A.

PATIENTS IDENTITY
Name : Mrs. AN
Age : 36 years old
Date of Birth : November 19th, 1979
Marital Status : Married
Religion : Moeslem
Address : Jln. Konggoasa 110
Registration Number : 770354
Date of Admission : August 31st, 2016

B. HISTORY
Chief Complaint : Seizure
History of Disease : Seizure has occurred one week before she was admitted to the
Wahidin Sudirohusodo hospital, more than twenty times on frequency, 4-5 minutes on duration,
tonic-clonic, and happened to all of her body, for two days (according to her family). After that
until she was discharged, she has never had a seizure again. There was a history of fever, and
occured for one month, not constantly. At that time, she was hospitalized at Kolaka Hospital, and
was diagnosed dengue hemorrhagic fever and typhoid fever. According to her family, she had a
decrease of consciousness one week ago, and sometimes she was looked iritable. She appeared
confused if her family tried to make a conversation. There was a history of vomiting, she also
complains an epigastric pain. There was no history of diarrhea. There was a history of prolonged
cough, but she never takes anti tuberculosis drugs. There was no history of headache. There are
no related family with the same disease.

C. PHYSICAL EXAMINATION
Vital Sign:
Blood Pressure : 110/70 mmHg
Heart Rate : 92 times/minute, regularly
Respiration Rate : 20 times/minute
o
Temperature : 37,3 C

Internal State:
Head : pale conjunctiva +/+, yellowish sclera -/-
Neck : Within normal limit
Thorax : Heart : normal heart sound, no gallop and murmur
Lungs: vesicular, ronchi -/-, wheezing -/-
Abdomen : Liver and spleen were not palpable
Local state:
Cheeks : Hyperpigmented lesion (+)
Mouth : oral ulcer (+)

1
Neurological State:
GCS : E4M6V5
Higher Cortical Function : normal
Meningeal Sign : Negative
Cranial Nerve : Round pupils, isocoria, 2,5mm/2,5mm
Direct Light Reflex +/+
Indirect Light Reflex +/+
Other Cranial Nerve : within normal limit
Motoric Function :
Movement: Muscles Strength:
N N 4+ 4+
4+ 4+
N N
Tones: N N Physiologic Reflex:BPR/TPR N N
N N KPR/APRR N N
Pathologic Reflex:HT

B
Sensoric Function : within normal limit
Autonomic Function : Urination : normal
Defecation : normal

D. WORKING DIAGNOSIS
Clinical Diagnosis : post seizure + post decrease of consciousness
Topical Diagnosis : bilateral cerebral hemispheres
Etiological Diagnosis : suspect typhoid encephalopaty

E. TREATMENT
1. IVFD Ringer Laktat 20 drops/minute
2. Citicholin 500 mg/12 hours/intravena
3. Ranitidin 50 mg/12 hours/intravena
4. Sohobion 1 ampul/24 hours/intramuscular
5. Ceftriakson 1 gr/12 hours/intravena

2
 This picture had been taken by patients
husband at August 27th, 2016

F. SUPPORTING EXAMINATIONS
Laboratory Examinations:
Date and time: September 1st, 2016; 14.16
EXAMINATION RESULT NORMAL RANGE UNIT
Blood Chemistry and Other Hematology Examinations
Coagulation
PT 10,5 10 14 second
INR 0,98 --
APTT 22,3 22,0 30,0 second
Immunoserology
HbsAg 0,00 (non reactive) < 0,13 COI
Anti-HCV 0,12 (non reactive) < 1,00 COI
Renal Function
Ureum 34 10 50 mg/dl
Creatinine 1,07 L(< 1,3), P(< 1,1) mg/dl
Liver Function
SGOT 113 < 38 U/L

3
SGPT 63 < 41 U/L
Total bilirubine 0,44 < 1,1 mg/dl
Direct bilirubine 0,29 < 0,30 mg/dl
Phosphatase alkali 89 L: < 270, P: <240 U/L
Total protein 4,5 6,6 8,7 gr/dl
Albumine 2,4 3,5 5,0 gr/dl
Globuline 2,1 1,5 5 gr/dl
Gamma-GT 194 L(11 50), P(7 32) U/L
LE cell Negative negative negative

