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副肿瘤性神经病的共济失调型与疼痛型

Ataxic vs painful form of paraneoplastic neuropathy.

作者信息

Oki Y, Koike H, Iijima M, Mori K, Hattori N, Katsuno M, Nakamura T, Hirayama M, Tanaka F, Shiraishi M, Yazaki S, Nokura K, Yamamoto H, Sobue G

机构信息

Department of Neurology, Nagoya University, Graduate School of Medicine, Nagoya, Japan.

出版信息

Neurology. 2007 Aug 7;69(6):564-72. doi: 10.1212/01.wnl.0000266668.03638.94.

Abstract

OBJECTIVE

To characterize the clinicopathologic features of ataxic and painful forms of paraneoplastic neuropathy.

METHODS

Clinical, electrophysiologic, and histopathologic findings were assessed in 17 patients with paraneoplastic neuropathy.

RESULTS

Clinical features can be categorized into two groups: one group (13 patients) with predominantly deep sensory disturbance and a second group (4 patients) with predominantly superficial sensory disturbance. The former group showed severe sensory ataxia and predominantly large myelinated fiber loss in the sural nerve. The latter group showed marked pain, in particular, severe mechanical hyperalgesia, and predominantly small myelinated and unmyelinated fiber loss. Nerve conduction assessment indicated an axonal neuropathy pattern in both groups, while sensory action potentials were more markedly diminished in the sensory ataxic form. Anti-Hu antibodies were detected in half of the patients in both groups. Treatment for cancer was effective to improve or stabilize neuropathic symptoms in some cases from both groups. Immunotherapy was effective only for a short time.

CONCLUSIONS

Paraneoplastic neuropathy can be characterized into two groups by the presence of sensory ataxia or severe spontaneous pain and severe mechanical hyperalgesia. Preferential small myelinated and unmyelinated fiber loss correlated to the cases of severe pain.

摘要

目的

描述副肿瘤性神经病共济失调型和疼痛型的临床病理特征。

方法

对17例副肿瘤性神经病患者的临床、电生理和组织病理学检查结果进行评估。

结果

临床特征可分为两组:一组(13例患者)主要表现为深感觉障碍,另一组(4例患者)主要表现为浅感觉障碍。前一组表现为严重的感觉性共济失调,腓肠神经主要为大的有髓纤维丢失。后一组表现为明显疼痛,尤其是严重的机械性痛觉过敏,主要为小的有髓和无髓纤维丢失。神经传导评估显示两组均为轴索性神经病模式,而感觉性共济失调型的感觉动作电位减弱更明显。两组各有一半患者检测到抗Hu抗体。在两组的一些病例中,癌症治疗对改善或稳定神经病变症状有效。免疫治疗仅在短期内有效。

结论

副肿瘤性神经病可根据是否存在感觉性共济失调或严重的自发疼痛及严重的机械性痛觉过敏分为两组。小的有髓和无髓纤维优先丢失与严重疼痛病例相关。

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