Cranial Nerves II–VI

Debnam, J. Matthew

doi:10.1007/978-3-031-17479-7_10

J. Matthew Debnam²

638 Accesses

Abstract

The five cranial nerves (CNs) of the orbit relay important motor and sensory information about orbital muscle movement, vision, and facial sensation. These cranial nerves include the optic nerve (CN II), oculomotor nerve (CN III), trochlear nerve (CN IV), trigeminal nerve (CN V), and abducens nerve (CN VI). Lesions may arise within the nerve, such as an optic nerve glioma or from the nerve sheath, e.g., meningiomas and schwannomas. Other primary and secondary tumors can spread along the nerves, including perineural tumor spread, leptomeningeal disease, and neurolymphomatosis. Infection and other conditions such as idiopathic orbital inflammation and IgG4-related disease can also involve these CNs. Thus, imaging plays an important role in defining the presence and extent of disease and refining the differential diagnosis.

Access provided by Autonomous University of Puebla. Download chapter PDF

Cranial Nerves

Peripheral Nerve Tumors

Keywords

The five cranial nerves (CNs) of the orbit relay important motor and sensory information about orbital muscle movement, vision, and facial sensation. These cranial nerves include the optic nerve (CN II), oculomotor nerve (CN III), trochlear nerve (CN IV), trigeminal nerve (CN V), and abducens nerve (CN VI). Lesions may arise within the nerve, such as an optic nerve glioma or from the nerve sheath, e.g., meningiomas and schwannomas. Other primary and secondary tumors can spread along the nerves, including perineural tumor spread, leptomeningeal disease, and neurolymphomatosis. Infection and other conditions such as idiopathic orbital inflammation and IgG4-related disease can also involve these CNs. Thus, imaging plays an important role in defining the presence and extent of disease and refining the differential diagnosis.

The imaging modalities used to evaluate the CNs include CT, MRI, and PET/CT. CT is used to assess the bony neural foramen for widening and destruction. MRI visualizes enhancing lesions of the CNs due to a superior contrast resolution. Both MRI and PET/CT provide important information for treatment planning and response.

The purpose of this chapter is to describe the demographics and imaging appearance of common and uncommon malignancies and tumor mimics that involve the CNs. This is accomplished with a review of the disease background, clinical presentation, and imaging features on various modalities. This chapter should provide the radiologist with a means to narrow their differential diagnosis when evaluating CN lesions. Lesions affecting the orbital CNs that occur in the skull base, sinonasal cavity, pituitary gland, cavernous sinus, and brain including the optic radiations are discussed in other chapters.

Orbital Cranial Nerve Anatomy

CN II (Optic Nerve)

Figures 10.1a and b, 10.2a, and 10.4 demonstrate the course of the optic nerve.

Carries visual information from the globe to the visual cortex.
Axons are part of the white-matter tract; myelinated by oligodendrocytes.
Surrounded by subarachnoid space and covered by meninges.
- Prone to gliomas and meningiomas.
Divided into four segments: intraocular, intraorbital, intracanalicular, and prechiasmatic.
Optic nerve emerges from the posterior globe.
Exits the orbit via the optic canal.
Bilateral optic nerves join to form the optic chiasm.
Nasal (medial) fibers of the optic nerves decussate (cross) in the optic chiasm; temporal (lateral) fibers do not cross.
Fibers from the optic chiasm travel in a posterolateral direction to the lateral geniculate body.
Optic radiations originate in the lateral geniculate nucleus and travel posteriorly to reach the primary visual cortex in the occipital lobe (see Chap. 9 for further details).

Six M R I images of the cranial nerves in the skull area labeled (a) to (f). The prechiasmatic optic nerves, the optic chiasm, oculomotor nerves, right trochlear nerve, trigeminal nerve roots, bilateral Meckel's cave, and abducens nerves are indicated by arrows. — **Fig. 10.1**

A series of six C T scan images of the orbital and base skull foramina labeled a to f. The arrows indicate optic canals in image (a), superior orbital fissures in (b), Foremen rotundum in (c), Pterygopalatine fossae in (d), inferior orbital fissures in (e), and Foreman ovale in (f). — **Fig. 10.2**

CN III (Oculomotor Nerve)

Figures 10.1c, 10.2b, 10.3, and 10.4 demonstrate the course of the oculomotor nerve.

Provides motor function to the superior rectus, inferior rectus, and medial rectus muscles, the inferior oblique muscles, and the levator palpebrae superioris muscles.
The oculomotor nucleus is located in the midbrain anterior to the periaqueductal grey matter.
Parasympathetic fibers that innervate the pupillary constrictor and ciliary muscles originate in the Edinger-Westphal nucleus, which is located posterior to the oculomotor nucleus.
- Pupillary light reflex and lens accommodation response.
Emerges from the medial aspect of the cerebral peduncle into the interpeduncular cistern and traverses the perimesencephalic cistern.
Courses anteriorly below the posterior cerebral artery and above the superior cerebellar artery.
Enters the cavernous sinus in the lateral wall as the most superior nerve.
Enters the orbit via the superior orbital fissure.
Superior division gives branches to the superior rectus and levator palpebrae superioris muscles (superior rectus-levator complex).
Inferior division innervates the inferior and medial rectus and inferior oblique muscles.