Date and time: September 2nd, 2016; 13.02

ROUTINE BLOOD RESULT NORMAL RANGE
RBC 5,57 [10^/uL] 4,00 10,0
HGB 6,8 [g/dL] 12,0 16,0
HCT 20,7 [%] 37,0 48,0
MCV 79,9 [fL] 80,0 97,0
MCH 26,3 [pg] 26,5 33,5
MCHC 32,9 [g/dL] 31,5 35,0
PLT 59 [103/mm3] 150 400
WBC 5,57 [103/mm3] 4,0 10,0
NEUT 91,1 [%] 52,0 75,0
LYMPH 3,8 [%] 20,0 40,0
MONO 4,7 [%] 2,0 8,0
EOS 0,2 [%] 1,0 3,0
BASO 0,2 [%] 0,0 0,1

Urinalysis
ITEM NAME RESULT REFERENCE UNIT
Color Cloudy yellow Clear yellow
pH 6,5 4,5 8,0
SG 1,010 1,005 1,035
PRO 1+ Negative
GLU Negative Negative
BIL Negative Negative
UBG Normal Normal
KET +/- Negative
NIT Negative Negative
Blood 3+ Negative
LEU +/- Negative
VC 0 Negative

Date and time: September 2nd, 2016; 14.36

EXAMINATION RESULT NORMAL RANGE UNIT
Blood Chemistry and Other Hematology Examinations
Anemia

4
Fe (Iron) 13 L(59 148), P(37 148) g/dl
TIBC 118 274 389 g/dl
Coombs test Positive/2 negative
Immunoserology
Ferritine >1200 13,00 400,00 ng/ml
Blood smear
Eritrocyte Normocytic normochrome, anisocytosis, ovalocyte (+), inclusion cell
(+), normoblast (-)
Leukocyte The ammount is sufficient, PMN > lymphocytes, toxic granulation
(+), blast cell (-)
Thrombocyte The ammount is decrease, giant thrombocyte (+)
Conclusion Bisitopenic due to infection

Date and time: September 6th, 2016; 15.01

ROUTINE BLOOD RESULT NORMAL RANGE
RBC 2,77 [10^/uL] 4,00 10,0
HGB 7,3 [g/dL] 12,0 16,0
HCT 22,7 [%] 37,0 48,0
MCV 81,9 [fL] 80,0 97,0
MCH 26,4 [pg] 26,5 33,5
MCHC 32,2 [g/dL] 31,5 35,0
PLT 183 [103/mm3] 150 400
WBC 6,74 [103/mm3] 4,0 10,0
NEUT 94,0 [%] 52,0 75,0
LYMPH 3,4 [%] 20,0 40,0
MONO 0,17 [%] 2,0 8,0
EOS 0,0 [%] 1,0 3,0
BASO 0,1 [%] 0,0 0,1

Date and time: September 13th, 2016; 13.50

ROUTINE BLOOD RESULT NORMAL RANGE
RBC 2,20 [10^/uL] 4,00 10,0
HGB 6,1 [g/dL] 12,0 16,0
HCT 19,9 [%] 37,0 48,0
MCV 90,5 [fL] 80,0 97,0
MCH 27,7 [pg] 26,5 33,5
MCHC 30,7 [g/dL] 31,5 35,0
PLT 120 [103/mm3] 150 400
WBC 2,45 [103/mm3] 4,0 10,0
NEUT 76,7 [%] 52,0 75,0
LYMPH 13,9 [%] 20,0 40,0
MONO 8,6 [%] 2,0 8,0
EOS 0,4 [%] 1,0 3,0
BASO 0,4 [%] 0,0 0,1

5
Date and time: September 13th, 2016; 14.40
EXAMINATION RESULT NORMAL RANGE UNIT
Blood Chemistry and Other Hematology Examinations
Liver Function
Albumine 2,1 3,5 5,0 g/dl
Electrolytes
Natrium 142 136 145 mmol/l
Kalium 3,1 3,5 5,1 mmol/l
Chloride 104 97 111 mmol/l

