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Open Access Research article

Painful ophthalmoplegia with normal cranial imaging

Chih-Hsien Hung12, Kuo-Hsuan Chang3, Chun-Che Chu3, Ming-Feng Liao3, Hong-Shiu Chang3, Rong-Kuo Lyu3, Yi-Ming Wu4, Yao-Liang Chen5, Chiou-Lian Lai2, Hsiao-Jung Tseng6 and Long-Sun Ro3*

Author Affiliations

1 Department of Neurology, Kaohsiung Municipal Hsiaokang Hospital, Kaohsiung, Taiwan

2 Department of Neurology, Kaohsiung Medical University Chung-Ho Memorial Hospital; Kaohsiung Medical University, Kaohsiung, Taiwan

3 Department of Neurology, Linkou Campus, Chang Gung Memorial Hospital and University College of Medicine, Gueishan Township, Taiwan

4 Department of Radiology, Linkou Campus, Chang Gung Memorial Hospital and University College of Medicine, Gueishan Township, Taiwan

5 Department of Radiology, Keelung campus, Chang Gung Memorial Hospital and University College of Medicine, Keelung City, Taiwan

6 Biostatistical Center for Clinical Research, Chang Gung Memorial Hospital, Gueishan Township, Taiwan

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BMC Neurology 2014, 14:7  doi:10.1186/1471-2377-14-7

Published: 9 January 2014

Abstract

Background

Painful ophthalmoplegia with normal cranial imaging is rare and confined to limited etiologies. In this study, we aimed to elucidate these causes by evaluating clinical presentations and treatment responses.

Methods

Cases of painful ophthalmoplegia with normal cranial MRI at a single center between January 2001 and June 2011 were retrospectively reviewed. Diagnoses of painful ophthalmoplegia were made according to the recommendations of the International Headache Society.

Results

Of the 58 painful ophthalmoplegia cases (53 patients), 26 (44.8%) were diagnosed as ocular diabetic neuropathy, 27 (46.6%) as benign Tolosa-Hunt syndrome (THS), and 5 (8.6%) as ophthalmoplegic migraine (OM). Patients with ocular diabetic neuropathy were significantly older (62.8 ± 7.8 years) than those with benign THS (56.3 ±12.0 years) or OM (45.8 ± 23.0 years) (p < 0.05). Cranial nerve involvement was similar among groups. Pupil sparing was dominant in each group. Patients with benign THS and OM responded exquisitely to glucocorticoid treatment with resolved diplopia, whereas patients with ocular diabetic neuropathy didn’t (p < 0.05). Patients with OM recovered more rapidly than the other groups did (p < 0.05). Overall, most patients (94.8%) recovered completely during the follow-up period.

Conclusions

Ocular diabetic neuropathy and benign THS accounted for most of the painful ophthalmoplegias in patients with normal cranial imaging. Patient outcomes were generally good.

Keywords:
Headache; Ocular diabetic neuropathy; Ophthalmoplegic migraine; Painful ophthalmoplegia; Tolosa-Hunt syndrome