Department of Neurosurgery, Microsurgical Neuroanatomy Laboratory, FLENI, Buenos Aires, Argentina.
Department of Neurology, FLENI, Buenos Aires, Argentina.
Clin Neurol Neurosurg. 2022 Feb;213:107098. doi: 10.1016/j.clineuro.2021.107098. Epub 2021 Dec 26.
We report 3 cases of Global rostral midbrain syndrome (GRMS) and Corpus Callosum (CC) infarction, in the context of hydrocephalus followed by shunt dysfunction and slit ventricles. Prior shunt implantation had been indicated for adult-onset hydrocephalus secondary to aqueductal stenosis of varying causes. All three patients had been stable for months before developing repeated shunt dysfunctions, ultimately progressing to parkinsonism, Parinaud syndrome, akinetic mutism, pyramidal signs, cognitive impairment, CC infarction and slit ventricles, in the context of CSF overdrainage. Parkinsonism-related symptoms responded to dopa in all cases, but Parinaud syndrome and cognitive impairment persisted. Although GRMS has been described in the context of a transtentorial pressure gradient after shunt blockage, in these three cases with similar clinical presentation, reverse transtentorial pressure gradient and slit ventricles due to shunt overdrainage was the likely cause. The authors discuss the role of CC infarction and provide a detailed analysis after gathering previously described data, to unify information under a recognizable clinical entity and better understand the underlying pathophysiology, treatment options and outcome.
我们报告了 3 例全球颅前段脑桥综合征(GRMS)和胼胝体(CC)梗死病例,这些病例与脑积水相关,随后发生分流功能障碍和狭缝脑室。先前的分流植入是为各种原因导致的导水管狭窄引起的成人获得性脑积水而指示的。所有 3 名患者在出现反复分流功能障碍之前都已经稳定数月,最终进展为帕金森病、Parinaud 综合征、无动性缄默、锥体束征、认知障碍、CC 梗死和狭缝脑室,同时伴有 CSF 过度引流。在所有病例中,帕金森病相关症状对多巴反应良好,但 Parinaud 综合征和认知障碍持续存在。尽管 GRMS 已在分流阻塞后颅腔压力梯度的情况下描述过,但在这 3 例具有相似临床表现的病例中,分流过度引流引起的逆向颅腔压力梯度和狭缝脑室可能是导致 GRMS 的原因。作者讨论了 CC 梗死的作用,并在收集了先前描述的数据后进行了详细分析,以便将信息统一在一个可识别的临床实体下,更好地了解潜在的病理生理学、治疗选择和结果。