Case Report

Autoimmune Ensephalitis

Created By :
Chaliza Soliha (130100211)
Rathigka a/p Palani (130100475)

Supervisor :
Dr. dr. Hj. Oke Rina Ramayani, Sp.A (K)

PEDIATRIC DEPARTMENT
HAJI ADAM MALIK HOSPITAL
MEDICINE FACULTY
UNIVERSITY OF NORTH SUMATERA
MEDAN
2017
 CHAPTER 1

INTRODUCTION

1.1. Background

In Indonesia, central nervous system infection ranked 10th out of the order of disease
prevalence. Inflammation that occurs in the central nervous system includes by 3 difficult
diagnoses clinical, such as meningitis, encephalitis and
meningoencephalitis.1-2
Case of central nervous system infection turned out provide a great burden on health
care at many countries, for example in the United States. There is nearly 19,000 patients per
year are hospitalized due to encephalitis with 230,000 days stay at hospital and 1,400 deaths
with total cost issued by the government US $ 650 million per year.3
Based on data obtained by Singh & Promes, incidence of encephalitis is reported as
many as three to four cases per 100,000 population.4 Most cases of encephalitis are not
recognized or experiencing misdiagnosis due to the development of pain quickly in some
cases. Children aged less of 1 year, patients aged over 65 years, and patients with weak
immune systems have a great risk exposed to acute encephalitis. Accurate information about
all events encephalitis is lacking because of the large number the causative agent and not the
case definition. Nevertheless, there is an incidence rate encephalitis is estimated at 1.5 to 8.8
per 100,000 population.5-6
Encephalitis is a broad term encompassing any inflammatory diseases process of the
brain that manifests clinically with alterations of consciousness and/or behavioral changes.
Associated signs and symptoms of encephalitis may include (but are not limited to) seizures,
movement abnormalities (e.g., dyskinesias, choreoathetosis), ataxia, dysautonomia, and
focal neurological deficits. Encephalitis may occur as the result of a primary infection of the
central
nervous system (CNS) or through an autoimmune process triggered by an infection, vaccine,
or occult neoplasm.7
Encephalitis is generally an acute process, but it may be a post-infectious
encephalomyelitis ( acute disseminated encephalomyelitis, ADEM ) that caused by measles
or chickenpox infections, a degenerative chronic disease, or a slow-moving viral infection.8
 Viral infection is the most common form of central nervous system in the world.
Thus, it is now accepted that there are diseases mediated by antibodies from both peripherals
and the central nervous system. Immune-mediated disorders affecting childeren’s brains
have been regarded as a rather rare, if intriguing, group of diseases.9-11
Autoimmune encephalitis may be triggered by infection in which case the term "Post-
infectious Encephalitis" is used. ADEM( Acute Disseminated Encephalomyelitis ) is a Post-
infectious Encephalitis. The illness usually follows in the wake of a mild viral infection (such
as those that cause rashes in childhood) or immunisations. Typically there is a delay of days
to two to three weeks between the triggering infection and development of the Encephalitis.
It has recently been recognised that there are other types of autoimmune encephalitis
resulting from an attack of the brain by the body's immune system. Some of these types of
autoimmune Encephalitis are identified by finding a specific antibody in blood. These
conditions include Potassium channel complex antibody associated encephalitis, NMDAR
and Hashimoto's Encephalitis. But, Anti-NMDAR encephalitis is now considered to be the
most frequently diagnosed of the autoimmune encephalitis and is one of the commonest
causes of encephalitis.12-13
Many types of autoimmune encephalitis have only become understood recently. It is
now known that there are many causes of encephalitis associated with autoantibodies. The
autoantibodies target areas on the outside of nerve cells which changes how the nerves
function, especially how they transmit nerve impluses. These types of autoimmune
encephalitis are given the biochemical names of their targets which can be long and
complex. The trigger that causes autoantibodies to be produced cannot always be found.12

1.1. Objective
The aim from this study is to explore more about encephalitis in children, especially
to know and learn about encephalitis that caused by autoimmune.

CHAPTER 2
LITERATURE REVIEW
2.1. Defenition
Autoimmune disorders are conditions that occur when the immune system (the
body’s defence system) attacks the body’s own tissues as if they were foreign. Our immune
system’s essential function is to continually patrol the body for foreign invaders, such as
viruses and bacteria, and other substances that are injurious to health, then eliminate
them. The down side is the potential for our immune system to mistake something that is
part of ourself as harmful. This inappropriate immune response is known as autoimmunity
and leads to destruction of healthy tissue or changes in how cells function.12
Encephalitis is inflammation or swelling of the brain. The inflammation is caused
either by an infection invading the brain (infectious); or through the immune system
attacking the brain in error (post-infectious or autoimmune Encephalitis). So, autoimmune
encephalitis is an inflammatory diseases process of the brain that manifests clinically with
alterations of consciousness and/or behavioral changes caused by autoimmune process in the
brain.7,12
ADEM (Acute disseminated encephalomyelitis) is a type of autoimmune
encephalitis that has been known for some time. It occurs relatively soon after an acute viral
infection and is also called Post-infectious encephalitis. It is more common in children than
adults. Immune cells attack myelin, which covers nerves, because it “looks like” the virus
they are fighting. Destruction of myelin slows down the rate at which an impulse is
transmitted along nerves. Although a serious illness, it is usually short lived with a good
recovery.12,14

