3. History
• A 21 yrs old female from gondar town presented
with abnormal body movement of her left side of the
body since 10 years ago.
• It involved her upper and lower extremities with
tonic extension movements preceeded by visual
hallucinations and associated left side mouth
deviation and drooling of saliva.
• each episode was lasting ~2-4 min. with no
alteration of consciousness, but had post ictal
weakness of involved extremities which was lasting
about 30 min..
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4. History…
• Each episodes were coming every 2- 3 month initially and
later every 2-3 weeks after which she was seen at this
hospital and started on phenytoin 100mg po bid since 7 yrs
back.
• 6 months later she started to have difficulty of walking and
maintaining posture at which time she was put on valproic
acid 200mg po bid and phenytoin tapered and discontinued
for dx. Of cerebellar ataxia 2 ? Phenytoin side effect and
she get improved.
• Currently she is on valproic acid 250mg poTID and she has
episodes coming every 2-3 month ;most of the episodes
were coming during night times and complains that
duration of single episode prolonged to about 10 min. since
6 months back.
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5. History…
• she was born in this hospital and she is the first
child of her mother who is a mother of 3; after
9 month of amenorhea and uneventfull
pregnancy, having had regular ANC follow up at
HC in. her vicinity in debark.
• Delivery was via /vaccum assisted vaginaal
delivery for poor maternal effort And the
mother stated that she has cried immediately
after birth and started breast feed within an
hour.
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6. History…
• At 7 days of age she had failure to have
breastfeed and was admitted to NICU for
about 3 weeks receiving IV antibiotics. The
mother also claimed to notice yellowish
discoloration of skin and she has been put on
phototherapy for few days.
• She also had history of left side eye lateral
deviation since early childhood and it was
corrected surgically at the age of 10.
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7. History…
• She joined school at age 7 and has
discontinued 2× at grade 5 due to her illness.
She had medium school performance and
currently she is grade 9 student for the 2nd
time.
• She complains difficulty participating in sport
activities at school due to easy fatiguability
but had no history of body weakness.
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8. History…
• And፡
– She has no history of headache.
– She has no history of altered mentation.
– no history of urinary or bowel incontinence.
– She has no history of fever or neck pain/stiffness.
– She had no history of hearing disturbance
– No history of trauma or surgical procedure to the head.
– No history of forgetfullness
– No hx similar illness in the family.
– No other drug history.
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9. Physical Examination
GA
• well looking
V/s
• BP = 100/60 RR=22bpm
• To = 36.5 oC PR = 78bpm(R)
HEENT
• Pink conjunctiva, Non icteric sclera
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11. Physical Examination…
Cardiovascular system
• JVP flat, s1 and s2 well heard, no murmur or gallop.
Abdomen
• Soft, no mass or organomegally, no sign of fluid
collection
Genitourinary system
• No CVAT
Musculoskeletal, integumentary system
• No edema, no rash
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12. Physical Examination…
Nervous system
• Mental status conscious፡
• oriented to person , place and time=9/10
• Registration=3
• Attention and calculation=5
• Recall=3
• Language=8/9
MMSE = 28/30
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13. Physical Examination…
Cranial nerves
• extraocular movements are intact;
• temporal and masseter strength intact,
• facial movements are intact;
• Can shrug her shoulders ;
• tongue is in midline
• Others including visual acuity and visual fields not
done .
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14. Physical Examination…
Motor RUE RLE LUE LLE
Tone N N N N
Power 5/5 5/5 5/5 5/5
• DTR
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ANKLE Knee Biceps Triceps Plantar
Lt +++ +++ +++ +++ Upgoing
Rt ++ ++ ++ ++ Downgo
ing
15. Physical Examination…
Sensory: intact for pinprick, touch and position
senses
Discriminatory sensation
• two point discrimination
0.5cm in rt finger
1.5cm in lt finger
• no astereognosis
• Graphestesia intact bilaterally
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16. Physical Examination…
Coordination
• Romborg test : negative
• can perform finger to nose and hill to shin
bilaterally
• no pronator drift
• No gait abnormality including tandem walking,
walk in the heels and walk in the toes
Meningeal signs = negative
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23. Rasmussen's encephalitis
Rasmussen’s encephalitis is a progressive
disease characterised by
drug-resistant focal epilepsy,
progressive hemiplegia, and
cognitive decline, with unihemispheric brain
atrophy.
• The disorder is rare and affects mostly children
or young adults.
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24. • Incidence 1.7 – 2.5 in 10M; M=F
• The mean age of presentation is between 6 to
8 years. Both sexes are equally affected
• cytotoxic T cell reaction against the neuron
leads to expression of major
histocompatibility complex (MHC) class I and
apoptotic neuronal death, resulting in
progressive deterioration of neurological
status.
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25. • Three disease stages of Rasmussen’s encephalitis
• Prodromal stage: Non-specifi c, low seizure
frequency, and mild hemiplegia
• Acute stage: Frequent seizures, often epilepsia
partialis continua; progressive hemiparesis,
hemianopia, cognitive deterioration, and aphasia
(if dominant hemisphere aff ected)
• Residual stage: Permanent and stable neurological
defi cits and continuing seizures
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