Neuro-Behcet's disease

Discussion:

The diagnostic criteria for Behcet's disease are all clinical, and rely upon the identification of recurrent oral ulceration, with at least two of the following: recurrent genital ulceration, eye lesions, skin lesions or positive pathergy test (minor trauma, eg phlebotomy, leading to skin lesions/ulcers that are resistant to healing). All these findings were positive in our patient. 

Neuro-Behcet's disease usually manifests itself in the 20-40 age range. The neuroradiological findings can be non-specific, but two major categories of CNS involvement are generally accepted: parenchymal and non-parenchymal. Parenchymal involvement characteristically involves the upper brainstem and extends into the thalamus/basal ganglia. Other sites of parenchymal involvement are the subcortical white matter and the spinal cord, and if there is no known history of Behcet's in these cases, the differential is wide eg sarcoidosis, connective tissue disorders, vasculitis, infectious, vitamin B12 deficiency or tumor. Non-parenchymal involvement is mainly in the form of venous sinus thrombosis.

Our patient was treated with a combination of prednisolone, methotrexate and infliximab, and has not had any further neurological relapses since.

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