Brainstem Gliomas Clinical Presentation

  • Author: Joseph Landolfi, DO; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP   more...
 
Updated: Jul 22, 2011
 

History

  • Common presenting symptoms include double vision, weakness, unsteady gait, difficulty in swallowing, dysarthria, headache, drowsiness, nausea, and vomiting. Rarely, behavioral changes or seizures may be seen in children. Older children may have deterioration of handwriting and speech.
  • Pontine lesions usually present with any or all of the above signs and symptoms, depending on location and extension. Midbrain and lower brainstem/upper spinal cord signs and symptoms may be seen with extension of the neoplasm to involve these structures.
  • In infants and children presenting with failure to thrive, pontine glioma should be considered in the differential diagnosis.
  • Tectal lesions typically present with headache, nausea, and vomiting.
  • Hydrocephalus is a common presentation, especially for tumors in periaqueductal or fourth ventricle outflow locations, because these regions have less tolerance of growth and higher risk of obstructive hydrocephalus.
  • Cervicomedullary lesions usually present with dysphagia, unsteadiness, nasal speech, vomiting, and weakness.
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Physical

  • Common clinical findings can be summarized as constituting a triad of cranial nerve deficits, long tract signs, and ataxia (of trunk and limbs). Papilledema may be seen.
  • Sixth and seventh cranial nerves are involved commonly. Facial sensory loss and a primary position, upbeating nystagmus may be seen. Involvement of cranial nerve III or IV suggests a mesencephalic component.
  • Tectal lesions may present with diplopia reflecting an internuclear ophthalmoplegia, indicating involvement of the medial longitudinal fasciculus. Parinaud syndrome also may be seen, with paralysis of upward gaze and accommodation, light-near dissociation (loss of pupillary reflex to light with preservation of pupilloconstriction in response to convergence), eyelid retraction, and convergence-retraction nystagmus.
  • Cervicomedullary lesions may present with sensory loss of the face (involvement of the trigeminal nucleus), dysphagia and/or dysphonia from lower cranial nerve involvement (commonly IX and X), long tract signs, and ataxia. Downbeating nystagmus and oculomyoclonus often are seen with medullary involvement.
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Causes

  • Although no familial tendency is prominent overall, an increased incidence of brainstem glioma has been observed consistently in patients with neurofibromatosis (up to 14% in some reports).
  • Thus far, no genetic or molecular markers have been recognized for brainstem gliomas.
  • In children irradiated for tinea capitis, an increased incidence of CNS tumors, especially meningiomas, gliomas, and nerve sheath tumors, has been reported. No specific reference is made in these reports to tumors of the brain stem. Radiotherapy-induced neoplasms tend to be more aggressive in their natural history than their de novo counterparts.
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Contributor Information and Disclosures
Author

Joseph Landolfi, DO  Director of Neuro-oncology, New Jersey Neuroscience Institute Brain Tumor Center; Medical Director, Gamma Knife Program, JFK Medical Center; Associate Professor of Neurology, Seton Hall University School of Graduate Medical Education

Joseph Landolfi, DO is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Schering-Plough Honoraria Speaking and teaching; Genetech Honoraria Speaking and teaching

Coauthor(s)

Anita Venkataramana, MBBS  Clinical Instructor, Department of Neurology, Division of Neuroimmunology/HIV, Johns Hopkins University

Disclosure: Nothing to disclose.

Specialty Editor Board

Edward L Hogan, MD  Professor, Department of Neurology, Medical College of Georgia; Emeritus Professor and Chair, Department of Neurology, Medical University of South Carolina

Edward L Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Neurological Association, American Society for Biochemistry and Molecular Biology, Phi Beta Kappa, Sigma Xi, Society for Neuroscience, and Southern Clinical Neurological Society

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

Jorge 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

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

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T2-weighted image of a diffuse intrinsic pontine glioma.
T2-weighted image of a right tectal glioma.
 
 
 
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