2.2. Epidemiology
Based on data obtained by Singh & Promes, incidence of encephalitis is reported as
many as three to four cases per 100,000 population.4 Most cases of encephalitis are not
recognized or experiencing misdiagnosis due to the development of pain quickly in some
cases. Children aged less of 1 year, patients aged over 65 years, and patients with weak
immune systems have a great risk exposed to acute encephalitis. Accurate information about
all events encephalitis is lacking because of the large number the causative agent and not the
case definition. Nevertheless, there is an incidence rate encephalitis is estimated at 1.5 to 8.8
per 100,000 population.5-6
 Viral infection is the most common form of central nervous system in the world.
Thus, it is now accepted that there are diseases mediated by antibodies from both peripherals
and the central nervous system. Immune-mediated disorders affecting childeren’s brains
have been regarded as a rather rare, if intriguing, group of diseases.9-11
Many types of autoimmune encephalitis have only become understood
recently. Many cases of autoimmune encephalitis associated with antibodies against the N-
Methyl D-Aspartate receptor have recently been reported. Anti-NMDAR encephalitis has
been increasingly recognised with over 700 cases reported by 2014. Anti-NMDAR
encephalitis is now considered to be the most frequently diagnosed of the autoimmune
encephalitis and is one of the commonest causes of encephalitis. NMDA receptor is a protein
in the brain that helps control the electrical activity of nerves and therefore antibodies against
these receptors are likely to have an important role in directly causing the disease.13-14
After the discovery of its pathogenic autoantibody in 2007, anti-NMDAR
encephalitis has become a well recognized autoimmune, inflammatory syndrome.15 To date,
>400 cases have been described in children and adolescents, including those as young as 8
months of age.16-17 An estimated 40% of all reported patients are younger than 18 years, and
young women constitute 80% of all pediatric cases.17 Although the exact prevalence of this
entity has yet to be determined, large-scale studies in both the United Kingdom and Australia
have shown anti-NMDAR encephalitis to be the leading identified cause of
antibodymediated autoimmune encephalitis.18-19

2.3. Pathogenesis
The NMDA receptors are glutamate-gated cationic channels found throughout the
brain that play an important role in synaptic transmission and plasticity. These diheteromeric
and triheteromeric structures are composed of two GluN1 subunits in combination with
either two GluN2 subunits or a GluN2 and a GluN3 subunit. Autoantibodies of
immunoglobulin G (IgG) subclass G1 bind the extracellular domain of the GluN1 subunits,
resulting in antibodymediated capping and internalization of surface NMDA receptors.
The presence of the autoantibody is correlated with a reversible decrease in the surface
expression of these receptors.20,21 The loss of receptors reduces NMDARmediated synaptic
function, resulting in the unique clinical manifestations of the disease.
 The clinical phenotype of anti-NMDAR encephalitis evolves through several stages
of disease progression. In approximately 50% of patients, a prodromal phase is evident days
to weeks before disease onset and may consist of fever, malaise, headache, and/or symptoms
of gastrointestinal or upper respiratory illness. This prodrome is followed by psychiatric
(delusions, hallucinations, paranoia, insomnia, agitation) and neurological (seizures, speech
impairment, ataxia, abnormal movements, autonomic instability) symptoms. Younger
patients tend to present with seizures and abnormal movements, whereas adults typically
present with psychiatric manifestations. In spite of the influence of age of onset on presenting
symptoms, most cases ultimately evolve into a similar syndrome that contains a mixture of
varied neurological and psychiatric manifestations.7,17,22
Seizures are eventually present in up to 80% of patients.17,22 Seizures may appear
focal or generalized at onset, and status epilepticus has been reported. The vast majority of
patients (90% to 100%) have an abnormal electroencephalograph (EEG), typically showing
focal or diffuse slowing and/or epileptiform discharges. An EEG pattern known as extreme
delta brush has been described in anti-NMDAR encephalitis and may be detected in up to
30% of adult patients. This EEG pattern, though not pathognomonic, may support a
diagnosis of anti-NMDAR encephalitis.7,17
Hyperkinetic movements are frequently noted in pediatric anti-NMDAR encephalitis
and include dyskinesias, choreoathetosis, tremor, and dystonia.7 Orofacial dyskinesias are
commonly seen and consist of semirepetitive grimacing, chewing, or biting movements.23
Continuous video-EEG monitoring may assist in excluding an epileptic etiology as the cause
of these paroxysmal movements. Signs of autonomic dysfunction, including hyperthermia,
tachycardia, hypertension, urinary incontinence, central hypoventilation, and cardiac
dysrhythmia, occur in about 40% of preadolescent children and 50% of adolescents.22 Brain
MRI in anti-NMDAR encephalitis demonstrates abnormalities in less than half of all
pediatric patients.17,22 If present, these imaging findings are relatively nonspecific and may
include cortical and/or subcortical, basal ganglia, and infratentorial T2 hyperintensities with
or without transient meningeal enhancement. In addition, children with a primarily
demyelinating appearance on MRI have been reported, some with imaging features
mimicking those found in neuromyelitis optica, ADEM, and multiple sclerosis. The extent
and location of imaging abnormalities does not appear to have a reliable correlation with
clinical course.7,15,22
Evaluation of a child with suspected anti-NMDAR encephalitis should include both
serum and CSF analysis to detect the presence of pathogenic anti-NMDAR autoantibodies.
Antibody testing is more sensitive in the CSF than in the serum, with up to 7% of patients
demonstrating positive CSF titers with concurrent negative serum titers. The CSF antibody
titers correlate strongly with the clinical disease course and remain elevated in those who
experience a relapse or do not show primary clinical improvement.24
Anti-NMDAR encephalitis can be associated with an underlying tumor that
stimulates the production of anti-NMDAR antibodies. An ovarian teratoma is the most
commonly associated tumor and is reported to be present in over half of adult female cases.23
If an associated tumor is discovered, complete tumor resection is important for maximal
recovery.7,22,23 Though not as common in pediatric anti-NMDAR encephalitis, a unilateral
or bilateral ovarian teratoma is discovered in approximately 30% of girls aged 18 years or
younger. In those aged less than 14 years, the prevalence of ovarian teratoma is <10%.17
Given this association, all female patients should undergo MRI of the abdomen and pelvis
in search for an ovarian teratoma. Testicular teratoma in male patients is rare and has not yet
been reported in pediatric cases of anti-NMDAR encephalitis. Extraovarian tumors can
occur but are much more common in older adult cohorts.22

