假瘤型炎性脱髓鞘病的临床及病理

Clinical and pathological study of tumor-like inflammatory demyelinating diseases

目的 :探讨假瘤型炎性脱髓病的临床病理特点。方法 :分析 5例假瘤型炎性脱髓鞘病的临床、影像及病理特点。结果 :本组临床特点 :1亚急性或慢性起病 ,逐渐进展。 2症状、体征及 MRI均提示为单一病灶。 3MRI所见病灶比 CT大 ,可有占位效应 ,也可呈周边花边样或环形强化 (例 2呈均匀强化 )。病理呈炎性脱髓鞘改变 ;白质内大片脱髓鞘区 ,血管周围淋巴细胞浸润 ,呈袖套样 ,伴有胶质细胞增生。也可主要累及灰质。结论 :1假瘤型炎症性脱髓鞘病可能是多发性硬化 (MS)中的一种特殊类型 ,或者是 MS与急性播散性脑脊髓炎之间的过渡类型。 2假瘤型炎性脱髓鞘病与胶质瘤不易鉴别 ,可试用肾上腺皮质激素治疗或者进行组织活检 ,不可急于手术。

Objective: To analyze the clinicopathological changes of tumor-like inflammatory demyelinating diseases. Methods: The clinical situation, magnetic resonance imaging(MRI), and pathology of 5 cases of tumor-like inflammatory demyelinating diseases were reported and analyzed. Result: The 5 patients' clinical features were as follows:①Onset was a subacute or chronic form. The course of diseases was gradually progressive with the increased or aggravated symptoms. ②The clinical symptoms, signs, and MRI showed a single focal lesion in every case. ③The focal lesions could be space-occupying with enhanced edges. Pathological condition was shown as typical inflammatory-demyalination: demyelinating in the white matter, perivascular inflammatory infiltrates consisting predominantly of lymphocytes, and glial cells proliferating. Grey matter could mainly be involved. Conclusion: ①Timor-like inflammatory demyelinating diseases are special type of multiple sclerosis(MS), or occupy an intermediate entity between MS and acute disseminated encephalomyelitis(ADEM). ②It is difficult to make a differential diagnosis between glioma and tumor-like inflammtory emyelinating diseases from clinical manifestation. Corticoids on trial treatment or even biopsy is available, rather than surgical operation.