Case of the month
By
Dr Nirjala Aryal
1st year Resident
Dept of Pediatrics
TUTH
November 2006
Patient profile
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Name-Nabina Kafle
Age -6yrs/F
Address-Januka Nagar Sarlahi
Date of admission- 2063/6/27
Date of Discharge- 2063/7/3
• Complaints:
• Sudden onset of Inability to move left half
of the body – 1 month.
• Also had complaint of double vision and
deviation of the angle of mouth to the left
side for the same duration.
• H/o Slurring of speech for one month.
Case description….
• Both upper and lower limbs were noted to be
weak simultaneously.
• The weakness was not progressive but
gradually improving over the days.
• There was no h/O loss of sensation.
• Along with the weakness, there was also
complaint of double vision and unable to see
the objects on right side.
• Also had deviation of angle of mouth to left
side with drooling of saliva from right corner
of mouth. There was also history of
accumulation of food on the right side of
mouth.
• There was also history of slurring of speech. She
can speak but the speech is not clear as before.
• There was no h/O difficulty in swallowing or
nasal regurgitation.
• There was no history of:
• Fever, headache, ear discharge, vomiting,
loss of consciousness, convulsion, bleeding
from any sites, head injury, dyspnea,
palpitation, bowel and bladder
incontinence.
• No h/O rashes and joint pain
• No h/O syncopal attack
• No h/O loose motion at the initiation of
weakness
• Past History- Not significant.
• Treatment history
• Physiotherapy- improvement in power of
the limb
• No history of contact with TB
• Family history- 5 siblings, 4th child of the family.
Others normal.
• Birth History- FT/SVD/Home, No Perinatal
Complication
• Developmental History- Normal according to the
age
• Immunization- completed as per EPI schedule
• Dietary History- taking adequate calories
Examination
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GC – Fair,
Weight-13kg(68% of expected)
OFC- 49.5cm
Vital signs
– Pulse 100/min
– BP 100/60mmHg
– RR 25/min
• JALCCOD-Nil
CNS Examination…..
• Higher Mental Function
• Conscious, oriented to
time, person and place,
Memory normal
• Speech slurred
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Cranial Nerves
• Right sided Lateral Rectus
palsy
• Right sided Facial PalsyLMN type
• Other cranial nerves:
intact
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Motor system
• Bulk- Normal
Motor system Examination…..
Upper limb
Right
Tone
Power
Coordination
Abnormal Movement
Left
Normal
Lower limb
Right
Left
Normal
5/5
3/5
Normal
Normal
No
No
5/5
Normal
No
4/5
Normal
No
Deep Reflexes Examination…..
Upper limb
Right
Biceps
Triceps
Supinator
Knee
Ankle
Left
Lower limb
Right
Left
CNS Examination…..
• Superficial Reflexes
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Plantar – up going on left side
Abdominal - Absent on left side
Sensations- Intact
Celebellar signs- Absent
Meningeal signs- Absent
Skull and Spine- Normal
Systemic Examination
• R/S -NAD
• CVS-NAD
• Abdominal - NAD
Diagnosis: Left hemiparesis – UMN type
with
RT. 6th and 7th LMN palsy
(Cross hemiparesis)
DIFFERENTIAL DIAGNOSIS
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Brain stem tumors
Brain stem stroke: Infarction
Hemorrhage
Cyst anterior to brain stem
CP angle tumors
Brain stem encephalitis
Investigation
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TLC- 10,000/Cu mm
DLC- N72,L25, M2,E1
Hb - 15.6 gm%
ESR- 40mm/1st hour
Platelet – 250000/cu mm
ECHO – Normal
• MRI Head- Pontine Glioma
Treatment
• Neuro surgical consultation done: advised
radiotherapy
• Patient discharged on request
Brain stem tumors
• Brain stem-area
between the aqueduct
of sylvius and the
fourth ventricle
-the midbrain, pons
and medulla
Introduction of brain stem glioma
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Classification - diffuse intrinsic pontine
- tectal
- cervicomedullary
Most common -pontine glioma
-grave prognosis
All have fatal termination
Malignant for practical purposes
-location
-transient responsiveness to
irradiation
Brain stem glioma…………
• Incidence -account for 10 percent of
pediatric brain tumors.
- peak between ages 5 and 10.
