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Case Conference

Case Conference. K. Myra Lalas PGY 3. CC: “walking funny”. History of Present Illness. 2 days PTA, started walking funny (like a drunk person), loses balance when standing, has been worsening When he talks, his mouth turns to the L side, once yesterday, twice today No seizures No LOC

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Case Conference

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  1. Case Conference K. Myra Lalas PGY 3

  2. CC: “walking funny”

  3. History of Present Illness • 2 days PTA, started walking funny (like a drunk person), loses balance when standing, has been worsening • When he talks, his mouth turns to the L side, once yesterday, twice today • No seizures • No LOC • No fevers

  4. HPI • No staring spells • (+) frontal headache this morning • No vomiting • Last week, (+) belly pain and NBNB emesis for 6 days • No diarrhea • No rash

  5. HPI • No incontinence • Able to move neck • No head trauma • No known sick conracts • Mom takes anti-anxiety meds (no missing meds.) • No sick contacts

  6. Went to CA 4 months ago. • Usually “hyper” but has been more calm the past 2 days

  7. Past Medical History • Dental surgery at 3 yo • Surgery for trigger thumb at 2 yo • No seizures • No asthma

  8. Birth History • FT via NSVD; no complications Immunization History • Up to date

  9. Developmental History • Walked before 12 mns of age • In SPED; has an IEP- 1st grade but failing his classes

  10. Family Medical History • Mom has anxiety d/o. • No seizures • No mental retardation • No schizophrenia • No malignancies

  11. Personal/ Social History • Lives with mom • No pets or smokers at home

  12. Physical Exam VS Temp 99.8 HR 88 RR 20 BP 100/60 100%RA Gen awake, alert, oriented x 3, speech slurred mildly HEENT PERRLA, EOMI, (+) vertical and horizontal nystagmus, clear OP, no LAD CHEST CTA b/l HEART N S1/S2, no murmurs ABD soft, (+) BS, no HSM EXT FEP, CRT < 2 secs

  13. Neuro Exam • Cranial nerves: I grossly intact II PERRLA, visual field testing normal III, IV, VI EOMI; (+) vertical and horizontal nystagmus V intact VII depressed RNLF with smile, weak eye closure and eye wrinkling on the R

  14. Neuro Exam VIII grossly intact IX, X no gag XI able to shrug shoulders XII no tongue fasciculation, tongue midline

  15. Neuro Exam Motors 5/5 Sensory no deficits Reflexes 1+, L toes upgoing Cerebellar ataxic gait, R dysmetria

  16. Differentials?

  17. Acute Ataxia • Brain Tumor • Drug Ingestion • Encephalitis (Brainstem) • Genetic Disorders • Dominant recurrent ataxia • Episodic ataxia • Hartnup Disease • MSUD • Pyruvate dehydrogenase deficiency

  18. Acute Ataxia • Migraine • Basilar • BPPV • Postinfectious- immune • Acute postinfectious cerebellitis • Miller Fisher Syndrome • MS • Neuroblastoma • Pseudoataxia (epileptic)

  19. Acute Ataxia • Trauma • Hematoma • Postconcussion • Vertebrobasilar occlusion • Vascular Disorders Cerebellar hemorrhage Kawasaki disease

  20. Where is the lesion? • Ataxia • R facial palsy • Upgoing toes on the L • R dysmetria

  21. What labs will you order? • MRI • CBC • Lyme titers • ESR/ CRP • Urine toxicology

  22. MRI • Axial mass in the R posterior brainstem, R middle cerebellar peduncle with surrounding vasogenic edema compressing the 4th ventricle with 2 separate areas of cystic changes

  23. CNS tumors in Childhood • Primary malignant CNS tumors are the second most common childhood malignancies (after hematologic malignancies.) • Most common pediatric solid organ tumor

  24. Signs and Symptoms • Headache • Nausea and vomiting • Abnormal gait or coordination • Papilledema • Seizures • Squint • Change in behavior or school performance • Macrocephaly • Cranial nerve palsies

  25. Signs and Symptoms • Lethargy • Abnormal eye movements • Hemiplegia • Weight loss • Unspecified visual or eye abnormalities • Altered level of consciousness

  26. Diagnosis • MRI *Compared to CT, it provides more detailed images of parenchymal lesions *More sensitive in detecting lesions within the posterior fossa, subarachnoid spaces, and leptomeninges • CT Scan

  27. Diagnosis • Fluorodeoxyglucose PET imaging or 11C-methionine PET may indicate foci of higher grade within a predominantly low-grade tumor.  • 11C-methionine PET may also be able to differentiate astrocytic from oligodendroglial tumors.  • Histology

  28. Brainstem Gliomas • Occur in the brain stem, which is the area between the aqueduct of Sylvius and the fourth ventricle. • Originate from glial cells or their stem cell precursors and include astrocytoma, oligodendroglioma, and ependymoma.

  29. Brainstem gliomas are divided into 3 distinct anatomic locations—diffuse intrinsic pontine,1tectal, and cervicomedullary. 

  30. Anatomic location determines the pathophysiological manifestation of the tumor. • With tectal lesions- hydrocephalus may occur as a result of fourth ventricular compression. • With pontine and cervicomedullary lesions, cranial nerve or long tract signs are observed commonly.

  31. WHO Grading • Histopathologically, brainstem gliomas can range from WHO Grade 1 to 4. Based on nuclear atypia, vascular proliferation, mitoses, necrosis • Grade 1 - juvenile pilocytic astrocytoma • Grade 2 - diffuse astrocytoma • Grade 3 - anaplastic astrocytoma • Grade 4 - glioblastoma multiforme.

  32. A low grade glioma or astrocytoma may show only a low density area (dark area) whereas high grade gliomas (Glioblastoma) usually show more contrast enhancement (white on the outside) and  necrosis in the middle (looks black on the MRI,)

  33. Treatment • Low-grade astrocytomas, which include Grade 1 (juvenile pilocytic astrocytomas) and Grade 2 (diffuse astrocytomas) astrocytomas Complete surgical resection. If additional treatment is required, radiation therapy is usually used for the older children and chemotherapy is used for the younger children.

  34. Treatment High-grade astrocytomas include Grade 3 (anaplastic astrocytomas) and Grade 4 (glioblastoma multiforme) astrocytomas Surgery, radiation therapy, and chemotherapy are usually recommended.

  35. References • Fenichel, G. Clinical Pediatric Neurology. 1997: WB Saunders Company, USA. • Rowland, LP et al. Merrit’s Neurology. 2010: Lippincott and Williams, USA. • www.uptodate.com • www.emedicine.com • www.aan.com • www.mskcc.org

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