A 30 -year- old Female

with
Fever and Disorientation
Dr. Farin Hossain
FCPS Part-2 Trainee
Department of Medicine, Unit 11
Dhaka Medical College Hospital
Case Summary
Mrs. Shamsunnahar, a 30-year-old female,
Muslim, married, normotensive, non-diabetic,
housewife, hailing from Feni, was admitted
into Medicine Unit-11, DMCH on 4/08/2023
with the complaints of
Contd.

• Fever for 3 days

• Disorientation for 2 days
• History of convulsion for 2 days
Contd.
According to the statement of the patient’s
attendant, she was reasonably well three
days back. Then she developed fever which
was high grade, continuous, with highest
recorded temperature 104°F.
Contd.
The fever subsided by taking antipyretic and
was not associated with chills & rigor,
hemoptysis, hematemesis, respiratory
distress, profuse sweating, burning micturition
or diarrhea. There was no travel history or
contact with TB patient but
Contd.
she complained of headache during fever.
The headache was new in onset, dull aching,
bilateral and continuous. But not associated
with aura, vomiting or blurring of vision.
Contd.
Then she got admitted to a local hospital for
these complaints. But her condition
deteriorated, and the patient’s attendant
notified that her movement of right side was
reduced compared to the left side.
Contd.
The weakness was sudden in onset,
developed in right upper and lower limb at the
same time and was non progressive.
Contd.
Patient developed disorientation which was
characterised by loss of orientation of time,
place and person, impaired memory &
antisocial, aggressive behaviour.
Contd.
There was history of convulsion. First
convulsion occurred on the 4th day of her
fever. It was episodic, sudden in onset,
paroxysmal, first involving the right upper limb
then became generalized and duration was
about 1 minute.
Contd.
In post ictal-phase the patient was drowsy,
partially responsive to verbal commands,
easily arousable with responding to mild
painful stimuli.
Contd.
There was no history of photophobia,
tongue bite, injury, spontaneous urination or
defecation, eyeball rolling, frothy sputum
from the angle of mouth and any triggering
factor.
Contd.
There was also no history of trauma to the
head, joint pain, skin rash, recent
vaccination, fetal loss, significant weight
gain or weight loss.
Contd.
She had no history of significant medical or
surgical complaints before the course of her
illness.
She is an occasional betel nut chewer and
has no history of high-risk sexual exposure
or substance abuse.
Contd.
She has been married for 14 years and has
a son and a daughter. Among her children,
her 5-year-old son was admitted into
Bangladesh Shishu Hospital with the
complaints of fever and disorientation during
the course her illness.
Contd.
Her menstrual cycle is regular with normal
flow and duration. She is not using any type
of contraceptive method.
Contd.
She belongs to lower middle-class family.
She lives in semi pakka house and drinks
water from arsenic free tube well.
Contd.
She was immunized under EPI schedule and
two doses of Covid 19 vaccine.
Examination
General Examination
• Appearance: Ill looking
• Body build: average
• Decubitus: supine
• Nutritional status: average
• Anemia: mild
• Jaundice: absent
• Cyanosis: absent
Contd.
• Clubbing
• Koilonychia
• Leukonychia
absent
• Dehydration
• Edema
• Bony tenderness
Contd.
• Pulse: 85 bpm
• BP: 110/70 mm Hg
• Temperature: 99°F
• Respiratory rate: 20 breaths per minute
• Lymph node: not palpable
Contd.
• Thyroid gland: not enlarged
• Neck vein: not engorged
• Hair distribution : normal
• Bedside urine (protein, sugar) : absent
Systemic Examination
Neurological Examination (5/8/2023)
Higher cerebral function
• Level of consciousness: disoriented
• Emotional state : labile
• Speech and language: impaired
• Memory , intelligence, judgment: impaired
• GCS: 11/15 E3V4M4
Cranial nerve examination
On admission we could not perform the cranial
nerve examination. But later on it was done and
revealed no abnormalities

Fundoscopy
Normal, no papilloedema
Motor system
Upper limb Right Left

Inspection Normal Normal

Muscle bulk Normal Normal

Tone Increased Normal

Power 1/5 4/5

Lower limb Right Left

Inspection Normal Normal

Muscle bulk Normal Normal

Tone Increased Normal

Power 1/5 4/5

Clonus (ankle & patella) Absent Absent

Reflex Right Left

Biceps Exaggerated Normal

Supinator Exaggerated Normal
Triceps Exaggerated Normal
Knee Exaggerated Normal
Ankle Exaggerated Normal
Planter Extensor Extensor
Cerebellar sign
Coordination: Right side could not be
evaluated due to patient’s weakness, left side
was normal
Romberg sign and gait: Could Not be
evaluated
Sensory system: Intact
Signs of meningeal irritation
Neck stiffness: absent
Kernig sign : absent
Brudzinski sign: absent
Cardiovascular Examination

Pulse: all the peripheral pulses were normal. No

irregularly irregular pulse.
Precordium:
Inspection
Palpation Revealed first and second heart sound
were normal
Auscultation There was no thrill or murmur
Rheumatological Examination
Revealed no abnormality

Other Systemic Examination

Revealed no abnormality
Problem List
High grade fever Disorientation

30 year female

Right sided Convulsion (3-4

weakness episodes)
Provisional
Diagnosis?
 Viral encephalitis
with
right sided hemiparesis
Differential Diagnosis
● Acute disseminated encephalomyelitis
(ADEM)
● Antiphospholipid syndrome (APS)
Investigations
CBC
Date 02/08/23 03/08/23 05/08/23 07/08/23 13/08/23

