Clinicopathological features of acute autonomic and sensory neuropathy
Clinicopathological features of acute autonomic and sensory neuropathy
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DOI：
10.1093/brain/awq214
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发表时间：
2010-10-01
期刊：
影响因子：
14.5
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Koike, Haruki;Atsuta, Naoki;Sobue, Gen
急性自主神经和感觉神经病变是一种罕见的疾病，只有轶事报道。我们描述了21例急性自主神经和感觉神经病患者的临床、电生理、病理和预后特征。三分之二的患者报告了既往事件，主要是上呼吸道或胃肠道感染。深度自主神经功能衰竭与不同程度的感觉障碍的特点，在所有患者的神经病理特征。所有患者的初始症状均与自主神经功能紊乱或浅表感觉障碍有关，而12例患者随后出现了伴有感觉共济失调的深部感觉障碍。感觉性共济失调的严重程度随发病至高峰期时间的延长而加重（P < 0.001）。感觉器官的分布以四肢近端、面部、头皮和躯干多见。它往往是不对称和节段性的，而不是表现为对称性多发性神经病。受累部位疼痛是常见且严重的症状。除了自主神经和感觉症状、咳嗽发作、精神症状、睡眠呼吸暂停和误吸外，肺炎使临床状况难以管理。神经传导研究显示感觉性共济失调患者感觉神经动作电位降低，而无感觉性共济失调患者感觉神经动作电位相对保留。脊髓磁共振成像显示，在T-2* 加权梯度回波图像的后柱高信号区的感觉性共济失调患者，但不是在那些没有它。腓肠神经活检显示小纤维为主的轴突损失没有证据的神经再生。在一例浅表和深部感觉受损的尸检病例中，我们观察到胸交感神经节和背根神经节以及奥尔巴赫神经丛的神经元细胞严重丢失，前骨细胞保存完好。脊髓前根中的有髓纤维得以保留，而脊髓后根和脊髓后柱中的有髓纤维被耗尽。虽然感觉障碍的恢复较差，但大多数患者的自主神经功能障碍在几个月内得到一定程度的改善。总之，免疫介导的机制可能与急性自主神经和感觉神经病变。自主神经节和感觉神经节中的小神经元细胞可能在初始阶段受到影响，随后，感觉神经节中的大神经元细胞受损。
Acute autonomic and sensory neuropathy is a rare disorder that has been only anecdotally reported. We characterized the clinical, electrophysiological, pathological and prognostic features of 21 patients with acute autonomic and sensory neuropathy. An antecedent event, mostly an upper respiratory tract or gastrointestinal tract infection, was reported in two-thirds of patients. Profound autonomic failure with various degrees of sensory impairment characterized the neuropathic features in all patients. The initial symptoms were those related to autonomic disturbance or superficial sensory impairment in all patients, while deep sensory impairment accompanied by sensory ataxia subsequently appeared in 12 patients. The severity of sensory ataxia tended to become worse as the duration from the onset to the peak phase of neuropathy became longer (P < 0.001). The distribution of sensory manifestations included the proximal regions of the limbs, face, scalp and trunk in most patients. It tended to be asymmetrical and segmental, rather than presenting as a symmetric polyneuropathy. Pain of the involved region was a common and serious symptom. In addition to autonomic and sensory symptoms, coughing episodes, psychiatric symptoms, sleep apnoea and aspiration, pneumonia made it difficult to manage the clinical condition. Nerve conduction studies revealed the reduction of sensory nerve action potentials in patients with sensory ataxia, while it was relatively preserved in patients without sensory ataxia. Magnetic resonance imaging of the spinal cord revealed a high-intensity area in the posterior column on T-2*-weighted gradient echo image in patients with sensory ataxia but not in those without it. Sural nerve biopsy revealed small-fibre predominant axonal loss without evidence of nerve regeneration. In an autopsy case with impairment of both superficial and deep sensations, we observed severe neuronal cell loss in the thoracic sympathetic and dorsal root ganglia, and Auerbach's plexus with well preserved anterior hone cells. Myelinated fibres in the anterior spinal root were preserved, while those in the posterior spinal root and the posterior column of the spinal cord were depleted. Although recovery of sensory impairment was poor, autonomic dysfunction was ameliorated to some degree within several months in most patients. In conclusion, an immune-mediated mechanism may be associated with acute autonomic and sensory neuropathy. Small neuronal cells in the autonomic and sensory ganglia may be affected in the initial phase, and subsequently, large neuronal cells in the sensory ganglia are damaged.