Table 1.

Clinical and electrographic data from cases.

	Case 1	Case 2	Case 3
Age	3 years, 6 months	19 years, 2 months	54 years, 2 months
Sex	Male	Female	Female
Days to diagnosis	10 days	28 days	14 days
Clinical symptomsa
Encephalopathy	Y	Y	Y
Movement Disorder	Y	Y	Y
Dysautonomia	Y	N	Y
Initial Lumbar Puncture	WBC: 38 cells/μL	WBC: 48 cells/μL	WBC: 99 cells/μL
	% Lymphocytes: 78	% Lympphocytes: 90	% Lymphocytes: 85
	RBC: 3 cells/μL	RBC: 12 cells/μL	RBC: 0 cells/μL
	Glucose: 45 mg/dL	Glucose: 57 mg/dL	Glucose: 42 mg/dL
	Total Protein: 55 mg/dL	Total Protein: 59 mg/dL	Total Protein: 74 mg/dL
	Oligoclonal Bands: negative	Oligoclonal Bands: positive	Oligoclonal Bands: negative
Initial MRI Brain	Normal	Normal	Normal
Neoplasia	No	No	Ovarian Teratoma
Anti-NMDAr antibody titer at diagnosis (CSF)	1:1280	1:320	1:2560
Immunomodulatory treatments	IVIg (2 g/kg over 5 days) × 1	IVIg (2 g/kg) × 2	IVIg (2 g/kg over 2 days) × 2
	– at diagnosis only	– at diagnosis and 6 mo	– at diagnosis and 3 mo
	IV methylprednisolone (30 mg/kg/d × 5 days) × 1	IV methylprednisolone (1 g/d × 5 days) × 2	IV methylprednisolone (1 g/kg × 5 days) × 1
	– at diagnosis only	– at diagnosis and 6 mo	– at diagnosis only
	Rituximab × 1	Rituximab × 2	Rituximab × 7
	– 14 days after diagnosis	– 10 days after diagnosis and at 6 mo	– at diagnosis and then monthly for 7 mo
Time from diagnosis to 1st Seizure	8 days	5 days	12 days
Seizure Semiology	Focal hemibody tonic extension with secondary generalization. No aura.	Generalized convulsion without aura.	Focal motor seizure with Jacksonian march prior to secondary generalization. Sensory aura preceding.
Inter-ictal EEG abnormalities	Bi-frontal spike and wave activity with right sided slowing and sharp activity	Multi-focal sharp activity and bi-temporal slowing with sharp activity	Bi-temporal sharp activity with generalized slowing and periodic lateralizing discharges
AEDs utilized (in order of administration)	Levetiracetam	Levetiracetam	Levetiracetam
	Valproic Acid	Phenytoin	Lorazepam
	Clobazam	Valproic Acid	Phenytoin
	Midazolam infusion	Lacosamide	Phenobarbital
	Pentobarbital	Phenobarbital	Diazepam
	Ketogenic Diet	Ketamine	Gabapentin
	Ketamine		Ketamine
Time from first seizure to SE	27 days	15 days	94 days
	(day 35)	(day 20)	(day 106)
Non-convulsive SE?	No	Yes	Yes
Seizure onset localization on EEG	Left temporal	Multi-focal (right temporal and left fronto-temporal)	Right fronto-temporal
Time from SE to ketamine	9 days	4 days	32 days
	(day 44)	(day 24)	(day 138)
Days to improve clinically or electrographically after ketamine	≪ 1 day	1 day	2 days
	(day 45)	(day 25)	(day 140)
Seizures after ketamine use	0 seizures in 24 h	0 seizures in 24 h	0 seizures in 48 h
Anti-epileptics at 12 months	Yes: Levetiracetam	Yes: Levetiracetam and Valproic acid	Patient Expired
Symptoms at 12 months	No	Yes: neuropsychiatric only	Patient Expired

Legend: g/kg: grams per kilogram; IVIg: intravenous immunoglobuin; MRI: magnetic resonance imaging; RBC: red blood cell count; WBC: white blood cell count.

^aWithin 4 weeks of diagnosis.