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Saremi et al.

MRI of Cranial Nerve Enhancement

N e u ro r a d i o l og y P i c t o r i a l E s s ay

MRI of Cranial Nerve Enhancement


Farhood Saremi1 Mohammad Helmy1 Sahar Farzin1 Chi S. Zee2 John L. Go2
Saremi F, Helmy M, Farzin S, Zee CS, Go JL RI is invaluable in characterizing disease of the cranial nerves. Gadolinium administration increases the ability of MRI to detect such abnormalities. We begin this pictorial essay with a description of the histologic anatomy of the cranial nerves and patterns of normal cranial nerve enhancement. After briefly discussing the pathophysiology, we review the MR appearance of abnormal cranial nerve enhancement in various diseases ranging from common neoplastic and infectious conditions to rare conditions such as ophthalmoplegic migraine and idiopathic pachymeningitis. In some cases, abnormal cranial nerve enhancement on MRI may be the only clue to the underlying disease. OBJECTIVE. In this pictorial essay, we review the MR appearance of cranial nerve enhancement in a variety of entities including neoplastic, infectious, and idiopathic diseases. CONCLUSION. MRI with contrast enhancement is a valuable tool for detecting and characterizing disease of the cranial nerves. Abnormal cranial nerve enhancement on MRI may sometimes be the first or only indication of an underlying disease process.

DOI:10.2214/AJR.04.1518 Received September 25, 2004; accepted after revision March 7, 2005.
1Department

of Radiological Sciences, University of California, Irvine, 101 The City Dr., Rte. 140, Orange, CA 92868. Address correspondence to F. Saremi (fsaremi@uci.edu).

2Department

of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA.

AJR 2005; 185:14871497 0361803X/05/18561487 American Roentgen Ray Society

Anatomy and Pathophysiology The cranial nerves are surrounded by a series of connective tissue sheaths called endoneurium, perineurium, and epineurium. The bloodnerve barrier of cranial nerves is maintained by the combined actions of tight junctions in the endothelium of the endoneural capillaries and tight junctions in the inner layers of the perineurium. Various insults disrupt the bloodnerve barrier, allowing leakage and accumulation of contrast material with resultant perineural enhancement. Such disruption may arise secondary to neoplasm, autoimmune disease, inflammation, demyelination, ischemia, trauma, radiation, and axonal degeneration, all resulting in abnormal cranial nerve enhancement (Appendix 1).

Normal Cranial Nerve Enhancement There are instances of normal cranial nerve enhancement. The geniculate, tympanic, and mastoid segments of the facial nerve possess peri- and epineural venous plexuses that may cause moderate enhancement by an increased vascular pool of contrast material [1]. The intracanalicularlabyrinthine segment does not normally enhance. The trigeminal ganglion and the proximal portions of its divisions are seen as discrete nonenhancing structures surrounded by an enhancing perineural vascular plexus. Enhancement of the trigeminal ganglion or its maxillary or mandibular divisions is infrequently seen as evidenced by their avascular appearance in cadaveric specimens [2]. When such enhancement is seen on MRI, it may be related to suboptimal imaging parameters, avid enhancement of the perivascular plexus, or a combination of both. Neoplasm Neoplastic meningitis refers to the disseminated seeding of the leptomeninges by malignant cells. This includes carcinomatous meningitis in patients with solid tumors and lymphomatous and leukemic meningitis when involvement is related to these underlying diseases. The most common cancers to involve the leptomeninges are breast (5%), lung (925%), and melanoma (23%) [3] (Fig. 1). MRI findings include pial enhancement and nodularity, smooth or nodular cranial nerve enhancement, hydrocephalus, and coexisting brain or bone metastases [4]. Primary diffuse leptomeningeal gliomatosis

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Fig. 148-year-old woman with metastatic melanoma and meningeal carcinomatosis. AE, Contrast-enhanced axial (A, B, D, and E) and coronal (C) T1-weighted images show enhancement and involvement of multiple cranial nerves: oculomotor nerves (arrows, A); trigeminal nerves (arrows, B); complex of seventh and eighth cranial nerves (arrows, C); complex of ninth, tenth, and eleventh cranial nerves (long arrows, D and E); and hypoglossal nerves (short arrows, E).

is a rare condition whereby a glioma arises from heterotopic cell nests in the leptomeninges. Leptomeningeal dissemination is an un-

common complication of gliomas and other primary intraaxial malignancies. The presence of a single unexplained en-

hancing cranial nerve in a patient with cancer raises the possibility of leptomeningeal dissemination.

