Case of the monthCase of the month

ByDr Nirjala Aryal

1st year ResidentDept of Pediatrics

TUTHNovember 2006

Patient profilePatient profile

• 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….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

ExaminationExamination

• 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…..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

Motor system Examination…..Motor system Examination…..

Upper limb

RightRight Left

Lower limb

Left

Tone

Power

Coordination

Abnormal Movement

Normal Normal

5/5 3/5 5/5 4/5

Normal Normal Normal Normal

No No No No

Deep Reflexes Examination…..Deep Reflexes Examination…..

Upper limb

RightRight Left

Lower limb

Left

Biceps

Triceps

Supinator

Knee

Ankle

CNS Examination…..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

Systemic ExaminationSystemic Examination

• R/S -NAD

• CVS-NAD

• Abdominal - NAD

Diagnosis: Left hemiparesis – UMN type Diagnosis: Left hemiparesis – UMN type withwith

RT. 6RT. 6thth and 7 and 7thth LMN palsy LMN palsy (Cross hemiparesis) (Cross hemiparesis)

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS• Brain stem tumors

• Brain stem stroke: Infarction

• Hemorrhage

• Cyst anterior to brain stem

• CP angle tumors

• Brain stem encephalitis

InvestigationInvestigation

• 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

TreatmentTreatment

• Neuro surgical consultation done: advised radiotherapy

• Patient discharged on request

Brain stem tumorsBrain stem tumors

• Brain stem-area between the aqueduct of sylvius and the fourth ventricle

-the midbrain, pons and medulla

Introduction of brain stem gliomaIntroduction 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

Brain stem glioma…………Brain stem glioma…………

• Incidence -account for 10 percent of pediatric brain tumors.

- peak between ages 5 and 10.

Brain stem glioma…………Brain stem glioma…………

• Clinical features • four major features-

1. cranial nerve palsies

2. Pyramidal tract signs

3. Cerebellar signs

4. Progression to advance stages usually without in the intracranial pressures

Clinical featuresClinical features• symptoms

Vomiting unaccompanied by headacheGait disturbances cerebellum or its

peduncles involvement ,result hemiparesis

Personality changesGradual or rapid onset of hemiweakness

of the bodyEvidence of cranial nerve involvement - facial weakness ,strabismus,

swallowing difficulty

Clinical features……..Clinical features……..• Signs

Spastic hemiparesisIncrease deep tendon reflexesExtensor plantar response6th (horizontal conjugate gaze palsy) and

7th (LMN) cranial nerve palsyDysfunction of 9th and 10th cranial nerveHemisensory deficit –rareChange in personality, sleeping pattern,

drowiness and coma-reticular substance infiltration

Clinical features……..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……..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……..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…………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…………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…………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…………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

TreatmentTreatment

• 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…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

Treatment…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.

ReferencesReferences

• Nelson Text Book of Pediatric

• Essential pediatric- O P Ghai

• Text Book of Child Neurology- John H Menkes

• Internet Articles

Thank youThank you