Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome

Abstract

Tolosa-Hunt syndrome is characterized by unilateral retro-orbital headaches and cranial nerve palsies, usually involving cranial nerves III-VI. It is rare for other cranial nerves to be involved, although this has previously been reported. We report a 19-year-old woman presenting with typical features of Tolosa-Hunt syndrome but ultimately developing bilateral facial nerve palsies and enhancement of both facial nerves on magnetic resonance imaging. The patient presented with unilateral retro-orbital headaches and palsies of cranial nerves III-VI. She was diagnosed with Tolosa-Hunt syndrome but was non-compliant with her corticosteroid treatment due to side effects. She returned with progressive left followed by right facial nerve palsy. Her corresponding follow-up magnetic resonance imaging scans showed sequential enhancement of the left and right facial nerves. She ultimately had clinical improvement with IV methylprednisolone. To our knowledge, Tolosa-Hunt syndrome associated with bilateral facial nerve palsy and corroborative facial nerve enhancement on magnetic resonance imaging has not previously been described. Moreover, our patient's clinical course is instructive, as it demonstrates that this atypical presentation of Tolosa-Hunt syndrome can indeed respond to corticosteroid treatment and should not be mistaken for other entities such as Bell's palsy.

Keywords: Bell’s palsy; Facial nerve palsy; Tolosa–Hunt syndrome; cavernous sinus inflammation; facial nerve enhancement; pseudotumor.

Figure 1.

Coronal T1-weighted post-contrast images (a)–(d) posterior to anterior, obtained at the time of presentation demonstrate enhancement and enlargement of the left Meckel’s cave (a, arrow), the cavernous sinus (b, solid arrow), V3 (b, dashed arrow), the superior orbital fissure (c, arrow), and V2 (d, arrow). Axial T2-weighted image demonstrates T2 hypointense soft tissue thickening in the left Meckel’s cave and cavernous sinus (e, white arrows) with a normal appearance of Meckel’s cave on the right (e, black arrow). Coronal F18 fluorodeoxyglucose positron emission tomography/computed tomography image through the orbits demonstrates asymmetric radiotracer activity in the right medial and inferior rectus muscles with no uptake on the left (f, arrows).

Figure 2.

Axial (a) and sagittal oblique (b) T1-weighted post-contrast images 2 months after initial presentation show new enhancement of the left facial nerve extending into the mastoid segment (a–b, arrows). Coronal T1 post-contrast image from the same exam shows persistent enhancement of the left cavernous sinus and V3 (c, arrows). Axial post-contrast image several months later shows new enhancement of the right facial nerve (d, arrows).