Presentation
Gradually decreased vision of the right eye.
Patient Data
Fusiform enlargement of the right optic nerve mainly the intracranial segment with tortuous course with hydrops of the anterior part of nerve sheath. It shows isointense signal intensity on T2/FLAIR sequences and no post-contrast enhancement. It is sparing the optic chiasm and has no extension to the left optic nerve. Features are suggestive of right optic nerve glioma.
There are abnormal hyperintensities on FLAIR, and T2WI with no enhancement of the dentate nuclei, posterior aspect of the pons and midbrain, thalami, globi pallidi, and the splenium of the corpus callosum. These mostly represent focal areas of signal intensity (FASI).
Case Discussion
The association of optic pathway gliomas and areas of T2/FLAIR hyperintensity with no contrast enhancement within the white matter and basal ganglia are most consistent with neurofibromatosis type 1.
Focal areas of signal intensity (FASI) tend to occur in the basal ganglia (often globus pallidus and thalamus), brainstem, cerebellum, and hippocampus.
Optic nerve gliomas can have variable signal intensity and variable post-contrast enhancement.
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