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Fig. 256-year-old woman after resection of adenoid cystic carcinoma of right hard palate. A, Axial bone window CT image shows widening of right pterygopalatine fossa (arrow). B and C, Contrast-enhanced axial T1-weighted MR images reveal infiltrating mass in right pterygopalatine fossa (short arrow, B) and cavernous sinus (short arrow, C). Note abnormal signal intensity in right masticator space (long arrows, B) and right medial temporal lobe (long arrows, C).

Fig. 343-year-old man with acute lymphoblastic leukemia. A, Axial FLAIR image reveals leukemic infiltrate of left pons and brachium pontis (arrow). (Fig. 3 continues on next page)

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B
Fig. 3 (continued)43-year-old man with acute lymphoblastic leukemia. B and C, Contrast-enhanced axial T1-weighted images show antegrade perineural extension along course of left spinal trigeminal tract and nuclei (arrow, B) into preganglionic segment of left trigeminal nerve (arrow, C).

Fig. 47-year-old girl with tuberculous meningitis. A and B, Contrast-enhanced axial (A) and coronal (B) T1-weighted images show abnormal peripheral enhancement of oculomotor nerves (long arrows). In addition, there is leptomeningeal enhancement of anterior surface of brainstem (short arrows, A).

Perineural tumor extension, a form of metastatic disease, involves the spread of primary mucosal or cutaneous tumors to noncontiguous regions along nerve sheaths. Perineural tumor spread has been shown in perineural or endoneural tissue planes along a path of least resistance. Retrograde spread is significantly more common than ante-

grade spread. A series by Parker and Harnsberger [5] found perineural spread occurs most commonly with squamous cell carcinoma and adenoid cystic carcinoma, with the facial nerve and second and third divisions of the trigeminal nerve most frequently involved (Fig. 2). Other neoplastic and aggressive infectious processes, such as

acute lymphoblastic leukemia, nonHodgkins lymphoma, malignant schwannoma, aspergillosis, mucormycosis, and actinomycosis, also show perineural extension (Fig. 3). MRI findings of perineural involvement include smooth thickening and enhancement of the nerve, concentric expansion of the skull base foramina with

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Fig. 532-year-old man with cryptococcal meningitis and perioptic neuritis. Contrast-enhanced axial T1-weighted image with fat suppression reveals thickening and enhancement of perineural structures of left optic nerve.

Fig. 661-year-old man with perineural spread of rhinocerebral mucormycosis who presented for follow-up after right orbital exenteration. A and B, Axial T2-weighted (A) and contrast-enhanced T1-weighted (B) images show recurrence of infection with invasion of right cavernous sinus (long arrows, A) and retrograde involvement of trigeminal nerve along cavernous, ganglionic, and cisternal segments (arrows, B). Abnormal signal within right pons indicates edema (short arrow, A).

obliteration of normal fatty contents, enlargement of the cavernous sinus, and neuropathic muscular atrophy [6].

Infection Infectious meningitis results from viral, bacterial, fungal, or parasitic infection. Lep-

tomeningitis is the most common form of intracranial tuberculosis, particularly in the pediatric population. Cranial nerve involvement

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Fig. 717-year-old boy with neuroschistosomiasis. Contrast-enhanced T1-weighted images show range of involvement of CNS in schistosomiasis. A, Sagittal image shows enhancing masses within chiasmatichypothalamic (short arrow) and pineal (long arrow) regions. B, Coronal image shows thickened and enhancing trigeminal nerves (arrows). C, Axial image reveals enhancing mass (arrow) in right cerebellopontine angle with extension into internal auditory canal.

is seen in 1770% of patients and occurs in the setting of diffuse leptomeningeal tuberculosis. Impairment has been attributed to ischemia of the nerve or entrapment of the nerve in basal exudates [7] (Fig. 4). Cryptococcus neoformans is the most common fungus to involve the CNS. Cryptococcal meningitis is one of the typical pathologic manifestations and can result in optic neuropathy in both immunocompetent and immunocompromised patients (Fig. 5).

