Anti-Lactosylceramide antibody positive combined central peripheral demyelination emerging from long-standing juvenile-onset chronic inflammatory polyradiculoneuropathy; a report of two cases

• Published in: Journal of neuroimmunology Vol. 378; p. 578086
• Main Authors: Takegami, Naoki, Sakuishi, Kaori, Yamaguchi-Takegami, Nanaka, Egashira, Shuhei, Komaki, Shogo, Mutoh, Tatsuro, Toda, Tatsushi
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.05.2023
• Subjects: Adolescent; Anti-neutral glycolipid antibody; Central Nervous System; Chronic inflammatory demyelinating polyradiculoneuropathy; Combined central and peripheral demyelination; Encephalomyeloradiculoneuropathy; Humans; Inflammation; Lactosylceramide; Polyradiculoneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - diagnosis; Encephalomyeloradiculoneuropathy; Lactosylceramide; Combined central and peripheral demyelination; Anti-neutral glycolipid antibody; Chronic inflammatory demyelinating polyradiculoneuropathy
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2023.578086

Anti-Lactosylceramide (LacCer) antibodies are associated with neurological inflammation involving both the peripheral and central nervous system (PNS, CNS respectively), however, the documented number of cases is small. Uncertainty remains whether its positivity can identify a unique clinical entity. Here, we describe two anti-LacCer antibody positive cases, both with long histories (> 30 years) of teenage-diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). CNS lesions including the medulla oblongata were observed for the first time in adulthood. We suggest that this secondary progression of CNS lesions in juvenile-onset CIDP can be one of the characteristic features of anti-LacCer antibody associated neurological disorder.