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Brainstem Gliomas Clinical Presentation

  • Author: Joseph C Landolfi, DO; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
 
Updated: Jun 09, 2016
 

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 C Landolfi, DO Director of Neurology, New Jersey Neuroscience Institute; Director of Neuro-oncology, Medical Director, Gamma Knife Program, JFK Brain Tumor Center; Chairman, Institutional Review Board, JFK Health System; Medical Director of Neuro-oncology, Meridian Health System; Professor of Neurology, Seton Hall University School of Graduate Medical Education

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

Disclosure: Received honoraria from Novacure for speaking and teaching; Received honoraria from Genetech for speaking and teaching.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

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, Society for Neuroscience, American Society for Biochemistry and Molecular Biology, American Academy of Neurology, American Neurological Association, Phi Beta Kappa, Sigma Xi, Southern Clinical Neurological Society

Disclosure: Nothing to disclose.

Acknowledgements

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

Disclosure: Nothing to disclose.

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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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