A diagram of the brainstem and the cranial nerves network. Six parts are labeled in the sensory nuclei, on the left, and 12 parts are labeled under motor nuclei, on the right. — **Fig. 10.3**

A drawing of the optic, oculomotor, trochlear, and abducens nerves. A total of 15 labeled parts, including trochlea, superior optic muscle, superior rectus muscle, optic nerve, and optic chiasm, are indicated. — **Fig. 10.4**

CN IV (Trochlear Nerve)

Figures 10.1c, 10.2b, 10.3, and 10.4 demonstrate the course of the trochlear nerve.

Innervates the superior oblique muscle.
Nucleus lies in the midbrain, anterior to the periaqueductal grey matter.
Fibers course posteriorly in the midbrain, decussate posterior to the periaqueductal grey matter, and then exit the pons below the inferior colliculus.
Runs anteriorly around the cerebral peduncles in the ambient cistern.
Courses along the free margin of the tentorium and pierces the dura between the free and attached edges of the tentorium.
Passes into the lateral wall of the cavernous sinus.
- Located inferior to the oculomotor nerve (CN III) and above the ophthalmic division of the trigeminal nerve (CN V₁).
Enters the orbit via the superior orbital fissure.
In the orbit, it extends superiorly and medially above the superior rectus-levator complex to innervate the superior oblique muscle.

CN V (Trigeminal Nerve)

Figures 10.1d and e, 10.2b–f, 10.3, and 10.5 demonstrate the course of the trigeminal nerve.

Largest of the cranial nerves; contains sensory and motor roots.
Supplies sensations to the face and mucous membranes, and motor function for the muscles of mastication.
Four trigeminal nuclei: mesencephalic nucleus, chief sensory nucleus, nucleus of the spinal tract, and motor nucleus.
- Nuclei extend from the midbrain inferiorly to the upper medulla.
The trigeminal nerve root exits the brainstem from the lateral pons. It passes through the prepontine cistern into a dural recess at the petrous apex (Meckel’s cave) where the trigeminal ganglion (Gasserian ganglion) is located.
Nerve splits into three divisions: ophthalmic (V₁), maxillary (V₂), and mandibular (V₃) nerves.
V₁ and V₂ course in the lateral wall of the cavernous sinus.
V₁ enters the orbit through the superior orbital fissure.
- Three terminal branches, each passing separately into the orbit via the superior orbital fissure.
- The lacrimal nerve innervates the lacrimal gland and a portion of the upper eyelid.
- The frontal nerve exits through the supraorbital foramen to provide sensory innervation to the upper face and scalp.
- The nasociliary nerve provides sensory innervation to the eyelids, conjunctiva, cornea, ethmoid air cells, and nasal cavity mucosa.
V₂ exits the skull base via the foramen rotundum and passes through the pterygopalatine fossa.
V₂ enters the orbit through the inferior orbital fissure, passing within the infraorbital canal, and extends to the face via the infraorbital foramen. Provides sensory innervation of the mid-face.
V₃ exits the skull base through the foramen ovale into the infratemporal fossa and branches into the lingual (sensation of the anterior 2/3 of the tongue) and inferior alveolar nerves (sensation of the lower teeth and chin; motor fibers to the muscles of mastication).

A schematic drawing of the trigeminal nerve network. 18 labeled parts including supraorbital nerves, superior orbital fissure, trigeminal ganglion, and ciliary ganglion are marked. — **Fig. 10.5**

CN VI (Abducens Nerve)

Figures 10.1f, 10.2b, 10.3, and 10.4 demonstrate the course of the abducens nerve.

Innervates the lateral rectus muscle.
Abducens nucleus is located in the dorsal pons just inferior to the floor of the fourth ventricle.
The abducens nerve emerges at the pontomedullary junction and courses through the prepontine cistern.
Most medial of the nerves exiting the brainstem at the pontomedullary junction (facial nerve [CN VII] and vestibulocochlear nerve [CN VIII] are more lateral).
Courses anteriorly through the prepontine cistern toward the clivus and runs superiorly along the clivus in a fibrous sheath named Dorello’s canal.
Courses over the medial petrous apex toward the cavernous sinus.
Lies in the central venous portion of the cavernous sinus inferolateral to the internal carotid artery.
Enters the orbit via the superior orbital fissure to innervate the lateral rectus muscle.

Optic Nerve Sheath Meningioma

Figures 10.6, 10.7, and 10.8 show cases of optic nerve sheath meningiomas.

Six M R I images of a patient with a history of N F 2 and papilledema due to an optic nerve sheath meningioma are labeled (a) to (f). The arrows indicate the intracranial meningioma on the optic nerve in image (b). — **Fig. 10.7**

Four M R I images of a patient with left vision loss labeled (a) to (d). The arrows indicate the enhancement of the mass on the optic nerve in image (b), suggestive of meningioma. — **Fig. 10.8**

Background

Benign tumor of the optic nerve sheath arising from arachnoid cap cells.
Located inside the dura [1].
Associated with neurofibromatosis type II (NF-2) [2].

Presentation

Mean age at presentation: 40 years [2].
Up to 25% present in children; tend to be more aggressive [3].
Female predilection [2].
Usually unilateral [2, 4].
Bilateral involvement can occur with tumor extension to the optic chiasm and contralateral nerve [2].
Symptoms: vision loss, proptosis [3].

Imaging

Tubular (65%), exophytic (25%), or fusiform (10%) [2].
Tram-track sign: appearance of the enhancing meningioma as it surrounds the non-enhancing optic nerve in the axial and sagittal oblique planes [5].
In the coronal plane, enhancement of the meningioma surrounds the optic nerve [6].
Optic nerve meningiomas in the optic canal can cause widening or hyperostosis [2].