2.4. Clinical Symptoms

Although the cause is different, the clinical symptoms of encephalitis are more or
less identical and distinctive, so they can be used as diagnostic criteria. Encephalitis is
generally preceded by non-specific prodormal fever symptoms such as cough, sore throat,
fever, headache, and abdominal complaints. After that, the more typical symptoms of
progressive lettuce, and the consequent involvement of the brain parenchyme, will result in
decreased consciousness (delirium, apathy, somnolence or coma) and cause behavioral
changes, such as children to become more aggressive.
In addition, multiple symptomatic serebrum symptoms may develop independently
or together such as ataxia, focal neurologic seizures and deficits (limb weakness, focal
seizures, cranial nerve palsies or aphasia) and signs of increased intakranial pressure.
Seizures can be general or focal or just twitching only. Seizures can occur for hours.
Cerebrospinal fluid often within normal limits, sometimes can be found slightly
elevated cell count, protein or glucose levels. In the postinfectious encephalitis group, the
symptoms of primary disease alone may help diagnose. Electroencelography (EEG) often
exhibits low electrical activity corresponding to decreased consciousness.7,8,9,25
If based on the type of clinical characteristics of autoimmune encephalitis can be
seen in this table :
TABLE 1. Clinical Characteristics of Individual Antibody-Associated Encephalitides in
Childhood7

2.5. Diagnosis

1. History taking
Enforcement of the diagnosis of encephalitis begins with a complete history process. History
of infection, immunization status, clinical symptoms and a history of previous symptoms
and presence of risk factors.

2. Physical examination

We can find an increase in physiological reflexes and the discovery of pathological

reflexes.

3. Supporting investigation

a. Lumbar puncture

Is a way of diagnosing encephalitis through brain fluid analysis. In autoimmune

encephalitis, cerebrospinal fluid often within normal limits, sometimes can be found slightly
elevated cell count, protein or glucose levels. There may also be an increase in intracranial
pressure.

b. Electroencephalography

This examination is a way to measure the electrical wave activity in the brain. Visible
decreased electrical activity, corresponding to a decreasing level of consciousness.

c. Brain imaging examination

Among them are CT Scan and MRI that can detect brain swelling. Helps localize the
lesion, locus and ventricular size, hematoma, cerebral region or tumor. If imaging imaging
has signs and symptoms leading to lungs encephalitism the puncture should be performed to
see if there is an increase in intracranial pressure.

Brain MRI in anti-NMDAR encephalitis demonstrates abnormalities in less than half

of all pediatric patients. If present, these imaging findings are relatively nonspecific and may
include cortical and/or subcortical, basal ganglia, and infratentorial T2 hyperintensities with
or without transient meningeal enhancement.

d. Serum and cerebrospinal fluid analysis

 Serum and CSF analysis to detect the presence of pathogenic anti-NMDAR
autoantibodies. Antibody testing is more sensitive in the CSF than in the serum, with up to
7% of patients demonstrating positive CSF titers with concurrent negative serum titers. The
CSF antibody titers correlate strongly with the clinical disease course and remain elevated
in those who experience a relapse or do not show primary clinical improvement.7,24

2.6. Treatment

If these symtomps and signs are recognised, other causes excluded (particularly
infections) and the amdtibody result is positive, treatment should be started. Treatment
consists of immune therapies and removal tumour, if present. The immune therapies use
medicines to dampen down the immune system.14 Treatment regimens used in autoimmune
encephalitis are based on the principles of treatment of other life threatening autoimmune
diseases. Various therapies, including corticosteroids, IVIG, plasma exchange, rituximab
and cyclophosphamide are currently used. However, there is no clear consensus and no
controlled randomised clinical trial data regarding which immunotherapies should be used,
the order in which they should be applied or the duration of therapy. Most experience has
been attained in the treatment of the two most common diseases – anti-NMDR and anti-
LGI1 encephalitis.
In a large multi-institutional observational cohort study of 577 patients with anti-
NMDAR encephalitis, 92% of patients received first-line immunotherapy including steroids,
IVIG and/or plasma exchange, combined with tumour removal (when applicable).
Improvement occurred within 4 weeks in 53% of patients. A total of 97% demonstrated a
good outcome at 24 months. The remaining 47% of patients who did not respond to first-
line treatment, generally had a poorer prognosis.5 However, non responders who
subsequently received rituximab and/or cyclophosphamide had better outcomes than those
who continued on first-line treatments or did not receive any further immunotherapy.13
Anti-NMDAR encephalitis can be associated with an underlying tumor that
stimulates the production of anti-NMDAR antibodies. An ovarian teratoma is the most
commonly associated tumor and is reported to be present in over half of adult female cases.23
If an associated tumor is discovered, complete tumor resection is important for maximal
recovery.7,22,23

