Prayson Richard A, Frater John L
Department of Anatomic Pathology, Cleveland Clinic Foundation, OH 44195, USA.
Am J Clin Pathol. 2002 May;117(5):776-82. doi: 10.1309/AD8R-560C-4V11-C5E2.
We retrospectively reviewed the clinicopathologic features and immunohistochemical profiles of 7 patients with Rasmussen encephalitis (age range, 3.5-15 years at surgery). All had medically intractable seizures (6 months' to 7 years' duration); all but 1 developed unilateral hemiparesis. Histologically, all cases were characterized by leptomeningeal and parenchymal perivascular chronic inflammation consisting primarily of T lymphocytes (CD3+, CD5+, CD7+). In all but 1 case, a predominance of CD8+ T-cytotoxic/suppressor lymphoid cells over CD4+ cells was observed. All cases had rare B lymphocytes (CD79a+, CD20+). Rare CD10+ and no CD56+ cells were noted. All cases were marked by diffuse proliferation of microglial cells, highlighted on CD68 immunostaining. Focal microglial nodule formations were observed in 4 cases and focal cortical atrophy in 5 cases. Viral inclusions were not noted. There was no evidence of Epstein-Barr virus by LMP-1 antibody immunostaining. The histologic findings of Rasmussen encephalitis resemble those of viral meningoencephalitis. The pathologicfindings may be only focally present, and missed, if diagnosis is made or confirmed with biopsy alone. Most lymphoid cells have a T-cell immunophenotype, with a predominance of CD8+ cells in most cases.
我们回顾性分析了7例拉斯穆森脑炎患者（手术时年龄范围为3.5至15岁）的临床病理特征和免疫组化结果。所有患者均有药物难治性癫痫（病程6个月至7年）；除1例患者外，其余均出现单侧偏瘫。组织学上，所有病例均以软脑膜和实质血管周围慢性炎症为特征，主要由T淋巴细胞（CD3 +、CD5 +、CD7 +）组成。除1例病例外，其余所有病例均观察到CD8 + T细胞毒性/抑制性淋巴细胞多于CD4 +细胞。所有病例均有罕见的B淋巴细胞（CD79a +、CD20 +）。可见罕见的CD10 +细胞，未见CD56 +细胞。所有病例均有小胶质细胞弥漫性增生，CD68免疫染色突出显示。4例观察到局灶性小胶质结节形成，5例观察到局灶性皮质萎缩。未发现病毒包涵体。LMP-1抗体免疫染色未显示爱泼斯坦-巴尔病毒感染证据。拉斯穆森脑炎的组织学表现类似于病毒性脑膜脑炎。如果仅通过活检进行诊断或确诊，病理结果可能仅局灶性存在且易被漏诊。大多数淋巴细胞具有T细胞免疫表型，大多数病例中以CD8 +细胞为主。
