亚急性自主神经和感觉神经节病临床与病理

Clinical and pathological characteristics of subacute autonomic and sensory ganglionopathy

目的探讨亚急性自主神经和感觉神经节病的病因、病理及临床特点。方法收集并分析7例亚急性自主神经和感觉神经节病患者的临床资料、神经电生理学、神经影像学和病理学检查结果。结果 7例患者主要临床表现包括四肢深感觉障碍、感觉性共济失调、手袜套样浅感觉减退或痛觉过敏、假性手足徐动征、神经痛。自主神经功能障碍包括Adie瞳孔、无汗、体位性低血压、假性肠梗阻。颈部MRI检查显示脊髓后索长T2信号。体感诱发电位检查显示外周传导异常,脊髓至皮质传导障碍。神经传导检查显示感觉神经动作电位波幅降低或消失。神经活检结果显示有髓和无髓神经均严重丢失,轴索变性。结论亚急性自主神经和感觉神经节病为累及后根神经节、交感神经节的免疫介导性疾病,具有独特的临床表现,预后不良。

Objective To explore the etiology, pathology and clinical characteristics of subacute autonomic and sensory ganglionopathy. Methods The clinical, neurophysiological, neuroimaging and pathological manifestations were analyzed in 7 cases with subacute autonomic and sensory ganglionopathy. Results The main clinical manifestations included proprioceptive sensory disturbance of extremities, sensory ataxia, impaired pain sensation or hyperalgesia with a glove and stocking pattern, pseudoathetosis, and neuralgia. Several lines of evidence indicated autonomic insufficiency, including Adie＇ s pupils, anhidrosis, orthostatic hypotension and intestinal pseudo-obstruction. MRI revealed high T2 intensity in the posterior columns of the spinal cord. SEP disclosed abnormalities in peripheral nerve conduction and central sensory pathway conduction. Nerve conduction studies showed absent or depressed amplitude of sensory action potentials. In sural nerve biopsy there were severe loss of myelinated and unmyelinated fibres, and axonal degeneration. Conclusions Subacute autonomic and sensory ganglionopathy was an immune-mediated disease, which selectively targets dorsal root ganglia and sympathetic ganglia. The disease had characteristic clinical presentations and poor prognosis.