关键词: IgLON5 anti-IgLON5 disease autoimmune encephalitis case report cognitive impairment

来  源:   DOI:10.3389/fneur.2024.1340284   PDF(Pubmed)

Abstract:
Anti-IgLON5 disease is a recently discovered autoimmune encephalopathy with sleep disorder as a hallmark in the majority of reported cases. Additional neurological manifestations include bulbar dysfunction, gait problems, movement disorders, oculomotor abnormalities, and hyperexcitability of the nervous system. At present, an increasing number of publications have dealt with the course and possible treatment options for anti-IgLON5 disease, and its clinical spectrum has expanded wider and more heterogeneous. Here, we report a case of a 66-year-old female with cognitive impairment accompanied by slow reaction, impaired memory, and decreased orientation. A positive cerebral MRI change and serum and cerebrospinal fluid (CSF) antibodies against IgLON5 were found during the diagnostic course. Subsequently the patient received immunotherapy and was generally in good health with no new symptoms during follow-up. Early testing for IgLON5 antibodies should be considered in patients with atypical neurological symptoms such as cognitive impairment, slow reaction, or decreased orientation. In clinical practice, immunotherapy should be considered in all cases of anti-IgLON5 encephalopathies.
摘要:
抗IgLON5疾病是最近发现的一种自身免疫性脑病,在大多数报道的病例中具有睡眠障碍的特征。其他神经系统表现包括球功能障碍,步态问题,运动障碍,动眼异常,和神经系统的过度兴奋。目前,越来越多的出版物涉及抗IgLON5疾病的病程和可能的治疗选择,它的临床范围扩大了更广泛和更异质。这里,我们报告了一例66岁的女性,伴有缓慢反应的认知障碍,记忆受损,和减少的方向。在诊断过程中发现了阳性的脑MRI改变以及针对IgLON5的血清和脑脊液(CSF)抗体。随后,患者接受了免疫治疗,总体健康状况良好,随访期间没有新的症状。早期检测IgLON5抗体应考虑在患者的非典型神经症状,如认知障碍,慢反应,或减少定向。在临床实践中,所有抗IgLON5脑病病例均应考虑免疫治疗.
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