未经证实:抗IgLON5病是一种以睡眠障碍为特征的自身免疫性脑病。其他临床症状是异质性的,包括运动障碍,球功能障碍,自主神经紊乱,和神经认知障碍。
未经评估:这里,我们报道了一例87岁女性患者的孤立性进展性偏球症。广泛的诊断检查显示血清中针对IgLON5的抗体。病史和多导睡眠图(PSG)均未揭示抗IgLON5疾病中通常报道的睡眠功能障碍的体征和特征。
UNASSIGNED:在广泛的文献综述中,我们确定了其他12项研究报告了确诊的抗IgLON5疾病和舞蹈病患者的临床表型为锥体外系运动障碍。随后,对这些患者的临床特征进行了仔细评估.
未经证实:我们的结果支持抗IgLON5疾病临床表型的多样性,在出现症状的范围内增加孤立的偏球症。由于睡眠相关障碍通常不是咨询的主要原因,只有PSG检查才能发现,我们建议在患有无法解释的运动障碍的患者中,应考虑筛查针对IgLON5的抗体,包括孤立的半球虫病。
UNASSIGNED: Anti-
IgLON5 disease is an autoimmune encephalopathy with sleep disturbances as a hallmark in the majority of reported cases. Additional clinical symptoms are heterogenous and include movement disorders, bulbar dysfunction, autonomic disorders, and neurocognitive impairment.
UNASSIGNED: Here, we report the case of an 87-year-old woman presenting with isolated progressive hemichorea. An extensive diagnostic work-up revealed antibodies against
IgLON5 in the serum. Neither history nor polysomnography (PSG) unveiled signs and features of sleep dysfunction typically reported in anti-IgLON5 disease.
UNASSIGNED: In an extensive literature
review we identified twelve other studies reporting about patients with confirmed anti-
IgLON5 disease and chorea as extrapyramidal movement disorder in their clinical phenotype. Subsequently, clinical characteristics of these patients were carefully evaluated.
UNASSIGNED: Our results support the diversity of clinical phenotypes in anti-
IgLON5 disease, adding isolated hemichorea to the spectrum of presenting symptoms. As sleep-related disorders are often not the leading reason for consultation and only revealed by PSG examination, we suggest that screening for antibodies against IgLON5 should be considered in patients presenting with unexplained movement disorders, including isolated hemichorea.