Time-sensitive treatment with tumor removal (if one is present) and prompt
immunotherapy appears to improve patient outcome.22 First-line immunotherapy includes
high-dose intravenous corticosteroids, intravenous immunoglobulin (IVIg), plasma
exchange (PLEX), or a combination of the above. In spite of appropriate first-line treatment,
up to 35% of pediatric patients do not respond adequately.7,17 These patients often require
more-potent second-line therapies, which carry a greater risk of adverse events. These
second-line therapies most commonly include rituximab, a B-celledepleting monoclonal
antibody, and/or cyclophosphamide, an alkylating agent that interferes with DNA
transcription.7

2.7. Prognosis
Patients need to be followed closely after recovery for signs of relapse. Relapses in
anti-NMDAR encephalitis patients with non-paraneoplastic-associated disease have been
reported to occur in around 10–25% of patients. Relapses may be separated by periods of
months to years and occur more frequently in patients who are undertreated or not treated
with immunotherapy during the first episode of disease.
Although recovery is typically protracted, the clinical outcome is often good in
children and young adults. Up to 80% of patients have substantial or full recovery, with
reports of gradual, continuing improvement noted up to 2 years after presentation. Clinical
relapse occurs in up to 25% of patients, independent of age of onset, and may occur months
after the initial clinical manifestations. As such, young women who have recovered from
anti-NMDAR encephalitis should undergo yearly tumor surveillance imaging even in the
absence of a tumor at disease onset. How long to follow tumor-negative, anti-NMDAR
patients with imaging surveillance remains unknown. Given relapse rates of 12% to 5%,
tumor-negative patients may require maintenance immunosuppression with a steroid-sparing
agent (e.g., mycophenolate mofetil or azathioprine) for up to 1 year. The effect of long-term
immunosuppression on the risk of relapse is not known.7,13
CHAPTER III

CASE REPORT
Patient Status
Name : ES
Age : 7 years /1 month /14 days
Sex : Female
Address : Jalan Menteng II Gg. Jermal no. 24 Medan
No MR : 00.70.49.19
Date of Admission : April 14th, 2017

History Taking
Main Problem : Loss of conciousness
Study :
 It was experienced by patient in 10 days. Beginning with the seizure experienced
by patient since 10 days ago, long about 15 minutes, spasm after spasm patients are
not aware, seizures in all parts of the body are encountered, spastic encountered.
 A history of seizures was first experienced by patient 2 weeks ago without preceded
fever, after the seizure the patient was unconscious.
 Headache also found in this patient before the seizure came.
 Fever was not found. A history of fever not found.
 Coughing was found in 1 month. Cough phlegm also found. Bloody cough not found
 A history of contact with adults sufferers coughing found. History of father suffers
from pulmonary TB and experienced of consuming OAT for 6 months.
 Nausea and vomiting was not found.
 Feses found normal. Urine found normal.
 The patient refer from the RS Budget Perbaungan and has been treated for 12 days
with diagnosed of encephalitis.
 History of Pregnancy: Not clear.
 History of Birth: The patient is their first child and born quite a month with the help
of a midwife at the clinic. Abnormalities was not found. Child born immediately
crying, breathing spontaneously, blue was not found. BW Born: 3.3 kg, Normal
Births.
 Historyof Immunisation: Not clear.
  History of breastfeeding: There is no history of breast milk. Patient given milk
formula and the mashed banana.
 History of previous illness: Patient already being treated at RS Perbaungan Jasmine
for 10 days and 5 days hospitalized in the ICU.
 History Of Drug Uses :Not clear.

Physical Examination

Present Status:Sensorium:GCS 11 (E4V1M6)

Anemia (+), Icteric (-), Oedem (-), Cyanosis (-), Dyspnoe (-)

Localized Status:

Head:

Eyes: Light reflex (+/+), isochoric pupil2 mm/2 mm, pale inferior palpebra conjunctiva (-/-),

Ears: within normal range

Nose: Plugged NGT, Attached O2 nasal kanul 1 Litre/minute

Mouth:Trismus (-), Oral thrush (+)

Neck: enlargement of the lymph nodes (-)

Thorax: Symmetrical fusiform, Retraction (-)

HR : 100times/minute, regular, noise (-)

RR : 32 times/minute, regular, ronkhi (-/-)
Abdomen : Soft, Normal Peristaltic (+)
Hepar
/Lien : No enlargedpalpable
Ekstremity : Nadi : 100times/minute , regular, pressure
/Volume : adequate, warm
extremity, CRT < 3”, Bp: 100/70 mmHg, stereotipic (+)
Anogenital : female, within normal limit
Neurologic Examination :
 MeningealReflex : Rigid occipital (+) , Kerniq (-) , Laseq (-), Brudzinsky I/II (-)
Pathological Reflex : Babinsky (+/+), Gordon (+/+), Oppenheim(+/+), Chaddock
(+/+) Klonus (+/+)
physiologicalReflex : APR (+/+)
KPR(+/+)
Nutritional Status:

