Department of Neurology, Klinikum Grosshadern der Ludwig-Maximilians-Universität München, Munich, Germany.
J Neurol Sci. 2010 Apr 15;291(1-2):118-20. doi: 10.1016/j.jns.2009.12.025. Epub 2010 Jan 27.
Stiff-person syndrome (SPS), a rare neuroimmunological disorder, is characterized by symmetrical rigidity and muscle stiffness, particularly of axial and proximal limb muscles. Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a variant of SPS which includes additional clinical features (e.g. sensory symptoms, brain stem signs and pathological CSF findings). An association of both SPS and PERM with (solid) malignancies has been previously reported. Beyond this, there have been single reports of SPS in the setting of Hodgkin's lymphoma (HL). Here, we present a case of PERM associated with HL, with PERM preceding occurrence of lymphoma by more than seven months. Our observation has obvious implications for the management and, in particular, diagnostic evaluation of patients with PERM.
僵人综合征（SPS）是一种罕见的神经免疫性疾病，其特征为对称性僵硬和肌肉强直，尤其以轴性和近端肢体肌肉为主。进行性脑脊髓炎伴僵硬和肌阵挛（PERM）是 SPS 的一种变异型，其包括其他临床特征（例如感觉症状、脑干体征和病理性 CSF 发现）。先前已有报道称 SPS 和 PERM 均与（实体）恶性肿瘤相关。除此之外，还有 SPS 合并霍奇金淋巴瘤（HL）的个案报告。在此，我们报告了一例 PERM 合并 HL 的病例，且 PERM 在淋巴瘤发生前已超过七个月。我们的观察结果对 PERM 患者的管理具有明显的意义，特别是对其诊断评估具有重要意义。
