Pagaling Gerald T, Espiritu Adrian I, Leochico Carl Froilan D, Andal Vida Margarette D, Blasco Krystle Anne R, Bagnas Marjorie Anne C, Pasco Paul Matthew D
Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Philippines.
Department of Clinical Epidemiology, College of Medicine, University of the Philippines Manila, Philippines.
Neurohospitalist. 2021 Apr;11(2):141-147. doi: 10.1177/1941874420953027. Epub 2020 Sep 4.
Wernicke-Korsakoff syndrome (WKS) is a triad of ophthalmoplegia, ataxia and memory deficits due to thiamine insufficiency resulting from under- or untreated Wernicke's encephalopathy (WE), which may be associated with hyperemesis gravidarum (HEG).
We present a case of a 36-year-old Filipino woman in her first trimester with HEG, along with the WKS triad and abnormal hyperintense signals in the bilateral thalami and midbrain, left occipital lobe, periaqueductal gray matter and pontine periventricular areas. Neurologic deficits partially improved but persisted despite intravenous thiamine administration.
A review of current treatments for WE, and the prevention and neurocognitive recovery of WKS was done. The beneficial effects of thiamine for acute WKS are supported by several case reports and clinical experience. Evidence from one randomized controlled trial wherein thiamine was given in various doses for treating WE or preventing WKS in an alcohol-dependent population is limited by methodological issues. Rehabilitation and pharmacotherapy for neurocognitive recovery seems promising, but they have inadequate evidentiary support. More robust studies on multi-modal strategies are warranted to facilitate the neurocognitive recovery of patients with WKS.
韦尼克-科尔萨科夫综合征(WKS)是一种由韦尼克脑病(WE)未充分治疗或未治疗导致硫胺素不足引起的眼肌麻痹、共济失调和记忆缺陷三联征,其可能与妊娠剧吐(HEG)有关。
我们报告一例36岁处于孕早期的菲律宾女性,患有妊娠剧吐,同时伴有韦尼克-科尔萨科夫综合征三联征以及双侧丘脑、中脑、左侧枕叶、导水管周围灰质和脑桥脑室周围区域的异常高信号。尽管静脉注射硫胺素,神经功能缺损有所改善但仍持续存在。
对韦尼克脑病的当前治疗方法以及韦尼克-科尔萨科夫综合征的预防和神经认知恢复进行了综述。硫胺素对急性韦尼克-科尔萨科夫综合征的有益作用得到了几例病例报告和临床经验的支持。一项随机对照试验的证据表明,在酒精依赖人群中给予不同剂量硫胺素治疗韦尼克脑病或预防韦尼克-科尔萨科夫综合征,因方法学问题而受到限制。神经认知恢复的康复治疗和药物治疗似乎很有前景,但证据支持不足。有必要开展更有力的多模式策略研究,以促进韦尼克-科尔萨科夫综合征患者的神经认知恢复。