Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, 130021, China.
Department of Radiology, The First Hospital of Jilin University, Changchun, 130021, China.
BMC Neurol. 2020 Apr 6;20(1):120. doi: 10.1186/s12883-020-01698-0.
Anomalies of pyramidal tract decussation are rare phenomena that can be caused by ectodermal dysplasia. Herein, we describe a patient with ichthyosis who exhibited ipsilateral hemiparesis after stroke and whose neuroimaging results showed evidence of motor control being provided by the ipsilateral motor cortex.
A 24-year-old right-handed man presented with skin abnormalities, sudden-onset left hemiparesis, and dysarthria. He exhibited a mild-to-moderate left-sided weakness (grade 4 on the Medical Research Council scale). Magnetic resonance imaging revealed an acute infarct in the left corona radiata. Diffusion tensor imaging revealed uncrossed corticospinal tracts. Next-generation sequencing identified heterozygous FLG mutations. The patient was diagnosed with cerebral infarction and ichthyosis vulgaris and was treated with aspirin (100 mg/d). His symptoms gradually dissipated.
This case suggests that pyramidal decussation anomalies can be associated with ichthyosis. Patients with ichthyosis should therefore be evaluated for nerve involvement.
锥体束交叉异常是一种罕见的现象，可由外胚层发育不良引起。在此，我们描述了 1 例鱼鳞癣患者，其在中风后出现同侧偏瘫，神经影像学结果显示同侧运动皮层提供运动控制。
1 名 24 岁的右利手男性，存在皮肤异常、突发左侧偏瘫和构音障碍。他表现出轻度至中度左侧无力（MRC 量表 4 级）。磁共振成像显示左侧放射冠急性梗死。弥散张量成像显示未交叉皮质脊髓束。下一代测序发现 FLG 杂合突变。患者被诊断为脑梗死和寻常性鱼鳞病，给予阿司匹林（100mg/d）治疗。他的症状逐渐消失。
本病例提示锥体束交叉异常可与鱼鳞癣相关。因此，应评估患有鱼鳞病的患者是否存在神经受累。
