Boudin L, Romeo E, Mavrovi E, Tsitsi Nding P, Blade J-S, de Jaureguiberry J-P, Gisserot O
Service de médecine interne - oncologie, HIA Sainte-Anne, 2, boulevard Sainte-Anne, 83800 Toulon cedex 9, France.
Service de médecine interne - oncologie, HIA Sainte-Anne, 2, boulevard Sainte-Anne, 83800 Toulon cedex 9, France.
Rev Med Interne. 2015 Jun;36(6):418-22. doi: 10.1016/j.revmed.2014.05.003. Epub 2014 Jun 3.
Neurological manifestations during Waldenstrom disease are common and are usually related to immune peripheral neuropathy or serum hyperviscosity syndrome. The infiltration of the central nervous system by the lymphoproliferative syndrome is known as the Bing-Neel syndrome. This extremely rare entity remains poorly described in the literature.
We report on 4 cases of patients for whom central neurological disorders led to the diagnosis of a Bing and Neel syndrome. These four cases illustrate different clinical presentations, diagnosis, therapeutic options, and outcome in this syndrome. Based on our literature review, we discuss about these differences.
The polymorphic clinical manifestations of Bing and Neel syndrome can mimic many diagnoses. However, it may be necessary to consider this diagnosis. Cerebrospinal fluid analysis and MRI may allow rapid diagnosis or guide a biopsy. Prolonged remissions are possible with appropriate treatment.
华氏巨球蛋白血症期间的神经学表现很常见，通常与免疫性周围神经病或血清高黏滞综合征有关。淋巴增殖综合征对中枢神经系统的浸润被称为宾-尼尔综合征。这种极其罕见的病症在文献中描述甚少。
我们报告了4例因中枢神经系统疾病而诊断为宾-尼尔综合征的患者。这4例病例说明了该综合征不同的临床表现、诊断、治疗选择及预后。基于我们的文献综述，我们讨论了这些差异。
宾-尼尔综合征的多形性临床表现可能会与多种诊断相混淆。然而，可能有必要考虑这一诊断。脑脊液分析和磁共振成像（MRI）可能有助于快速诊断或指导活检。适当治疗有可能实现长期缓解。
