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Questions posted in the
Neurology and Neurosurgery Forum have been answered by doctors from The Cleveland Clinic Foundation.
Question Title: Behcets and CNS InvolvementForum: Neurology Forum
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I have diagnosed with SLE and Behcets. I have seizures because of the SLE that have been fairly well controlled mostly by prednisone. After developing huge hard palate mouth sores, nasal sores and sometimes genital sores, the doctors diagnosed Behcets. I am being encouraged to try Thalidomide and will probably start this as of the beginning of the year. I do know that Behcets can affect the CNS also and Im wondering what the symptoms might be and how would you differenciate the SLE from the Behcets? Is the treatment the same? The docs are concerned since I also have slight kidney involvement and the ulcers are now appearing on my hands and feet. It seems that each time these terrible ulcers and joint pain, excruciating headaches occur the only thing that alleviates them at all is prednisone in fairly large doses, is this the normal treatment for these two diseases? It is very difficult to research this myself as there is very little information on Behcets to be found. Thank you for your time and this wonderful forum! Dear Kelsey: I am sorry to hear of your illness. It is very unusual to have two distinct diseases at the same time, especially since both are autoimmune. Since both are autoimmune diseases, both are treated with agents that will suppress the immune system such as steriods. There is no combination of historical or laboratory finding that are specific for Behcet's disease. The criteria for diagnosis depends on the presence of oral ulcerations plus two of the following: genital ulcerations, typical defined eye lesions, typical defined skin lesions, or a positive pathergy test. Men are affected two to three times more often that females. Neurologic involvement is common, occurring in up to 30% of the patients, within 5 years of diagnosis and is the initial presentation in 5%. The intermittency of the neurologic features is one of the most characteristic findings. Any portion of the neuraxis may be involved and neurologic complication can vary from one relapse to the next. Three common patterns of central neurologic involvement are noted: a brainstem syndrome affecting many cranial nerves, a meningoencephalitic syndrome and an organic confusional state. Meningoencephalitis can occur in put to 12 percent as the presenting neurologic picture and can vary from an uncomplicated aseptic meningitis to a fulminant and fetal meningoencephalomyelitis. Typically, fever, headache, meningismus, and CSF pleocytosis are present. The treatment is difficult. Topical steroids for oral, genital and ocular lesions are recommended. Corticosteroids remian the mainstay of systemic tratment and are used alone or in combination with other immunosuppressant agents. I had to be an antagnoist, but since both diseases are autoimmune, and both can present similarily, I would think about getting a second opinion about the Behcet's disease diagnosis. Not having diagnosed you, or having examined you, but it seems that you do not fit the entire criteria for Behcet's. Thalamide is a viable alternative treatment, I am assuming that your on birth control pills or other means of controlling the possibility of pregnacy. Best of luck and let us know if we can help. Sincerely, CCF Neuro[P] MD, RPS
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