1 / 38

Case of the month

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:

lester
Download Presentation

Case of the month

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Case of the month By Dr Nirjala Aryal 1st year Resident Dept of Pediatrics TUTH November 2006

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

  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.

  4. 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.

  5. 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

  6. 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

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

  8. 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

  9. Motor system Examination….. Upper limb Lower limb Right Left Right Left Tone Normal Normal 5/5 4/5 Power 5/5 3/5 Normal Normal Coordination Normal Normal Abnormal Movement No No No No

  10. Deep Reflexes Examination….. Upper limb Lower limb Right Left Right Left Biceps Triceps Supinator Knee Ankle

  11. 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

  12. Systemic Examination • R/S -NAD • CVS-NAD • Abdominal - NAD

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

  14. Brain stem tumors Brain stem stroke: Infarction Hemorrhage Cyst anterior to brain stem CP angle tumors Brain stem encephalitis DIFFERENTIAL DIAGNOSIS

  15. 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

  16. MRI Head- Pontine Glioma

  17. Treatment • Neuro surgical consultation done: advised radiotherapy • Patient discharged on request

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

  19. 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

  20. Brain stem glioma………… • Incidence -account for 10 percent of pediatric brain tumors. - peak between ages 5 and 10.

  21. 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

  22. 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

  23. 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

  24. 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

  25. 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

  26. 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

  27. 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

  28. 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

  29. 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

  30. 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

  31. 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

  32. 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

  33. 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.

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

  35. Thank you

More Related