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AB0101 Transverse myelitis: a rare manifestation of behÇet’s disease
  1. C Macieira,
  2. C Miranda Rosa,
  3. JC Teixeira da Costa,
  4. M Viana Queiróz
  1. Unit of Rheumatology and Metabolic Bone Diseases, Santa Maria Hospital, Lisbon, Portugal

Abstract

Background The diagnosis of Behçet’s disease is based on well-known criteria. Although skin, eye and joints are the most frequently involved organs, other systems may be affected such as the nervous system. Transverse myelitis is a rare and serious manifestation of the disease.

We present the case of a 51 year-old woman with a 2 year diagnosis of Behçet’s disease, initially presented as oral aphtosis, genital ulcerations, symmetrical polyarthritis and a maculo-papular hand rash. Eye examination was normal.

The patient remained well for 2 years, when suddenly presented with severe headache, back pain, a rapidly progressive tetraparesia and a global sensitive deficit.

Erythrocyte sedimentation rate, C-reactive protein, total blood cell count, lisozyme, angiotensin-converting enzyme and complement factors were within the normal laboratory ranges. The serologic tests for rickettsial and Lyme?s diseases were negative. The immunological study was positive for anti-Scl70 antibody and lupus anticoagulant.

Cerebrospinal fluid analysis revealed a 48-cell count with predominance of mononuclear forms and normal cultural and pathology examinations.

Spinal cord magnetic resonance imaging (MRI) identified a cervico-thoracic lesion suggestive of transverse myelitis. The cranial MRI was normal.

Pulsed methylprednisolone (1 g/day) was initiated, with rapid clinical improvement. After 45 days, with a maintenance dose of prednisone (30 mg/day) the patient was doing well referring residual sensitive deficit. With a one-year follow-up and daily prednisone (20 mg) and azathioprine (50 mg), the patient is assymptomatic and there are no neurological sequelae.

A literature review was performed. Central nervous system involvement occurs in less than 5% of cases of Behcet’s disease and in this group 69% have clinical manifestations suggestive of transverse myelitis. High methylprednisolone doses are fundamental for favourable clinical outcome.

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