalitis in a Turkish woman and she indeed re-
ceived a complete anti-tuberculosis therapy (4). is treatment
ineciency, the exclusion of drug toxicity and the reoccurrence
of articular inammation strongly suggested to role of RP. Cen-
tral nervous system (CNS) involvement in RP is a rare compli-
cation and treatment empirical. e eectiveness of anti-tumor
necrosis factor (TNF) agents in these clinical presentation, such
as iniximab in our case report, have been related in a few re-
ports (5).
DISCLOSURE
e authors have no potential conicts of interest to disclose.
AUTHOR CONTRIBUTION
Investigation: Terrier B, Cottier JP, Bastides F. Writing - original
draft: Le Marec J, Jobard S, Diot E. Writing - review & editing: Le
Marec J, Jobard S, Bigot A, Terrier B, Cottier JP, Bastides F, Diot E.
ORCID
Julien Le Marec http://orcid.org/0000-0003-1062-7694
Stéphanie Jobard http://orcid.org/0000-0003-3149-9775
Adrien Bigot http://orcid.org/0000-0002-9488-9669
Benjamin Terrier http://orcid.org/0000-0001-6612-7336
Jean-Philippe Cottier http://orcid.org/0000-0001-9166-212X
Frédéric Bastides http://orcid.org/0000-0001-6335-172X
Elisabeth Diot http://orcid.org/0000-0003-3507-8337
REFERENCES
1. Jeon CH. Relapsing polychondritis with central nervous system involve-
ment: experience of three dierent cases in a single center. J Korean Med
Sci 2016; 31: 1846-50.
2. McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing poly-
chondritis: prospective study of 23 patients and a review of the literature.
Medicine (Baltimore) 1976; 55: 193-215.
3. Hawboldt J, Bader M. Intramuscular methotrexate-induced aseptic men-
ingitis. Ann Pharmacother 2007; 41: 1906-11.
4. Török ME. Tuberculous meningitis: advances in diagnosis and treatment.
Br Med Bull 2015; 113: 117-31.
5. Ferfar Y, Mirault T, Desbois AC, Comarmond C, Messas E, Savey L, Domont
F, Cacoub P, Saadoun D. Biotherapies in large vessel vasculitis. Autoim-
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Address for Correspondence:
Julien Le Marec, MD
CHRU de Tours, Service de Médecine Interne, Université François-Rabelais de Tours, 2 bis
boulevard Tonnellé, 37032 Tours Cedex, France
E-mail: julienlemarec@msn.com
Received: 17 January 2017 / Accepted: 23 March 2017
... After adequately ruling out other possible etiologies, such as infectious diseases, tumors, paraneoplastic syndrome, and adverse drug reactions, the diagnosis of RP with CNS involvement was established by clinical signs and symptoms, cerebrospinal fluid (CSF) examinations, cranial magnetic resonance imaging (MRI) findings, and treatment responses. 11 Specifically, a diagnosis of autoimmune limbic encephalitis was made if the patient met the following four criteria: (1) subacute onset of working memory deficits, seizures, or psychiatric symptoms, suggesting involvement of the limbic system; (2) bilateral brain abnormalities on T2-weighted fluid-attenuated inversion recovery MRI that were highly restricted to the medial temporal lobes, or 18 fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET) showing a decrease in FDG uptake in medial temporal lobes; (3) a CSF white blood cell (WBC) count of more than five cells per mm 3 , or an electroencephalogram (EEG) with epileptic or slow-wave activity involving the temporal lobes; and (4) reasonable exclusion of alternative causes, such as herpes simplex virus encephalitis, HHV-6 encephalitis, glioma, status epilepticus, neurosyphilis, Whipple, human immunodeficiency virus. 12 Two of the five patients with autoimmune limbic encephalitis were tested for anti-glutamic acid decarboxylase (GAD), anti-N-methyl-D-aspartate receptor (NMDAR), anti-Hu, anti-Yo, and anti-Ri antibody. ...
Comparison of relapsing polychondritis patients with and without central nervous system involvement: A retrospective study of 181 patients
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Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center
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Relapsing polychondritis: Prospective study of 23 patients and a review of the literature
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