Date and time: September 19th, 2016; 11.05

ROUTINE BLOOD RESULT NORMAL RANGE
RBC 2,54 [10^/uL] 4,00 10,0
HGB 6,9 [g/dL] 12,0 16,0
HCT 22,0 [%] 37,0 48,0
MCV 87 [fL] 80,0 97,0
MCH 27,3 [pg] 26,5 33,5
MCHC 31,4 [g/dL] 31,5 35,0
PLT 204 [103/mm3] 150 400
WBC 2,3 [103/mm3] 4,0 10,0
NEUT 90,0 [%] 52,0 75,0
LYMPH 4,4 [%] 20,0 40,0
MONO 5,0 [%] 2,0 8,0
EOS 0,3 [%] 1,0 3,0
BASO 0,3 [%] 0,0 0,1

Date and time: September 21st, 2016; 10.22

ROUTINE BLOOD RESULT NORMAL RANGE
RBC 2,42 [10^/uL] 4,00 10,0
HGB 6,5 [g/dL] 12,0 16,0
HCT 20,8 [%] 37,0 48,0
MCV 86,0 [fL] 80,0 97,0
MCH 26,9 [pg] 26,5 33,5
MCHC 31,3 [g/dL] 31,5 35,0
PLT 219 [103/mm3] 150 400
WBC 2,14 [103/mm3] 4,0 10,0
NEUT 74,3 [%] 52,0 75,0
LYMPH 15,4 [%] 20,0 40,0
MONO 9,3 [%] 2,0 8,0
EOS 0,5 [%] 1,0 3,0
BASO 0,5 [%] 0,0 0,1

Date and time: September 23rd, 2016; 10.47

ROUTINE BLOOD RESULT NORMAL RANGE

6
RBC 2,56 [10^/uL] 4,00 10,0
HGB 6,9 [g/dL] 12,0 16,0
HCT 22,4 [%] 37,0 48,0
MCV 87 [fL] 80,0 97,0
MCH 27,0 [pg] 26,5 33,5
MCHC 31,0 [g/dL] 31,5 35,0
PLT 256 [103/mm3] 150 400
WBC 2,4 [103/mm3] 4,0 10,0
NEUT 76,2 [%] 52,0 75,0
LYMPH 15,1 [%] 20,0 40,0
MONO 6,7 [%] 2,0 8,0
EOS 2,0 [%] 1,0 3,0
BASO 0,0 [%] 0,0 0,1

Electrocardiography:

7
Conclusion:
- Sinus rythm, 80 bpm
- Lateral and inferior wall ischemic

Thorax PA (August 31st, 2016):

- Bronchovascular complexion is within normal limit
- There is no specific process at both lungs
- Heart: dilated, aortic calcification
- Both sinuses and diaphragms are good
- Bones are intact
Conclusion: Cardiomegaly and aortic artherosclerosis, normal lungs

Non-contrast head CT-Scan (September 1st, 2016):

8
Non-contrast and axial section of head CT Scan examination:
- White and gray matter differentiation is normal
- There is no pathological intracranial lesion, hypodens or hyperdens
- Sulci and gyri are normal
- There is no midline shift
- Ventricle system and subarachnoid space are normal
- Pons, CPA, and cerebellum are normal
- There is physiologic calcification at pineal body
- There is calcification at bilateral basal ganglia
- Paranasal sinuses and air cell mastoid are normal
- Ocular bulbs and retrobulbar space are normal
- Bones are intacts
Conclusion: There is no hypodens/hyperdens intracranial lesion
Brain MRI with contrast (September 9th, 2016)

9
 T1-weighted images T1-weighted images + contrast

Brain MRI with contrast: images

T2-weighted FLAIR
- There is hypointense lesion at T1WI, hyperintense lesion at T2WI, without contrast
enhancement, at right and left basal ganglias.

