TY - JOUR

T1 - Differentiating anti-IgLON5 disease and Lewy body dementia

T2 - a systematic review

AU - McWilliam, Oskar

AU - Gramkow, Mathias H.

AU - Blaabjerg, Morten

AU - Clemmensen, Frederikke Kragh

AU - Hasselbalch, Steen G.

AU - Frederiksen, Kristian Steen

N1 - Publisher Copyright: © 2024, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.

PY - 2024

Y1 - 2024

N2 - Background: Anti-IgLON5 disease is a rare but potentially reversible cause of cognitive impairment, sleep disturbances, dysautonomia, and movement disorders. It is an autoimmune encephalitis which, due to its insidious onset, could mimic neurodegenerative disorders, and multiple symptoms overlap with those seen in dementia with Lewy bodies (DLB). We hypothesized that the symptomatology and findings in patients with anti-IgLON5 disease overlapped with that of DLB. Objectives: To assess the commonality of features in anti-IgLON5 disease and DLB and identify potential red flags for anti-IgLON5 disease in patients undergoing diagnostic evaluation for DLB. Methods: We searched in MEDLINE, Web of Science, and Embase from inception on December the 8th, 2022 with the search term “IgLON5”. We performed a systematic review of case reports and case series of anti-IgLON5 disease, and two reviewers independently extracted data on symptoms and findings. Frequencies of symptoms were compared with consensus criteria for DLB. Results: We included 57 studies with 127 individual case reports of anti-IgLON5 disease (mean age 63 years at diagnosis, median symptom duration 2 years). Cognitive dysfunction was reported in 45% of cases, REM—sleep behavioral disorder in 15%, and 14% had parkinsonism. Respiratory insufficiency was reported in 37%, and bulbar symptoms in 67%. Conclusions: We found a significant overlap between anti-IgLON5 disease and DLB. We propose that anti-IgLON5 disease should be considered in young patients with DLB with chorea, gaze palsy, early dysphagia, or prominent respiratory symptoms. Our study contributes to the emerging knowledge on symptoms and biomarkers in anti-IgLON5 disease.

AB - Background: Anti-IgLON5 disease is a rare but potentially reversible cause of cognitive impairment, sleep disturbances, dysautonomia, and movement disorders. It is an autoimmune encephalitis which, due to its insidious onset, could mimic neurodegenerative disorders, and multiple symptoms overlap with those seen in dementia with Lewy bodies (DLB). We hypothesized that the symptomatology and findings in patients with anti-IgLON5 disease overlapped with that of DLB. Objectives: To assess the commonality of features in anti-IgLON5 disease and DLB and identify potential red flags for anti-IgLON5 disease in patients undergoing diagnostic evaluation for DLB. Methods: We searched in MEDLINE, Web of Science, and Embase from inception on December the 8th, 2022 with the search term “IgLON5”. We performed a systematic review of case reports and case series of anti-IgLON5 disease, and two reviewers independently extracted data on symptoms and findings. Frequencies of symptoms were compared with consensus criteria for DLB. Results: We included 57 studies with 127 individual case reports of anti-IgLON5 disease (mean age 63 years at diagnosis, median symptom duration 2 years). Cognitive dysfunction was reported in 45% of cases, REM—sleep behavioral disorder in 15%, and 14% had parkinsonism. Respiratory insufficiency was reported in 37%, and bulbar symptoms in 67%. Conclusions: We found a significant overlap between anti-IgLON5 disease and DLB. We propose that anti-IgLON5 disease should be considered in young patients with DLB with chorea, gaze palsy, early dysphagia, or prominent respiratory symptoms. Our study contributes to the emerging knowledge on symptoms and biomarkers in anti-IgLON5 disease.

KW - Anti-iglon5 disease

KW - Autoimmune encephalitis

KW - Cognitive dysfunction

KW - Lewy body dementia

KW - Movement disorders

U2 - 10.1007/s00415-023-12145-8

DO - 10.1007/s00415-023-12145-8

M3 - Review

C2 - 38195895

AN - SCOPUS:85181910176

VL - 271

SP - 1707

EP - 1716

JO - Deutsche Zeitschrift fur Nervenheilkunde

JF - Deutsche Zeitschrift fur Nervenheilkunde

SN - 0939-1517

IS - 4

ER -