CT

Isointense to the optic nerve with homogeneous enhancement.
Calcifications form a “sleeve-like case” around the optic nerve [2].
Useful to detect calcification and evaluate the optic nerve canal [3].

MRI

T1 iso- to hypointense to the optic nerve with homogeneous enhancement.
T2 iso- to hyperintense to the optic nerve [7, 8].

PET/CT

⁶⁸Ga-DOTATATE PET/CT has been reported to confirm the presence of optic sheath meningioma [9, 10].
⁶⁸Ga-DOTATATE is a radiolabeled somatostatin receptor ligand.
Meningiomas have overexpression of somatostatin receptors.
Neuroendocrine and pituitary tumors can also be ⁶⁸Ga-DOTATATE avid [9].

Key Points

Review the orbital apex and optic canal.
Tram-track sign can be seen in other lesions such as perioptic lymphoma, leukemia, metastases, idiopathic orbital inflammation, sarcoidosis, Erdheim-Chester disease, perioptic hemorrhage, and optic neuritis.

Optic Nerve Glioma

Figures 10.9, 10.10, and 10.11 show cases of optic nerve gliomas.

Four M R I images of a patient with N F 1 and right eye vision loss are labeled (a) to (d). The bilateral optic nerve gliomas are indicated by the arrows in images (a) and (b). — **Fig. 10.10**

Three M R I images and a 68 G a-D O T A T A T E P E T, C T scan image of a patient with vision changes over the past 2 years are labeled (a) to (d). The expanded left optic nerve lesion is indicated by an arrow in image (a). — **Fig. 10.11**

Background

The majority are pilocytic astrocytomas (WHO grade I) [11].
Usually associated with NF-1 [11, 12].

Presentation

Most common in children (75%); no gender bias [11].
More aggressive in adults; in which case, there may be no association with NF-1 [12].
Can be bilateral and multifocal if associated with NF-1.
Optic nerve is the most common site in patients with NF-1.
Optic chiasm is the most common site in patients without NF-1 [13].
Symptoms: decreased vision, proptosis [14, 15].

Imaging

Fusiform or exophytic optic nerve enlargement.
Optic nerve may be elongated with kinking or buckling [16].
Absence of calcifications.
Most are lobulated solid tumors.
- Cystic components have been described, particularly in patients without NF-1 [15].

CT

Isointense to optic nerve with homogeneous enhancement.

MRI

T1 iso- to hypointense to contralateral optic nerve with variable enhancement.
T2 iso- to hyperintense [13, 15].

Key Points

Review the orbital apex, optic nerve to the optic chiasm, hypothalamus, and the optic tracts.

Optic Neuritis

Figures 10.12 and 10.13 show cases of optic neuritis.

Four M R I images of a patient with acute lymphoid leukemia and right eye blindness labeled (a) to (d). The arrows indicate the location of the hyperintense signal emanated by the intraorbital and canalicular segments of the right optic nerve in images (a) and (b). — **Fig. 10.12**

Four M R I images in axial, sagittal, and coronal view of a male patient with metastatic melanoma, are labeled (a), (b), (c), and (d). Arrows point to the enhancement of the left optic nerve, and sheath in images (a) and (c). — **Fig. 10.13**

Background

Inflammatory process involving the optic nerve [13].
Etiologies: autoimmune diseases, e.g., multiple sclerosis, neuromyelitis optica; systemic diseases, e.g., system lupus erythematosus, sarcoidosis, Wegener disease, Sjogren’s syndrome, and Behcet disease [17].

Presentation

Symptoms: vision loss, painful eye movements [8].

Imaging

Optic nerve appears swollen [8].
Fat stranding around the nerve from inflammation .

MRI

T2 hyperintense.
Enhances with contrast [8].

Key Points

Search for fat stranding.

Nerve Sheath Tumors

Figures 10.14, 10.15, and 10.16 show cases of nerve sheath tumors.

Four M R I images of a patient with left orbital schwannoma are labeled (a) to (d). Arrows point to the location of the enhanced schwannoma in the cavernous sinus component with the elevation of the left side of the optic chiasm in image (d). — **Fig. 10.14**

Two M R I images of a patient with left proptosis and left orbital mass are labeled (a) and (b). The arrows indicate the mass in the left orbit in image (a) and the enhancement of the schwannoma indicated in images (b), (c), and (d). — **Fig. 10.15**

Four M R I images of a patient with N F 2 and right plexiform neurofibroma are labeled (a) to (d). Arrows indicate the position of the neurofibroma in the right cavernous sinus area in image (d). — **Fig. 10.16**

Background

Benign nerve sheath tumors are comprised of spindle cells arranged in compact (Antoni type A) or loose (Antoni type B) tissues [18].
The most involved nerves about the orbit are the trigeminal (CN V) and facial (CN VII) nerves.
Soft tissue sarcomas that originate from the peripheral nerve sheath are termed malignant peripheral nerve sheath tumors (MPNSTs) [19].
Intracranial schwannomas may be associated with NF-2.
- Genetic condition with multiple schwannomas including bilateral vestibular schwannoma, meningiomas, and ependymomas [20].
Neurofibromas are typically associated with NF-1.
- Genetic condition of neurofibromas with iris hamartomas, optic nerve gliomas, osseous lesions, café au lait spots, and axillary or inguinal freckling.
- Can show malignant transformation.
- Rarely involve the cranial nerves [21].

