RESUMEN
BACKGROUND: Behcet's disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet's disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases. METHODS: We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD. RESULTS: The median age was 35.86 (12-59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (n = 31), pyramidal syndrome (n = 28), cerebellar syndrome (n = 5), behavioral changes (n = 5), and pseudobulbar signs (n = 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (n = 15, 37.5%) and the brainstem (n = 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (n = 40, 93%) and immunosuppressive agents (n = 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients. CONCLUSION: PT NBD is a rare but life-threatening condition.
Asunto(s)
Síndrome de Behçet , Enfermedades Cerebelosas , Adulto , Femenino , Humanos , Masculino , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Inmunosupresores/uso terapéutico , Imagen por Resonancia Magnética , Recurrencia Local de Neoplasia , Niño , Adolescente , Adulto Joven , Persona de Mediana EdadRESUMEN
Neurological cytomegalovirus (CMV) infections especially extensive longitudinal myelitis are extremely rare in immunocompetent adults. However, we hereby report a case of cervical, thoracic, and lumbosacral myelitis caused by CMV infection in a healthy adult patient. The patient was treated properly and had a good outcome. The etiopathogenesis and the prognostic factors for this affection are not well established and are still being debated by authors. Further clinical data would contribute to a better understanding of this pathology in order to provide a better prognosis.