Table 2.

Summary of prior reported cases of motor-neuron-disease-like manifestation with IgLON5 disease

Study (number of cases)	Age at diagnosis/sex	Initial symptom	Symptoms suggestive of IgLON5 autoimmunity	Time from symptom onset to diagnosis	Treatment regimen	Treatment outcome
Videnovic et al. (2022) [9]	67/male	Postural instability, falls, dysarthria, dysphagia, and clumsiness of hands	Sleep disturbances (RBD, EDS, OSA), bulbar issues (dysarthria, dysphagia, hypophonia), gait instability, cognitive disturbance, urinary urgency and frequency	3.5 years	Riluzole, IVMP 1 g/day × 5 days, IVIG 2 g/kg per month × 2 followed by Rituximab 1 gm × 2 given 2 weeks apart	Improvement of RBD and EDS
Werner et al. (2021) [7]	74/male	OSA	Recurrent episodic dyspnea necessitating tracheostomy, sleep disturbance (PLMD, NREM parasomnia), bilateral vocal cord paresis, stridor, dysautonomia	2 years	Mercaptopurine 75 mg/day PO	Improvement of dysphagia resulting in PEG removal
	52/male	Dysphagia	Recurrent episodes of dyspnea with stridor with recommendation for tracheostomy, RBD	3.5 years	IVMP 1 g/day × 5 days, alternate day PLEX, Rituximab 375 mg/m2, once)	Stable at 6 months
	77/male	Dysarthria	Recurrent episodic dyspnea necessitating tracheostomy, bilateral vocal cord paresis, dysautonomia	2 years	PLEX, monthly IVIG (0.4 g/kg for 5 days), twice; rituximab (375 mg/m2, twice)	Partial improvement with dysphagia
	63/male	Episodic dyspnea, OSA, parasomnia	Recurrent episodic dyspnea necessitating tracheostomy, sleep issues (PLMD, RBD, REM parasomnia), bilateral vocal cord paresis, recurrent depression, cognitive disturbance (reduced impulse control and semantic fluency), dysautonomia (ED, palpitations, signs of BPH)	1.5 years	IVMP 1 g/day × 5 days, twice; PLEX, rituximab (375 mg/m2, once)	Stable, persistent symptoms without deterioration
	70/male	OSA	Recurrent episodic dyspnea necessitating tracheostomy, sleep issues (RBD, OSA, PLMD, REM motor activity), bilateral vocal cord paresis, dysautonomia (hyperhidrosis)	7 months	IVMP 1 g/day X5 days, PLEX, rituximab 375 mg/m2, once	Stable, persistent symptoms without deterioration
Tao et al. (2018) [6]	57/male	Dysphagia	Recurrent respiratory issues necessitating intubation, involuntary movements, sleep abnormalities (snoring), cognitive issues (abnormal behavior and consciousness)	2 years	IVMP 1 g/day × 3, 500 mg/day × 3, oral prednisone; IVIG 0.4 g/kg × 5	Initial transient improvement followed by disease progression

ED erectile dysfunction, EDS excessive day time sleep, IVIG intravenous immuno-globulin, IVMP intravenous methyl prednisolone, OSA obstructive sleep apnea, PEG percutaneous endoscopic gastrostomy, PLEX plasma exchange, PLMS periodic limb movements of sleep, RBD rapid eye movement behavioral disturbance, REM rapid eye movement