Beçhet’s disease (BD) is a chronic, heterogeneous, multisystem disease that affects young males and females around the Mediterranean region, as well as from Far and Middle East. Its etiology is vague with vasculitis being its main pathological feature. International diagnostic criteria have been established and they require the presence of recurrent oral ulcerations plus two of the following: Recurrent genital ulceration, eye lesions, skin lesions and positive pathergy test. A significant number of patients with Beçhet’s disease suffers from symptoms from the central nervous system (CNS), while the most common clinical symptoms are pyramidal signs, mental-behavioral changes, hemiparesis and brain stem syndrome. The existence of mental-behavioral changes seems to be one of the most common findings in patients with Neuro-Beçhet (N-BD). These changes seem to be related with memory and attention deficits, and the process of deterioration continues even in attack-free periods, suggesting a continuously active disease process in the CNS. The prevalence of anxiety, depression and general psychiatric symptoms is higher among patients with BD compared to healthy individuals. However, the association between psychiatric symptoms and BD is not clearly understood. On the other hand, syndromes like psychosis or bipolar disorder appear to be less frequent, especially in attack-free periods. We describe the case of a 52-year old woman with Beçhet‘s disease who developed a single manic episode 13 years after the onset of Beçhet‘s disease. A 52-year old woman, suffering from Beçhet‘s disease since the age of 39, developed manic symptoms, namely elevated mood, pressured speech, flight of ideas, distractibility and decreased need for sleep. The above symptoms developed during a period that no other symptoms of Beçhet‘s disease were present. Moreover there was no other manifestation from the nervous system. A brain MRI was unremarkable, while a brain SPECT study revealed severe hypoperfusion of the left prefrontal cortex. Neuropsychological examination revealed severe disturbance in attention, working memory and learning ability, while her visuaspatial ability and executive functions were well spared. Her symptoms were well controlled after treated with quetiapine 800 mg. The manic episode developed in the absence of any neurological manifestation (Neuro-Beçhet), or other symptom of Beçhet‘s disease, and was clearly distinguishable from euphoria, disinhibition or irritability that are common in patients with Beçhet‘s disease. It looks probable that CNS damage caused by the disease constitutes a biological substrate for the development of manic episodes in patients suffering from Beçhet’s disease.

Key words: Beçhet’s disease, manic episode, SPECT, neuropsychological impairment. 

V. Bozikas, A. Ramnalis, J. Dittopoulos, J. Iakovou, G. Garyfallos, K. Fokas (page 295) - Full article (Greek)