Neurofibromatosis type 2

Case contributed by Prashant Mudgal


Patient presented with headache off and on, vertigo and giddiness since 6 months.

Patient Data

Age: 20 years
Gender: Female

Two lobulated extra-axial heterogeneously enhancing lesions are noted in the bilateral cerebello-pontine angle cisterns. Both lesions are extending into bilateral internal acoustic meatus and resulting in widening of IAM. Medially the lesions are abutting and compressing the inferior cerebellar peduncles. The findings are suggestive of bilateral vestibulocochlear schwannomas.

Multiple extra-axial homogeneously enhancing lesions are seen involving left high frontal parafalcine region with varying degree of vasogenic edema surrounding it, and along the inferior aspect of tentorium cerebelli bilaterally. The lesions in the left parafalcine and left subtentorium show thick calcific foci within. The findings are suggestive of meningiomas.

Imaging features are suggestive of neurofibromatosis type 2.

Case Discussion

Neurofibromatosis type 2 is rare autosomal dominant neurocutaneous disorder characterized by:

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