Optic nerve glioma (pilocytic astrocytoma) - neurofibromatosis type 1
Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease is an autosomal dominant disorder, caused by a mutation in the NF1 gene.
It is a multisystem disease, affecting the nervous, musculoskeletal, cardiopulmonary, integumentary, and other systems.
Focal areas of signal intensity (FASI), also known as unidentified bright objects (UBO) is the most common intracranial finding in patients with neurofibromatosis type 1 1.
Optic nerve gliomas may affect the optic nerves, the optic chiasm and optic tracts, so it is called optic pathway gliomas (OPG). It is usually unilateral and commonly shows fusiform enlargement of the optic pathway.
On MRI: the optic nerve glioma is:
iso to hypointense onT1
iso to hyperintense on T2
the enhancement is variable on T1C+ (from absent to intense)
The diagnosis was confirmed by pathology (pilocytic astrocytoma, WHO grade I).
- Basal ganglia
- Basal ganglia and thalamus signal abnormalities
- Basal ganglia T1 hyperintensity
- Basal ganglia T2 hyperintensity
- Café au lait spots
- Focal areas of signal intensity (brain)
- Hydrocephalus (summary)
- Neurofibromatosis
- Neurofibromatosis type 1
- Neurofibromatosis type 1 (CNS manifestations)
- Neurofibromatosis type 1 (cutaneous manifestations)
- Neurofibromatosis type 1 (mnemonic)
- Neurofibromatosis type 1 (orbital manifestations)
- Optic canal
- Optic canal enlargement
- Optic chiasm
- Optic nerve
- Optic nerve enlargement
- Optic nerve sheath diameter
- Optic pathway glioma
- Optic tract
- Pilocytic astrocytoma
- Pituitary region masses