eMedicine Specialties > Neurology > Pediatric Neurology

Neurofibromatosis, Type 2: Follow-up

Author: Beth A Pletcher, MD, Associate Professor, Co-Director of The Neurofibromatosis Center of New Jersey, Department of Pediatrics, University of Medicine and Dentistry of New Jersey
Contributor Information and Disclosures

Updated: Dec 10, 2008

Follow-up

Further Inpatient Care

Hospitalization is necessary for neurosurgical procedures as well as ABIs.

Further Outpatient Care

Although care of the patient with neurofibromatosis type 2 theoretically can be done in the primary care setting, the complexity, rarity, and multisystem involvement encountered in this condition suggest that medical care in a disease-specific multidisciplinary clinic may permit optimal management. The following is an outline of reasonable guidelines in the care of the patient with NF2:

  • Annual neurologic examination looking for subtle deficits or changes in neurologic status that might suggest disease progression
  • Annual hearing screening with BAER with referral to an audiologist for amplification, augmentation, or speech therapy recommendations
  • Annual MRI to monitor existing lesions or look for presymptomatic lesions
  • Annual ophthalmologic evaluations to monitor visual acuity

Complications

  • Unilateral or, frequently, bilateral vestibular schwannomas leading to tinnitus, hearing loss, and/or problems with balance
  • Meningiomas, gliomas, ependymomas, and other cerebral, cerebellar, or spinal cord lesions that may result in neurological deficits, seizures, and/or hydrocephalus
  • Peripheral nerve schwannomas, mixed tumors, and occasionally neurofibromas
  • Peripheral neuropathies
  • Visually significant juvenile cataracts

Prognosis

The prognosis of NF2 depends upon a number of factors including age of onset of symptoms, degree of hearing deficit, and number and location of various tumors. Although age of onset is relatively similar within families, the age range can vary from 2-70 years. While the tumors themselves are relatively indolent and do not undergo malignant transformation, studies performed in the late 1980s and early 1990s showed clearly that significant rates of mortality as well as morbidity are associated with the diagnosis of NF2.

One such study suggested that the survival from the time of actual diagnosis averages 15 years; however, this may be changing for the better with improved diagnosis, surgical techniques, surveillance, screening, and recognition of mild disease due in part to increased physician awareness and availability of molecular diagnostic options.

Patient Education

  • Patients and at-risk family members should be made aware of specific symptoms such as tinnitus, hearing deficits, focal weakness, sensory changes, or balance problems that might suggest tumor growth and should prompt immediate medical attention.
  • Patients with vestibular schwannomas should be cautioned about diving and underwater activities because of increased risks for disorientation and potential for drowning.
  • Patients and their families may be referred to NF-specific regional and national support groups for continuous updates on advances in treatment as well as for emotional support. Neurofibromatosis, Inc. has a toll-free number (1-800-942-6825) and can be reached by e-mail at nfinc1@aol.com. The Children's Tumor Foundation has a toll-free number (1-800-323-7938) for information and to sign up for their newsletter. The NF2 Review, located in Los Angeles, CA, can be reached at 1-213-483-4431 and not only has an NF2-specific newsletter but also has a particular research interest in NF2 with a team of specialists working on auditory brainstem implants and the newest surgical approaches to vestibular schwannomas. Online resources include the NIH Web site and an NF2 person-to-person support group known as the NF2 crew.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose vestibular schwannomas early enough to permit intervention that may prolong hearing
  • Failure to identify spinal cord lesions early on to permit intervention prior to permanent neurologic damage
  • Failure to provide appropriate genetic counseling
  • Failure to provide complete informed consent prior to undertaking molecular testing
 


More on Neurofibromatosis, Type 2

Overview: Neurofibromatosis, Type 2
Differential Diagnoses & Workup: Neurofibromatosis, Type 2
Treatment & Medication: Neurofibromatosis, Type 2
Follow-up: Neurofibromatosis, Type 2
Multimedia: Neurofibromatosis, Type 2
References
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References

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Further Reading

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Keywords

bilateral acoustic neurofibromatosis, bilateral acoustic neuroma, central neurofibromatosis, NF2, spinal cord tumor, spinal cord schwannoma, vestibular schwannoma, meningioma, multiple meningiomas, glioma, juvenile cataracts, neurofibromatosis type 2

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Contributor Information and Disclosures

Author

Beth A Pletcher, MD, Associate Professor, Co-Director of The Neurofibromatosis Center of New Jersey, Department of Pediatrics, University of Medicine and Dentistry of New Jersey
Beth A Pletcher, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics, American Medical Association, and American Society of Human Genetics
Disclosure: Nothing to disclose.

Medical Editor

David A Griesemer, MD, Professor, Departments of Neuroscience and Pediatrics, Medical University of South Carolina
David A Griesemer, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Epilepsy Society, Child Neurology Society, and Society for Neuroscience
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Division of Pediatrics, Department of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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