The Clinical Features and Risk Factors of Parenchymal Neuro-Behcet’s Disease

Abstract EN

To investigate the clinical features of parenchymal neuro-Behcet’s disease (p-NBD), we retrospectively reviewed the medical records of 1009 BD patients admitted to Peking Union Medical College Hospital from 2000 to 2016.

Forty-two patients (25 males and 17 females) with p-NBD and eighty-four age- and sex-matched BD patients without neurological involvement who were served as controls were enrolled.

Neurological onset was concomitant with the onset of BD in six cases (14.3%).

Pyramidal signs (50.0%) and headache (33.3%) were the most common manifestations.

On MRI, the lesions were mainly in the midline structures and hyperintense in the T2-weighted image.

The most common lesion was the brainstem (54.8%).

Spinal cord involvement was observed in five cases, four of which with cervical cord involvement.

Multifocal lesions were observed in 13 patients.

Ocular involvement was more prevalent in p-NBD (35.7%) (P=0.041, OR=2.36, 95% CI=1.03-5.44) compared with controls.

All patients received corticosteroids and immunosuppressants, mainly cyclophosphamide (39/42).

Six patients with severe/refractory condition received biological agents and achieved response measured by decreased Rankin score (P=0.002).

With a median follow-up of 28 months, 22 patients (61.1%) achieved clinical improvements, while 10 (27.8%) relapsed and 4 died (mortality rate 11.1%).

p-NBD is a rare yet disabling and life-threatening complication of BD.

Ocular involvement is a risk factor for p-NBD.

Promptly aggressive treatment is essential for improving prognosis, and biological agents might be a promising approach for severe/refractory p-NBD.