Supranuclear lesions: Isolated lesions are rare and are typically associated with other neurologic findings. They are not associated with tongue fasciculations or atrophy. Spastic dysarthria and pseudobulbar palsy especially with bilateral cortical lesions are possible. When asked to protrude the tongue, the tongue will deviate away from the side of the lesion [3].

Jugular foramen and extracranial lesions: The most common lesion to involve the hypoglossal nerve is the hypoglossal schwannoma which accounts for only 5% of non-vestibular intracranial schwannomas. This lesion is a benign tumor of differentiated Schwann cells which may arise anywhere along the course of the hypoglossal nerve. A sharply marginated, fusiform mass which may exhibit a dumbbell shape is seen on CT or MRI [4, 5]. The most common MRI characteristics of this lesion include T1 isointensity to gray matter, T2 hyperintensity, and uniform enhancement. Intramural cysts may be identified if the lesion is large. Multiple lesions including skull base metastasis, glomus jugular paraganglioma, vascular variant persistent hypoglossal artery, or jugular foramen meningioma can mimic a hypoglossal schwannoma. Skull base traumatic fractures can also present with hypoglossal nerve dysfunction.