New face of Neuroinfection; Autoimune encephalitis A 26-year-old female presented with abnormal behavior and seizures over 6 weeks. Testing found antibodies in her cerebrospinal fluid, suggesting autoimmune encephalitis (AIE). AIE is inflammation of the brain caused by antibodies against neuronal antigens and can be associated with infections or tumors. Clinical presentation varies but often includes seizures, psychosis, and cognitive impairment. Diagnosis involves CSF analysis, EEG, MRI and detecting antibodies. Treatment is usually steroids, IVIG, or plasma exchange with immunosuppressants, though relapses can occur. Early detection and immunotherapy improve prognosis. AIE is an important consideration as it often initially appears as psychiatric problems

1. New face of Neuroinfection;
Autoimune encephalitis
Andika Okparasta
Divisi Neuroinfeksi – Neuroimunologi
Bagian Neurologi FK Unsri – RSUP dr. Moh. Hoesin
Palembang

2. Case ilustration:
• S: Female, 26 y.o, presented with
abn fluctuating behaviour (paranoid
ideation) and focal seizure over 6
weeks, after 1-2 weeks  abn
posturing of the right hands,
orrofacial dyskinesia, refractory
status epilepticus and mutism, no
history of fever, headache, visual
complaints or any systemic
complaints.
• O: CSF: cells 7/µL (MN 75%), protein
56 g/dL, glucose 78 g/dL (plasma
106), MRI normal, EEG : delta waves
• A: possible AIE
• P: MP 1 gr/ day for 5 day, supportif
Significant
improvement

4. Outline
• Definition
• Clinical presentation
• Diagnostic
• Treatment
• Prognostic

5. • Encephalitis is defined as inflammation of the
brain parenchyma associated with neurology
dysfunction
• New molecular markers (antibodies) are being
discovered in patients presenting with
encephalitic syndromes –> AUTOIMMUNE
ENCEPHALITIS (AIE)

6. 1960; Limbic
Encephalitis;
lesion involved the
medial temporal
lobe and
hippocampus
Auto-AB often affect
not only limbic
structures
Autoimmune
enceohalitis (AIE)
2007; anti-NMDAR
encephalitis

8. • AIE may be associated with the precence of
specific autoantibodies
• Infections as possible triggers  HSV
encephalitis around 25 % cases
• AIE is also associated with tumors

9. AIE associated with AB against
neuronal cell surface antigens:
• Anti-NMDAR , Anti-
AMPAR, Anti-GABAb, Anti-
LGI-1, Anti-Gly-R, Anti-
DPPX, Anti-mGLUR5, Anti-
Adenylate-kinase 5
AIE associated with AB againts
intracelluler antigens:
• Anti-Hu, Anti-Ri, Anti-Yo,
Anti-Ma2/Ta, Anti
amphiphysin, Anti-GAD65

10. • The target antigens in
AIE involved in neuronal
sygnaling and synaptic
plasticity
• Three mechanisms for
the resulting symptoms:
– Receptor
internalization
– Antibody blockade
of ion entry
– Complement
mediated cell lysis.

11. 81.2
90
90.4
69.1
70.2
21.8
51.3
33.3
47
78.5
63.6
71.3
22.1
17
37.7
43.6
anti-NMDAR
anti-VGKC
anti-GABAb
AIE associated with intracelullar
antibodies
Clinical presentation
seizure psychosis cognitive impaiment malignancy

12. Nawa-apisak et al.2016. Neuroimmunol Neuroinflamm 3: 79-85

13. Diagnostic tools
• CSF  moderate lymphocytic pleocytosis
(<100 cells/µl)
• The sensitivity of antibody testing has only
been investigated in a few types AIE 
primarily in NMDAR encephalitis

14. Diagnostic tools (cont’)
• EEG is often abnormal  extreme delta brush

15. Diagnostic tools (cont’)
MRI is frequently normal or shows only slight
alterations  not spesific; show high uni/bilateral
T2/Flair-signals on medial temporal lobe

16. • Tumors screening is important

21. Treatment

22. AIE is not
always
monophasic
and relapse of
AIE has been
noted even
after 5-10
years
Oral corticosteroids + monthly
IVIG or PLEX
Azathioprine
Mycophenolate mofetil

23. Prognostic factor
• Delay in immunotherapy
• Altered consciousness
• ICU admission
• Older age (??)
• The presence of status epilepticus
• CSF abnormality and MRI changes

24. Take home messages..
• Almost cases of AIE is appear as psychiatric
problem
• Tumors screening is important
• Early detection and management are
important for good outcome

25. Harga khusus untuk
peserta DIES NATALIS FK
UNSRI 2019