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Relapsing “encephalo” polychondritis
  1. Sarosh R Irani1,
  2. Anushka Soni2,
  3. Huw Beynon3,
  4. Bal S Athwal4
  1. 1Senior House Officer in Neurology, Academic Department of Clinical Neurosciences, Medical School, Royal Free Hospital, London, UK
  2. 2Senior House Officer in Rheumatology, Nuffield Orthopaedic Centre, Oxford, UK
  3. 3Consultant Rheumatologist, Rheumatology Department, Royal Free Hospital, London, UK
  4. 4Consultant Neurologist, Academic Department of Clinical Neurosciences, Medical School, Royal Free Hospital, London, UK
  1. Correspondence to:
 Dr S R Irani, Academic Department of Clinical Neurosciences, Upper Third Floor, Medical School, Royal Free Hospital, London, UK;
 saroshirani{at}doctors.net.uk

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Inflammed auricular cartilage in relapsing polychondritis.

We were referred a 65 year old right-handed Cypriot gentleman who had presented with subacute onset of confusion. Two months beforehand he had been managing his shop and accounts independently. Two years previously he had developed bifrontal, dull headaches, associated with fever and swelling of the small joints in his hand, which all resolved over some weeks. Six months later he then experienced myalgia and finger arthralgia lasting for two weeks, again resolving spontaneously. Twelve months after that he complained of painful, reddened, tender, and swollen ears that worsened gradually over weeks. At the same time there was redness and pain of both eyes with no visual impairment. Over this period, he lost 14 lb in weight and developed a persistent fever. These recent symptoms had only partially settled when he developed fluctuating headache, confusion, and deterioration in gait worsening over four weeks. The major cognitive features were memory loss, personality change, aggression, and paranoia. He was found lost and wandering in familiar places and his family began to supervise him at all times. His clinical state then followed a fluctuant course with periods of aphasia and psychomotor retardation. There was no neck stiffness, incontinence, seizures, genital or oral ulceration, rash or cranial nerve or limb dysfunction.

His previous medical history was unremarkable, apart from mild hypertension well controlled on atenolol. There was a 20 pack-year history of smoking and he drank little alcohol. He travelled to Cyprus annually, last doing so two months before presentation. There was no family history of any similar symptoms, or indeed of any neurological or “rheumatological” disease.

ON EXAMINATION

He had …

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