Presentation

Denervation atrophy of innervated muscle or a sensory deficit can aid in identifying the nerve of origin or an adjacent cranial nerve from long-standing mass effect [20].

Imaging

Slow growth characterized by smooth expansion of an affected neural foramen, bone remodeling, and/or mass effect on adjacent soft tissues [20, 22].
Clinical symptoms suggesting transformation to an MPNST: nonspecific and include new pain, increased growth, and new neurologic deficits [23].

CT

Hypo- to isodense [20].
Cranial nerve schwannomas demonstrate variable enhancement [22, 24].

MRI

Appearance depends on the components of Antoni type A and B tissues [18].
T1 hypo- to isointense with avid enhancement.
T2 heterogeneously hyperintense due to compactly arranged cells (Antoni type A pattern - hypointense) intermixed with areas of loosely arranged cells (Antoni type B pattern - hyperintense) with variable water content and cellularity [18, 25]. Therefore, nerve sheath tumors can be hypo- to isointense [26].
Larger lesions may demonstrate heterogeneous enhancement, internal cysts, and hypointense foci of hemosiderin related to internal hemorrhage [20].
MRI findings of MPNSTs: interval growth, larger size, heterogeneous signal and enhancement, internal necrosis without enhancement, irregular margins, and local invasion [26].
Restricted diffusion associated with MPNSTs has been described [26]; however, further work on this topic is needed [19].

PET

In addition to intense ¹⁸F-FDG avidity, both schwannomas and MPNSTs may be large and demonstrate a heterogeneous appearance [26].

Key Points

Review the cranial nerve to detect all lesions.
Search for signs to suggest transformation to a MPNST.

Perineural Tumor Spread

Figures 10.17, 10.18, and 10.19 show cases of perineural tumor spread.

Three M R I images of a patient with left forehead squamous cell carcinoma are labeled (a) to (c). Arrows point to the location of the enhancing mass in the left upper eyelid in image (a). — **Fig. 10.17**

Four M R I images of a patient with squamous cell carcinoma of the skin are labeled (a) to (d). An arrow points to the ophthalmic division of the right trigeminal nerve in image (a), a thin arrow indicates the foramen rotundum in image (b), and another thin arrow points to the left Meckel’s cave in image (c). — **Fig. 10.18**

Six M R I images of a patient with hard palate adenoid cystic carcinoma and facial numbness are labeled (a) to (f). An arrow indicates denervation atrophy of the left muscles of mastication in image (f). — **Fig. 10.19**

Background

Microscopic spread of tumor along the nerve sheaths [27].
May extend a substantial distance from the primary tumor [28].
Disruption of the blood-nerve barrier results in increased endoneural capillary permeability allowing for the leakage and accumulation of contrast material leading to enhancement of the nerve [29].
Most common tumors: adenoid cystic and squamous cell carcinoma. It can also occur with basal cell carcinoma, lymphoma, melanoma, melanoma, rhabdomyosarcoma, and juvenile angiofibroma [21].
Associated with decreased overall survival [30].

Presentation

Symptoms: pain, dysesthesias (abnormal sensations such as painful burning, itching, stinging), or denervation atrophy of innervated muscle [21].
Most affected nerves: maxillary (V₂) and mandibular (V₃) divisions of the trigeminal nerve and the facial nerve [21].

Imaging

Signs of perineural tumor involvement: nerve enlargement with enhancement; replacement of neuroforaminal fat, enlargement or destruction of the foramen, denervation atrophy of innervated muscle [28].
An affected nerve can maintain its normal size while passing through the skull base foramina.
Neuritis from chemoradiation may occur months to years after treatment and may be difficult to distinguish from perineural tumor spread [30, 31].

MRI

Invasion of the skull base may present as the replacement of normal fatty marrow signal [21].
Denervated muscles have characteristic MRI patterns [32].
- Acute (<1 month): T2 hyperintensity, enhancement, and increased volume.
- Subacute (up to 12–20 months): T1 hyperintensity due to fat deposition without volume loss.
- Chronic (>12–20 months): Fatty atrophy with volume loss.

Key Points

MRI with fat suppression can aid in visualization.
Comment on the number of lesions and other sites of involvement.
Evaluate the entire nerve to assess for skip lesions.
Differentiate between extension from the primary tumor versus a separate metastasis.
In selected cases, abnormal soft tissue adjacent to cranial nerves can be sampled with image-guided fine-needle aspiration [33].
Nerve enhancement may persist indefinitely even if there is clinical evidence of improvement. Radiographic and clinical progression are important indicators of recurrent tumor [34].

Leptomeningeal Disease

Figures 10.20, 10.21, 10.22, and 10.23 show cases of leptomeningeal disease.

