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面部痉挛而非半面痉挛:一例病例报告及文献综述

Facial spasms, but not hemifacial spasm: a case report and review of literature.

作者信息

McDowell Michael M, Zhu Xiao, Hughes Marion A, Sekula Raymond F

机构信息

Department of Neurosurgery, University of Pittsburgh Medical Center, 200 Lothrop Street, B-400, Pittsburgh, PA, 15213, USA.

Department of Radiology, Division of Neuroradiology, University of Pittsburgh Medical Center, Pittsburgh, PA, 15213, USA.

出版信息

Childs Nerv Syst. 2016 Sep;32(9):1735-9. doi: 10.1007/s00381-016-3057-7. Epub 2016 Mar 16.

Abstract

INTRODUCTION

Facial spasms represent a complicated array of neurological motor disorders with unique diagnostic and treatment algorithms. Due to the rarity of many of these disorders in the pediatric population, special care must be taken in identifying subtle differences in presentation of these disorders.

METHODS

We present a case of a 3-year-old boy diagnosed with a brainstem ganglioglioma, Chiari 1 malformation, and a 2-year history of left-sided facial spasms. Stereotyped facial contractions and subtle eye deviation occurred every 10 s, with downward movement rather than upward elevation of the eyebrow.

RESULTS

MRI revealed absence of a clear compressive vessel of the centrally-myelinized portion of the facial nerve, and EMG of the left facial nerve demonstrated no abnormal motor response or evidence of "lateral spread." Given these findings, a diagnosis of hemifacial seizures was made. Microvascular decompression was not recommended, and botulinum toxin injection was not pursued; however, the patient has remained refractory to antiepileptic drugs, possibly due to biochemical alteration by his ganglioglioma. He may eventually require surgical debulking should his symptoms progress.

CONCLUSION

Hemifacial spasm is a well-recognized disorder, but similar conditions can, at times, imitate its appearance. While our patient presented with facial spasms, his clinical history, examination, and radiographic and electrophysiological findings were more consistent with hemifacial seizures secondary to a brainstem lesion, rather than hemifacial spasms. It is important to distinguish the two entities, as misdiagnosis and inappropriate diagnostic or therapeutic measures may be taken inadvertently.

摘要

引言

面部痉挛是一系列复杂的神经运动障碍,具有独特的诊断和治疗方法。由于这些疾病在儿科人群中很多都很罕见,因此在识别这些疾病表现的细微差异时必须格外小心。

方法

我们报告一例3岁男孩,诊断为脑干神经节胶质瘤、Chiari 1畸形,并有2年左侧面部痉挛病史。刻板的面部收缩和轻微的眼球偏斜每10秒出现一次,眉毛向下移动而非向上抬起。

结果

MRI显示面神经中央髓鞘化部分没有明显的压迫血管,左侧面神经肌电图未显示异常运动反应或“侧向扩散”迹象。基于这些发现,诊断为半侧面部癫痫。不建议进行微血管减压,也未采用肉毒杆菌毒素注射;然而,该患者对抗癫痫药物仍然无效,可能是由于其神经节胶质瘤引起的生化改变。如果他的症状进展,最终可能需要手术切除肿瘤。

结论

半侧面部痉挛是一种公认的疾病,但有时类似情况可能会模仿其表现。虽然我们的患者表现为面部痉挛,但其临床病史、检查以及影像学和电生理检查结果更符合脑干病变继发的半侧面部癫痫,而非半侧面部痉挛。区分这两种疾病很重要,因为可能会无意中做出误诊并采取不适当的诊断或治疗措施。

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