Online edition:ISSN 2758-089X

Neuro-Behcet’s Syndrome and Brain Stem Encephalitis. A Neuropathologic Study

The neuropathologic findings of a case of neuro-Behcet's syndrome were described in comparison with those of a case of brain stem encephalitis of Iizuka type and the pathogenesis of the central nervous system lesions of the former case was discussed. The patient was a 51-year-old man who had recurrent genital ulcers from an early age. Nine and a half years before death he noticed visual disturbance. Fourteen months before, dysarthria, gait disturbance and mental deterioration developed together with oral ulcers and skin eruptions. Neuropathologic examination revealed widespread demyelinated lesions and gliosis scattered with small softened lesions and perivascular lymphocytic infiltrations mainly in the brain stem. In addition, small softened lesions were also observed in the cerebrum, cerebellum, thalamus and basal ganglia. These findings were quite similar to those for the case of brain stem encephalitis of Iizuka type. It was therefore suggested that brain stem encephalitis of Iizuka type might be a subtype of neuro-Behcet's syndrome lacking ocular and mucocutaneous lesions. The central nervous system lesions in the case of neuro-Behcet's syndrome were suspected to have been induced not by a secondary circular disturbance due to vasculitis but by the effect of some kind of noxa resulting from a perivascular immunological reaction.

Shirabe T, et al