10
 - Sulci and gyri are normal
- There is no midline shift
- Ventricular system and subarachnoid space are normal
- Cerebellum, pons are normal
- Ocular bulbs are intact; the shape, size, and intensity of anterior and posterior chambers
are normal; lateral and medial rectus muscles, optic nerves, other retrobulbar tissue are
normal
- Paranasal sinuses are normal
- Bones are intact
Conclusion: bilateral cerebral infarction

Abdominal Ultrasonography (September 9th, 2016)

Liver: normal size, regular shape,

parenchymal homogen echo. There is
no SOL, vascular and biliary system are
normal
Gallbladder: contractile
Spleen: normal size, parenchymal echo
is normal, vascular system is normal
Pancreas: normal size and shape, there
is no SOL, pancretic duct is not dilated
Kidneys: normal size and contour.
Pelvocalyceal system is not dilated,
cortical echo is normal, there is no
stone or SOL
Bladder: there is no wall thickening,
regular mucose, there is no stone
There is free fluid echo in right pleural
space, and minimal fluid in Monsson
pouch

Conclusion:
right pleural effusion and minimal
ascites
ANA Profile (September 16th, 2016):
Antigen Class Explanation Specific for
RNP/Sm + Positive MCTD, SLE, systemic sclerosis,
polymyositis, dermatomyositis
Sm (+) Borderline SLE
dsDNA (+) Borderline SLE
Nucleosomes + Positive SLE
Ribosomal P-protein (+) Borderline SLE

11
G. FOLLOW UP
Sept 1st, 2016 Sept 8th, 2016 Sept 18th, 2016 Sept 25th, 2016
Time
(ER) (Lontara 3) (Lontara 3) (Lontara 3)
- Seizure (-) - Seizure (-) - Abdominal - No complaint
- Fever (-) - Abdominal discomfort (+)
Subjective - Abdominal discomfort (+) - Pallor (+)
discomfort (+) - Difficulty of
- Oral ulcer (+) sleeping (+)
Blood
140/80 mmHg 140/80 mmHg 110/70 mmHg 110/70 mmHg
Pressure
96 times/minute, 88 times/ minute, 84 times/ minute, 80 times/ minute,
Pulse
regularly regularly regularly regularly
Respiration 20 times/minute 20 times/ minute 20 times/ minute 18 times/ minute
Temperature 36,4oC 36,6oC 36,60C 36,50C
Anaemia +/+, Anaemia +/+, Anaemia +/+, Anaemia +/+,
hyperpigmentation butterfly rash (+), butterfly rash (+), butterfly rash
Local state
on cheeks, oral oral ulcer (+) oral ulcer (+) (+), oral ulcer (-)
ulcer (+)
GCS E4M6V5 E4M6V5 E4M6V5 E4M6V5
Higher
Cortical Normal Normal Normal Normal
Function
Meningeal
Negative Negative Negative Negative
Sign
Round pupils Round pupils Round pupils Round pupils
Cranial isocoria 2,5/2,5 isocoria 2,5/2,5 isocoria 2,5/2,5 isocoria 2,5/2,5
Nerve mm mm mm mm
DLR +/+, ILR +/+ DLR +/+, ILR +/+ DLR +/+, ILR +/+ DLR +/+, ILR +/+
Other Cr.
Normal Normal Normal Normal
Nerves
Movement N N N N N N N N

N N N N N N N N
Strength 5 5 5 5
4+ 4+ 5 5
4+ 4+ 4+ 4+ 5 5 5 5
Tones N N N N N N N N

N N N N N N N N
Physiologic N N N N N N N N
Reflex
BPR|TPR N N N N N N N N
KPR|APR
Pathologic
Reflex

