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Letters to the Editor

A Limited Form of Neuromyelitis Optica With a Lesion of the Fourth Nerve Nucleus

Ogasawara, Mikihide MD; Sakai, Tsutomu MD, PhD; Kono, Yu MD, PhD; Shikishima, Keigo MD, PhD; Tsuneoka, Hiroshi MD, PhD

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Journal of Neuro-Ophthalmology: December 2013 - Volume 33 - Issue 4 - p 414-416
doi: 10.1097/WNO.0b013e31829e0b24
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We read with great interest the review article “Neuromyelitis optica” by Morrow and Wingerchuk (1). The authors referred to several reports of abnormal eye movements in association with brainstem lesions in NMO (2–4). We evaluated a patient with a limited form of NMO with a fourth nerve palsy.

A 62-year-old woman with a history of numbness in the legs, nausea, and repeated vomiting presented with vertical diplopia and numbness in the left arm and chest. Neuro-ophthalmic testing demonstrated visual acuity of 20/20 in both eyes, full visual fields, and normal ophthalmoscopy. The patient had a right hypertropia that increased in left gaze and head tilt to the right. Ocular motility was otherwise unremarkable.

Magnetic resonance imaging (MRI) showed hyperintense areas in the midbrain, pons, and medulla (Fig. 1) and in the spinal cord from C5 to T1 (Fig. 2). NMO antibody was positive, whereas acetylcholine receptor, nuclear, SS-A, and SS-B antibodies were all negative. Cerebrospinal fluid analysis revealed no oligoclonal bands.

FIG. 1
FIG. 1:
A. Sagittal T2 magnetic resonance imaging (MRI) shows hyperintense lesions in the midbrain and pons (arrowheads). Axial FLAIR MRI reveals hyperintense lesions in the midbrain (B, arrowhead), pons (C, arrowhead), and left side of the medulla oblongata (D, arrowhead).
FIG. 2
FIG. 2:
Sagittal T2 spine magnetic resonance imaging demonstrates a hyperintense lesion extending for five vertebral segments (C5 to T1;arrowheads).

The patient was diagnosed with a limited form of NMO due to the presence of NMO antibody and longitudinally extensive spinal cord lesions. She was treated with intravenous methylprednisolone pulse therapy (1,000 mg daily for 3 days) followed by oral prednisolone (10 mg/day with gradual taper). Two months after the third pulse, vertical diplopia had almost totally resolved with a decrease of the hyperintense areas on MRI.

Our patient with a right fourth nerve palsy had a form of NMO, a subtype of NMO spectrum disorder. Reports of cases of NMO or NMO spectrum disorder with ophthalmoplegia are rare. Gilmore et al (5) described a patient with Parinaud syndrome, in which MRI abnormalities were found in periaqueductal gray matter. Shinoda et al (3) found a midbrain tegmentum lesion adjacent to the aqueduct on MRI causing wall-eyed bilateral internuclear ophthalmoplegia syndrome in a patient with NMO spectrum disorder. In our patient, MRI showed a high-intensity lesion in the area of the fourth nerve nucleus causing vertical diplopia. In addition, our patient had a history of nausea and vomiting likely due to NMO involving the medullary floor of the fourth ventricle and area postrema (6,7).


1. Morrow MJ, Wingerchuk D. Neuromyelitis optica. J Neuroophthalmol. 2012;32:154–166.
2. Hage R Jr, Merle H, Jeannin S, Cabre P. Ocular oscillations in the neuromyelitis optica spectrum. J Neuroophthalmol. 2011;31:255–259.
3. Shinoda K, Matsushita T, Furuta K, Isobe N, Yonekawa T, Ohyagi Y, Kira J. Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome in a patient with neuromyelitis optica spectrum disorder and anti-aquaporin-4 antibody. Mult Scler. 2011;17:885–887.
4. Wang KC, Lee CL, Chen SY, Lin KH, Tsai CP. Prominent brainstem symptoms/signs in patients with neuromyelitis optica in a Taiwanese population. J Clin Neurosci. 2011;18:1197–1200.
5. Gilmore CP, Jacob A, Evangelou N. A case of neuromyelitis optica with gadolinium-enhancing brain lesions and Parinaud syndrome. Arch Neurol. 2009;66:140–141.
6. Patel V, Griffith NC, Blackwood E, Dias M, Cordato DJ. Spectrum disorder of neuromyelitis optica in a patient presenting with intractable vomiting and hiccups, transverse myelitis and acute encephalopathy. J Clin Neurosci. 2012;19:1576–1578.
7. Popescu BF, Lennon VA, Parisi JE, Howe CL, Weigand SD, Cabrera-Gómez JA, Newell K, Mandler RN, Pittock SJ, Weinshenker BG, Lucchinetti CF. Neuromyelitis optica unique area postrema lesions: nausea, vomiting, and pathogenic implications. Neurology. 2011;76:1229–1237.
© 2013 by North American Neuro-Ophthalmology Society