RT Journal Article
SR Electronic
T1 Anti-IgLON5 Disease
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e962
DO 10.1212/NXI.0000000000000962
VO 8
IS 2
A1 Jana Werner
A1 Ilijas Jelcic
A1 Esther Irene Schwarz
A1 Elisabeth Probst-Müller
A1 Jakob Nilsson
A1 Bernhard Schwizer
A1 Konrad Ernst Bloch
A1 Andreas Lutterotti
A1 Hans-Heinrich Jung
A1 Bettina Schreiner
YR 2021
UL http://nn.neurology.org/content/8/2/e962.abstract
AB Objective To expand the spectrum of anti-IgLON5 disease by adding 5 novel anti-IgLON5–seropositive cases with bulbar motor neuron disease-like phenotype.Methods We characterized the clinical course, brain MRI and laboratory findings, and therapy response in these 5 patients.Results Patients were severely affected by bulbar impairment and its respiratory consequences. Sleep-related breathing disorders and parasomnias were common. All patients showed clinical or electrophysiologic signs of motor neuron disease without fulfilling the diagnostic criteria for amyotrophic lateral sclerosis. One patient regained autonomy in swallowing and eating, possibly related to immunotherapy.Conclusion IgLON5 disease is an important differential diagnosis to evaluate in patients with bulbar motor neuron disease-like phenotype and sleep disorders. There is need for a deeper understanding of the underlying pathobiology to determine whether IgLON5 disease is an immunotherapy-responsive condition.AHI=apnea-hypopnoea-index; ALS=amyotrophic lateral sclerosis; ASV=adaptive servo-ventilation; CPAP=continuous positive airway pressure; FOIS=Functional Oral Intake Scale; OSA=obstructive sleep apnea; PEG=percutaneous endoscopic gastrostomy; PSG=polysomnography