Unité des rickettsies, URMITE CNRS-IRD UMR 6236, IFR 48, Faculté de médecine, Université de la Méditerranée, Marseille, France.
BMC Infect Dis. 2011 Jun 15;11:171. doi: 10.1186/1471-2334-11-171.
Tropheryma whipplei, the agent of Whipple's disease, causes localised infections in the absence of histological digestive involvement. Our objective is to describe T. whipplei encephalitis.
We first diagnosed a patient presenting dementia and obesity whose brain biopsy and cerebrospinal fluid specimens contained T. whipplei DNA and who responded dramatically to antibiotic treatment. We subsequently tested cerebrospinal fluid specimens and brain biopsies sent to our laboratory using T. whipplei PCR assays. PAS-staining and T. whipplei immunohistochemistry were also performed on brain biopsies. Analysis was conducted for 824 cerebrospinal fluid specimens and 16 brain biopsies.
We diagnosed seven patients with T. whipplei encephalitis who demonstrated no digestive involvement. Detailed clinical histories were available for 5 of them. Regular PCR that targeted a monocopy sequence, PAS-staining and immunohistochemistry were negative; however, several highly sensitive and specific PCR assays targeting a repeated sequence were positive. Cognitive impairments and ataxia were the most common neurologic manifestations. Weight gain was paradoxically observed for 2 patients. The patients' responses to the antibiotic treatment were dramatic and included weight loss in the obese patients.
We describe a new clinical condition in patients with dementia and obesity or ataxia linked to T. whipplei that may be cured with antibiotics.
导致 Whipple 病的 Whipple 螺旋体在没有组织学消化参与的情况下引起局部感染。我们的目的是描述 Whipple 螺旋体脑炎。
我们首先诊断了一名表现为痴呆和肥胖的患者，其脑活检和脑脊液标本中含有 Whipple 螺旋体 DNA，对抗生素治疗有明显反应。随后，我们使用 Whipple 螺旋体 PCR 检测试剂盒检测了送往我们实验室的脑脊液标本和脑活检。还对脑活检进行了 PAS 染色和 Whipple 螺旋体免疫组化染色。分析了 824 份脑脊液标本和 16 份脑活检。
我们诊断了 7 例 Whipple 螺旋体脑炎患者，这些患者均无消化道受累。其中 5 例有详细的临床病史。常规针对单拷贝序列的 PCR、PAS 染色和免疫组化染色均为阴性；然而，几种针对重复序列的高度敏感和特异性 PCR 检测呈阳性。认知障碍和共济失调是最常见的神经系统表现。2 例患者出现体重增加的反常现象。患者对抗生素治疗的反应非常显著，包括肥胖患者的体重减轻。
我们描述了一种新的临床情况，即痴呆和肥胖或共济失调与 Whipple 螺旋体有关，抗生素治疗可能有效。
