Case of the month

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

Patient 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….

• • • • • 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, 4 th Others normal.

child of the family. 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

• • • • • 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 Cranial Nerves • Right sided Lateral Rectus palsy • Right sided Facial Palsy LMN type • Other cranial nerves: intact Motor system • Bulk- Normal

Motor system Examination…..

Right Tone Power Normal 5/5 Coordination Normal Abnormal Movement No Upper limb Left 3/5 Normal No Right Lower limb Left Normal 5/5 Normal 4/5 Normal No No

Deep Reflexes Examination…..

Right Upper limb Left Right Lower limb Left Biceps Triceps Supinator Knee Ankle

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 Examination

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

Diagnosis: Left hemiparesis – UMN type RT. 6 th with and 7 th LMN palsy (Cross hemiparesis)

DIFFERENTIAL DIAGNOSIS

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

Investigation

• • • • • • TLC- 10,000/Cu mm DLC- N72,L25, M2,E1 Hb - 15.6 gm% ESR- 40mm/1 st 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

• • • • 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…………

• 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 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  6 th 7 th (horizontal conjugate gaze palsy) and (LMN) cranial nerve palsy  Dysfunction of 9 th and 10 th  Hemisensory deficit –rare cranial nerve  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 -7 th 64/78 -9 th -6 th and 10 th 54/78 48/78 -5 th (sonsory) 38/78 -5th(motor) 13/48 -12 th 13/78 -8 th 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 • • • • 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