Optic neuropathy is a rare complication of cryptococcal meningitis and usually occurs in non-AIDS patients. Necrosis of the optic nerves and infiltration of the meninges around the optic tracts, nerves, and chiasm by cryptococcal organisms have been observed [8]. Rhinocerebral mucormycosis is a potentially devastating fungal infection in diabetic and immunocompromised patients. Sinonasal disease often progresses to the orbit and

cavernous sinus and may be complicated by vascular and perineural invasion and local thrombotic infarction [9] (Fig. 6). Cranial neuroschistosomiasis occurs less commonly than the spinal variety and may arise with any of the clinical forms of this parasitic infection. Eggs within the CNS induce a cell-mediated periovular granulomatous reaction that leads to signs and symptoms of increased intracranial pressure and focal neurologic signs [10]. Although

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Fig. 832-year-old man with Bells palsy. AC, Contrast-enhanced axial (A) and coronal (B and C) T1-weighted images show abnormal enhancement of right facial nerve extending from distal intracanalicular segment (arrows, A and B) to distal mastoid segment (arrow, C).

meningeal spread of infection involving cauda equina nerve roots has been reported, rare instances of cranial nerve involvement may also be seen as in our case (Fig. 7). Cranial neuritis in Lyme disease may involve any of cranial nerves III through VII, with the facial nerve most frequently affected and often associated with cochleovestibular nerve abnormalities. The affected segments appear thickened and enhance. Viral infections related to herpes simplex virus type 1, cytomegalovirus, and Varicella zoster organisms also manifest with cranial nerve involvement and show abnormal enhancement on MRI.

Postinfectious and Demyelinating Disorders Bells palsy is the most common cause of unilateral peripheral facial neuropathy. In addition to normal enhancement of the facial nerve segments discussed earlier, there is pathologic enhancement of the intracanalicularlabyrinthine portion (Fig. 8). Martin-Duverneuil et al. [11] suggest three criteria for pathologic enhancement of the facial nerve: enhancement outside the facial canal, extension of enhancement to cranial nerve VIII, and intense enhancement of the labyrinthine and mastoid segments. In Ramsay Hunt syndrome, abnormal facial nerve enhancement is

accompanied by enhancement of the vestibular and cochlear nerves as a result of extension of inflammation from cranial nerve VII to the intracanalicular portions of these cranial nerve VIII divisions. Ophthalmoplegic migraine is a rare condition characterized by headache and oculomotor nerve palsy lasting days to weeks. MRI findings include reversible enhancement of the cisternal segment of the oculomotor nerve and focal thickening at the exit of the nerve in the interpeduncular cistern (Fig. 9). Involvement of cranial nerves IV, V1, and VI also occurs. Multiple cranial nerve involvement is also present in a group

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Fig. 957-year-old man with ophthalmoplegic migraine. AC, Unenhanced axial (A) and enhanced axial (B) and coronal (C) T1-weighted images reveal smooth enlargement and homogeneous enhancement of cisternal segment of left oculomotor nerve (arrows).

of inflammatory demyelinating polyneuropathies that include Guillain-Barr and variants, such as Miller Fisher syndrome and polyneuritis cranialis. Granulomatosis Intracranial neurosarcoidosis has a predilection for the basal leptomeninges, and involvement of every cranial nerve has been described. MRI shows a spectrum of CNS abnormalities including diffuse or nodular thickening and abnormal enhancement of the leptomeninges in the basal cisterns and hypothalamic regions [12] (Fig. 10). Perineural spread has also been reported in sarcoidosis [13]. Clinical involvement and

imaging cranial nerve involvement frequently do not coincide, and clinical resolution may not imply imaging resolution [14]. Idiopathic hypertrophic cranial pachymeningitis is a rare disease characterized by inflammation and fibrosis of the dura mater. It remains a diagnosis of exclusion but may be the presenting manifestation of granulomatous diseases such as sarcoidosis, Wegeners granulomatosis, or tuberculosis. MRI shows focal or diffuse thickening and enhancement of the dura that encase cranial nerves causing recurrent cranial neuropathies. The oculomotor, abducens, and facial nerves are more frequently involved [15].

Tolosa-Hunt syndrome consists of painful ophthalmoplegia related to a granulomatous inflammatory process in the cavernous sinus. MRI findings are nonspecific and include enhancement and abnormal soft tissue in the ipsilateral cavernous sinus and orbital apex [16] (Fig. 11). Postradiation Neuritis Radiation-induced cranial nerve injury is an uncommon, usually delayed, complication of radiation therapy or radiosurgery. Cranial nerve deficits may be permanent or resolve spontaneously. Loss of the nerveblood barrier due to demyelination and ischemia, coagulation necrosis, or peripheral fibrosis results

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Fig. 1058-year-old man with neurosarcoidosis. A and B, Contrast-enhanced axial T1-weighted images show enhancement and involvement of cisternal segments of right and left seventh and eighth cranial nerve complexes (arrows, A) and root entry zones of preganglionic trigeminal nerves (arrows, B).

