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痙攣性てんかんと最初に診断された超エクプレックス症を呈する基底性症候群のトップへ
Top of Basilar Syndrome Presenting With Hyperekplexia Initially Diagnosed as a Convulsive Status Epilepticus.
PMID: 32451184 DOI: 10.1016/j.jemermed.2020.04.038.
抄録
背景:
高エクプレックス症は、触覚または音響刺激に対する顕著な驚愕反応とトーンの増加を特徴とするまれな神経学的障害である。後天性の多錐体症は通常、脳幹の病理学的疾患で見られ、急性に発症すると痙攣発作と誤診されることがあります。
BACKGROUND: Hyperekplexia is a rare neurologic disorder characterized by pronounced startle responses to tactile or acoustic stimuli and increase tone. Acquired hyperekplexia is usually seen in brainstem pathologies and when it develops acutely it can be easily misdiagnosed as a convulsive seizure.
ケースレポート:
38歳の男性が急性発症し，短い不随意のジャラジャラした運動と意識レベルの低下を呈した．当初，痙攣性てんかんと診断され，複数の抗てんかん薬を投与されたが，改善はみられなかった．その後の検査で眼頭反射反応に異常が認められ，右椎骨動脈解離に伴う脳幹梗塞に伴う脳底動脈血栓を伴う多動性てんかんであることが判明した．緊急血栓摘出術が行われ、最終的にはリハビリテーション施設に退院した。なぜ救急医はこのことに注意すべきなのか？これら2つの重篤な病態の病理と緊急治療は異なるため、救急医は多錐体症と痙攣性てんかんとの鑑別方法に注意すべきである。また、CT画像が正常であっても、非典型的な痙攣のような運動と脳幹病理学的徴候が認められる場合には、後天的な脳幹病理（特に脳底動脈血栓塞栓症）を疑うべきである。
CASE REPORT: A 38-year-old man presented with acute onset generalized brief involuntary jerky movements and a decreased level of consciousness. He was initially diagnosed with convulsive status epilepticus for which he received multiple antiseizure medications without any improvement. Further investigations revealed abnormal oculocephalic reflex response and that his movements were in fact hyperkeplexia caused by brainstem infarction with basilar artery thrombus secondary to right vertebral artery dissection. Emergent thrombectomy was performed and he was eventually discharged to a rehabilitation facility. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should be aware of hyperekplexia and how to differentiate it from convulsive stats epilepticus because the pathology and the emergent treatment of these 2 serious conditions are different. An underlying acquired brainstem pathology (especially basilar artery thromboembolism) should be suspected in any patient with untypical convulsive like movements along with focal neurologic signs compatible with brain stem pathology even when computed tomography imaging is normal. © 2020 Elsevier Inc.
Copyright © 2020 Elsevier Inc. All rights reserved.