Two M R I images of a patient with breast cancer labeled (a) and (b). The arrows indicate the enhancement of oculomotor nerves from leptomeningeal tumor spread in image (a). — **Fig. 10.20**

Four M R I images of a patient with mantle cell lymphoma are labeled (a) to (d). The arrows indicate the fat saturation around the optic and infraorbital nerves in image (b). — **Fig. 10.21**

Three M R I images of a male patient with acute myeloid leukemia who presented with facial pain due to neurolymphomatosis are labeled (a) to (c). The fat saturation with enhancing L M D in the cerebellar folia is indicated by arrows in image (c). — **Fig. 10.22**

Three M R I images of a male patient with right temporal glioblastoma are labeled (a) to (c). The arrows indicate fat saturation with enhancement of the right oculomotor nerves in image (b). — **Fig. 10.23**

Background

Leptomeningeal disease (LMD) is a complication of late-stage systemic cancers.
- Infiltration of the leptomeninges by tumor cells [35].
Both primary and secondary tumors can spread through the subarachnoid spaces and along cranial nerves.
- Primary tumors: medulloblastoma, oligodendroglioma ependymoma, and glioblastoma.
- Secondary tumors: breast and lung cancer and melanoma.
CN VII and VIII are the most affected cranial nerves [35].
Neurolymphomatosis: term used when hematologic malignancies such as lymphoma spread along cranial and peripheral nerves [36].
- Most often encountered with diffuse large B cell lymphoma.
- Can occur months to years after the original diagnosis.

Presentation

Symptoms: cranial neuropathies, mental status changes [35, 36].
Ocular disturbance (cranial nerve deficits, optic disc or retinal infiltration, papilledema) reported in 67% of patients with LMD [37].

Imaging

Nerves are enlarged and enhanced [35].
Neurolymphomatosis of the orbital cranial nerves most commonly occurs via direct spread from a solid mass in the orbit or maxillofacial region [36].

Key Points

Lack of detection can lead to delayed diagnosis [38].
MRI with fat suppression can aid in visualization.
T1 non-contrast-enhanced MRI can sometimes better detect disease in the pterygopalatine fossae, characterized by fat replacement.
Comment on the number of lesions and other sites of involvement.
Evaluate the entire nerve to assess for skip lesions.

Idiopathic Orbital Inflammation (IOI)

Figures 10.24 and 10.25 show cases of idiopathic orbital inflammation.

Four M R I images of a patient with decreased vision in the right eye due to I O I are labeled (a) to (d). The fat saturation with a hyperintense appearance of the right optic nerve is indicated by the arrows in image (b). — **Fig. 10.24**

Four M R I images of a patient with right conjunctival redness and periorbital pain due to biopsy proven I O I are labeled (a) to (d). The arrows indicate the fat saturation and infiltrative tissue in the intraconal space of the right orbit and enlargement of the medial and lateral rectus muscles in image (a). — **Fig. 10.25**

Background

Idiopathic orbital inflammation (IOI) was previously referred to as orbital pseudotumor.
IOI is an inflammatory condition characterized by polymorphous infiltration and variable degrees of fibrosis [39, 40].
After thyroid orbitopathy and lymphoproliferative disorders, IOI is the third most common disease to affect the orbit [41].

Presentation

Symptoms: proptosis, headache, periorbital pain, and inflammatory signs, e.g., swelling and erythema.
Compression upon the orbital apex and cavernous sinus involvement may lead to decreased visual acuity and cranial nerve palsies [42].

Imaging

Retro-orbital involvement may occur from spread through the superior and inferior orbital fissures and the optic canal, extending to the cavernous sinus.
Other sites of involvement: optic nerve, including the junction with the globe, lacrimal gland, and adjacent periorbital soft tissues.
When the extraocular muscles are involved, IOI can include the tendinous portion of the muscles [29].

CT

Enhancement with contrast has been reported [42].

MRI

T1 isointense and T2 hypointense due to fibrosis.
Variable contrast enhancement [42].

Key Points

Assess for cranial nerve and cavernous sinus involvement.
Describe involvement and signs of compression at the orbital apex.

IgG4-Related Disease

Figures 10.26 and 10.27 show cases of IgG4-related disease.

Five M R I images and an axial 18 F-F D G P E T, C T scan images of a patient with right orbital swelling due to l g 4 immunoglobulin-related disease are labeled (a) to (f). Fat saturation with enhancing soft tissue in the right pterygopalatine fossa with involvement of the right infraorbital nerve, indicated by arrows in image (c). — **Fig. 10.26**

Four M R I images and 2 axial 18 F-F D G P E T, C T scan images of a patient with slowly progressive right proptosis are labeled (a) to (f). The arrows indicate the enhancing tissue in the right intraconal space with infiltration of the right lateral rectus muscle in image (a). — **Fig. 10.27**

Background

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease of unknown etiology.
Characterized by tissue infiltration with plasma cells that express IgG4, inflammation, and fibrosis.
Various organs may be involved, including the pancreas, bile duct, liver, retroperitoneal soft tissues, lung, thyroid, salivary glands, and lymph nodes, either alone or systematically [43, 44].
The head and neck is the second most affected site after the pancreas [45].

Presentation

Occurs primarily in older men.
Often with elevated serum IgG4 levels [46].
Symptoms: hypophysitis, thyroiditis, pancreatitis, cholecystitis, retroperitoneal fibrosis, and lymphadenopathy [45,46,47,48].

Imaging

Cavernous sinus disease often accompanies orbital and dural involvement.
The nerves appear thickened with enhancement when the cavernous sinus is involved [49].

CT

Soft tissue density of the disease.

MRI

T1 hypointensity, T2 hypo- to hyperintensity with homogeneous enhancement [47, 48, 50].
IgG4-related hypophysitis has also been described with thickened enhancement of the pituitary infundibulum [51].

PET

IgG4-RD is ¹⁸F-FDG-avid, and PET is useful to detect multiorgan involvement, guide biopsies, and assess treatment response [46, 52].

