Notturno Francesca, Caporale Christina M, Uncini Antonino
Department of Human Motor Sciences and Neuromuscular Diseases Unit, Institute of Aging (Ce.S.I), Foundation University G. d'Annunzio, Chieti-Pescara Clinica Neurologica, Ospedale S.S. Annunziata, via Dei Vestini, 66013 Chieti, Italy.
Muscle Nerve. 2008 Feb;37(2):265-8. doi: 10.1002/mus.20875.
Three patients developed acute pure sensory ataxic neuropathy. Two of the three patients had a recent Campylobacter jejuni infection. Patient 1 had monospecific IgG anti-GD1b. Patients 2 and 3 had cross-reactive IgG anti-GQ1b and anti-GD1b and patient 2 also had IgG anti-GT1a. Motor nerve conduction studies were completely normal. Sensory conductions showed reduced amplitude or absent sensory nerve action potentials with normal or slightly slowed conduction velocities. In patient 2, serial electrophysiological studies showed reappearance and improvement of sensory nerve potential amplitudes in 4 weeks. All patients recovered completely in 2 months and sensory potential amplitudes normalized in 3-5 months. Our findings: (1) confirm the existence of a pure acute sensory ataxic neuropathy with cross-reactive IgG anti-GQ1b and anti-GD1b as a variant of Guillain-Barré syndrome; (2) expand the clinical presentation of Guillain-Barré syndrome after C. jejuni infection and suggest that molecular mimicry is at the basis of acute sensory ataxic neuropathy; and (3) indicate that, in acute sensory ataxic neuropathy with prompt recovery, the site of the lesion is not in the primary sensory neurons and the pathophysiological mechanism may be functional in nature.
三名患者发生急性纯感觉性共济失调性神经病。三名患者中有两名近期感染空肠弯曲菌。患者1有单特异性IgG抗GD1b。患者2和3有交叉反应性IgG抗GQ1b和抗GD1b，患者2还有IgG抗GT1a。运动神经传导研究完全正常。感觉传导显示感觉神经动作电位波幅降低或消失，传导速度正常或略有减慢。在患者2中，系列电生理研究显示4周内感觉神经电位波幅再现并改善。所有患者在2个月内完全恢复，感觉电位波幅在3 - 5个月内恢复正常。我们的发现：(1)证实存在一种以交叉反应性IgG抗GQ1b和抗GD1b为特征的纯急性感觉性共济失调性神经病，作为吉兰 - 巴雷综合征的一种变异型；(2)扩展了空肠弯曲菌感染后吉兰 - 巴雷综合征的临床表现，并提示分子模拟是急性感觉性共济失调性神经病的基础；(3)表明，在急性感觉性共济失调性神经病且恢复迅速的情况下，病变部位不在初级感觉神经元，病理生理机制可能本质上是功能性的。
