Neuro-Behcet’s Disease

This 38 year old woman came to the ED with a 2 wk history of progressively escalating headache. After she was admitted, she complained of L sided numbness, clumsiness and weakness.

Three yrs before this, she had developed a painful monarthritis of the left knee with acute erythema nodosa. Six months later she developed oral and vaginal ulcers, and she was diagnosed with Bechet’s disease and treated for 1-yr with colchicince. She had no recent symptoms from Bechets. She also had a histry of classical migraine headaches.

Her exam showed L facial weakness, L pronator drift, and L sided dsymetria.

Her brain CT was normal. Her lumbar puncture showed a marked pleocytosis with almost 500 WBC, half polys. MRI of the brain showed a mildly ring enhancing lesion that extended from the R midbrain into the R thalaus:
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She was treated with high dose intravenous steroids and her symptoms improved over the next 5-days.

Bechet’s disease is a rare immune-mediated systemic vasculitis that presents with oral aphthous ulcers, painful genital ulceration around the anus, vulva, or scrotum and erythema nodosum, and uveitis.
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Neurological involvement can include aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss. Brainstem lesions, particulary lesons extending from the midbrain into the diencephalon, as in our case, are most common.
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Neuro-Behcet’s patients are usually treated with corticosteroids. Azathioprine, cyclophosphamide, chlorambucil and cyclosporin A are often added as steroid sparers, because relapses are common when steroids are discontinued.

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