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Table 1 Summary of physical, laboratory, imaging findings, treatment and outcomes for 4 cases of suspected autoimmune encephalitis. Clinical features suggestive of autoimmune encephalitis are bolded

From: Immune mediated pediatric encephalitis – need for comprehensive evaluation and consensus guidelines

Case1234
Age in years/sex13/M17/F9/F17/M
Symptom duration8 months3 months1 year6 months
Physical symptomsAnorexia
Motor slowing
Slow, ataxic gait
Abnormal movements of lip/tongue
Mutism
Food refusal
Dizziness
Autonomic instability
Headaches
Abdominal pain
Emesis
Urinary retention
Vibratory tactile sensation in head
Dysphagia
Constipation
Periods of confusion
Disorientation
Decreased speech fluency
Language regression Nonsensical speech
Decreased movements
Decreased speech
Soft, scripted speech
Staring spells
Fatigue
Psychiatric symptomsSocial isolation
Catatonia
Memory difficulties
Decline in academic performance
Social isolation
Anxiety
Panic attacks
Agitation
Insomnia
Paranoia
Inappropriate laughter
Talking to imaginary friends
Disengagement in school
Aggression
Defiance
Social Isolation
Visual hallucinations
Paranoia
Disorganized behavior
Decreased attention and concentration
Social isolation
Not caring for self
Auditory and visual hallucinations
Talking to self
Irritability
Difficulties with multistep commands
Increased sleep
Family History of autoimmune diseaseNoneNoneFather with multiple sclerosis
Paternal aunt with Myasthenia Gravis
None
Serum and urineMildly elevated
GAD65 antibody (0.15 nmol/L), otherwise unremarkable including rest of encephalopathy panel, electrolytes including calcium (with exception of low phosphorus), folate, B12, Lyme serology, herpes simplex, enterovirus, cryptococcus, VDRL, whole exome sequencing
Mildly elevated
GAD65 antibody (0.15 nmol/L), otherwise unremarkable including
CBC, electrolytes, thyroid function, liver function, C-reactive protein, B12, ceruloplasmin, toxicology, heavy metals, blood smear, urinalysis
Unremarkable including outside hospital testing
CBC, CMP, inflammatory markers, thyroid studies, ammonia, folate, copper, ceruloplasmin, heavy metals, plasma amino acids, urine organic acids, very long chain fatty acids, lysosomal disorders screen, chromosomal microarray, Noonan panel. Repeat inpatient testing unremarkable
Unremarkable except for low ferritin (12 mcg/L), including toxicology screen, CBC, electrolytes, inflammatory markers, thyroid function
MRIUnremarkable other than evidence of malnutritionSlit third ventricle with narrowed lateral ventricles, no cerebral hypotension and diffuse changes consistent with perinatal insultUnremarkableUnremarkable
CSFMildly elevated protein (45 mg/dL), otherwise unremarkable; no presence of bandsTotal protein elevated (144 mg/dL; 110 mg/dL 6 months later); no presence of bandsUnremarkable; no presence of bandsPositive GFAP antibodies from outside hospital, repeat negative; no presence of bands
EEGUnremarkableUnremarkableIntermittent diffuse nonspecific bifrontal slowing, bifrontal spikes and sharp waves without clinical correlate, intermittent independent left/right temporal slowingMild nonspecific background slowing
Treatment with IVIG or corticosteroids5 days IV methylprednisolone; 5 days IVIG then monthly infusions for 3 months5 days IVIG then intermittent doses; 5 days methylprednisolone upon readmission3 days high dose IV methylprednisolone then oral prednisone;
single dose IVIG then twice-weekly IVIG, then monthly IVIG;
rituximab
5 days IV methylprednisolone
ResponseImproved over timeInitial significant improvement, not sustained with both treatment course. Return to baseline after neurosurgical interventionSome initial improvement in mood, speech, social interactions; not sustainedInitial improvement, not sustained