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CEREBROVASCULAR EVENTS UNMASKING A RARE BUT TREATABLE DISEASE
  1. L Zhang*,
  2. DC Paviour,
  3. U Khan
  1. St George's Hospital NHS Trust

    Abstract

    Behcet's disease is a systemic vasculitis with an unknown etiology. Central nervous system involvement occurs in 5–10% of cases. We report two cases with different neurological presentations.

    Case 1 A 26-year-old man, presented with sudden onset of head and neck pain, unsteady gait, and a right hemiparesis with sensory loss. MRI showed swelling of the left side of the Pons and midbrain. DW-MRI showed intermediate high signal, suggesting a vascular event. He was treated with anti-platelets and made a full recovery. MRI at 4 months showed signal abnormality consistent with haemosiderin. He represented 18 months later with an acute onset headache, diplopia, dysarthria and ataxia. MRI showed brainstem swelling and a small hemorrhage in the Pons. A review revealed a history of genital ulceration and the presence of a mouth ulcer.

    Case 2 A 26-year-old man, presented with a headache worsening over 6 months with 2 weeks of blurred vision. He had been taking tetracycline for acne for 2 years. He had papilloedema and bilateral VI nerve palsies. After an unremarkable MRI, a lumbar puncture (LP) confirmed intracranial hypertension with normal csf. Tetracycline was stopped. His headache returned a month later. Questioning revealed an episode of painless loss of vision in his right eye as a teenager. Examination revealed new mouth and genital ulcers. A repeat MRI revealed cerebral venous sinus thrombosis.

    Behcet's is a rare condition. In both cases phenotypic features of the disease had been present but their significance missed until the patients presented with neurological symptoms.

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