12
Sensibility Normal Normal Normal Normal
Urination and Urination and Urination and Urination and
Autonomic
defecation are defecation are defecation are defecation are
Function
normal normal normal normal
- Post seizure + - Post seizure + - Post seizure + - Post seizure +
post decrease of post decrease of post decrease of post decrease of
consciousness consciousness consciousness consciousness
due to suspect due to suspect due to central due to central
Diagnosis
thypoid central nervous nervous system nervous system
encephalopaty system lupus lupus lupus
- Anemia
- Oral ulcer
Ceftriaxone 1 Ceftriaxone 1 Omeprazole 40 Omeprazole 20
gr/12 hours/IV gr/12 hours/IV mg/12 hours/IV mg/12 hours/oral
Omeprazole 40 Omeprazole 40 Sohobion 1 Sohobion 1
mg/12 hours/IV mg/12 hours/IV tab/24 hour/oral tab/24 hour/oral
Sohobion 1 Sohobion 1 KSR 1 tab/12 KSR 1 tab/12
amp/24 hour/IM tab/24 hour/oral hours/oral hours/oral
KSR 1 tab/12 KSR 1 tab/12 Vit C 50 mg/24 Vit C 50 mg/24
Treatment hours/oral hours/oral hour/oral hour/oral
Vit C 50 mg/24 Vit C 50 mg/24 Aspirin 80 mg/24 Aspirin 80 mg/24
hour/oral hour/oral hour/oral hour/oral
Consult to Hemato- Consult to
oncology division Psychiatry division
depressive
affect, auditory
hallucination (+)
- Bisitopenia due - Autoimmune - Autoimmune - Autoimmune
to autoimmune hemolytic hemolytic hemolytic
disease DD/ SLE anemia anemia anemia
- Imbalance - Suspect SLE - SLE - SLE
Diagnosis electrolyte - Mental and - Mental and - Mental and
from other - Lateral wall conduct disorder conduct disorder conduct disorder
division ischemic due to organ due to organ due to organ
dysfunction DD/ dysfunction dysfunction
severe - Severe - Severe
depression with depression with depression with
psychosis psychosis psychosis
Treatment Methylprednisolone - Methylprednisol - Methylprednisol - Sandimmun 100
from other 250 mg/12 hours/IV one 125 mg/12 one 125 mg/24 mg (cyclosporin)
division drips for 3 days, hours/IV hour/IV 1 tab/8
then continue 125 - Risperidone 2 - Sandimmun 100 hours/oral
mg/12 hours IV, mg/ tab/12 mg (cyclosporin) - Risperidone 2
tappering off hours/oral 1 tab/8 mg/ tab/12

13
 hours/oral
- Risperidone 2
hours/oral
- Sandepril 50 mg mg/ tab/12
- Sandepril 50 mg
(Maprotiline hours/oral
(Maprotiline
hydrochloride)/ - Sandepril 50 mg
hydrochloride)/
tab/24 (Maprotiline
tab/24
hour/oral hydrochloride)/
hour/oral
- Trihexyphenidil tab/24
- Trihexyphenidil
2 mg tab/12 hour/oral
2 mg tab/12
hours/oral (if - Trihexyphenidil
hours/oral (if
EPS happened) 2 mg tab/12
EPS happened)
hours/oral (if
EPS happened)

This picture was taken by myself at

September 25th, 2016

H. FINAL DIAGNOSIS
Clinical Diagnosis: post seizure + post decrease of consciousness
Topical Diagnosis: bilateral cerebral hemispheres (intracranial vessels and soft tissues)
Etiological Diagnosis: Neuropsychiatric Systemic Lupus Erythematosus

