Behcet's syndrome: a report of 41 patients with emphasis on neurological manifestations

Abstract

Forty one patients with the clinical diagnosis of Behçet's syndrome from two teaching hospitals in Kuwait were studied. There were 34 male and seven female patients. Age at presentation ranged from 14 to 48 years. Neurological manifestations were present in 24 patients. Eleven patients showed evidence of increased intracranial pressure, and 10 of these had radiologically confirmed dural sinus thrombosis. Five patients presented with a meningoencephalitic or meningomyelitic picture, three with a stroke-like picture, and three with primarily brain stem signs. One patient developed trigeminal neuritis, and five patients exhibited (along with other features) variable degrees of psychological manifestations. All patients with neurological involvement were treated with steroids, and some also had courses of other immunosuppressant drugs and colchicine. The disease took a relatively benign course, except those patients with meningoencephalitic and meningomyelitic presentation, one of whom died from the disease. Those treated early had a better prognosis. The incidence of dural sinus thrombosis in this series of patients is unusually high. In most patients, the course of the disease was more favourable than reported in the literature. This may be attributed to early and aggressive treatment. (J Neurol Neurosurg Psychiatry 1998;64:382-384)

Rapidly Reversible Neurological Manifestations in a Case of NeuroBehcet Disease

“…Those who were diagnosed and treated early and vigorously had a far better outcome than the ones in whom diagnosis and treatment were delayed (2). The rapid improvement in the present case underscores the importance of vigorous therapy early in the acute phase.…”

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…The frequency of neurological involvement in BD varies greatly (1.3-59.0 %) [140,141], and CNS involvement can be either parenchymal or vascular Bneuro-BD^ [142]. However, in some studies, up to 20 % of patients can present with a combination of the 2 forms [143,144].…”

“…This inflammatory perivasculitis may affect practically all areas of the brain, whereas inflammatory changes are also observed in the CSF. Clinical manifestations mainly include headaches, cognitive dysfunction, sensory and motor deficits, and psychosis, and, less commonly, seizures, ataxia, and extrapyramidal manifestations [140,142,145]. Transverse myelitis may also occur in the context of parenchymal neuro-BD (10 % of total neuro-BD cases), while isolated myelitis is rather uncommon.…”

Tratamiento de la enfermedad de Behçet

“…La polineuritis es poco frecuente. Se han descrito cuadros confusionales, síntomas psiquiátricos y casos de demencia (18)(19)(20).…”

“…La utilización de corticoides como terapia única se ha demostrado insuficiente y con un papel meramente paliativo (18). Los inmunosupresores más habituales son la Ciclofosfamida a dosis de 2 mg/kg/día o en "bolus" de 1g, la Azatioprina a dosis de 2 mg/kg/día y el Clorambucil a dosis de 0,1-0,2 mg/kg/día (12,(23)(24).…”