Resident, Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India.
Assistant Professor, Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India.
J Assoc Physicians India. 2023 Nov;71(11):103-104. doi: 10.59556/japi.71.0385.
Acute motor axonal neuropathy (AMAN) is a variant of Guillain-Barré syndrome (GBS), characterized by acute areflexic flaccid quadriparesis with motor axonal changes and absence of demyelinating findings in electrophysiological studies. A 30-year-old man presented with acute onset flaccid type of weakness involving all four limbs, along with drooping of eyelids. Examination revealed ptosis with restricted horizontal and vertical eye movements. Spinomotor system examination revealed acute flaccid areflexic quadriparesis. Nerve conduction studies (NCS) showed features suggestive of motor axonal neuropathy changes. Cerebrospinal fluid (CSF) revealed albuminocytological dissociation. The diagnosis of AMAN was made, and the patient was treated with intravenous immunoglobulin (IVIg). His weakness gradually improved over 1 month, with partial improvement in ptosis and eye movements. This case highlights the occurrence of ophthalmoparesis in the AMAN variant of GBS. The presence of ophthalmoparesis and areflexia makes it necessary to exclude Miller-Fisher syndrome. But, the presence of axonal changes in nerve conduction study and the profound weakness with negative serum anti-GQ1b antibody profile, supports the diagnosis of AMAN. : Budumuru U, Muralidharan K, Sowmini PR, AMAN with Ophthalmoparesis: A Rare Presentation. J Assoc Physicians India 2023;71(11):103-104.
急性运动轴索性神经病（AMAN）是吉兰-巴雷综合征（GBS）的一种变体，其特征是急性弛缓性四肢瘫痪伴运动轴索改变，电生理研究中无脱髓鞘发现。一名 30 岁男性出现急性无力，表现为四肢弛缓性瘫痪，伴有眼睑下垂。检查发现上睑下垂，伴有水平和垂直眼球运动受限。脊髓运动系统检查显示急性弛缓性无反射性四肢瘫痪。神经传导研究（NCS）显示提示运动轴索性神经病改变的特征。脑脊液（CSF）显示白蛋白细胞分离。诊断为 AMAN，并给予静脉注射免疫球蛋白（IVIg）治疗。他的无力在 1 个月内逐渐改善，上睑下垂和眼球运动部分改善。本例强调了 GBS 的 AMAN 变体中出现眼肌瘫痪。眼肌瘫痪和无反射使得有必要排除米勒-费舍尔综合征。但是，神经传导研究中存在轴索改变，以及血清抗 GQ1b 抗体阴性的严重无力，支持 AMAN 的诊断。
