Hiraga A, Kuwabara S, Ogawara K, Misawa S, Kanesaka T, Koga M, Yuki N, Hattori T, Mori M
Department of Neurology, Chiba University Graduate School of Medicine, Japan.
Neurology. 2005 Mar 8;64(5):856-60. doi: 10.1212/01.WNL.0000153071.71335.E9.
In Guillain-Barré syndrome (GBS), anti-ganglioside antibodies are strongly associated with the acute motor axonal neuropathy (AMAN) form, but there are also cases of the demyelinating form of GBS (acute inflammatory demyelinating polyneuropathy [AIDP]) with anti-ganglioside antibodies.
To elucidate the patterns and sequential changes in electrodiagnostic abnormalities of anti-ganglioside-positive GBS.
Detailed serial electrodiagnostic findings were reviewed for 51 patients with GBS. Anti-ganglioside antibodies were measured by ELISA.
Antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a were present in 25 patients. Of these, 12 (48%) showed the AMAN pattern, 5 (20%) the AIDP pattern, and 3 (12%) isolated F-wave absence in the first examination. All five patients with the AIDP pattern showed prolonged distal latencies, but three eventually showed the AMAN pattern or rapid normalization. The remaining two still had similarly prolonged distal latencies in weeks 4 to 6, but the serial changes were distinct from those in the anti-ganglioside-negative AIDP patients who showed progressive increases in distal latencies over 2 months after onset.
Besides the simple axonal degeneration pattern, patients with anti-ganglioside-positive Guillain-Barre syndrome can show transient conduction slowing/block in the distal or proximal nerve segments, mimicking demyelination, but anti-ganglioside antibodies do not appear to be associated with acute inflammatory demyelinating polyneuropathy.
在吉兰 - 巴雷综合征(GBS)中,抗神经节苷脂抗体与急性运动轴索性神经病(AMAN)型密切相关,但也有抗神经节苷脂抗体阳性的脱髓鞘型GBS(急性炎症性脱髓鞘性多发性神经病[AIDP])病例。
阐明抗神经节苷脂抗体阳性GBS患者电诊断异常的模式及连续变化情况。
回顾了51例GBS患者详细的系列电诊断结果。通过酶联免疫吸附测定法检测抗神经节苷脂抗体。
25例患者存在针对GM1、GM1b、GD1a或GalNAc - GD1a的抗体。其中,12例(48%)在首次检查时表现为AMAN模式,5例(20%)为AIDP模式,3例(12%)表现为单纯F波消失。所有5例AIDP模式的患者均表现为远端潜伏期延长,但3例最终表现为AMAN模式或迅速恢复正常。其余2例在第4至6周时仍有类似的远端潜伏期延长,但系列变化与抗神经节苷脂抗体阴性的AIDP患者不同,后者在起病后2个月内远端潜伏期呈进行性延长。
除了单纯的轴索性变性模式外,抗神经节苷脂抗体阳性的吉兰 - 巴雷综合征患者可在远端或近端神经节段出现短暂的传导减慢/阻滞,类似脱髓鞘表现,但抗神经节苷脂抗体似乎与急性炎症性脱髓鞘性多发性神经病无关。