Hb% g/dl 10.2 10.3 11 11.3 10.2

TC of WBC 4x10^3 4x10^3 5.4x10^3 8.9x10^3 8.5x10^3

TPC k/ul 155 158 157 156 265

ESR mm/hr 50 50 33 29 33

HCT% 30.3 30.5 32 33.5 29.7

Dengue test
Dengue NS1- negative
Dengue IgM - negative
Dengue IgG - negative
Serum Electrolytes (05/08/23)

Sodium 139 mmol/L

Potassium 3.7mmol/l

Chloride 103 mmol/l

Tco2 28 mmol/l
Routine Investigations

Urine R/M/E Normal

CXR (P/A): Normal
ECG Normal
RBS 7 mmol/L
CSF Study (08/08/23)
Cytological examination:
 Appearance: normal
 Total WBC : 02 /cmm
 Total RBC: nil
 Neutrophil: absent
 Lymphocytes: 100%
 Gram staining: no bacteria found
Contd.
Biochemical test
 CSF-Protein: 37 mg/dl ( normal 15-48)
 CSF glucose: 74 mg/dl ( normal 40-70)
Fluid for ADA
 Negative
CSF Fluid for Gene xpert
 MTB Not detected
Other Investigations

Anti-phospholipid antibody (IgM,

IgG):
ANA Negative
p-ANCA
c-ANCA
Echocardiography Normal
Contd.
D-dimer
LDH
Normal
PT and INR
Fasting lipid profile
VDRL Not reactive
HIV 1 & 2 Negative
Contd.

Serum lactate
Protein C Pending
Protein S
Carotid Duplex Study

● Normal blood flow

in both carotid
artery and Normal
vessel wall
MRI of Brain
Contd.
Contd.
Findings of MRI
● Large lesion having T1 Hypointensity &T2
FLAIR hyperintensity seen in the left fronto
-parietal lobe (11/4 cm) and right frontal
lobe (8/3 cm)
● Suggestive of subacute infarction
MRA of Brain
MRA of Brain
Findings of MRA
● Intracranial ICA are severely attenuated
in caliber, left ACA, left MCA are
severely attenuated in caliber. Both PCA
appear unremarkable. There is paucity
of distal cortical vessels
MRV of Brain
Findings of MRV
● Hypoplastic left transverse sinus, left
sigmoid sinus and left internal jugular
vein. (Normal variant )
● There is no luminal irregularity or flow.
MRI, MRA & MRV Report
 Subacute infracts in left
fronto-parietal lobe & right
frontal lobe
 Suggestive of Moya Moya
disease [Suzuki grade ii/iii]
with stenosis of
intracranial ICA, left ACA &
left MCA
 Normal brain MRV
Confirmatory Diagnosis
 Moyamoya disease
with
right sided hemiparesis
MRI & MRA Report of Patient’s Son
● Acute infract in left fronto
parieto occipital region
● Significant narrowing of
both internal carotid artery
● Significant narrowing of
right and left ACAs &
MCAs
Definition of Moyamoya
● Moyamoya disease is a nonatherosclerotic progressive
steno-occlusive arteriopathy that most frequently affects
the intracranial ICAs and proximal segments of the
MCAs and ACAs. It may also involve the posterior
circulation.
● Spontaneous occlusion of the major intracranial arteries
is typically accompanied by the appearance of a tuft of
fine collateral vessels at the base of the brain.
Contd.
● Moyamoya is a Japanese word meaning puff of
smoke. The direct cause of moyamoya is unknown,
though there are likely both genetic and
environmental factors involved. The inheritance mode
of moyamoya disease is multifactorial and thought to
be autosomal dominant with incomplete penetrance.
DSA of Moyamoya Normal Brain DSA
MRA of Moyamoya Normal Brain MRA
Types of Moyamoya disease

● Primary Moyamoya disease which is truly

idiopathic
● Moyamoya syndrome
Moyamoya syndrome
1. Cranial radiotherapy
2. Atherosclerosis
3. Phakomatosis, neurofibromatosis type 1
(NF1), tuberous sclerosis (TS)
4. Infection, tuberculous meningitis, bacterial
leptomeningitis, post-varicella vasculitis
Contd.
5. Connective tissue disorders: systemic lupus
erythematosus (SLE)
6. Hematological disorders: cell disease,
antiphospholipid syndrome
Clinical feature

● Ischemic events more frequent in children

● Hemorrhagic stroke more in adult
● Epilepsy, chorea
 Suzuki grading
Grade Definition
i) Narrowing the apex of the ICA
ii) Incipient moyamoya vessels
iii) intensification of moyamoya vessels and progressive stenosis
of the ICA
iv) Development of collaterals from the ECA
v) Reduction of moyamoya vessels and Intensification of ECA
collaterals
vi) ICA occlusion and disappearance of moyamoya vessels
Treatment
1. Medical treatment with vasodilators,
corticosteroids, antiplatelet agents etc. has
been tried with doubtful efficacy.
2. Surgery is the definitive treatment where the
purpose is to create collateralization on the
brain surface.
Contd.
There are two ways
1. Indirect revascularization
2. Direct revascularization procedures such
as superficial temporal-middle cerebral
artery bypass
Thank you