Case of the month

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Case of the month

• By
• Dr Nirjala Aryal
• 1st year Resident
• Dept of Pediatrics
• TUTH
• November 2006

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Patient profile

• Name-Nabina Kafle
• Age -6yrs/F
• Address-Januka Nagar Sarlahi
• Date of admission- 2063/6/27
• Date of Discharge- 2063/7/3

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• 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.

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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.

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• 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

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• 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

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Examination

• GC Fair,
• Weight-13kg(68 of expected)
• OFC- 49.5cm
• Vital signs
• Pulse 100/min
• BP 100/60mmHg
• RR 25/min
• JALCCOD-Nil

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CNS Examination..

• Higher Mental Function
• Conscious, oriented to time, person and place,
  Memory normal
• Speech slurred
• Cranial Nerves
• Right sided Lateral Rectus palsy
• Right sided Facial Palsy- LMN type
• Other cranial nerves intact
• Motor system
• Bulk- Normal

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Motor system Examination..
Upper limb
Lower limb
Right
Right
Left
Left
Tone
Normal
Normal
5/5
4/5
Power
5/5
3/5
Normal
Normal
Coordination
Normal
Normal
Abnormal Movement
No
No
No
No
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Deep Reflexes Examination..
Upper limb
Lower limb
Right
Right
Left
Left
Biceps
Triceps
Supinator
Knee
Ankle
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CNS Examination..

• Superficial Reflexes
• Plantar up going on left side
• Abdominal - Absent on left side
• Sensations- Intact
• Celebellar signs- Absent
• Meningeal signs- Absent
• Skull and Spine- Normal

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Systemic Examination

• R/S -NAD
• CVS-NAD
• Abdominal - NAD

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Diagnosis Left hemiparesis UMN type withRT.
6th and 7th LMN palsy (Cross hemiparesis)
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DIFFERENTIAL DIAGNOSIS

• Brain stem tumors
• Brain stem stroke Infarction
• Hemorrhage
• Cyst anterior to brain stem
• CP angle tumors
• Brain stem encephalitis

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Investigation

• TLC- 10,000/Cu mm
• DLC- N72,L25, M2,E1
• Hb - 15.6 gm
• ESR- 40mm/1st hour
• Platelet 250000/cu mm
• ECHO Normal

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• MRI Head- Pontine Glioma

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Treatment

• Neuro surgical consultation done advised
  radiotherapy
• Patient discharged on request

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Brain stem tumors

• Brain stem-area between the aqueduct of sylvius
  and the fourth ventricle
• -the midbrain, pons and medulla

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Introduction of brain stem glioma

• 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

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Brain stem glioma

• Incidence -account for 10 percent of
  pediatric brain tumors.
• - peak between ages 5 and 10.

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Brain stem glioma

• Clinical features
• four major features-
• cranial nerve palsies
• Pyramidal tract signs
• Cerebellar signs
• Progression to advance stages usually without
  in the intracranial pressures

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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

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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

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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

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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

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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 patients first hospital
  admission

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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

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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

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Brain stem glioma

• Imaging Studies
• MRI
• the diagnostic test of choice.
• differentiate vascular malformations and other
  processes that can be misdiagnosed as a brainstem
  glioma on CT scan.
• 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
• delineate the extent of infiltration of the
  leptomeninges and the surrounding structures
• contrast enhancement in a tectal lesion should
  raise suspicion of a metastatic lesion

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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

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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

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Treatment

• Radiotherapy
• Response to radiotherapy depends on
• 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

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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.

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References

• Nelson Text Book of Pediatric
• Essential pediatric- O P Ghai
• Text Book of Child Neurology- John H Menkes
• Internet Articles

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Thank you