Rapid Atypical Progression of Neuro-Behçet's Disease Involving Whole Brainstem and Bilateral Thalami

Abstract

We present a case of Neuro-Behçet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

Keywords: Behçet's disease; Central Nerve System manifestation; Neuro-Behçet's disease; Neurological deterioration.

Fig. 1

Abnormal high signal intensity in both T1 weighted image (A) and T2 weighted image (B) in the left pons and left mid brain (cerebral peduncle). At this time, abnormal signals were noted only in small limited portion of the brainstem.

Fig. 2

This brain MRI scan taken at hospital at 3 days post-presentation revealing abnormal signal intensity initially observed in the brainstem that were not enhanced strongly (pre-enhanced T1 weighted image (E) and post-enhanced T1 weighted image (F). It had extended to the whole brain stem and the both thalami, both basal ganglia, periventricular white matter, both mid brain cerebral peduncles, and involved the whole stem, whole ventral and dorsal aspects, and that edema associated with the lesion was compressing surrounding tissue (A and B : Axial fluid-attenuated inversion-recovery (FLAIR) images, C : Sagittal FLAIR image, D : Coronal FLAIR image).

Fig. 3

Brains MR scan taken one month later showing a marked reduction in the abnormal signal as compared with the scan taken at 10 days without brain swelling. Minimal abnormal signals were noted in the mid brain bilaterally. (A and B : Axial T1 and T2 weighted image, C : Sagittal FLAIR image, D : coronal FLAIR image).