White Matter Lesions in Behcet's Disease: Neuro-Behcet's Disease or Multiple Sclerosis? (P02.150)

Abstract

Objective: To evaluate patients with Behcet9s disease (BD) and white matter lesions (WML). Background BD is a systemic auto-inflammatory disorder of unknown cause. It affects CNS in around 5% of the patients, usually causing a brainstem meningoencephalitis. Occasionally patients with WML can be seen which necessitates differentiating from multiple sclerosis (MS). Design/Methods: Files of neuro-BD clinic between 1990-2010 were evaluated to find cases with BD and WML. All the MRIs were re-evaluated masked to the clinical status. Barkhof/Tintore criteria for MS were applied. Clinical characteristics of the patients were compared with the patients who had typical parenchymal neuro-BD. Results: 43 patients (32F, 11M) had WML. 22 (18F, 4M) had abnormal findings on neurological exam (Group 1); the remaining had primary headache disorders (Group2). In Group 1, 16 patient fulfilled Barkhof/Tintore criteria. One had a single lesion resembling a demyelination, and 5 had nonspecific WML. In Group 2 none fulfilled Barkhof/Tintore criteria; all showed nonspecific WML. Therefore, patients in Group 1 were compared with 63 consecutive parenchymal neuro-BD cases (Group 3; 12F, 51M). There were significantly more females in Group I. Distribution of systemic BD findings, age at onset, disease duration, attack numbers were similar in both groups. CSF oligoclonal band positivity was significantly higher in Group 1 (9/17 vs 5/47). All patients received steroids at the acute setting. As long term treatment, Group 1 patients did not respond sufficiently to azathioprine, usually interferon beta or glatiramer acetate had to be added; whereas Group 3 patients usually responded to azathioprine. Conclusions: A subgroup of the patients with BD have predominantly WML resembling MS. Although the diagnostic criteria for MS require that all other possible diagnoses should be ruled out, patients with BD and predominantly MS-like WML tend to behave like MS cases, rather than neuro-BD. This should be kept in mind when chosing disease modifying treatments. Supported by: Neuroimmunology Society, Turkey. Disclosure: Dr. Akman-Demir has received personal compensation from Merck Serono, Bayer, Novartis, Teva, for consulting or giving lectures. Dr. Mutlu has nothing to disclose. Dr. Kiyat-Atamer has nothing to disclose. Dr. Shugaiv has nothing to disclose. Dr. Kurtuncu has nothing to disclose. Dr. Tugal-Tutkun has nothing to disclose. Dr. Tuzun has nothing to disclose. Dr. Bahar has nothing to disclose.