Mazzacane F, Ferrari F, Malvaso A, Mottese Y, Gastaldi M, Costa A, Pichiecchio A, Cavallini A
Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.
Department of Emergency Neurology and Stroke Unit, IRCCS Fondazione Mondino, Pavia, Italy.
Front Neurol. 2024 Jan 25;15:1338291. doi: 10.3389/fneur.2024.1338291. eCollection 2024.
Acute amnestic syndrome is an uncommon clinical presentation of neurological disease. Differential diagnosis encompasses several syndromes including Wernicke-Korsakoff and transient global amnesia (TGA). Structural lesions of the fornix account for a minority of cases of acute amnestic syndromes. Etiology varies from iatrogenic injury to ischemic, inflammatory, or neoplastic lesions. A prompt diagnosis of the underlying pathology is essential but challenging. The aim of this review is to systematically review the existing literature regarding cases of acute amnestic syndrome associated with non-iatrogenic lesions of the fornix.
We performed a systematic literature search on PubMed, Scopus, and Web of Science up to September 2023 to identify case reports and case series of patients with amnestic syndrome due to fornix lesions. The systematic review was conducted according to PRISMA guidelines. The research was limited to articles written in English. Cases of fornix damage directly ascribable to a surgical procedure were excluded.
A total of 52 publications reporting 55 cases were included in the review. Focusing on acute/subacute onset, vascular etiology was highly prevalent, being responsible for 78% of cases, 40/55 (74%) of which were due to acute ischemic stroke. The amnestic syndrome was characterized by anterograde amnesia in all patients, associated with retrograde amnesia in 27% of cases. Amnesia was an isolated presentation in most cases. Up to two thirds of patients had persistent memory deficits of any severity at follow-up.
Acute amnestic syndrome can be rarely caused by fornix lesions. In most cases of acute/subacute presentation, the etiology is ischemic stroke, mainly caused by strokes involving the subcallosal artery territory. The differential diagnosis is challenging and a distinction from common mimics is often difficult on a clinical basis. A high index of suspicion should be maintained to avoid misdiagnosis and provide adequate acute treatment to patients with time-dependent disease, also employing advanced neuroimaging. More research is needed to better understand the outcome and identify prognostic factors in patients with amnestic syndrome due to fornix lesions.
急性遗忘综合征是一种罕见的神经系统疾病临床表现。鉴别诊断包括多种综合征，如韦尼克 - 科萨科夫综合征和短暂性全面遗忘症（TGA）。穹窿的结构性病变占急性遗忘综合征病例的少数。病因范围从医源性损伤到缺血性、炎症性或肿瘤性病变。及时诊断潜在病理状况至关重要但具有挑战性。本综述的目的是系统回顾关于与非医源性穹窿病变相关的急性遗忘综合征病例的现有文献。
我们在截至2023年9月的PubMed、Scopus和科学网进行了系统的文献检索，以识别因穹窿病变导致遗忘综合征患者的病例报告和病例系列。系统综述按照PRISMA指南进行。研究仅限于用英文撰写的文章。直接归因于外科手术的穹窿损伤病例被排除。
本综述纳入了共52篇报告55例病例的出版物。关注急性/亚急性起病，血管病因非常普遍，占病例的78%，其中40/55（74%）是由于急性缺血性卒中。遗忘综合征在所有患者中均以顺行性遗忘为特征，27%的病例伴有逆行性遗忘。在大多数情况下，遗忘是一种孤立的表现。高达三分之二的患者在随访时有任何严重程度的持续性记忆缺陷。
急性遗忘综合征很少由穹窿病变引起。在大多数急性/亚急性表现的病例中，病因是缺血性卒中，主要由累及胼胝体下动脉区域的卒中引起。鉴别诊断具有挑战性，在临床基础上通常难以与常见的类似疾病区分开来。应保持高度的怀疑指数，以避免误诊，并为患有时间依赖性疾病的患者提供充分的急性治疗，同时也采用先进的神经影像学检查。需要更多的研究来更好地了解因穹窿病变导致遗忘综合征患者的预后并确定预后因素。
