Autoimmune Encephalitis in Tunisia: Report of a Pediatric Cohort

Table 1

Demographic, clinical, and laboratory features, imaging and accessory tests, treatment, and outcome of 19 children with autoimmune encephalitis.

Case	Gender	Age at diagnosis	Antibodies	Intensity of positive test	Seizure	Associated features	CSF findings	MRI findings	Treatment	Outcome/follow-up duration
1	F	10 months	Anti-NMDAR	CSF: ++	Yes	Behavioral disorders, speech disorders, orofacial dyskinesia, dystonia chorea	Normal	Increased temporal T2 signal	Methylprednisolone; IVIG	Full recovery/3 years
2	M	9 years	Anti-NMDAR	CSF: +++; Serum: +	Yes	Behavioral disorders, decreased level of consciousness, sleepiness, speech disorders, orofacial dyskinesia, dystonia	Normal	Normal	Methylprednisolone; IVIG	Partial recovery/3 months
3	M	8 years	Anti-NMDAR	CSF: +++	Yes	Behavioral disorders, decreased level of consciousness, orofacial dyskinesia	Hyperproteinorrhachia, pleocytosis	Normal	Methylprednisolone; IVIG	Full recovery/6 years
4	M	12 years	Anti-NMDAR	CSF: ++; Serum: ++	Yes	Behavioral disorders, hallucination, speech disorders, dystonia	Normal	Normal	Methylprednisolone; IVIG	Full recovery/3 years
5	F	13 years	Anti-NMDAR	CSF: +; Serum: -	Yes	Behavioral disorders, decreased level of consciousness	Normal	Normal	Methylprednisolone; IVIG; Azathioprine	Full recovery/4.5 years
6	F	5.5 years	Anti-NMDAR	CSF: +; Serum: -	Yes	Behavioral disorders, speech disorders, sleepiness	Normal	T2/FLAIR hyperintensity with no contrast enhancement in deep white matter	Methylprednisolone; IVIG; Azathioprine	Partial recovery/6.5 years
7	F	13 years	Anti-NMDAR	CSF: +; Serum: -	Yes	Behavioral disorders, orofacial dyskinesia	Pleocytosis	Normal	Methylprednisolone; IVIG	Full recovery/6 years
8	M	8.5 years	Anti-NMDAR	CSF: +; Serum: -	Yes	Behavioral disorders, speech disorders, orofacial dyskinesia, sleepiness	Pleocytosis	Normal	Methylprednisolone; IVIG	Full recovery/3 months
9	F	13 years	Anti-NMDAR	CSF: +	No	Behavioral disorders, speech disorders, sleepiness	Normal	Increased temporal T2 signal	Methylprednisolone; IVIG	Partial recovery/9.5 years
10	F	6 years	Anti-NMDAR	CSF: +; Serum: +/-	Yes	Behavioral disorders, decreased level of consciousness, speech disorders, dystonia, orofacial dyskinesia	Hyperproteinorrhachia	Increased temporal T2 signal	Methylprednisolone; IVIG; Azathioprine	Full recovery/4.5 years
11	F	8 years	Anti-NMDAR	CSF: +++; Serum: -	Yes	Behavioral disorders, hallucination, decreased level of consciousness sleepiness, speech disorders, orofacial dyskinesia	Pleocytosis	Increased thalamus T2 signal	Methylprednisolone; IVIG	Full recovery/3 months
12	F	6 years	Anti-Ma2	Serum: +	Yes	Behavioral disorder, speech disorders	Normal	Normal	Methylprednisolone; IVIG	Partial recovery/5.5 years
13	M	8 years	Anti-Ma2	Serum: ++	Yes	Behavioral disorders, decreased level of consciousness, hallucination	Pleocytosis	External capsule; Increased signal	Methylprednisolone; IVIG; Azathioprine	Full recovery/1.5 years
14	F	23 months	Anti-Ma2	CSF: +; Serum: +	Yes	Decreased level of consciousness, speech disorders, behavioral disorders, hallucination	Normal	Increased left temporoparietal, basifrontal, and occipital T2 signal with leptomeningeal enhancement	Methylprednisolone	Partial recovery/3 months
15	M	2.5 years	Anti-Ma2	CSF: ++; Serum: +	Yes	Behavioral disorders, decreased level of consciousness, speech disorders, swallowing and chewing disorders, drooling	Normal	Increased right frontal signal	Methylprednisolone; IVIG; Azathioprine	Partial recovery/6.5 years
16	F	4.5 years	Anti-GAD65	CSF: 45 UI/ml ( UI/ml)	Yes	Behavioral disorders, decreased level of consciousness, orofacial dyskinesia, sleepiness	Normal	Normal	Methylprednisolone; IVIG; Azathioprine	Partial recovery/6 years
17	F	9 years	Anti-GAD65	CSF: 32 UI/ml ( UI/ml)	Yes	Behavioral disorders, decreased level of consciousness, speech disorders, autonomic disturbances, hallucination, sleepiness	Normal	Normal	Methylprednisolone; IVIG; Rituximab	Deceased/6 months
18	M	9.5 years	Anti-GAD65	CSF: 71 U/ml ( UI/ml)	Yes	Decreased level of consciousness, acute confusional state, visual hallucinations, behavioral disorders, tremor	Pleocytosis	Normal	Methylprednisolone; IVIG; Azathioprine	Partial recovery/1.5 years
19	F	12 years	Anti-SOX1	Serum: +++	Yes	Behavioral disorders, decreased level of consciousness, distal upper limb tremor and myoclonia	Pleocytosis	Increased temporal T2 signal	Methylprednisolone; IVIG	Partial recovery/1 year

M: male; F: female; CSF: cerebrospinal fluid; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery; NV: normal value; NMDAR: N-methyl-D-aspartate receptor; GAD65: glutamic acid decarboxylase 65; IVIG: intravenous immunoglobulin; SOX1: Sry-like high mobility group box 1.