Neurological involvement in Behçet’s disease (BD) is well recognised with a prevalence of 5.3 to 30 per cent. The purpose of this retrospective study was to analyse the clinical patterns of neuro-Behçet’s (NB) and to compare them with features of 925 NBs recorded in the literature during the same period.
All patients with NB fulfilled the International Study Group criteria for the diagnosis. Ninety-three patients with headache alone were excluded. Clinical findings were complemented by CSF studies, CT and MRI scanning and angiography.
NB was present in 16.4 per cent of BD patients. The male: female ratio was 4: 3. The mean age of NB patients was 31.76 years; the average age at onset was 29.83 years; and the mean duration was 3.81 years. The clinical features were categorised into 2 major types: those with and without parenchymal involvement. Some patients had mixed features. Ninety four patients (61.03 per cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, a pseudo-bulbar syndrome and cerebellar signs. Twenty seven patients (17.53 per cent) without parenchymal involvement were divided into: a) those with intracranial hypertension - 24 patients (15.58 percent) - presenting with headache, vomiting and bilateral papilloedema; b) those with cerebral arterial involvement - 3 cases, one with cerebral aneurysms. A mixed pattern was found in 9 patients. Erythema nodosum and vascular involvement were more frequently found with intracranial hypertension than in BD without neurological involvement.
Other clinical features that were reported included a pure meningeal pattern in 15 cases, a pure peripheral neuropathy in 4 cases, isolated cranial nerve palsies in 8 cases, chorea in 1 patient and pseudo-tumor of the cervical cord in another. Six cases of juvenile BD and 1 instance of familial BD were found.
One hundred and thirty three patients were treated (86.36 per cent). The mean duration of treatment was 7.46 months. Corticosteroids, immunosuppressive agents (cyclophosphamide, azathioprine, chlorambucil) and anticoagulants (in intracranial hypertension) were used.
The course of the disease was favourable in only 54.13 per cent of cases and poor in 18.79 percent.
The paper concludes that NB occurrs frequently in men and and is more severe, especially when there is parenchymal involvement. 7 deaths were recorded of which 6 had the parenchymal form of the disease.
© 2006 Elsevier Masson SAS. Tous droits réservés.