Brain stem glioma…………
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Clinical features
•
four major features1. cranial nerve palsies
2. Pyramidal tract signs
3. Cerebellar signs
4. Progression to advance stages usually
without in the intracranial pressures
Clinical features
• symptoms
Vomiting unaccompanied by headache
Gait disturbances cerebellum or its
peduncles involvement ,result
hemiparesis
Personality changes
Gradual or rapid onset of
hemiweakness of the body
Evidence of cranial nerve involvement
- facial weakness ,strabismus,
swallowing difficulty
Clinical features……..
• Signs
Spastic hemiparesis
Increase deep tendon reflexes
Extensor plantar response
6th (horizontal conjugate gaze palsy) and
7th (LMN) cranial nerve palsy
Dysfunction of 9th and 10th cranial nerve
Hemisensory deficit –rare
Change in personality, sleeping pattern,
drowiness and coma-reticular substance
infiltration
Clinical features……..
Incidence of Neurological symptoms in 48 children (From
Bray et al)
Symptoms
number
Gait disturbance
47
Squint
25
Vomiting
22
Headache
21
Dysarthria
19
Facial weakness
15
Personality change
11
Dysphagia
10
Drowsiness
10
Head tilt
05
Hearing loss
04
Clinical features……..
Incidence of Neurological signs (from Bray
et al and Ingraham Matson)
Signs
Number
Pyramidal tract signs
41/48
Cranial nerve involvement
-7th
64/78
-9th and 10th
54/78
-6th
48/78
-5th(sonsory)
38/78
-5th(motor)
13/48
-12th
13/78
-8th
12/78
Cerebellar signs
Nystagmus-horizontal
26/48
-vertical
24/78
Gaze paralysis-horizontal
22/48
-vertical
5/48
Hemisensory deficit
5/48
Clinical features……..
Fate of the disease
Swallowing and speaking difficulty
complete paralysis of the extremities
impairment of consciousness with
deepening coma respiratory or cardiac
irregularities DEATH
• Average survival without treatment is 15
months from the date of the patient’s first
hospital admission
Brain stem glioma…………
• Causes
Increased incidence in patient with
neurofibromatosis (up to 14% in some
reports).
children irradiated for tinea capitis –
increased incidence of CNS tumors,
especially meningiomas, gliomas, and
nerve sheath tumors
no genetic or molecular markers have
been recognized
Brain stem glioma…………
• Work up
• Lab Studies
– Blood chemistry not useful for diagnosis
– cerebrospinal fluid (CSF) examination
protein may be elevated
• Tissue confirmation only in case of exophytic
growth
Brain stem glioma…………
• Imaging Studies
• MRI
o the diagnostic test of choice.
o differentiate vascular malformations and other processes
that can be misdiagnosed as a brainstem glioma on CT
scan.
o an expansile, infiltrative process with low-to-normal
signal intensity on T1-weighted images and
heterogeneous high-signal intensity on T2-weighted
images, with or without contrast enhancement
o delineate the extent of infiltration of the leptomeninges
and the surrounding structures
o contrast enhancement in a tectal lesion should raise
suspicion of a metastatic lesion
Brain stem glioma…………
• CT Scan
– appropriate choice when MRI is not available
– sensitivity of and characterization of tumors by CT are
poorer
– calcifications, cystic changes, and displacement of the
ventricular system
– lower brainstem lesions often not apparent
• Arteriography
– in differentiating vascular lesions, including tumors, from
gliomas
Treatment
• Medical Care
• Treatment frustrating –new therapy little benefit over
conventional treatment with radiotherapy alone.
• Adjuvant chemotherapy is not used in children
• effectiveness of chemotherapy at relapse is uncertain
• Focal radiotherapy
– cornerstone of treatment
– can improve or stabilize the patient's condition
– 54-60 Gy, with doses up to 72 Gy given with
hyperfractionation
– not demonstrated efficacy in children
Treatment…
• Radiotherapy…
– Response to radiotherapy depends on
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tumor location,
histological type, and
response to early treatment
exophytic tumors better survival rates than without an
exophytic component
– transient clinical remission in 60% of the children
– first improvement seen after 3-6 weeks of treatment
– improvement noted by partial clearing of cranial
nerve signs
Treatment…
• Surgical Care
• impossible due to location of tumor
• Palliative Care
• hydrocephalus
• ventriculostomy or ventriculoperitoneal shunting
• difficulties in swallowing and diminished gag reflex
• gastrostomy such as the percutaneous
esophagogastrostomy (PEG).
• multiple upper respiratory infections, pneumonia,
or altered voice
• ventilatory assistance.
References
• Nelson Text Book of Pediatric
• Essential pediatric- O P Ghai
• Text Book of Child Neurology- John H
Menkes
• Internet Articles
Thank you