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  • Title: [Neuro-Behçet's syndrome: encephalitis and cerebral venous thrombosis--clinical aspects and neuroradiology of 5 cases].
    Author: Huss GP, Mattle H, Wessel K, Mumenthaler M.
    Journal: Nervenarzt; 1992 Jun; 63(6):352-8. PubMed ID: 1635618.
    Abstract:
    The value of brain CT-scan, magnetic resonance imaging (MRI) and angiography for diagnosis, differential diagnosis and follow up in Neuro-Behçet-Syndrome is assessed in 5 cases. Three of the patients presented with clinical signs of encephalitis. Further investigations led to the diagnosis of Behçet-Syndrome. CT-scan was negative in two of these cases, but MRI showed multiple, predominantly periventricular lesions with high signal intensity on T2-weighted spin-echo images in all three. Clinical symptoms improved with steroid and chlorambucil therapy in all three cases. In two patients the MRI-lesions resolved at least partially after 1.5 and 3 years of treatment respectively. In one patient the initial MRI-findings were still present after 7 months of treatment. The other two patients presented with headache, papilledema and increased CSF-pressure. The cause was superior sagittal sinus thrombosis, confirmed by angiography in both cases. Additional symptoms appeared later and led to the diagnosis of Behçet syndrome. One patient died of pulmonary aneurysms 28 months after the diagnosis had been established. The course of disease of the remaining patient is so far favorable.
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