| OCR Text |
Show PHOTO ESSAY ‘‘Ophthalmoplegic Migraine'' With Reversible MRI Enhancement of the Cisternal Sixth Cranial Nerve Patrick J. M. Lavin, MD, Joseph M. Aulino, MD, and David Uskavitch, MD FIG. 1. Axial MRI through the pons. Symmetrically reformatted, thin-section MRI at the pontine level performed 3 weeks after onset of left sixth cranial nerve palsy in our patient. A. Precontrast T1 image shows the left sixth cranial nerve in the prepontine cistern (white arrowhead). B. Postcontrast T1 image shows enhancement of the nerve (white arrow). C. Gradient echo image shows the nerve as a linear hypointense signal outlined by a hyperintense area of surrounding cerebrospinal fluid. D. Five-month follow-up study. Postcontrast T1 image shows no abnormal enhancement of the left sixth cranial nerve. Abstract: A 45-year-old woman reported multiple episodes of reversible left eye pain and diplopia stretching over 12 years. Ophthalmic examinations had repeatedly disclosed a left sixth cranial nerve Departments of Neurology (PJML, DU), Ophthalmology (PJML), and Radiology (JMA), Vanderbilt University Medical Center, Nashville, Tennessee. This study was supported by an unrestricted grant from Research to Prevent Blindness, Inc. Address correspondence to Patrick J. M. Lavin, MD, Department of Neurology, Vanderbilt University Medical Center, 1161 21st Avenue South, Suite A-0118 MCN, Nashville, TN 37232-2551; E-mail: patrick. lavin@vanderbilt.edu palsy. Postcontrast brain MRI performed 3 weeks after clinical onset of the most recent episode demonstrated enhancement of the cisternal segment of the left sixth cranial nerve. Five months later, when symptoms and signs had largely abated, postcontrast brain MRI was normal. The clinical diagnosis satisfies the criteria for ‘‘ophthalmoplegic migraine.'' Although reversible cisternal enhancement of the third cranial nerve has been often described in this condition, this is the first report of cisternal enhancement of the sixth cranial nerve. (J Neuro-Ophthalmol 2009;29:151-153) J Neuro-Ophthalmol, Vol. 29, No. 2, 2009 151 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. J Neuro-Ophthalmol, Vol. 29, No. 2, 2009 Lavin et al A45-year-old woman had episodic severe left eye pain and double vision for 12 years. The episodes occurred 2-3 times per year and symptoms usually lasted about 4 weeks. Each episode started abruptly with steady left periocular pain (rated 8 of 10 in severity) that often awakened her from sleep. She reported photophobia and phonophonia but no obvious pupil size changes, tearing, or nasal discharge. The pain usually abated a few hours after she received parenteral ketorolac, meperidine, or cortico-steroids. After receiving treatment, she usually slept but always awoke to binocular diplopia that was horizontal and worse at distance and on left gaze. The pain never lasted for more than 2 or 3 days, but the diplopia usually lasted for at least 4 weeks. Propranolol, topiramate, and indomethacin did not prevent the attacks. Abortive agents, including nonpre-scription medications and triptans, neither helped the headaches nor prevented the diplopia. She had a history of depression, hypertension, two cesarean deliveries, an appen-dectomy, and a fractured elbow. Her medications were valsartan, metoprolol, alprazolam, a daily vitamin, and topiramate (50 mg daily). At the time of our first examination, the patient reported a typical episode starting 3 months earlier, with diplopia that was initially present in the primary gaze position and gradually improved so that it was present on left gaze. Visual acuity was 20/20 in each eye at distance and near viewing. Her eyes were aligned in primary position but she had only 75% abduction of the left eye. The right pupil was fractionally larger than the left, but both reacted briskly to light and near stimuli. The palpebral fissures were equal in height. The ophthalmologic and neurologic examinations were otherwise normal. When examined 2 months later, 5 months after the onset of the current episode, she had diplopia only on extreme left gaze and 90% abduction of the left eye. Brain and orbit MRI, which was performed 3 weeks after the onset of the current episode, demonstrated enhancement of the left sixth cranial nerve in the pre-pontine