Key Points

Assess for involvement of the salivary glands, e.g., parotid and submandibular glands.
Assess for involvement of cranial nerves (e.g., infraorbital nerve), lacrimal gland, extraocular muscles, cavernous sinus, and Meckel’s cave.
Describe involvement and signs of compression at the orbital apex.

References

Newman SA, Jane JA. Meningiomas of the optic nerve, orbit, and anterior visual pathways. In: Al-Mefty O, editor. Meningiomas. New York, NY: Raven Press; 1991. p. 461–94.
Google Scholar
Ortiz O, Schochet SS, Kotzan JM, Kostick D. Radiologic-pathologic correlation: meningioma of the optic nerve sheath. AJNR Am J Neuroradiol. 1996;17:901–6.
CAS PubMed PubMed Central Google Scholar
Parker RT, Ovens CA, Fraser CL, Samarawickrama C. Optic nerve sheath meningiomas: prevalence, impact, and management strategies. Eye Brain. 2018;10:85–99. https://doi.org/10.2147/EB.S144345.
Article PubMed PubMed Central Google Scholar
Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37:167–83. https://doi.org/10.1016/0039-6257(92)90135-g.
Article CAS PubMed Google Scholar
Kanamalla US. The optic nerve tram-track sign. Radiology. 2003;227:718–9. https://doi.org/10.1148/radiol.2273010758.
Article PubMed Google Scholar
Badr MA, Elkhamary SM, Al Sabbagh S, Al TA. Bilateral Optic Nerve Sheath Meningioma with Intracanalicular and Intracranial Component in a 25-year-old Saudi Patient. Middle East Afr J Ophthalmol. 2008;15:138–41. https://doi.org/10.4103/0974-9233.51990.
Article PubMed Google Scholar
Mafee MF, Goodwin J, Dorodi S. Optic nerve sheath meningiomas. Role of MR imaging. Radiol Clin N Am. 1999;37:37–58, ix. https://doi.org/10.1016/s0033-8389(05)70077-4.
Article CAS PubMed Google Scholar
Zimmerman CF, Schatz NJ, Glaser JS. Magnetic resonance imaging of optic nerve meningiomas. Enhancement with gadolinium-DTPA. Ophthalmology. 1990;97:585–91. https://doi.org/10.1016/s0161-6420(90)32538-1.
Article CAS PubMed Google Scholar
Klingenstein A, Haug AR, Miller C, Hintschich C. Ga-68-DOTA-TATE PET/CT for discrimination of tumors of the optic pathway. Orbit. 2015;34:16–22. https://doi.org/10.3109/01676830.2014.959185.
Article PubMed Google Scholar
Vay SU, Werner JM, Kabbasch C, Schmidt M, Drzezga A, Fink GR, et al. Uncovering an optic nerve sheath meningioma using 68Ga-DOTATATE PET/CT. Clin Nucl Med. 2021;46(9):e464–5. https://doi.org/10.1097/RLU.0000000000003619.
Article PubMed Google Scholar
Taylor T, Jaspan T, Milano G, Gregson R, Parker T, Ritzmann T, et al. Radiological classification of optic pathway gliomas: experience of a modified functional classification system. Br J Radiol. 2008;81:761–6. https://doi.org/10.1259/bjr/65246351.
Article CAS PubMed Google Scholar
Millar WS, Tartaglino LM, Sergott RC, Friedman DP, Flanders AE. MR of malignant optic glioma of adulthood. AJNR Am J Neuroradiol. 1995;16:1673–6.
CAS PubMed PubMed Central Google Scholar
Gala F. Magnetic resonance imaging of optic nerve. Indian J Radiol Imaging. 2015;25:421–38. https://doi.org/10.4103/0971-3026.169462.
Article PubMed PubMed Central Google Scholar
Listernick R, Darling C, Greenwald M, Strauss L, Charrow J. Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr. 1995;127:718–22. https://doi.org/10.1016/s0022-3476(95)70159-1.
Article CAS PubMed Google Scholar
Jahraus CD, Tarbell NJ. Optic pathway gliomas. Pediatr Blood Cancer. 2006;46:586–96. https://doi.org/10.1002/pbc.20655.
Article PubMed Google Scholar
Jakobiec FA, Depot MJ, Kennerdell JS, Shults WT, Anderson RL, Alper ME, et al. Combined clinical and computed tomographic diagnosis of orbital glioma and meningioma. Ophthalmology. 1984;91:137–55. https://doi.org/10.1016/s0161-6420(84)34316-0.
Article CAS PubMed Google Scholar
Razek AA, Castillo M. Imaging lesions of the cavernous sinus. AJNR Am J Neuroradiol. 2009;30:444–52. https://doi.org/10.3174/ajnr.A1398.
Article PubMed PubMed Central Google Scholar
Wippold FJ 2nd, Lubner M, Perrin RJ, Lämmle M, Perry A. Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns. AJNR Am J Neuroradiol. 2007;28:1633–8. https://doi.org/10.3174/ajnr.A0682.
Article PubMed PubMed Central Google Scholar
Wilson MP, Katlariwala P, Low G, Murad MH, McInnes MDF, Jacques L, et al. Diagnostic accuracy of MRI for the detection of malignant peripheral nerve sheath tumors: a systematic review and meta-analysis. AJR Am J Roentgenol. 2021;217:31–9. https://doi.org/10.2214/AJR.20.23403.
Article PubMed Google Scholar
Skolnik AD, Loevner LA, Sampathu DM, Newman JG, Lee JY, Bagley LJ, et al. Cranial nerve schwannomas: diagnostic imaging approach. Radiographics. 2016;36:1463–77. https://doi.org/10.1148/rg.2016150199.
Article Google Scholar
Romano N, Federici M, Castaldi A. Imaging of cranial nerves: a pictorial overview. Insights Imaging. 2019;10:33. https://doi.org/10.1186/s13244-019-0719-5.
Article PubMed PubMed Central Google Scholar
Kapur R, Mafee MF, Lamba R, Edward DP. Orbital schwannoma and neurofibroma: role of imaging. Neuroimaging Clin N Am. 2005;15:159–74. https://doi.org/10.1016/j.nic.2005.02.004.
Article PubMed Google Scholar
Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 2003;61:696–8. https://doi.org/10.1212/01.wnl.0000078813.05925.2c.
Article PubMed Google Scholar
Chung SY, Kim DI, Lee BH, Yoon PH, Jeon P, Chung TS. Facial nerve schwannomas: CT and MR findings. Yonsei Med J. 1998;39:148–53. https://doi.org/10.3349/ymj.1998.39.2.148.
Article CAS PubMed Google Scholar
Koga H, Matsumoto S, Manabe J, Tanizawa T, Kawaguchi N. Definition of the target sign and its use for the diagnosis of schwannomas. Clin Orthop Relat Res. 2007;464:224–9. https://doi.org/10.1097/BLO.0b013e3181583422.
Article PubMed Google Scholar
Dewey BJ, Howe BM, Spinner RJ, Johnson GB, Nathan MA, Wenger DE, et al. FDG PET/CT and MRI features of pathologically proven schwannomas. Clin Nucl Med. 2021;46:289–96. https://doi.org/10.1097/RLU.0000000000003485.