Body Weight: 20 kg

Birth weight: 3.3 grams

Body Height: 117 cm

W/U:86.9%,

H/U:96%,

W/H:95.2%

LABORATORIUM RESULT

14thApril 2017
Complete Blood Count

Test Result Satuan Referral

Hemoglobin 10.3 g/dL 10.8 – 15.6

Erytrocye 3.61 Juta/μL 4.50 – 6.50

Leucocyte 17.600 /μL 4,500 – 13,500

Hematocrit 30 % 33 – 45

Thrombocyte 678.000 /μL 181,000 – 521,000

MCV 83 fL 69 – 93
 MCH 28.5 Pg 22 – 34

MCHC 34.3 g/dL 32 – 36

RDW 13.0 % 11 – 15

MPV 8.8 fL 6.5 – 9.5

PCT 0.600 % 0.100 – 0.500

PDW 8.7 % 10.0 – 18.0

Hitung Jenis

Neutrophyl 79.30 % 25.00 – 60.00

Lymphocyte 13.20 % 25.00 – 50.00

Monocyte 6.70 % 1.00 – 6.00

Eosinophyl 0.60 % 1.00 – 5.00

Basophyl 0.20 % 0.00 – 1.00

AbsoluteNeutrophyl 13.96 103/μL 2.4 – 7.3

AbsoluteLymphocyte 2.32 103/μL 1.7 – 5.1

AbsoluteMonocyte 1.18 103/μL 0.2 – 0.6

AbsoluteEosinophyl 0.11 103/μL 0.10 – 0.30

AbsoluteBasophyl 0.03 103/μL 0 – 0.1

Clinical chemistry

Test Result Satuan Referral

 Carbohydrate Metabolism

Blood glucose (Sewaktu) 106 mg/dL < 200

Kidney

Blood Urea Nitrogen 8 mg/dL 7 - 19

Ureum 17 mg/dL 15 – 40

Creatinin 0.56 mg/dL 0.6 – 1.1

Electrolyte

Calcium(Ca) 9.40 mg/dL 8.4 – 10.2

Sodium(Na) 139 mEq/L 135 – 155

Potassium (K+) 2.7 mEq/L 3.6 – 5.5

Chloride(Cl) 104 mEq/L 96 – 106

15th April 2017

Test Result Referral

Body fluid
Cerebrospinal fluid analysis
Colour Translucent Translucent
LDH U/L 30 <200
Total Protein mg/dl 13.00 <45
JumlahLeukosit 103/Ul 0.001 <0.003
JumlahEritrosit 106/Ul 0.000
Glukosa mg/dl 96 40 – 76
Ph 8.0 7-8
MN Sel % 100.0
 PMN Sel % 0.0

Differential Diagnosis : Meningoencephalitis

Working Diagnosis : Autoimmune Encephalitis
Therapy : - Elevated head 30 0 midline position
- O2 nasal canule 1-2 liter / minute
- IVFD NaCl 0.9% 20 gtt/menit mikro
- Inj. Ranitidine 20 mg/ 12 hour/IV
- Inj. Ceftriaxone 2 gr /24hour/IV
- Inj. Feniton MD 50 mg / 12 hour / IV in 20cc Nacl 0.9%
(finish within 20 minute)
- Inj. Sibital 50 mg in 20cc Nacl 0.9% (finish within 20 minute / 12 hour)
-Risperidon 1 x 0.20 mg for 4 days (H4) – continued dosage
1 x 0.5 mg
- Methylprednisolon 1 x 20 mg (5 Tab)
- Diet SV 1500 kcal + 40 gr / Protein
Rencana Penjajakan : - EEG
- MRI

FOLLOW UP

14th April 2017

S O A P
Unconcious Sens : GCS11 (E4V1M6) - Encephalitis - Elevated head 30 0 midline
(+) - Meningitis position
Temp : 36.1℃
- Meningoencep
-O2 nasal canule 1-2 litre /
Kepala : halitis
minute
Eyes: Light
- IVFD NaCl 0.9% 20
Reflex(+/+),isochoric
gtt/minute mikro
pupil2 mm/2 mm, pale
inferior palpebral - Inj. Ceftriaxone 1 gr / 12
conjunctiva(-/-), hour / IV
Ears:within normal range
- Inj. Fenitoin MD 50 mg / 12
Nose:Plugged NGT, hour in 20cc Nacl 0.9%
Attached O2 nasal canule
- Inj. Paracetamol 200 mg
Litre
1- 2 /minute
(K/P)
Mouth:within normal
- Diet SV 1500 kcal with 40
range
gram protein
Neck:enlargement of the
lymph nodes (-)

Thorax:SymmetricalFusi
form, Retraction (-)
HR : 100times/minute ,
reg, noise(-)
RR : 32 times/minute ,
reg, ronki (-/-)

Abdomen:Soft,
Peristaltic (+) Normal,
Hepar
/Lien : No enlarged
palpable
 Extremity:HR :
100times/minute , reg,
T
/Vadequate, warm
extremity, CRT < 3”, Bp:
100/70 mmHg, streotipic
movement (+)

Anogenital : female,
within normal range

NeurologicExamination:

Meningeal Reflex:Rigid
occipital (+) , Kerniq (-)
,Laseq (-), Brudzinsky
I/II (-)

PathologicalReflex:Babi
nsky (+/+) , Gordon
(+/+), Oppenheim(+/+),
Chaddock (+/+) , Klonus
(+/+)

Physiological Reflex:

APR (+/+) KPR(+/+)

18th April 2017

S O A P

Unconciouss Sens:CM - Autoimmune - Elevated head 30 0 midline

(+) , spasme Encephalitis position
Temp:37.6℃
(+) ,
- -O2 nasal canule 1-2 litre /
uncontrolled Head :
Meningoencephalitis minute
movement +
Eyes:Light Reflex( /+),
(+) , - IVFD NaCl 0.9% 20
isochoric pupil 2 mm/2
cough(+), gtt/minutemikro
mm, pale inferior
fever (-)
palpebral conjunctiva(-/-) -Inj. Methylprednisolon
600mg / 24 hour / IV
Ears: within normal
range -Inj. Ranitidine 20 mg / 12
hour /IV
Nose:Plugged NGT,
Attached O2 nasal canule -Inj. Ceftriaxone 2 gr / 24
1- 2Litre/minute hour / IV

