Neurological manifestations of Behçet's disease in a Caribbean population: clinical and imaging findings

Abstract

Behçet's disease (BD) is a chronic relapsing multisystem disorder. While most frequently occurring around the Mediterranean and in Japan, isolated cases of BD have been reported in Africa south of the Sahara and in the Caribbean. The aim of this study was to describe our experience of BD in Guadeloupe (French West Indies) where the presence of the disease has not been reported previously. We analysed retrospectively the charts, and clinical and imaging features of patients native to Guadeloupe who were diagnosed with neurological manifestations of BD between 1989 and 1999. In our series of 13 cases, seven had neurological involvement. Neurological manifestations included meningoencephalitis or meningoencephalomyelitis in four cases, cerebral venous thrombosis in one case and peripheral neuropathy in two cases associated with myositis in one. Patients received treatment with colchicine (n=7), corticosteroids (n=6), immunosuppressive therapy (azathioprine and/or cyclophosphamide; n=4), acetylsalicylic acid (n=2) and oral anticoagulation for venous thrombosis (n=1). Long-term sequelae occurred only in patients with recurrent neurological disease. This study suggests that the frequency of BD in this Afro-Caribbean population is higher than this reported in Caucasian populations. Meningoencephalitis is associated with a poor prognosis while other patients achieved recovery.

Cerebral Venous Thrombosis in Behçet's Disease Patients Compared to Other Causes of Cerebral Venous Thrombosis: a Retrospective Study

“…[2,9,[11][12][13][14][15][16][17]20,30,[34][35][36][37][38][39][40] Moreover, warfarin and acetyl salicylic acid have been given to some CVT patients with BD. [10][11][12]17,26,34 Wechsler et al 14 reported that treatment of CVT during acute phase consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others.…”

“…35 Colchium, prednisolone, azathioprine, cyclosporine, and cyclophosphamide have been administered to prevent the recurrence of CVT in BD. 2,9,15,17,[26][27][28]30,[36][37][38][39][40][41][42] Acetazolamide and lumboperitoneal shunts have been installed to reduce intracranial hypertension in some CVT patients with BD. 20,25 We administered colchium, azathioprine, and/or prednisolone to our patients to prevent recurrence and we have not observed any recurrence during follow-up in CVT patients with BD.…”

Acute inflammatory myelopathies

“…Of these 7 patients the myelitis was partial in 3, transverse in 2, and presented as a Brown-Séqard syndrome in 2. In addition to this series a number of case reports describe myelopathic involvement in Behçet's disease (Morrissey et al, 1993a;Yoshioka et al, 1996;Mascalchi et al, 1998;Kocer et al, 1999;Green and Mitchell, 2000;Harmouche et al, 2000;Lee et al, 2001;Lannuzel et al, 2002;Moskau et al, 2003;Calguneri et al, 2005;Deshpande et al, 2005;Mullins et al, 2009;Fukae et al, 2010;Metreau-Vastel et al, 2010). Behçet's associated myelitis is typically longitudinally extensive and extension of upper cervical cord lesions into brainstem structures can occur.…”

Subacute Longitudinal Myelitis Associated with Behcet's Disease

“…In most NBD patients with myelitis, highdose systemic corticosteroid therapy was reported to be effective for the acute or subacute phase (1,(11)(12)(13)19). However, there were some reports describing patients who showed a poor response to corticosteroid ther-apy (5,10,13,16,17). In those patients, other immunosuppressants or interferon α may be used (1,10,17 …”