Article PubMed Google Scholar
Brown IS. Pathology of perineural spread. J Neurol Surg B Skull Base. 2016;77(2):124–30. https://doi.org/10.1055/s-0036-1571837.
Article PubMed PubMed Central Google Scholar
Ong CK, Chong VF. Imaging of perineural spread in head and neck tumours. Cancer Imaging. 2010;10(1):S92–8. https://doi.org/10.1102/1470-7330.2010.9033.
Article PubMed PubMed Central Google Scholar
Carter RL, Foster CS, Dinsdale EA, Pittam MR. Perineural spread by squamous carcinomas of the head and neck: a morphological study using antiaxonal and antimyelin monoclonal antibodies. J Clin Pathol. 1983;36(3):269–75. https://doi.org/10.1136/jcp.36.3.269.
Article CAS PubMed PubMed Central Google Scholar
Raghavan P, Witek ME, Morales RE. Imaging of complications of chemoradiation. Neuroimaging Clin N Am. 2022;32(1):93–109. https://doi.org/10.1016/j.nic.2021.08.012.
Article PubMed Google Scholar
Saremi F, Helmy M, Farzin S, Zee CS, Go JL. MRI of cranial nerve enhancement. AJR Am J Roentgenol. 2005;185(6):1487–97. https://doi.org/10.2214/AJR.04.1518.
Article PubMed Google Scholar
Fischbein NJ, Kaplan MJ, Jackler RK, Dillon WP. MR imaging in two cases of subacute denervation change in the muscles of facial expression. AJNR Am J Neuroradiol. 2001;22:880–4.
CAS PubMed PubMed Central Google Scholar
Barakos JA, Dillon WP. Lesions of the foramen ovale: CT-guided fine-needle aspiration. Radiology. 1992;182(2):573–5. https://doi.org/10.1148/radiology.182.2.1732985.
Article CAS PubMed Google Scholar
Moonis G, Cunnane MB, Emerick K, Curtin H. Patterns of perineural tumor spread in head and neck cancer. Magn Reson Imaging Clin N Am. 2012;20(3):435–46. https://doi.org/10.1016/j.mric.2012.05.006.
Article PubMed Google Scholar
Debnam JM, Mayer RR, Chi TL, Ketonen L, Weinberg JS, Wei W, et al. Most common sites on MRI of intracranial neoplastic leptomeningeal disease. J Clin Neurosci. 2017;45:252–6. https://doi.org/10.1016/j.jocn.2017.07.020.
Article PubMed Google Scholar
Fritzhand SJ, Esmaeli B, Sun J, Debnam JM. Primary disease sites and patterns of spread in cases of neurolymphomatosis in the orbit associated with lymphoma. Cancer Imaging. 2021;21:39. https://doi.org/10.1186/s40644-021-00409-3.
Article PubMed PubMed Central Google Scholar
Mayer RR, Frankfort BJ, Strickland BA, Debnam JM, McCutcheon IE, Groves MD, et al. Leptomeningeal metastases presenting exclusively with ocular disturbance in 34 patients: a tertiary care cancer hospital experience. J Clin Neurosci. 2017;39:151–4. https://doi.org/10.1016/j.jocn.2017.01.024.
Article PubMed Google Scholar
Grisariu S, Avni B, Batchelor TT, van den Bent MJ, Bokstein F, Schiff D, et al. Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report. Blood. 2010;115:5005–11. https://doi.org/10.1182/blood-2009-12-258210.
Article CAS PubMed PubMed Central Google Scholar
Rothfus WE, Curtin HD. Extraocular muscle enlargement: a CT review. Radiology. 1984;151:677–81. https://doi.org/10.1148/radiology.151.3.6546996.
Article CAS PubMed Google Scholar
Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 2003;121:491–9. https://doi.org/10.1001/archopht.121.4.491.
Article PubMed Google Scholar
Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin N Am. 1999;37:151–68, xi. https://doi.org/10.1016/s0033-8389(05)70084-1.
Article CAS PubMed Google Scholar
Li Y, Lip G, Chong V, Yuan J, Ding Z. Idiopathic orbital inflammation syndrome with retro-orbital involvement: a retrospective study of eight patients. PLoS One. 2013;8:e57126. https://doi.org/10.1371/journal.pone.0057126.
Article CAS PubMed PubMed Central Google Scholar
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22:21–30. https://doi.org/10.1007/s10165-011-0571-z.
Article CAS PubMed Google Scholar
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51. https://doi.org/10.1056/NEJMra1104650.
Article CAS PubMed Google Scholar
Hayashi Y, Moriyama M, Maehara T, Goto Y, Kawano S, Ohta M, et al. A case of mantle cell lymphoma presenting as IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz’s disease. World J Surg Oncol. 2015;13:225. https://doi.org/10.1186/s12957-015-0644-0.
Article PubMed PubMed Central Google Scholar
Fujita A, Sakai O, Chapman MN, Sugimoto H. IgG4-related disease of the head and neck: CT and MR imaging manifestations. Radiographics. 2012;32(7):1945–58. https://doi.org/10.1148/rg.327125032.
Article PubMed Google Scholar
Himi T, Takano K, Yamamoto M, Naishiro Y, Takahashi H. A novel concept of Mikulicz’s disease as IgG4-related disease. Auris Nasus Larynx. 2012;39(1):9–17. https://doi.org/10.1016/j.anl.2011.01.023.
Article PubMed Google Scholar
Ginat DT, Freitag SK, Kieff D, Grove A, Fay A, Cunnane M, et al. Radiographic patterns of orbital involvement in IgG4-related disease. Ophthalmic Plast Reconstr Surg. 2013;29:261–6. https://doi.org/10.1097/IOP.0b013e31829165ad.
Article PubMed Google Scholar
Mahalingam HV, Mani SE, Patel B, Prabhu K, Alexander M, Fatterpekar GM, Chacko G. Imaging spectrum of cavernous sinus lesions with histopathologic correlation. Radiographics. 2019;39:795–819. https://doi.org/10.1148/rg.2019180122.
Article PubMed Google Scholar
Tiegs-Heiden CA, Eckel LJ, Hunt CH, Diehn FE, Schwartz KM, Kallmes DF, et al. Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 2014;35:1393–7. https://doi.org/10.3174/ajnr.A3865.
Article CAS PubMed PubMed Central Google Scholar
Caputo C, Bazargan A, McKelvie PA, Sutherland T, Su CS, Inder WJ. Hypophysitis due to IgG4-related disease responding to treatment with azathioprine: an alternative to corticosteroid therapy. Pituitary. 2014;17:251–6. https://doi.org/10.1007/s11102-013-0498-9.
Article CAS PubMed Google Scholar
Zhao Z, Wang Y, Guan Z, Jin J, Huang F, Zhu J. Utility of FDG-PET/CT in the diagnosis of IgG4-related diseases. Clin Exp Rheumatol. 2016;34:119–25.
PubMed Google Scholar