Mouth:within normal - Inj. phenytoin MD 50 mg /

range 12 hour in 20cc Nacl 0.9%

Neck:enlargement of the - Diet SV 1500 kcal + 40

lymph nodes (-) gram protein

Thorax:SymmetricalFusi R/- EEG

form, Retraction (-)
- MRI
HR : 104times/minute , reg,
noise(-)
RR : 28times/minute , reg,
ronki (-/-)

Abdomen: Soft,
Peristaltic (+) Normal,
 Hepar
/Lien : No enlarged
palpable

Extremity: HR :
104times/minute , reg,
T
/Vadequate, warm
extremity, CRT < 3”,
streotipic movement (+)

Anogenital: female,
within normal range

NeurologicExamination:

Meningeal Reflex :Rigid

occipital (-) , Kerniq (-) ,
Laseq (-), Brudzinsky I/II
(-)

PathologicalReflex:Babi
nsky (-/-) , Gordon (-/-),
Oppenheim(-/-),
Chaddock (-/-) ,Klonus
(+/+)

Physiological Reflex :

APR (++/++)
KPR(++/++)

19th April 2017

S O A P

Unconciouss Sens:GCS 11 Autoimmune - Elevated head 30 0 midline

(+) Encephalitis position
Temp:38℃
,uncontrolled
Meningoencephalitis -O2 nasal canule 1-3 litre /
movementl Head :
minute
(+) , cough +
Eyes: Light Reflex( /+),
(+), fever - IVFD NaCl 0.9% 20
isochoric pupil 2 mm/2
(+) gtt/menit mikro
mm, pale inferior
palpebral conjunctiva(-/-) - Inj. Methylprednisolon
600mg / 24 hour / IV
Ears: within normal
range -Inj. Ranitidine 20 mg / 12
hour /IV
Nose :Plugged NGT,
Attached O2 nasal kanul -Inj. Ceftriaxone 2 gr / 24
Mouth:within normal hour / IV
range
- Inj. Phenytoin MD 50 mg /
Neck:enlargement of the 12 hour in 20cc Nacl 0.9%
lymph nodes (-)
- Diet SV 1500 kcal with 40
Thorax:SymmetricalFusi gram protein
form, Retraksi (-)
- Inj. Sibitol 50 mg in 20cc
HR : 100times/minute , reg,
Nacl 0.9%
noise(-)
RR : 28times/minute , reg, - Risperidone 1 x 0.25 mg
ronki (-/-)
R/ - EEG
Abdomen : Soft,
- USG Abdomen
Peristaltik (+) Normal,
- MRI
Hepar
/Lien : No palpable
enlarged
 Ekstremity: HR :
100times/minute , reg,
T
/Vadequate, warm
extremity, CRT < 3”,
stereotypic movement
(+)

Anogenital: female,
within normal range

NeurologicExamination:

Meningeal Reflex :Rigid

occipital (-) , Kerniq (-) ,
Laseq (-),Brudzinsky I/II
(-)

PathologicalReflex:

Babinsky (-/-) , Gordon (-

/-), Oppenheim(-/-),
Chaddock (-/-) ,Klonus
(+/+)

Physiological Reflex :

APR (++/++)
KPR(++/++)

On the date of 19th April, conducted encephalography examination and found the results:
-Looks basic rhythm wave with frequency 4-5 spd, mixed 2-2.5 spd.
-Found wave-wave in right Midtemporal (T4)
-No significant asymmetry
-PS did not cause any significant changes
Impression:
-EEG abnormal
-Found epileptiform waves in T4
-Mild general hypofunction

Dx : Autoimmune ensephalitis

20th April 2017

S O A P

Unconciouss Sens :GCS 11 - Autoimmune - Elevated head 30 0 midline

(+), (E4V1M6) Encephalitis position
uncontrolled
Temp :36.8℃ -O2 nasal canule 1-2 liter /
movement
minute
(+) Head :
,cough(+), - IVFD NaCl 0.9% 20
Eyes: Light Reflex(+/+),
gtt/menit mikro
fever (-) isochoric pupil 2 mm/2
mm, pale inferior -Inj. Ranitidine 20 mg / 12
palpebral conjunctiva(-/-) hour /IV

Ears: within normal - Inj. Ceftriaxone 2 gr / 24

range hour / IV

Nose:Plugged NGT, - Inj. phenytoin MD 50 mg /

Attached O2 nasalcanule 12 hour in 20cc Nacl 0.9%

Mouth:within normal -Inj. Sibitol 50 mg in 20cc

range Nacl 0.9%

Neck:enlargement of the - Risperidone 1 x 0.25 mg

lymph nodes (-)
-Inj. Methylprednisolon 1 x
Thoraxs:SymmetricalFus 20 mg (5 tab)
iform, Retraction(-)
HR : 100times/minute , reg, - Diet SV 1500 kcal with 40
noise(-) gram protein
RR : 24times/minute , reg,
ronki (-/-)

Abdomen: Soft,
Peristaltic(+) Normal,
Hepar
/Lien : No enlarged
palpable

Ekstremity : HR:
100times/minute , reg,
T
/Vadequate, warm
extremity, CRT < 3”,
spastic (-),streotipic
movement(-)

Anogenital: female,
within normal range

NeurologicExamination:

Meningeal Reflex :Rigid

occipital (-) , Kerniq (-) ,
Laseq (-), Brudzinsky I/II
(-)

PathologicalReflex:Babi
nsky (-/-) , Gordon (-/-),
Oppenheim(-/-),
Chaddock (-/-) , Klonus
(+/+)

