Relapsing polychondritis revealed by basal ganglia lesions

Relapsing polychondritis (RP) is characterized by recurrent episodes of potentially destructive inflammation of cartilaginous structures (ears and nose), and of the cardiovascular system. Central nervous system involvement in RP is rare but can lead to serious complications.We describe an unusual case presenting association of RP with extensive deep brain lesions that initially manifested as severe apathy and parkinsonism. A 67-year-old man presented with a 2-month history of severe lack of initiative, eye tenderness, and metacarpophalangeal joint pain. Physical examination revealed bilateral episcleritis and moderate, symmetric parkinsonian signs (plastic rigidity and limb and axial hypokinesia without rest or postural tremor), and brisk tendon reflexes. Cognitive evaluation revealed severe apathy, reduced fronto-executive abilities, without depression or overt dementia. Sedimentation rate was elevated (53 mm/hour). Brain magnetic resonance imaging (MRI) showed extensive bilateral, hyperintensities on T2-weighted images and a heterogeneous gadolinium enhancement in deep gray matter nuclei (Fig. 1). MR spectroscopy (pointresolved spectroscopy [PRESS] at echo time [TE] 288 ms) revealed an increased choline/creatine ratio at 2.1 and lactate less than 2.5 mmol (lactate/Cr < 0.3) in both putamen, suggestive of inflammation. Cerebrospinal fluid (CSF) analysis, extensive autoimmune testing, thoracic computed tomography (CT), and echocardiogram revealed no abnormalities. The treatment decision was deferred because of spontaneous improvement of the apathy. A few months later the patient presented a painful bilateral ear swelling and persisting parkinsonism (Fig. 2). MRI showed diminishing cerebral lesions with persistent gadolinium enhancement. A Gallium-67 scintigraphy showed increased tracer accumulation in both external ears (Fig. 3). Auricular biopsy showed a dense inflammatory infiltrate formed of lymphoplasmacytes and macrophages. He had auricular chondritis, episcleritis, and seronegative arthritis, thus meeting the diagnosis criteria of RP. He was started on maintenance treatment with prednisone (1 mg.kg .day , per os). Clinicoradiological response was excellent and no further relapses were recorded. Three years later, the neurological examination was normal, while MRI revealed small periventricular abnormalities. The reported case highlights the difficulty of diagnosis of RP and the variety of neurological manifestations. Central nervous system (CNS) involvement, such as nonparaneoplastic encephalitis, cerebellar dysfunction, myelitis, or cranial nerve palsy has been reported, but it is uncommon and not included in the diagnosis criteria. The current manuscript adds another case of parkinsonism in a RP patient. Despite negative extensive workup, we can not exclude the possibility that a coexisting, undiagnosed autoimmune disease could have been responsible for this patient’s neurological features. Corticosteroids continue to be the mainstay of clinical management of RP but a wide array of immunosuppressive drugs has been used in case reports. While spontaneous resolution is rare, this patient’s apathy did improve without treatment. However, the parkinsonism only improved with chronic steroids, a treatment which also allowed a complete reversion of the neurological and systemic features to normality, with no relapses. RP may follow a fluctuating progression that challenges differential diagnosis. The present case demonstrates that parkinsonism and apathy may be found -----------------------------------------------------------*Correspondence to: Dr. Teodor Danaila, Department of Neurology, University-Hospital, Avenue de la Côte de Nacre, 14033, Caen cedex, France; danaila-t@chu-caen.fr Relevant conflicts of interest/financial disclosures: Nothing to report. Full financial disclosures and author roles may be found in the online version of this article.