儿童慢性炎症性脱鞘性多神经病的临床和病理改变特点

Clinical and pathological features in the childhood chronic inflammatory demyelinating polyneuropathy

目的探讨儿童慢性炎症性脱鞘性多神经病(chronic inflammatory demyelinating polyneuropathy,CI-DP)的临床及病理改变特点。方法根据欧洲神经肌肉病中心修订的儿童CIDP诊断标准诊断的10例17岁以下患者,收集其临床资料,进行周围神经电生理以及腓肠神经的病理检查。结果所有患者主要表现为肢体无力,分别有4例和3例出现四肢感觉减退和颅神经损害。9例有脑脊液蛋白细胞分离现象。10例均出现运动或感觉神经传导速度减慢及远端潜伏期延长,9例患者的动作电位波幅降低。所有患者的有髓神经纤维出现轻-重度减少,其中3例患者的纤维脱失程度在不同束间存在差异,6例患者以脱髓鞘为主;3例以轴索损害为主。1例患者仅出现轻微改变。9例患者存在炎细胞浸润。结论儿童CIDP以肢体无力为主。部分患者以轴索损害为主,神经纤维脱失程度可以存在束间差异。

Objective To study the clinical, electrophysiological and pathological features in the childhood chronic inflammatory demyelinating polyneuropathy （CIDP）. Methods Ten patients whose age at onset was between 2 - 17 years old were diagnosed by the European Neuromuscular Center criteria for CIDP. The clinical data were collected and electrophysiological examination as well as sural nerve biopsy were performed in all patients. Results The main clinical symptoms were proximal weakness in all cases, among them, 4 cases presented with sensory loss in the extremities and 3 cases with multiple cranial nerve palsy. Cerebrospinal fluid test showed albumino-cytological dissociation in 9 cases. Decrease in motor and sensory nerve conduction velocities with prolonged distal latency presented in 10 cases and decrease in amplitude of compound active potential in 9 cases. Mild to severe loss of myelinated fibers appeared in all cases and fiber loss a- mong the fascicules varied markedly in 3 cases. Six cases mainly presented with demyelination, 3 cases with marked Wallerian degeneration, 1 case with mild morphological changes and 9 cases inflammatory infiltrate. Conclusions Motor weakness is the major symptom of childhood CIDP. Axonal lesions are the main morphological changes in some patients and substantial variability of myelinated fibers loss exists among the different fascicules in other cases.