Neurologic Manifestations of Ependymoma Clinical Presentation

  • Author: Subrata Ghosh, MD, MBBS; Chief Editor: Stephen A Berman, MD, PhD, MBA   more...
 
Updated: Aug 29, 2011
 

History

Presenting features are insidious and progressive in nature.

  • Nausea and vomiting (80%) is the most common presenting symptom, secondary to increased intracranial pressure.
  • Headache (60%), due to the local effect of pressure or increased intracranial pressure, is usually worse in the morning.
  • Change in behavior (50%) includes lethargy, irritability, diminished social interaction, and loss of appetite (prevalent in younger children).
  • Difficulty with balance (30%) reflects cerebellar involvement or mass effect.
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Physical

  • Papilledema (60%)
  • Ataxia (45%)
  • Nystagmus with or without gaze palsy (40%)
  • Lower cranial nerve palsies (10%)
  • Apraxia or hemiparesis (20%)
  • Increase in head circumference in children younger than 2 years (10%)
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Causes

No particular genetic or molecular marker or familial predisposition has been identified for this tumor type. In one series, only a few ependymomas were reported to be hyperdiploid or tetraploid.

  • Other diagnostic considerations:
    • Meningitis
    • Encephalitis
    • Other brain tumors (astrocytoma, medulloblastoma, oligodendroglioma)
    • Meningitis and encephalitis can be readily differentiated by their more abrupt onset, associated fever, or signs of meningeal irritation.
    • Differentiation from other types of brain tumors (astrocytoma, medulloblastoma, oligodendroglioma) is radiological and pathological.
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Contributor Information and Disclosures
Author

Subrata Ghosh, MD, MBBS  Staff Physician, Division of Neurosurgery, St. Luke's Episcopal Hospital, Texas Medical Center, Houston; Assistant Professor of Neurosurgery, Baylor College of Medicine

Subrata Ghosh, MD, MBBS is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, Congress of Neurological Surgeons, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Draga Jichici, MD, FRCP, FAHA  Associate Clinical Professor, Department of Neurology and Critical Care Medicine, McMaster University School of Medicine, Canada

Draga Jichici, MD, FRCP, FAHA is a member of the following medical societies: American Academy of Neurology, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Critical Care Society, Canadian Medical Protective Association, Canadian Neurocritical Care Society, Neurocritical Care Society, Royal College of Physicians and Surgeons of Canada, and Society of Critical Care Medicine (USA)

Disclosure: Nothing to disclose.

Specialty Editor Board

Rodrigo O Kuljis, MD  Esther Lichtenstein Professor of Psychiatry and Neurology, Director, Division of Cognitive and Behavioral Neurology, Department of Neurology, University of Miami School of Medicine

Rodrigo O Kuljis, MD is a member of the following medical societies: American Academy of Neurology and Society for Neuroscience

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge C Kattah, MD  Head, Associate Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Chief Editor

Stephen A Berman, MD, PhD, MBA  Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

References

  1. Nazar GB, Hoffman HJ, Becker LE, et al. Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg. Mar 1990;72(3):408-17. [Medline].

  2. Moynihan TJ. Ependymal tumors. Curr Treat Options Oncol. Dec 2003;4(6):517-23. [Medline].

  3. [Best Evidence] Massimino M, Buttarelli FR, Antonelli M, Gandola L, Modena P, Giangaspero F. Intracranial ependymoma: factors affecting outcome. Future Oncology. March 2009;5(2):207-16. [Medline]. [Full Text].

  4. Bhattacharjee MB, Armstrong DD, Vogel H, Cooley LD. Cytogenetic analysis of 120 primary pediatric brain tumors and literature review. Cancer Genet Cytogenet. Aug 1997;97(1):39-53. [Medline].

  5. Bigner SH, McLendon RE, Fuchs H, et al. Chromosomal characteristics of childhood brain tumors. Cancer Genet Cytogenet. Sep 1997;97(2):125-34. [Medline].

  6. Ernestus RI, Schroder R, Stutzer H, Klug N. The clinical and prognostic relevance of grading in intracranial ependymomas. Br J Neurosurg. Oct 1997;11(5):421-8. [Medline].

  7. Graham DI, Lantos PL, eds. Greenfield's Neuropathology. 6th ed. Arnold Press; 1997:636-44.

  8. Kaye AH, Laws E Jr, eds. Brain Tumors: An Encyclopedic Approach. First ed. Churchill Livingstone; 1997:493-504.

  9. Kleihues P et al. Pathology & Genetics. Tumors of the Nervous System. International Agency for Research on Cancer (IARC)/World Health Organization. 1997;96-109.

  10. Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumours. Brain Pathol. Jul 1993;3(3):255-68. [Medline].

  11. Kun LE. Brain tumors. Challenges and directions. Pediatr Clin North Am. Aug 1997;44(4):907-17. [Medline].

  12. McLaughlin MP, Marcus RB, Buatti JM, et al. Ependymoma: results, prognostic factors and treatment recommendations. Int J Radiat Oncol Biol Phys. Mar 1 1998;40(4):845-50. [Medline].

  13. Osborn AG. Diagnostic Neuroradiology: A Text and Atlas. First ed. Mosby; 1994:566-70.

  14. Russell DS, et al. Pathology of Tumors of the Nervous System. 4th ed. Arnold Press; 1977:203-26.

 
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CT scan without contrast, axial view, demonstrates mixed but predominantly hyperdense tumor in the posterior fossa with severe obstructive hydrocephalus.
CT scan with contrast, axial view shows moderately intense contrast enhancement (compare with the previous image).
MRI, T2-weighted image, axial view, showing mixed (isodensity and hyperdensity) heterogenous nature of the tumor with some peritumoral edema.
MRI, T1-weighted image, without contrast, sagittal view, showing the posterior fossa location, mixed (hypodensity and isodensity) signal intensity and tending to grow out of the fourth ventricle.
 
 
 
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