A
Fig. 1135-year-old woman with Tolosa-Hunt syndrome presenting with painful ophthalmoplegia. A, Enhancement and enlargement of left cavernous sinus are illustrated on contrast-enhanced coronal T1-weighted image (arrow). B, Extension of enhancing tissue into left orbital apex (arrow) is seen on contrast-enhanced axial T1-weighted image.

in cranial nerve enhancement. Radiationinduced optic neuropathy occurs months to years after exposure of the anterior visual

pathways to ionizing radiation. MRI shows smooth enlargement and enhancement of the optic nerve and chiasm (Fig. 12).

Primary Nerve Tumors Vestibular schwannomas are the most common cranial nerve schwannomas, followed by

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Fig. 1248-year-old woman with postradiation optic neuritis who presented with loss of vision in left eye 8 months after radiation therapy. Patient had previously undergone resection of adenoid cystic carcinoma of right maxillary sinus. Contrastenhanced axial T1-weighted image shows enhancement of intracranial portion of left optic nerve (long arrow). Note large enhancing tumor (short arrows) with internal hemorrhage in right temporal lobe.

A
Fig. 1348-year-old woman with schwannoma arising from left inferior vestibular nerve. A and B, Contrast-enhanced axial (A) and coronal (B) T1-weighted images show small enhancing tumor (arrows).

trigeminal and facial schwannomas and then glossopharyngeal, vagus, and spinal accessory nerve schwannomas (Fig. 13). Neurofibromatosis 2 is characterized by bilateral vestibular schwannomas. Schwannomas of the other cranial nerves occur more frequently in neurofibromatosis 2. Enhancing hemangiomas, meningiomas, or metastases may mimic the appearance of early schwannomas.

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Graphics 1991; 11:383399 6. Caldemeyer KS, Mathews VP, Righi PD, Smith RR. Imaging features and clinical significance of perineural spread or extension of head and neck tumors. RadioGraphics 1998; 18:97110 7. Uysal G, Kose G, Guven A, Diren B. Magnetic resonance imaging in diagnosis of childhood central nervous system tuberculosis. Infection 2001; 29:148153 8. Cohen DB, Glasgow BJ. Bilateral optic nerve cryptococcosis in sudden blindness in patients with acquired immune deficiency syndrome. Ophthalmology 1993; 100:16891694 9. McLean FM, Ginsberg LE, Stanton CA. Perineural spread of rhinocerebral mucormycosis. Am J Neuroradiol 1996; 17:114116 10. Pittella JE. Neuroschistosomiasis. Brain Pathol 1997; 7:649662 11. Martin-Duverneuil N, Sola-Martinez MT, Miaux Y, et al. Contrast enhancement of the facial nerve on MRI: normal or pathological? Neuroradiology 1997; 39:207212 12. Nowak DA, Widenka DC. Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 2001; 248:363372 13. Mazziotti S, Gaeta M, Blandino A, Vinci S, Pandolfo I. Perineural spread in a case of sinonasal sarcoidosis: case report. Am J Neuroradiol 2001; 22:12071208 14. Christoforidis GA, Spickler EM, Recio MV, Mehta BM. MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment. Am J Neuroradiol 1999; 20:655669 15. Goyal M, Malik A, Mishra NK, Gaikwad SB. Idiopathic hypertrophic pachymeningitis: spectrum of the disease. Neuroradiology 1997; 39:619623 16. Yousem DM, Atlas SW, Grossman RI, Sergott RC, Savino PJ, Bosley TM. MR imaging of Tolosa-Hunt syndrome. AJR 1990; 154:167170

APPENDIX 1: Classification of Cranial Neuropathies Neoplastic: Carcinoma, lymphoma, leukemia, glioma, myeloma Infection: Tuberculosis, syphilis, leprosy, mycoplasma, Lyme disease, viral infections, fungal infections, parasitic infections Postinfectious and demyelinating: Bells palsy, Ramsay Hunt syndrome, ophthalmoplegic migraine, Miller Fisher syndrome, polyneuritides, multiple sclerosis Granulomatosis: Sarcoidosis, idiopathic granulomatosis, vasculitis, inflammatory granulomatosis Angiopathic: Wegeners granulomatosis, Churg-Strauss syndrome, Behets syndrome, diabetes Idiopathic: Idiopathic pachymeningitis, Tolosa-Hunt syndrome Physical or chemical: Radiation, trauma, surgery, toxins, drugs Hereditary: Dejerine-Sottas disease, Krabbes disease Primary nerve tumors: Schwannoma, neurofibromatosis

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