Neuro-Beh et, pseudotumor cerebri and ocular signs: a rare association []

[english] Introduction: The central nervous system involvement in Beh et’s disease occurs in 5–30% of cases. The diagnosis of pseudotumor cerebri is even rarer (only 22 cases reported worldwide). Purpose: To emphasize the importance of differential diagnosis in a case of pseudotumor cerebri in the context of ocular inflammation. Methods: V.A.V.R., a 31 year old female, was diagnosed with pan-uveitis on the left eye associated with recurrent bipolar aphthosis. During the etiological investigation, there was an onset of a left hemiparesis and facial palsy. Results: The central nervous system (CNS) neuroradiological investigation revealed a space-occupying lesion within the right hemisphere with intense signal enhancement with gadolinium. It globally reached the nucleo-basal structures and induced deviation of the middle structures (including homolateral ventricle). Cytochemical analysis of cerebrospinal fluid (CSF) was negative for atypical cells. The ophthalmological features regressed with the corticosteroid and immunosuppressive therapy instituted. The final diagnosis was of pseudotumor cerebri in the context of Beh et’s disease. Conclusion: In Beh et’s disease, a cerebral space-occupying lesion should lead to a diagnosis of pseudotumor cerebri. The correct diagnosis will determine an appropriate therapy and may prevent an inappropriate neurosurgical approach. The cortico and immunotherapy allowed a substantial regression of the lesion.