Physiological Reflex :
 APR (++/++)
KPR(++/++)

21st April 2017

S O A P

Unconciouss Sens :GCS 11 - Encephalitis - Elevated head 30 0

(+) , (E4V1M6) Autoimmune midline position
uncontrolled
Temp :36.2℃ -O2 nasal canule 1-2 liter
movement
/ minute
(+),Spasme Head :
(-), fever (-) - IVFD NaCl 0.9% 20
Eyes:Light Reflex(+/+),
gtt/menit mikro
isochoric pupil 2 mm/2
mm, pale inferior - Inj. Phenytoin MD 50
palpebral conjunctiva(-/-) mg / 12 hour in 20cc
Nacl 0.9%
Ears: within normal
range - Diet SV 1500 kcal with
40 gram protein
Nose:Plugged NGT,
Attached O2 nasal canule

Mouth:within normal
range

Neck:enlargement of the
lymph nodes (-)

Thorax:SymmetricalFusi
form, Retraction (-)
HR : 100kali/menit , reg,
noise(-)
RR : 24kali/menit , reg,
ronki (-/-)

Abdomen:Soft,
Peristaltik (+) Normal,
Hepar
/Lien : No enlarged
palpable

Extremity:HR:
100kali/menit , reg,
T
/Vadequate, extremity
warm, CRT < 3”,
streotipic movement(+)

Anogenital: female,

NeurologicExamination:

Meningeal Reflex :Rigid

occipital (-) ,Kerniq (-),
Laseq (-), Brudzinsky I/II
(-)

PathologicalReflex:

Babinsky (-/-) , Gordon (-

/-), Oppenheim(-/-),
Chaddock (+/+) ,Klonus
(+/+)

Physiological Reflex :

APR (+/+)
KPR(+/+)
On April 20th, 2017, an MRI examination was performed.

On 2nd May 2017, MRI results have been completed and found: There are hypertensive
lesions on T2 and FLAIR in the subcortical right temporal lobes.

4th May 2017

S O A P

Uncontrolled Sens :GCS 11 - Autoimmune - Elevated head 30 0

movement (E4V1M6) Encephalitis midline position
(+),fever (-)
Temp :36.8℃ -O2 nasal canule 1-2 liter
/ minute
Head :
- IVFD NaCl 0.9% 20
Eyes:Light Reflex(+/+),
gtt/menit mikro
isochoric pupil, pale
inferior palpebral - Inj. Phenytoin MD 50
conjunctiva(-/-) mg / 12 hour in 20cc
Nacl 0.9%
Ears: within normal
range - Methylprednisolon tab
1 x 4 mg (H14) for (H 13
Nose:Plugged NGT
– H15)
Mouth: within normal
- Nystatin syrup 4 x 1 cc
range
- Risperidone 1 x 0.5 mg

- Diet SV 1500 kcal with

Thorax:SymmetricalFusi
40 gram protein
form, Retraction (-)
R/ Chemotheraphy CPA
Extremity:HR:
at 11.00 pm ( after
100kali/menit , reg, T/V
 adequate, extremity hidration with Nacl 0,9%
warm, CRT < 3” fluid.

On 4th May 2017, Cyclophospamide (CPA) theraphy had been given at 11.45 pm.

CHAPTER 4
CASE DISCUSSION
Case Theory
Defenition :
On 14th April a girl came with Encephalitis is inflammation or swelling of the
conciousness complaint. It had brain. The inflammation is caused either by an
been started since the patient infection invading the brain (infectious); or through
suddenly had seizure. The patient the immune system attacking the brain in error
refer from the Perbaungan (post-infectious or autoimmune Encephalitis). So,
hospital and has been treated for autoimmune encephalitis is an inflammatory
12 days with diagnosed with diseases process of the brain that manifests
encephalitis. clinically with alterations of consciousness and/or
behavioral changes caused by autoimmune process
in the brain.

Clinical Manifestation :
The patient with a consciousness Encephalitis is generally preceded by non-specific
complaint, this complaint was prodormal fever symptoms such as cough, sore
experienced after the patient had throat, fever, headache, and abdominal complaints.
seizures on the whole body since After that, the more typical symptoms of
2 weeks ago. Cough also progressive lettuce, and the consequent
experienced by patients since 1 involvement of the brain parenchyme, will result in
month ago. decreased consciousness (delirium, apathy,
Headache also found in this somnolence or coma) and cause behavioral
patient before the seizure came. changes, such as children to become more
aggressive.

MeningealReflex : In encephalitis can be found an increase in

Rigid occipital (+) , physiological reflexes and the discovery of
Kerniq(-) , Laseq (-), pathological reflexes.
Brudzinsky I/II (-)
Pathological Reflex :
 Babinsky (+/+), Gordon
(+/+),Oppenheim(+/+),
Chaddock (+/+) Klonus (+/+)
PhysiologicalReflex :
APR (+/+)
KPR(+/+)

Diagnosis :

- In this patient had been found - Cerebrospinal fluid often within normal
the increased of glucose limits, sometimes can be found slightly
level in cerebrospinal elevated cell count, protein or glucose levels.
fluid, 96 mg/dl. (N : 40-
76 mg/dl)
- EEG Impression: - The vast majority of patients (90% to 100%)
1. EEG abnormal have an abnormal electroencephalograph
2. Found epileptiform (EEG), typically showing focal or diffuse
waves in T4 slowing and/or epileptiform discharges.
3. Mild general
hypofunction - Brain MRI in anti-NMDAR encephalitis
- MRI results have been demonstrates abnormalities in less than half
completed and found: of all pediatric patients. If present, these
There are imaging findings may include cortical and/or
hyperintensities subcortical, basal ganglia, and infratentorial
lesions on T2 and T2 hyperintensities with or without transient
FLAIR in the meningeal enhancement.
subcortical right
temporal lobes.

