Department of Internal Medicine and Inserm unit 644 and 1 Department of Neurology, Centre hospitalier universitaire de Rouen-Boisguillaume, 76031 Rouen Cedex, France
Correspondence to: I. Marie, Department of Internal Medicine, Centre hospitalier universitaire de Rouen-Boisguillaume, 76031 Rouen Cedex, France. E-mail: isabelle.marie{at}chu-rouen.fr
SIR, Relapsing polychondritis (RP) is a systemic disorder, which is characterized by recurrent inflammation and destruction of cartilage structure [1, 2]. Auricular, nasal, ocular, tracheobronchial and articular impairment are well-recognized manifestations of RP [1, 2]. Neurological involvement is considered to be rare in RP, and usually occurs during the course of the disease [17]. We recently observed a case which is of particular interest, since the patient developed facial diplegia revealing RP.
A 63-yr-old woman presented with a 3-day history of facial diplegia. She had a 2-yr history of bilateral auricle chondritis of unknown non-infectious origin, which had been successfully treated with steroids for 6 months. At admission, the patient had no fever and physical examination showed bilateral seventh nerve palsy; general physical examination was otherwise normal. Laboratory studies disclosed the following: erythrocyte sedimentation rate 36 mm/h, C-reactive protein 10 mg/l, haemoglobin 8 mmol/l, white blood cell count 7.1x109/l, platelets 387x109/l. Findings of renal and liver tests were normal, as was the fasting plasma glucose level (4.6 mmol/l). Other biochemical tests yielded normal values for blood electrophoresis and glycosylated haemoglobin (HbA1c) (5%); microalbuminuria and urinary protein excretion were also negative. Cerebral computed tomography (CT) and magnetic resonance imaging (MRI) were within normal limits. Cerebrospinal fluid analysis showed protein level 0.45 g/l, glucose level 3.2 mmol/l, white blood cells 9/mm3, red blood cells 19/mm3; the IgG index was normal, without oligoclonal bands. Gram staining, bacterial and mycobacterial cultures of the cerebrospinal fluid remained negative. Bacterial (Treponema pallidum, Borrelia burdorferi) and viral (Herpes simplex virus, human immunodeficiency virus) serologies were also negative. Autoantibody screening tests, i.e. rheumatoid factors, antinuclear antibodies, anticardiolipin and antiphospholipid antibodies, lupus-like anticoagulant, antineutrophil cytoplasmic antibodies, anticartilage and anticollagen II antibodies, serum angiotensin-converting enzyme and cryoglobulin were negative.
On the second week after admission to the hospital, the patient developed both conjunctivitis and episcleritis of the right eye, and then hoarseness of voice and polyarthritis involving the hands, wrists, elbows and knees within 3 weeks. Funduscopy was normal (e.g. diabetic retinopathy was absent) and otorhinopharyngeal examination showed no abnormality. Other investigations, i.e. electrocardiogram, trans-oesophageal echocardiography, cerebral arterial Doppler, electroencephalography, brainstem auditory and visual evoked responses, electromyogram, accessory salivary gland histological examination and both thoracic and abdominal CT, were within normal limits. The diagnosis of RP-related facial diplegia was made because of the following manifestations: (i) there was no evidence for other causes of facial diplegia; (ii) the 2-yr history of bilateral auricle chondritis of unknown non-infectious origin with favourable outcome after institution of steroid therapy; (iii) ocular inflammation; (iv) non-erosive seronegative inflammatory polyarthritis; and (v) hoarseness of the voice related to laryngeal cartilage inflammation. The patient was treated with prednisone (at an initial dose of 1 mg/kg daily), resulting in resolution of the facial diplegia, ophtalmological impairment, hoarseness of the voice and joint symptoms. At 3-months follow-up, the patient remained free of all clinical features, receiving steroid therapy at a dose of 35 mg daily.
Central nervous system involvement is rare in RP, being encountered in 09% of patients [17]. Neurological impairment may lead to life-threatening complications in patients with RP, such as hemiplegia, cerebellar dysfunction, aseptic meningoencephalitis, rhombencephalitis, limbic non-paraneoplastic encephalitis, polyneuritis, myelitis or cranial nerve palsy [17].
Our case is original in that neurological involvementi.e. facial diplegiarevealed RP. This is, to the best of our knowledge, the first case of facial diplegia associated with RP. The diagnosis of RP-related bilateral seventh nerve palsy could be made in our patient, since the extensive search for other causes (e.g. diabetes mellitus, LandryGuillainBarré syndrome, infections and other connective tissue diseases) was negative, and all manifestations resolved after initiation of steroid therapy. We suggest therefore that facial diplegia may be included within the spectrum of RP-related neurological manifestations. Because neurological involvement may precede other systemic signs, our findings also underscore that when unexplained neurological symptoms, including facial diplegia, are observed in patients, a clinical evaluation for an underlying RP may be done.
Finally, although MRI provides a useful method for diagnosis of cerebral vasculitis, it failed to demonstrate damage consistent with RP-related cerebral vasculitis in our patient (e.g. high signal intensity of the white matter/cortex on T2-weighted images, infarcts or cerebral atrophy). Recently, new brain imaging techniques have proven their superiority in the detection of connective tissue disease-related [mainly systemic lupus erythematosus (SLE)] cerebral vasculitis at an early stage. In patients with neuropsychiatric SLE and normal brain MRI, few investigators have therefore observed alterations in cerebral function: i.e. [810] (i) multifocal areas of low glucose metabolism, using FDG positron emission tomography (FDG-PET); (ii) patchy areas of cerebral blood flow defects with HMPAO single photon emission tomography (HMPAO-SPECT); and (iii) low N-acetyl aspartate/creatine ratio on magnetic resonance spectroscopy (MRS). These tests are not yet available in our university hospital; they would have probably revealed RP-related cerebral dysfunction in our patient. These data confirm that brain FDG-PET, HMPAO-SPECT or MRS should be performed in patients with connective tissue diseases presenting with neurological signs and inconspicuous brain MRI, in order to allow appropriate management [10].
The authors have declared no conflicts of interest.
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