Neuro-Behcet's disease

Case contributed by Yune Kwong
Diagnosis certain


Under treatment by opthalmologists for bilateral uveitis of ‘unknown’ cause. Now presents with acute left hemiplegia.

Patient Data

Age: 30 years
Gender: Male

MRI shows high signal intensity around the posterior limb of the right internal capsule, extending superiorly into the centrum semiovale, and inferiorly into the cerebral peduncle. Similar changes are seen on the left side, but these are less prominent. No evidence of restricted diffusion. Following gadolinium administration, patchy foci of enhancement are seen around the right internal capsule.

Summary of ancillary investigations.

1. HLA type B51 positive (known increased incidence in Behcet's disease).
2. CSF: mixed leukocytosis (130), raised protein (1.1g) and glucose of 2.5 (serum 5.3). Acid-fast staining for cerebral TB was negative. PCR for HSV, VZV and enteroviruses were also negative.
3. HIV serology, syphilis serology, ANA and ANCA were negative. Serum ACE and calcium in normal range.
4. Chest X-ray normal with no evidence of sarcoidosis.


Follow up study 3 years later

On this follow up MRI, gliotic changes are seen in the right internal capsule and centrum semiovale. There is also volume loss of the right cerebral peduncle and along the pyramidal tract, reflecting Wallerian degeneration along the corticospinal tract. No enhancement is seen to suggest active disease.

Case 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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