Medical Student, Damascus University, Damascus, Syria.
Department of Neurology, Damascus University, Damascus, Syria.
BMC Neurol. 2022 Mar 18;22(1):102. doi: 10.1186/s12883-022-02599-0.
Acute motor and sensory axonal neuropathy (AMSAN) is a subtype of Guillain-Barré syndrome (GBS) differentiated by nerve conduction studies (NCS) and characterized by symmetric ascending paralysis often involving respiratory muscles. While opsoclonus, which is involuntary chaotic rapid eye movements, is not a common manifestation of GBS. Moreover, little published data are available on the relation between liver enzymes elevation and GBS.
A 42-year-old man presented to Al Mouwassat University Hospital with weakness in all limbs and dyspnea. Examination showed an elevated respiratory rate, hyporeflexia, and decreased strength of upper and lower limbs. Analysis of cerebrospinal fluid revealed an albuminocyto-dissociation suggesting the diagnosis of GBS and subsequent plasmapheresis. NCS confirmed a diagnosis of AMSAN. Elevation in liver enzymes was noticed prompting further exploration with no positive findings. Despite treatment efforts, the patient developed severe dyspnea, deterioration in cognitive abilities, and opsoclonus with a normal brain MRI. Unfortunately, he developed respiratory failure which lead to his death.
In this case, we highlight the occurrence of opsoclonus which is a rarely-encountered manifestation of GBS, in addition to an unexplained elevated liver enzyme, the thing that could contribute to larger research to further comprehend the pathophysiology of GBS.
急性运动感觉轴索性神经病(AMSAN)是吉兰-巴雷综合征(GBS)的一个亚型,通过神经传导研究(NCS)进行区分,其特征为对称上升性瘫痪,常累及呼吸肌。而眼球震颤,即不自主的快速眼球运动,不是 GBS 的常见表现。此外,关于肝酶升高与 GBS 之间的关系,发表的数据很少。
一名 42 岁男性因四肢无力和呼吸困难就诊于 Al Mouwassat 大学医院。检查显示呼吸频率升高、反射减弱、上下肢肌力下降。脑脊液分析显示白蛋白细胞分离,提示诊断为 GBS,并随后进行了血浆置换。NCS 确诊为 AMSAN。注意到肝酶升高,进一步检查未发现阳性结果。尽管进行了治疗,但患者出现严重呼吸困难、认知能力恶化和眼球震颤,脑 MRI 正常。不幸的是,他出现呼吸衰竭,导致死亡。
在本例中,我们强调了眼球震颤的发生,这是 GBS 一种罕见的表现形式,此外还有不明原因的肝酶升高,这可能有助于进一步研究以更好地理解 GBS 的病理生理学。