[Neurological Behcet syndrome. Presentation of three cases]

Behçet's disease is a multisystemic illness in which neurological abnormalities are seen in 5-48% of cases.

We present 3 patients (2 men and 1 women) diagnosed as having Behçet's disease, according to the criteria of the 'International Study Group for Behçet's Disease' of 1990. We describe the clinical findings in these patients, the results of investigations done (MR or CT, evoked potentials-PEV, PESS, PEATC-EEG, ECN, CSF and neuropsychological tests), their course and response to treatment with hyperimmune human gammaglobulim at a dose of 400 mg/kg body weight in 2 patients. All three patients had repeated episodes of meningitis and alterations of the cranial nerves; two patients had epileptic seizures and two had signs of cerebellar disorders and dementia at some time during their illness. One patient died and the other two survived with severe disabilities. Cranial CT showed a cerebral infarct in one patient; evoked potentials showed axon damage in the two patients in whom this was studied. In the only patient in whom ECN was done, signs of axonal polyneuropathy were found. In all three patients there was pleocytosis in the CSF. In the patients in whom hyperimmune human gammaglobulin was given, the results were: one improved after this treatment; in the other there was clinical deterioration, but this was considered to be secondary to a curettage done the day before.

The presence of neurological findings in Behçet's disease not caused by cerebral vein thrombosis worsens the prognosis of these patients.