PMID- 31632341
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1664-2295 (Print)
IS  - 1664-2295 (Electronic)
IS  - 1664-2295 (Linking)
VI  - 10
DP  - 2019
TI  - Anti-IgLON5 Disease: A Case With 11-Year Clinical Course and Review of the 
      Literature.
PG  - 1056
LID - 10.3389/fneur.2019.01056 [doi]
LID - 1056
AB  - Background: Anti-IgLON5 disease is a novel disorder with a complex interplay 
      between inflammation and neurodegeneration. Patients develop antibodies against 
      IgLON5 but also deposition of neuronal tau protein. Symptoms often have an 
      insidious onset, slow progression and mimic other neurological disorders. Here we 
      report a case with severely prolonged 11-year disease course and provide a review 
      of current reported cases with focus on presentation, work-up, treatment, and 
      outcome. Method: All reported cases of anti-IgLON5 disease were evaluated. Cases 
      reported twice (in case series and as single case reports), were carefully 
      excluded. Results: Most patients display a characteristic sleep disorder with 
      severe insomnia, non rapid eye movement (NREM) parasomnia, with finalistic 
      movements and sleep disordered breathing (stridor and obstructive sleep apnea). 
      Other symptoms are bulbar involvement, gait instability, movement disorders, 
      oculomotor abnormalities, dysautonomia, and peripheral symptoms. Antibodies are 
      present in both serum and CSF and there is a strong correlation with human 
      leukocyte antigen (HLA) DRB1(*)10:01 and HLA-DQB1(*)05:01. Neuropathological 
      examination reveals neurodegeneration with neuronal tau deposits in regions that 
      correlate with the clinical presentation (e.g., predominantly hypothalamus and 
      tegmentum of the brain stem). Majority of cases respond partially to 
      immunotherapy. Cases, who received no treatment or treatment with IV 
      corticosteroids alone, had a higher mortality than cases treated with more potent 
      immunotherapy. Conclusion: The clinical spectrum of Anti-IgLON5 disease continues 
      to expand. Further studies are needed to elucidate the pathophysiology, 
      therapeutic strategies and outcome in this novel disorder. Aggressive 
      immunotherapy seems to increase survival.
CI  - Copyright (c) 2019 Nissen and Blaabjerg.
FAU - Nissen, Mette Scheller
AU  - Nissen MS
AD  - Department of Neurology, Odense University Hospital, Odense, Denmark.
AD  - Department of Clinical Research, University of Southern Denmark, Odense, Denmark.
AD  - BRIDGE, Brain Research - Inter-Disciplinary Guided Excellence, University of 
      Southern Denmark, Odense, Denmark.
FAU - Blaabjerg, Morten
AU  - Blaabjerg M
AD  - Department of Neurology, Odense University Hospital, Odense, Denmark.
AD  - Department of Clinical Research, University of Southern Denmark, Odense, Denmark.
AD  - BRIDGE, Brain Research - Inter-Disciplinary Guided Excellence, University of 
      Southern Denmark, Odense, Denmark.
LA  - eng
PT  - Case Reports
DEP - 20191002
PL  - Switzerland
TA  - Front Neurol
JT  - Frontiers in neurology
JID - 101546899
PMC - PMC6783555
OTO - NOTNLM
OT  - IgLON5
OT  - autoimmune encephalitis
OT  - immunology
OT  - inflammation
OT  - tau
EDAT- 2019/10/22 06:00
MHDA- 2019/10/22 06:01
PMCR- 2019/10/02
CRDT- 2019/10/22 06:00
PHST- 2019/07/06 00:00 [received]
PHST- 2019/09/18 00:00 [accepted]
PHST- 2019/10/22 06:00 [entrez]
PHST- 2019/10/22 06:00 [pubmed]
PHST- 2019/10/22 06:01 [medline]
PHST- 2019/10/02 00:00 [pmc-release]
AID - 10.3389/fneur.2019.01056 [doi]
PST - epublish
SO  - Front Neurol. 2019 Oct 2;10:1056. doi: 10.3389/fneur.2019.01056. eCollection 
      2019.