I. DISCUSSION

14
 A woman, 36 years old, was admitted to the hospital due to history of seizure and
history of decrease of consciousness. She had difficulty of sleeping, especially while she was at
hospital. There was also an auditory hallucination. On physical examination, we found pale
conjungtiva, butterfly rash, and oral ulcer. Vital sign: BP 110/70 mmHg, HR 92 beats per minute,
RR 20 times per minute, and temperature 37,3oC. On laboratory finding, we found a decreased
amount of thrombocyte and haemoglobine (bisitopenia), also haematuria on urinalysis. On
radiological finding, we found right pleural effusion and minimal ascites on abdominal
ultrasonography, also bilateral cerebral infarction at ganglia basalis on brain MRI with contrast.
The ANA profile showed an elevated level of antigen Sm, dsDNA, nucleosomes, and ribosomal
P-protein, which are specific for Systemic Lupus Erythematosus.
Based on history of disease, physical examination, and laboratory finding, diagnosis of
Neuropsychiatric Systemic Lupus Eritematosus (NPSLE) was established. Involvement of
Central nervous system (CNS) may become a severe complication of several autoimmune
disorders. Patients can present with either focal symptoms, consisting of stroke and/or transient
ischemic attacks, or with central nonfocal symptoms of cognitive dysfunction, acute confusional
state, seizures, or psychosis.1 This patient present a episodic seizure with psychosis symptoms.
The pathogenic etiologies of NPSLE manifestations are likely to be multifactorial and
may involve autoantibody production, microangiopathy, intrathecal production of
proinflammatory cytokines and premature atherosclerosis. Cellular and parenchymal changes in
lupus murine models include neuronal cytotoxicity and atrophy of dendritic spines. Cerebral
spinal fluid from lupus-prone mice and adult patients with NPSLE reduce the viability of
proliferating neural cells lines. Postmortem histopathologic studies reveal a wide range of brain
abnormalities caused by multifocal microinfarcts, cortical atrophy, gross infarcts, hemorrhage,
ischemic demyelination and patchy multiple-sclerosis-like demyelination in people with SLE. A
microvasculopathy which was formerly attributed to deposition of immune complexes but now is
suspected to arise from activation of complement, appears to be the most common microscopic
brain findings in SLE.1,2
It is becoming clearer that the integrity of the blood-brain-barrier is very important in
SLE related neuropathology. Processes leading to brain dysfunction in SLE probably involve
abnormal endothelial-white blood cell interactions that allow proteins or cells access to the
central nervous system (CNS). This may be a mechanism whereby autoantibody-mediated CNS
effects can occur. Vascular endothelial cells can be stimulated by proinflammatory cytokines or

15
autoantibodies that up-regulate the expression of adhesion proteins on their surface facilitating
lymphocyte entry into the central nervous system.2
Among the neurologic manifestations of systemic lupus erythematosus (SLE), the most
common are the organic encephalopathies (35-75% of case series), which basically comprise all
potential variations of acute confusion, lethargy, or coma; chronic dementias; depression, mania,
or other affective disturbances; or psychosis. Seizures are already known to occur in 14-25% of
patients with lupus compared with 0.5-1% in the general population. This patient had a seizure
and psychosis. Seizures may result from cerebral vasculitis (ischemic or hemorrhagic
manifestations), cardiac embolism, opportunistic infection, drug intoxication, or associated
metabolic derangements. A seizure focus may result from an acute insult or from the
development of a seizure focus in an area of previous brain insult. Partial or secondarily
generalized seizures are most common, but all seizure types have been reported. Psychosis is
reported in up to 3.5% and is characterised by either the presence of delusions or hallucinations.
The latter are most frequently auditory. 3,4 These theories are related to the patient because the
seizure is generalized type and she had an auditory hallucination.
Systemic Lupus Erythematosus, itself, has some clinical features depending on the
organs involmement, as explained below:
Mucocutaneous involvement is almost universal in SLE with both lupus-specific and non-
specific lesion. Lupus-specific lesions can be further classified as acute, subacute, and
chronic lesions. The classic lupus butterfly rash presents acutely as an erythematous,
elevated lesion, pruritic or painful, in a malar distribution, commonly precipitated by
exposure to sunlight. The rash may last from days to weeks and is commonly accompanied
by other inflammatory manifestations of the disease. 4,5 This patient show a butterfly rash on
her cheeks.
The musculoskeletal system is affected in 5395% of SLE patients, can be seen as
arthritis/arthropaty, myositis, and/or avascular bone necrosis.4,5 There is no musculoskeletal
involvement at this patient.
Renal involvement occurs in 4070% of all SLE patients and is a major cause of morbidity
and hospital admissions. Proteinuria of various levels is the dominant feature of lupus
nephritis (LN) and is usually accompanied by glomerular haematuria. Urinalysis is the most
important and effective method to detect and monitor disease renal activity. 4,5 We found
proteinuria (1+) and blood (3+) in her urine, so we can conclude there was a renal
involvement.