Theraphy:
- Elevated head 30 0 midline - Treatment regimens used in autoimmune
position encephalitis are based on the principles of treatment
-O2 nasal canule 1-2 liter / of other life threatening autoimmune diseases.
minute Various therapies, including corticosteroids, IVIG,
- IVFD NaCl 0.9% 20 gtt/menit plasma exchange, rituximab and
mikro cyclophosphamide are currently used. However,
- Inj. Phenytoin MD 50 mg / 12 there is no clear consensus and no controlled
hour in 20cc Nacl 0.9% randomised clinical trial data regarding which
- Methylprednisolon tab 1 x 4 immunotherapies should be used, the order in
mg (H14) for (H 13 – H15) which they should be applied or the duration of
- Nystatin syrup 4 x 1 cc therapy.
- Risperidone 1 x 0.5 mg
- Diet SV 1500 kcal with 40
gram protein
- Chemotheraphy CPA 750
mg/IV

REFERENCES

1. Saharso, D, Hidayati, SN 2000, Infeksi Susunan Saraf Pusat, Dalam Soetomenggolo,

T, S., Ismael, S, 74 Buku Ajar Neurologi Anak, Ikatan Dokter Anak Indonesia.
2. Carlo, WA 2011, ‘Nervous System Disorders’, in RM Kliegman, BF Stanton, NF
Schor, JW St, Gene & RE Behrman (eds), Nelson Textbook of Pediatrics, 19edn,
Elsevier Inc, Philadelphia.
3. Khetsuriani, N, Holman, RC & Anderson, LJ 2002, ‘Burden of encephalitis-
associated hospitalizations in the United States, 1988-1997’. Clin Inf Dis, vol. 35,
pp.175-82.
4. Singh, A & Promes, SB 2013, ‘Meningitis, Encephalitis, and Brain Abscess’, in JG
Adams (eds), Emergency Medicine, 2edn, Elsevier, pp.1443-1453.
5. Cizman, M & Jazbec, J 1993, ‘Etiology of acute encephalitis in childhood in
Slovenia’, Pediatr Infect Dis J, vol. 12, pp. 903–8.
6. Davison, KL, Crowcroft, NS, Ramsay ME, et al. 2003, ‘Viral encephalitisin England,
1989-1998: what did we miss?’, Emerg Infect Dis, vol. 9, pp. 234– 40.
7. Nicholas J, P Howard. ‘Antibody-Mediated Autoimmune Encephalitis in
Childhood’, Elsevier. 2016; 60: 13-23.
8. J Karen, M Robert, Jenson et al. Nelson Ilmu Kesehatan Anak Esensial. Singapore :
Elsevier; 2014.
9. Arifputera A, Calistania C, Klarisa C et al. Kapita Selekta Kedokteran. Jakarta :
Media Aesculapius; 2014.
10. Vincent A. ‘Autoimmune Encephalitis’, J BMJ. 2015; 85; 1-6.
11. Armangue T, Petit-Pedrol M, Dalman J. Autoimmune encephalitis in children. J
Child Neurol 2012; 27: 1460-9.
12. Dowell E. Understanding Encephalitis, Elaina dowell; 2015.
13. Newman M.P, Blum S, Wong W, et al. Autoimmune encephalitis. J Internal
Medicine. 2015; 148-157.
14. R Sarosh, Vincent A. NMDA-Receptor Antibody Encephalitis. J The Encephalitis
Society. 2010;1-3.
15. Dalmau J, Tuzun E, Wu HY, et al. Paraneoplastic anti-N-methyl-Daspartate receptor
encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61:25-36.
16. Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of
autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual
 viral etiologies in young individuals enrolled in the California Encephalitis Project.
Clin Infect Dis. 2012;54:899-904.
17. Florance NR, Davis RL, Lam C, et al. Anti-N-methyl-D-aspartate receptor
(NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009;66:11-18.
18. Granerod J, Ambrose HE, Davies NW, et al. Causes of encephalitis and differences
in their clinical presentations in England: a multicentre, population-based
prospective study. Lancet Infect Dis. 2010; 10:835-844.
19. Pillai SC, Hacohen Y, Tantsis E, et al. Infectious and autoantibodyassociated
encephalitis: clinical features and long-term outcome. Pediatrics. 2015;135:e974-
e984.
20. Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-
NMDA receptor encephalitis. J Neurosci. 2010; 30:5866-5875.
21. Moscato EH, Peng X, Jain A, Parsons TD, Dalmau J, Balice-Gordon RJ. Acute
mechanisms underlying antibody effects in anti-N-methyl-D-aspartate receptor
encephalitis. Ann Neurol. 2014;76:108-119.
22. Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for
long-term outcome in patients with anti-NMDA receptor encephalitis: an
observational cohort study. Lancet Neurol. 2013;12:157-165.
23. Dalmau J, Gleichman AJ, Hughes EG, et al. Anti-NMDA-receptor encephalitis: case
series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091-1098.
24. Gresa-Arribas N, Titulaer MJ, Torrents A, et al. Antibody titres at diagnosis and
during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet
Neurol. 2014;13:167-177.
25. M Abdoerrachman, M Affandi, Agusman S et al. Ilmu kesehatan anak FK UI.
Jakatra: Infomedika; 2007.