Anti-IgLON5 disease with distinctive brain MRI findings responding to immunotherapy: A case report. Issue 4 (29th January 2021)

• Record Type: Journal Article
• Authors: Pi, Yan; Zhang, Li-li; Li, Jing-cheng
• Other Names: Saranathan. Maya section editor.
• Is Part Of: Medicine. Volume 100:Issue 4(2021)
• Publication Date: 2021-01-29
• Subjects: anti-IgLON5 disease -- brain magnetic resonance imaging -- immunotherapy; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000024384
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract: Rationale: Anti-IgLON5 disease was first described as a progressive antibody-associated encephalopathy, with multiple non-specific clinical symptoms including sleep dysfunction, bulbar symptoms, progressive supranuclear palsy-like syndrome, cognitive impairment, and a variety of movement disorders. This newly discovered disease presents with unremarkable or unspecific brain magnetic resonance imagings (MRI), and have poor responsiveness to immunotherapy. Patient concerns: In this case, a 37-year-old man presented with 4-day history of gait instability, dysarthria, and oculomotor abnormalities. The initial neurologic examination revealed mild unsteady gait, subtle dysarthria, and left abducent paralysis. Diagnosis: The patient was diagnosed with anti-IgLON5 disease, based on clinical features and positive anti-IgLON5 antibodies in serum. Interventions: Initially, the patient was treated with high dosages of methylprednisolone and immunoglobulins. Outcomes: The symptoms of patient rapidly improved after high-dose intravenous methylprednisolone and immunoglobulins. Conclusions: In this paper, we report a new case of anti-IgLON5 disease with major symptoms of gait instability, dysarthria, and oculomotor abnormalities, with distinctive brain MRI findings, and responsive to immunotherapy.