Warren Jason D, Scott Grace, Blumbergs Peter C, Thompson Philip D
Department of Neurology, Royal Adelaide Hospital, and University Department of Medicine, University of Adelaide, South Australia.
J Clin Neurosci. 2002 May;9(3):328-9. doi: 10.1054/jocn.2001.1014.
We report the case of a 57 year old woman with typical clinical features of the stiff man syndrome (SMS) and antibodies to glutamic acid decarboxylase (antiGADAb), who developed a supranuclear gaze palsy, ileus and died of bronchopneumonia eight years after the onset of illness. Post mortem examination revealed perivascular lymphocyte cuffing throughout the cerebral hemispheres, brainstem and spinal cord and neuronal loss in medial anterior horns of the cervical spinal cord. These findings support the notion that the SMS and progressive encephalomyelitis with rigidity (PER) form a clinical and pathologic continuum.
我们报告了一例57岁女性患者，她具有僵人综合征（SMS）的典型临床特征且存在谷氨酸脱羧酶抗体（抗GADAb），发病八年后出现核上性凝视麻痹、肠梗阻，并死于支气管肺炎。尸检显示，整个大脑半球、脑干和脊髓均有血管周围淋巴细胞套袖现象，颈髓前内侧角有神经元丢失。这些发现支持了僵人综合征和进行性僵硬性脑脊髓炎（PER）构成临床和病理连续体的观点。