Download references

Author information

Authors and Affiliations

Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
J. Matthew Debnam

Authors

J. Matthew Debnam
View author publications
You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. Matthew Debnam .

Editor information

Editors and Affiliations

Department of Neuroradiology, Division of Diagnostic Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
J. Matthew Debnam

Rights and permissions

Reprints and permissions

Copyright information

About this chapter

Cite this chapter

Debnam, J.M. (2023). Cranial Nerves II–VI. In: Debnam, J.M. (eds) Imaging Atlas of Ophthalmic Tumors and Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-17479-7_10

Download citation

DOI: https://doi.org/10.1007/978-3-031-17479-7_10
Published: 10 March 2023
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-17478-0
Online ISBN: 978-3-031-17479-7
eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Cranial Nerves II–VI

Abstract

Similar content being viewed by others

Cranial Nerves

Cranial Nerves

Peripheral Nerve Tumors

Keywords

Orbital Cranial Nerve Anatomy

CN II (Optic Nerve)

CN III (Oculomotor Nerve)

CN IV (Trochlear Nerve)

CN V (Trigeminal Nerve)

CN VI (Abducens Nerve)

Optic Nerve Sheath Meningioma

Background

Presentation

Imaging

CT

MRI

PET/CT

Key Points

Optic Nerve Glioma

Background

Presentation

Imaging

CT

MRI

Key Points

Optic Neuritis

Background

Presentation

Imaging

MRI

Key Points

Nerve Sheath Tumors

Background

Presentation

Imaging

CT

MRI

PET

Key Points

Perineural Tumor Spread

Background

Presentation

Imaging

MRI

Key Points

Leptomeningeal Disease

Background

Presentation

Imaging

Key Points

Idiopathic Orbital Inflammation (IOI)

Background

Presentation

Imaging

CT

MRI

Key Points

IgG4-Related Disease

Background

Presentation

Imaging

CT

MRI

PET

Key Points

References

Author information

Authors and Affiliations

Corresponding author

Editor information

Editors and Affiliations

Rights and permissions

Copyright information

About this chapter

Cite this chapter

Download citation

Share this chapter