16
 Nervous system involvement in SLE remains one of the major causes of morbidity and
mortality. SLE affects both the central nervous system (CNS) and the peripheral nervous
system (PNS). At the peripheral nervous system, the manifestation can be acute
inflammatory demyelinating polyradiculoneuropathy (Guillain-Barre syndrome), autonomic
disorder, single/multiplex mononeuropathy, myasthenia gravis, neuropathy, plexopathy,
and/or polyneuropathy.4 In this case, we found central nervous system and psychiatric
involvement, such as seizure and auditory hallucination.
Pericarditis may occur in approximately 25% of SLE patients. Pericardial effusions may be
asymptomatic and are usually mild to moderate. Myocardial involvement is rare and
typically occurs in the presence of generalised lupus activity. The patient may present with
fever, dyspnoea, tachycardia, and congestive heart failure. Clinical features of left
ventricular dysfunction, non-specific ST-T wave changes, segmental wall motion
abnormalities, and decreased ejection fraction are found in >80% of patients. 4 We could not
define further if there is a cardiovascular involvement, because we just found fever and non-
specific ST-T wave changes on ECG. We did not perform echocardiography, so we could not
define if there was a left ventricular dysfunction or not.
The most common pleuropulmonary manifestation of SLE is pleuritis. Pleuritic pain is
present in 4560% of patients and may occur with or without a pleural effusion, with
clinically apparent pleural effusions reported in up to 50%.4 In this case, we found a sign of
pleuritis, based on abdominal ultrasonography which indicated a right pleural effusion.
Lymphadenopathy occurs in approximately 40% of patients, usually at the onset of disease
or during disease flares. Splenomegaly occurs in 1045% of patients, particularly during
active disease, and is not necessarily associated with cytopenias. 4 We neither found
lymphadenopathy nor splenomegaly.
The most frequent haematological manifestation of SLE is anaemia. This is usually due to
chronic inflammation, but sometimes an autoimmune haemolytic anaemia can be
demonstrated. Leukopenia, such as lymphopenia or less commonly neutropenia, is also well
recognised, but it rarely predisposes to infections. Thrombocytopenia may also be a
recurring feature.4,5 From what we have explained above, this patient had a haemolytic
anemia, there was also leukopenia (lymphopenia) and thrombocytopenia, which indicated
the haematological manifestation of SLE.
GI manifestations are reported in 2540% of patients with SLE, and represent either lupus
GI involvement or effects of medications. Dyspepsia has been reported in 1150%, and

17
 peptic ulcers (usually gastric) in 421%. Abdominal pain accompanied by nausea and
vomiting occurs in up to 30% of SLE patients. Liver chemistries (aspartate aminotransferase
(AST), alanine aminotransferase (ALT), LDH, alkaline phosphatase) may be abnormal in
patients with active disease or those receiving NSAIDs. 4 We found an abdominal discomfort
and an elevated liver chemistries, so we could conclude there was a GI and hepatic
manifestations in this patient.
The Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) group
has recently proposed a revised classification criteria, which hopes to replace the 1997 American
College of Rheumatology criteria, to improve on the sensitivity and specificity. These criteria are
useful and give consistency in the classification of the disease, mainly for the purpose of research
and surveillance. These criteria are explained below:5

Picture 1. 2012 SLICC classification criteria for SLE (based on the literature 5)
This patient fulfilled 8 from 11 clinical criterias, such as acute cutaneous lupus, oral
ulcers, serositis, renal, neurologic, haemolytic anaemia, leukopenia/lymphopenia, and
thrombocytopenia. She also fulfilled 4 laboratory criterias (ANA test, anti-dsDNA, anti-Sm,
Coombs test), so the diagnosis of definitive SLE was uncontested.