cistern (Fig. 1A-C). Brain MRA showed no abnormality. A second brain MRI (Fig. 1D), performed 5 months after the commencement of the current episode, demonstrated no sixth cranial nerve enhancement. ‘‘Ophthalmoplegic migraine'' (OM), a rare enigmatic disorder with an annual incidence of about 1 per million (1), was reclassified by the International Classification of Headache Disorders, 2nd ed. (ICHD-III) (2) as a recurrent neuralgia under the category ‘‘cranial neuralgias and central causes of facial pain.'' The onset of OM is usually, but not always, in the first decade. Involvement of the third cranial nerve is more common than involvement of the fourth or sixth cranial nerves, and simultaneous impairment of more than one ocular motor nerve is exceptional (1). Many patients with OM clinically involving the third cranial nerve demonstrate enhancement or thickening, or both, of the cisternal segment of the third cranial nerve (3,4) that is usually reversible within 7-9 weeks (3) (Fig. 2). Isolated enhancement of the fourth cranial nerve has been reported once in this condition (5) (Fig. 3). There has also been one previous report of reversible enhancement of the intraparenchymal portion of the sixth cranial nerve in OM (Fig. 4) (6), but no previous report of enhancement of the FIG. 2. Previously reported example of enhancing cisternal left third cranial nerve in ‘‘ophthalmoplegic migraine''. Postcontrast axial MRI shows the typical trapezoidal neural thickening and high signal (arrow). (Modified from Mark et al [3]). FIG. 3. Previously reported example of enhancing and thickening of the cisternal left fourth cranial nerve in ‘‘ophthalmoplegic migraine'' (arrow). (Modified from van der Dussen et al [5]). 152 © 2009 Lippincott Williams & Wilkins Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Ophthalmoplegic Migraine J Neuro-Ophthalmol, Vol. 29, No. 2, 2009 FIG. 4. Previously reported example of enhancing intra-pontine left sixth cranial nerve in ‘‘ophthalmoplegic migraine'' (arrow). (Modified from Lee et al [6]). cisternal portion of the sixth cranial nerve such as we are describing here. MRI enhancement of the cranial nerves occurs with infectious, inflammatory, demyelinating, and neoplastic disorders. Enhancement of the cisternal portion of the sixth cranial nerve is reported with trauma, ischemia, venous congestion associated with a medullary venous malforma-tion, leukemia, chemical meningitis, the Fisher variant of acute inflammatory demyelinating polyneuropathy (AIDP), polyneuritis cranialis (probably a variant of AIDP), multiple sclerosis, diabetes mellitus, meningoencephalitis, autoim-mune disorders such as the Tolosa-Hunt syndrome (7), Lyme disease (8), and vincristine therapy (9). This is the first report of enhancement of the cisternal portion of the sixth cranial nerve in OM. REFERENCES 1. Hansen SL, Borelli-Møller L, Strange P, et al. Ophthalmoplegic migraine: diagnostic criteria, incidence of hospitalization and possible etiology. Acta Neurol Scand 1990;81:54-60. 2. Headache Classification Subcommittee. The International Classifica-tion of Headache Disorders, 2nd edition. Cephalalgia 2004;24 (Suppl. 1):1-160. 3. Mark AS, Casselman J, Brown D, et al. Ophthalmoplegic migraine: reversible enhancement and thickening of the cisternal segment of the oculomotor nerve on contrast-enhanced MR images. AJNR Am J Neuroradiol 1998;19:1887-91. 4. Carlow TJ. Oculomotor ophthalmoplegic migraine: is it really migraine. J Neuroophthalmol 2002;22:215-21. 5. van der Dussen DH, Bloem BR, Liauw L, et al. Ophthalmoplegic migraine: migrainous or inflammatory? Cephalgia 2004;24: 312-5. 6. Lee TG, Choi SW, Chung KC. Ophthalmoplegic migraine with reversible enhancement of intraparenchymal abducens nerve on MRI. Headache 2002;42:140-1. 7. Hosoya T, Adachi M, Yamaguchi K, et al. Abducens nerve enhancement demonstrated by multiplanar reconstruction of con-trast- enhanced three-dimensional MRI. Neuroradiology 2001;43: 295-301. 8. Lell M, Schmid B, Stemper B, et al. Simultaneous involvement of third and sixth cranial nerve in a patient with Lyme disease. Neuroradiology 2003;45:85-7. 9. Toker E, Yenice O, Ogut MS. Isolated abducens palsy induced by vincristine therapy. J AAPOS 2004;8:69-71. 153 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |
