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1. Case Report :
Autoimmune Meningoencephalitis
Ashifa Maulidya S
Supervisor : dr. Andika Okparasta, Sp. S (K)

2. Patient Identity
● Name :Tn. R, Male
● Age : 29 Yo
● Occupation : Photographer
● Marital status : Married
● Address : Sungai Lilin

3. Timeline
High fever
Rash (+) vesicles (+)
Headache (-)
Dx with Varicella
Altered mental status
- Cognitive decline
- Not able to
communicate
Admitted to Hospital
Progressively worsens AMS:
- Somnolens
- Fever (-)
Patient referred to RSMH
hospital
5 days
RSUD Sekayu
Treated with :
- Dexamethasone 4x5 mg iv
- Ceftriaxone 2x2 gr iv
- Acyclovir 5x800 mg oral
- Miniaspi 1x80 mg PO
- CPZ 1x25 mg
Sens : E2M5V2 VS : at normal limit
Meningeal sign (+)
CSF analysis (10/10/23)
WBC : 2.0 , PMN : 47, MN : 53
Glucose : 81 (serum 93)
Lab results
Anti HIV (-) ,WBC : 17.620
Head contrast CT Scan
Meningeal enhancement in basal
cistern & interhemisphere fissures →
Suggestive meningitis
Tx.
- Acyclovir 5x800 mg
PO 5 days
- PCT 3x500 mg
7 days
1 Oct ‘23 7 Oct ‘23 12 Oct ‘23

4. Head contrast CT Scan (10/10/2023)
Meningeal enhancement in basal cistern
& interhemisphere fissures →
Suggestive meningitis

5. Timeline
Patient presents with decreased of conciousness
Weakness of all four limbs
Fever (-) seizure (-)
Abnormal movement of lips (+) occasionally
History of :
- Trauma (-)
- CVD (-)
- Free sex (-) IV drug use (-) tattoo (-)
- DM (-) HTN (-) and other systemic diseases (-)
- Cigarettes smoke (+) 2 packs / day
RSMH
13 Oct ‘23
Until Now

6. Physical Examination
● Sens : E3M5V2
● TD : 118/70 mmHg
● HR : 70 x/m
● RR : 20 x/m
● T : 37.5 °c
● SpO2 : 98%
Status Neurologis :
● N.III : pupil bulat, isokor
● N.III,IV,VI : kedudukan bola mata di tengah
● N.VII : plicanasolabialis simetris , sudut mulut
simetris
● N.XII: deviasi lidah bdd, disartria (-)

7. Physical Examination
● Lka/ Lki/ Tka/ Tki
Gerakan lateralisasi (-)
Kekuatan lateralisasi (-)
Tonus ↑ / ↑ / ↑/ ↑
Klonus - -
R. Fisiologis ↑ / ↑ / ↑/ ↑
R. Patologis - / - / BCO(+) / BO(+)
● GRM : kaku kuduk (+) , laseque +/+, kerniq +/+
● Gerakan abnormal : Orofacial dyskinesia

8. Laboratory Results (13/10/23)
● Hb : 12.8
● WBC : 27.530*(0/0/88*/5/7)
● Ht : 37%
● RBC : 4.650.000
● Albumin : 3.8
● D-dimer : 3.67
CSF Results (14/10/23)
● Warna : Tidak berwarna
● pH : 9.0
● Leukosit/ sel : 15.0
● MN / PMN : 100/ 0
● LDH : 30
● Glu LCS / Serum : 78 / 127 = 60%
● Protein : 39.3
● Cryptococcus : Negative
● GeneXpert : Negative
Kesan : viral infection

9. Diagnosis
DK
- Penurunan kesadaran
- Quadriparesis tipe spastik
- GRM +
DT
- Meningen, encephalon
DE
- Viral encephalitis
dd/ Autoimmune encephalitis
Treatment
Non Farmakologis
- R/ MRI kepala kontras
- R/ EEG
- Fisioterapi
Farmakologis
- Ceftriaxone 2x2gr iv
- Acyclovir 3x750 mg IV
- Aspilet 1x80 mg PO
- Inj. Dexametason 2x5mg iv tapering
off
- Inj. Omeprazol 1x40mg iv
- Paracetamol 3x500 mg

10. Follow Up
24 Oct
Orofacial dyskinesia dd/ focal
seizure
- As valproate 2x250 mg
- Clonazepam 1x0,5 mg
- THP 2x2 mg PO
Dexamethasone stop
28 Oct
Dx.
Susp. Autoimmune ME
Tx.
Methylprednisolone 1x1 gr 5
hari
17 Oct
Brain MRI + C :
Normal
EEG :
Perlambatan umum irama teta
22 Oct
Kultur LCS
Microorganism : S. Hemolyticus
Sensitive Vancomycin (MIC : 1)
Abx : Vancomycin 2x1 gr iv,
Acyclovir stop
LP evaluasi
- WBC : 7.0
- MN/ PMN : 100/0
- LDH : 30
- Glukosa LCS/ serum :
76/118 : 60%
- Protein : 41.6
- Kultur MO : STERIL

11. Follow Up
03 Nov
+ Azatriopine 1x50 mg
EEG evaluasi :
Perlambatan umum irama teta
8 Nov
Status Neurologis : STQA
R/ Plasmapharesis
14 Nov
Post Plasmapharesis ke-2:
E4M6V2 → Perbaikan klinis

12. Literature review

13. Introduction
● Antibody-mediated autoimmune encephalitis (AE) comprises a group of non-
infectious immune-mediated inflammatory disorders of the brain
parenchyma.
● Symptoms typically include subacute, progressive neuropsychiatric symptoms
with associated cognitive dysfunction, movement disorders, and autoimmune
seizures.
● The diagnosis should be based on objective neurologic dysfunction in
combination with auto antibody testing.

14. Immune mediated encephalitis includes paraneoplastic and autoimmune types
● Paraneoplastic encephalitidies : Always cancer related
● Autoimmune encephalitidies : Associated with antibodies against intracelular (group I) or neuronal cell surface and
synaptic proteins (group II)
The absence of autoantibodies does not exclude the possibility that a disorder is immune mediated, and a positive test
does not always imply an accurate diagnosis
Neuronal intracelullar
(Classic paraneoplastic)
Neuronal cell surface/synaptic
(autoimmune)
CRMP-5, Hu(ANNA-1), Yo, Ri, Ma,
amphiphysin, dll
NMDA, VGKC LGI-1, CASPR, AMPA,
GABA-B, VGCC, dll
Biasanya berhubungan dengan
keganasan
Tidak berhubungan dengan keganasan
(autoimun)
Respon sel T sitotoksik; Antibodi
merupakan epifenomena dari
autoimunitas terhadap tumor
Dimediasi komplemen/antibodi
Respon buruk terhadap terapi
imunosupresan
Respon baik terhadap terapi
imunosupresan

15. CLINICAL FEATURES
▶ Rapidly progressive dementia
▶ Psychiatric symptoms – hallucinations, psychosis
▶ Seizures
▶ Movement disorders : ataxia, dystonia, myoclonus, and orofacial
dyskinesia (NMDAR)
▶ Autonomic disturbances

18. Singkirkanpenyebab infeksi
Singkirkanadanya penyebab
medis lain (misalnya
ensefalitis Wernicke)
Singkirkan adanya
penyakit autoimun
lainnya
Lakukanbeberapa
pemriksaan
Pemeriksaan antibodi
Imaging
EEG
Biopsi
Skrining kanker
Alurdiagnosis ensefalitis autoimun
Diagnosis

19. In addition to the above criteria, patients
should be carefully examined for other
diseases that can mimic autoimmune
encephalitis.
These diseases should be excluded before
immunotherapy begins and in most
instances a detailed clinical history,
complete general and neurological
examination, routine blood and CSF
analysis, and brain MRl.
The most frequent differential diagnoses
are herpes simplex virus encephalitis and
other CNS infections. Importantly, CSF
herpes simplex virus PCR can be negative if
done too early (eg, within 24 h), and this
test should be repeated if the clinical
suspicion remains high

20. Autoimmune Limbic Encephalitis
● CM : Rapid development of confusion,
working memory deficit, mood changes,
and seizures.
● Short-term memory loss is considered the
hallmark of the disorder
● CSF analysis shows mild-to-moderate
lymphocytic pleocytosis in 60–80% of
patients, and elevated IgG index or
oligoclonal bands in approximately 50% of
cases
● Definite case: autoantiboy detected

21. Acute disseminated encephalomyelitis and other syndromes with MRI
features of demyelination
● Acute disseminated encephalomyelitis is a
monophasic, inflammatory disease of the CNS that
mainly occurs in children and adults younger than 40
years.The disorder can be preceded by an acute
systemic infection or vaccination.
● It is characterised by a variable extent of
encephalopathy (a mandatory criterion for a
definitive diagnosis; panel 3), and other neurological
signs, such as cranial nerve palsies, ataxia,
hemiparesis, myelopathy, or optic neuritis.
● MRI shows multiple, large (>2 cm) abnormalities on
T2-weighted FLAIR imaging that can be present in
the supratentorial white matter, basal ganglia,
brainstem, cerebellum, and spinal cord, with or
without contrast enhancement

23. Anti NMDA receptor encephalitis
● Predominantly affects children and young female
patients (12-45 years of age)
● Comonly associated with ovarian teratoma
(94%), followed by extraovarian teratomas
(2%), and other tumors (4%). Herpes simplex
virus-1 encephalitis appears to be a trigger for
anti-NMDAR encephalitis.

26. ● Patients may progress from behavioural changes to catatonia or mutism, so
an important differential diagnosis of anti-NMDAR encephalitis is neuroleptic
malignant syndrome, because many patients are initially treated with
neuroleptics for behavioral symptoms

27. Bickerstaff’s Brainstem Encephalitis
● Bickerstaff ’s brainstem encephalitis is
characterised by subacute onset, in less than 4
weeks, of progressive impairment of
consciousness along with ataxia and bilateral,
mostly symmetrical, ophthalmoparesis.
● The syndrome is usually preceded by an
infectious event, and has a good outcome.
● Additionally, patients frequently develop
pupillary abnormalities, bilateral facial palsy,
Babinski’s sign, and bulbar palsy. Generalised
limb weakness can occur, which overlaps with
features of Guillain-Barré syndrome.

28. ● After excluding all well characterised syndromes of autoimmune
encephalitis (with or without autoantibodies) and other syndromes
accompanied by well defined autoantibodies, a group of patients who
have possible autoimmune encephalitis will remain.
● Patients in this group can be regarded as having probable autoimmune
encephalitis if they satisfy criteria for Hashimoto’s encephalopathy
(panel 6) or the criteria proposed in panel 7

30. Tatalaksana

31. Penatalaksanaan Regimen
Imunoterapi lini pertama
Methylprednisolone
Imunoglobulin intravena
Pertukaran plasma /
imunoadsorpsi
Imunoterapi lini kedua
Rituximab
Siklofosfamid
Terapi alternatif
Tocilizumab
Interleukin-2 dosis rendah
(aldesleukin)
Agen pengatur steroid
digunakan untuk terapi
pemeliharaan
Azathioprine
Mycophenolate mofetil
1 g setiap hari, selama 3–5 hari
2 g / kg selama 5 hari (400 mg / kg / hari)
1 sesi setiap hari selama 5–7 siklus
375 mg / m2 infus IV mingguan selama 4 minggu
750 mg / m2 setiap bulan selama 3-6 bulan
Awalnya 4 mg / kg, diikuti dengan peningkatan hingga
8 mg / kg setiap bulan berdasarkan respon klinis
1,5 juta IU / hari, 4 injeksi subkutan dengan interval 3
minggu
Awalnya 1–1,5 mg / kg satu kali sehari atau dibagi dua
kali sehari, target 2–3 mg / kg / hari
Awalnya 500 mg dua kali sehari, target 1000 mg dua
kali sehari

32. Thankyou
Reference:
● A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15: 391–40 Published Online February 19,
2016
● Antibody-mediated autoimmune encephalitis: A practical approachJustin R. Abbatemarco, Chen Yan, Amy Kunchok, Alexander
Rae-Grant Cleveland Clinic Journal of Medicine Aug 2021, 88 (8) 459-471; DOI: 10.3949/ccjm.88a.20122