18
 Using structural MRI, the majority (40% to 80%) of abnormalities in NPSLE are small
focal lesions concentrating in periventricular and subcortical white matter. Cortical atrophy,
ventricular dilation, diffuse white matter and gross infarctions are also common. 2,6,7 This theory is
corresponded with the case, which we can found a bilateral cerebral infarction at basal ganglias
on brain MRI.
The management of patients with NPSLE includes symptomatic and
immunosuppressive therapies. The EULAR (European League Against Rheumatism)
recommendations for the management of NPSLE are: 2,3,8,9
1) Glucocorticoids and immunosuppressive therapy are indicated for neuropsychiatric
manifestations felt to reflect an immune/inflammatory process (eg, ACS, aseptic meningitis,
myelitis, cranial and peripheral neuropathies and psychosis) following exclusions of non-
SLE-related causes.
High-dose intravenous (IV) corticosteroid regimens consist of methylprednisolone 1-2 g
daily for 3-6 doses, followed by oral prednisone 60 mg daily, then tappering according to
clinical recovery.
Cyclosporine is a more potent immunosuppressive medication that works by blocking the
function of cells in immune system called T-lymphocytes
2) Antiplatelet/anticoagulation therapy is indicated when manifestations are related to
antiphospholipid antibodies, particularly in thrombotic CVD.
3) The use of symptomatic therapies (eg, anticonvulsants, antidepressants) and the treatment of
aggravating factors (eg, infection, hypertension and metabolic abnormalities) should also be
considered. Anti-epileptic drug (AED) therapy is not necessary in patients with single or
infrequent seizures
4) Antiplatelet agents may be considered for primary prevention in SLE patients with
persistently positive, moderate or high, antiphospholipid antibody titres.
On the other side, psychotropic medications (i.e anti-depressants and atypical
antipsychotics) may have an important adjunctive role in SLE patients with affective or
psychotic disorder manifestations.2
This patient was given glucocorticoids and immunosupressive therapy, also antiplatelet
because there was the evidence of cerebral infarction. Anti-epileptic drug was not given because
patient never had a seizure when hospitalized. For psychiatric manifestation, the patient was
given risperidon as an atypical antipsychotics, and sandepril (maprotiline hydrochloride) as an
antidepressant.

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 BIBLIOGRAPHY

1. Azad KAK, Sardar MH, Appolo AM, Malik MU, Sarker M. CNS lupus presenting as
encephalitis: a case report. J Dhaka Med Coll 2011; 20(1): 82-5.
2. Muscal E, Brey RL. Neurological manifestations of systemic lupus erythematosus in
children and adults. Neurol Clin 2010; 28(1): 61-73.
3. Ramachandran TS. CNS lupus [online]. 2016 [cited October 18th, 2016]. Available from:
http://emedicine.medscape.com/article/1146456-overview#showall
4. Bertsias G, Cervera R, Boumpas DT. Systemic lupus erythematosus: pathogenesis and
clinical features. EULAR Textbook on Rheumatic Diseases 2012. p. 477-505.
5. Apostolopoulos D, Hoi AYB. Systemic lupus erythematosus: when to consider and
management. Australian Family Physician 2013; 42(10): 696-700.
6. Rowshani AT, Remans P, Rozemuller A, Tak PP. Cerebral vasculitis as a primary
manifestation of systemic lupus erythematosus. Ann Rheum Dis 2005; 64: 784-6.
7. Kaichi Y, Kakeda S, Moriya J, Ohnari N, Saito K, Tanaka F, et al. Brain MR findings in
patients with systemic lupus erythematosus with and without antiphospholipid antibody
syndrome. American Journal Neuroradiology 2014; 1-5.
8. Bertsias GK, Ioannidis JPA, Aringer M, Bollen M, Bombardieri S, Bruce IN, et al. EULAR
recommendations for the management of systemic lupus erythematosus with
neuropsychiatric manifestations: report of a task force of the EULAR standing committee
for clinical affairs. Ann Rheum Dis 2010; 69: 2074-82.
9. The John Hopkins Lupus Center. Immunosuppresive medications [online]. 2016 [cited
October 18th, 2016]. Available from: http://www.hopkinslupus.org/lupus-treatment/lupus-
medications/immunosuppressive-medications/

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