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NIH Criteria for Diagnosis of Neurofibromatosis Type 2

Purpose: To evaluate a patient for neurofibromatosis using the NIH criteria.

Description

Neurofibromatosis can be inherited as an autosomal dominant genetic condition. Two familial disorders occur, which can be diagnosed by specific clinical criteria. Molecular diagnostic tests are available or being developed for confirmation of the affected individual and family.


The NIH reported criteria in 1987 with a revision in 1991.

 

A definite diagnosis of neurofibromatosis can be made wth one of the following:

(1) bilateral vestibular schwannomas

(2) a first-degree relative with neurofibromatosis 2 AND unilateral vestibular schwannoma

(3) a first-degree relative with neurofibromatosis 2 AND any one of: meningioma, glioma, schwannoma, juvenile posterior subcapsular lenticular opacity


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Specialty

Objective

ICD-10