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Table 1 Clinical Presentation of Six patients with Refractory Anti-NMDA Receptor Autoimmune Encephalitis

From: Effectiveness of intra-thecal methotrexate in refractory Anti-N-methyl-d-aspartate receptor encephalitis

Case

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

Age at onset

2 years

2 years

20 months

4 years

9 years

8 years

Gender

Female

Male

Female

Male

Male

Male

Initial symptoms

Fever

Fever

Fever

Seizure

Behavioral changes

Behavioral changes

Seizures

Focal unaware seizure

Focal unaware seizure

Focal unaware seizure

Focal unaware seizure

Focal unaware seizure

Focal unaware seizure

Seizure control

Yes

No

Yes

Yes

Yes

Yes

Psychiatric symptoms

Yes

Yes

Yes

Yes

Yes

Yes

Movement disorders

Yes

Yes

Yes

Yes

Yes

Yes

Time from onset to encephalopathy

7 days

4 days

2 days

20 days

21 days

6 days

Viral etiology

HHV6

Rhinovirus

HSV1

None

None

None

Initial EEG

Diffuse slowing

Diffuse slowing

Diffuse slowing

Diffuse slowing and bitemporal discharges

Diffuse slowing

Diffuse slowing

MRI brain

*

Normal

**

Mild brain volume loss

Normal

Normal

CSF

WBC

110

9

41

1

23

14

Protein

Normal

Normal

Normal

Normal

Normal

Normal

Initial NMDA titer

1:640

1:640

1:81

1:128

1:128

1:160

NMDA titer post IT-MTX

1:640

1:640

1:1

Not repeated

1:10

Not done

Antiviral treatment

Yes “Gancyclovir”

No

Yes

“Acyclovir”

No

No

No

Duration prior to initiation of IT-MTX

13 months

6 months

42 months

6 months

5 months

8 months

Initial intensive care unit admission

7 days

8 days

7 days

11 days

7 days

73 days

Total length of admission

32 months

15 months

6 years

14 months

2 months

6 months

  1. * High FLAIR signal in the bilateral medial and posterior thalami, bilateral frontal and parietal cortex
  2. ** Atrophy and encephalomalacia involving temporal, inferior frontal and limbic system bilaterally, more on the right side.