Vigliani M-C, Novero D, Cerrato P, Daniele D, Crasto S, Berardino M, Mutani R
Department of Neuroscience, University of Turin, Via Cherasco 15, 10126 Turin, Italy.
J Neurol Neurosurg Psychiatry. 2009 Jun;80(6):693-5. doi: 10.1136/jnnp.2008.145961.
A case of brainstem encephalitis in a man positive for both anti-Hu and anti-Ri antibodies is reported. This case had an unusual double step evolution and progressive involvement of different CNS subdivisions at MRI. Brainstem encephalitis developed abruptly, mimicking a posterior vascular deficit with vertigo and dizziness. These symptoms transiently remitted completely after a few days to relapse acutely 1 month later with sudden loss of consciousness, followed by confusion, disorientation, dysarthria, dysphagia and reduced thermic sensation on the right side. Within another few days, the patient developed acute respiratory failure and died some weeks later. MRI was negative at the beginning but later showed a progressive ascending involvement of the brainstem and thalamus. At autopsy, this picture corresponded to lymphocytic infiltration, preferentially B cells into the perivascular spaces and T cells in the brainstem parenchyma, confirming that T cells could be the effector of cytotoxicity, probably in the presence of cooperation with B cells that were well represented in this setting.
报告了一例抗Hu和抗Ri抗体均呈阳性的男性脑干脑炎病例。该病例有不寻常的双阶段病程，且在MRI上不同的中枢神经系统分区呈进行性受累。脑干脑炎突然发作，类似后循环血管病变，伴有眩晕和头晕。这些症状在数天后完全短暂缓解，但1个月后急性复发，突然意识丧失，随后出现意识模糊、定向障碍、构音障碍、吞咽困难以及右侧温度感觉减退。再过几天，患者出现急性呼吸衰竭，并在数周后死亡。MRI起初为阴性，但后来显示脑干和丘脑呈进行性向上累及。尸检时，此表现对应淋巴细胞浸润，血管周围间隙中以B细胞为主，脑干实质中以T细胞为主，证实T细胞可能是细胞毒性的效应细胞，可能是在与该病例中大量存在的B细胞协同作用的情况下。
