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Clinical characteristics and prognosis of Neuro-Behçet’s disease

Objective Neuro-Behçet’s disease (NBD) is a rare manifestation of Behçet’s disease (BD) and may cause severe disability. The aim of this study was to evaluate the treatment response in patients with NBD and to investigate the parameters that may influence the prognosis of the disease in patients with severe to mild-moderate disability. Methods The files of 60 patients admitted to our outpatient clinic for NBD between January 2007 and June 2014 were retrospectively reviewed. We compared the BD duration, time to NBD, NBD type and course, clinical findings of BD, functional neurological system involvement, localization of lesions on brain MRI, and all the medications between the severe and mild-moderate disability groups. Results The mean time to the onset of NBD was significantly longer (17.8±4.6 years) and the mean age was significantly higher (50.25±9.1 years) in patients with severe disability than in those with mild-moderate disability (7.5±8.0 years and 37.5±10.9 years; p=0.01 and p=0.03, respectively). Moreover, hemispheric involvement was significantly associated with severe disability (p=0.006). No difference was found with regard toother investigated parameters between the groups. Conclusion We believe that severe neurological disability may be associated with older age at the onset of NBD or longer time to NBD and hemispheric lesions on brain MRI. However, our results should be cautiously evaluated with further research.

For each NBD patient, information such as the age, sex, clinical findings of BD, date of diagnoses of BD and NBD, type and course of NBD, all medications, and functional neurological system involvement (assessed using the Kurtzke Functional System Scale for each system) was collected. BD duration was defined as the time from diagnosis of BD to the last examination. Period from the diagnosis of BD to the onset of the symptoms of NBD was defined as the time to NBD.
The severity of NBD was evaluated using the modified Rankin Scale (mRS) scores at the last examination. ThemRS score is commonly used for measuring the degree of disability or dependence in thedaily activitiesof individuals who have suffered a strokeor have other causes of neurological disability (0: no symptoms; 1: no significant disability; 2: slight disability; 3: moderate disability; 4: moderately severe disability; 5: severe disability; 6: dead). In addition, the localization of lesions on brain magnetic resonance imaging (MRI) was also recorded.
The patients with mRSscoresof 0-3 at the last examination were included in the mild-moderate disability group and those with scores of 4-6 in the severe disability group. We compared the BD duration, time to NBD, type and course of NBD, clinical findings of BD, functional neurological system involvement, localization of lesions on brain MRI, all medications, and mRS scores at the last examination in the severe and mild-moderate disability groups.
While 56 cases had mild-moderate disability, 4 had severe disability. The mRS score was 4 in 3patients and 5 in 1 patient with severe disability; 2 had a progressive and others had a relapsing course; 3 of 4 patients were male, and 1 was female. The mean age at the onset of NBD was 37.5±10.9 years in the mild-moderate disability group and 50.25±9.1 years in the severe disability group; the difference was statistically significant (p=0.03). The mean time to NBD was significantly longer (17.8±4.6 years) in patients with severe disability than in those with mild-moderate disability (7.5±8.0 years; p=0.01). All the patients with severe disability had parenchymal involvement, as seenon brain MRI, and pyramidal signs on examination (Table 2). Moreover, hemispheric involvement was significantly associated with severe disability (p=0.006). No differenceswere found in the age of BD onset, sex, mean duration of BD, type and course of NBD, clinical findings of BD, neurological functional system involvement, and treatment modalities between the groups (p>0.05 for each; Table 1).
Akman-Demir et al. (4) reported that one-third of their patients had severe sequelaedue to NBD. Parenchymal involvement, elevated protein level and/or pleocytosis in CSF, brainstem involvement with other brain regions, primary or secondary progressive course, and relapse during steroid tapering were associated with a poor prognosis (4). Siva et al. (19) reported that 45.1% of patients with NBD had EDSS of ≥6 (indicating the need of a walking aid) in 10 years from the onset of BD. Cerebellar symptoms at the onset or progressive course were associated with unfavorable outcomes, whereas a headache at the onset or diagnosis of CVT was associated with favorable outcomes (19). In contrast to these reports, in our study population, only 7% of patients had severe disability. In agreement with previous treatment recommendations (32), all our patients were treated with oral or IV high-dose prednisolone in case of acute attacks. Steroid treatment was continued for at least 6 months after acute attacks,immediately followed by preventive treatment; therefore, we did not observe any recurrence due to early steroid tapering. Because only a small percentage (7%) of our patients had a poor outcome, we speculated that the recommended treatment modality resulted in a better prognosis, unlike in previous reports that showed a worse prognosis (4,19). Peno et al. (13) suggested that delayed treatment and younger age could lead to an aggressive disease. In contrast to Peno et al. (13), we found that older age at the onset of NBD had a worse prognosis. This issue should be evaluated with further research. We also found that the time to NBD was longer in the severe disability group than in the mild-moderate disability group. Probably, when NBD begins at an older age, the time to NBD becomes longer; therefore, both age at the onset of NBD and time to NBD may have the same implications.
In other words, when brain involvement is seen relatively early based on the onset of BD or when NBD begins at a younger age, the response to the suggested treatment modality may be better; therefore, the prognosis may also be better. Conversely, when NBD is seen later based on the onset of BD or when NBD begins at an older age, the response to the suggested treatment modality may not be sufficiently good;therefore, the outcome may be worse.
In conclusion, severe neurological disability may be associated with older age at the onset of NBD or longer time to NBD and hemispheric lesions on brain MRI. Our results confirmedthe success of previous treatment recommendationswhich stated that high-dose steroids should be immediately administered in case of an acute NBD attack, steroid treatment should be continued for at least 6 months after the attack, and other immunosuppressive agents should also be administered after the attack to prevent recurrence and progression of NBD in patients with younger age or those with a shorter time to NBD. However, in patients with an older age at the onset of NBD or in those with a longer time to NBD, this treatment modality may be unsuccessful. However, our results should be cautiously evaluated because our severe disability group included only four cases; to decide whether the results represent all the older onset NBD or longer time to NBD cases, further research that includes more patients with severe disability is needed. Informed Consent: Written informed consent was obtained from all the patients who participated in this study.
Peer-review: Externally peer-reviewed. Financial Disclosure: The authors declared that this study has received no financial support.