Abstract
Background
Intracranial aneurysms are the most common vascular cause of painful ophthalmoplegia. Symptoms include retro-orbital pain, diplopia, ophthalmoplegia, trigeminal neuropathy, or a combination of these. Most single aneurysms cause ipsilateral, painful ophthalmoplegia. Here, we report the first, to our knowledge, case of bilateral painful ophthalmoplegia possibly caused by an aneurysm of the cavernous segment of the left internal carotid artery.
Case presentation
A 62-year-old male patient presented with headache and bilateral ptosis. Laboratory tests revealed hypopituitary function. Computerized tomography angiography showed a large aneurysm in the cavernous sinus segment of the left internal carotid artery. Aneurysm embolization was performed in the Nerve Interventional Department. Four months after surgery, the patient's symptoms returned to normal.
Conclusions
This case suggests that patients with bilateral painful ophthalmoplegia should be screened for aneurysms using computed tomography angiography or magnetic resonance angiography immediately.
Similar content being viewed by others
Background
Painful ophthalmoplegia, which involves periorbital or hemicranial pain with paralysis of ipsilateral extraocular motility, can have various causes, including trauma, neoplasms, vascular and inflammatory diseases [1]. Intracranial aneurysms are the most common vascular cause, which can compress the cranial nerves III-VI and cause retro-orbital pain, diplopia, ophthalmoplegia, trigeminal neuropathy, or a combination of the above [2]. Bilateral painful ophthalmoplegia is rare, and is mostly caused by infectious diseases, ophthalmoplegia migraine, cavernous sinus thrombosis, or intracranial hypotension [3,4,5,6]. Herein, we report the first case of bilateral painful ophthalmoplegia possibly caused by an aneurysm of the cavernous segment of the left internal carotid artery.
Case presentation
A 62-year-old male farmer was admitted to our hospital with a 2-month history of persistent headache and a 1-week history of ptosis. When the headache started, the patient received analgesic treatment, and the symptoms were relieved to some extent. One week prior to admission, the patient's headache worsened, and was accompanied by ptosis. Brain MRI at a local hospital indicated abnormal signals in the bilateral cavernous sinuses and adjacent meninges. Lumbar puncture showed normal opening pressure and slight leukocytosis (20×106/L reference range 0-8×106/L). Despite hormonal and symptomatic treatment, the patient's symptoms were not relieved. The patient was referred to our hospital for treatment. A previous medical history revealed diabetes for 2 months, with insulin prescribed. Physical examination on admission showed that the patient had paralysis of cranial nerves III, IV, and VI, which presented as bilateral ptosis and bilateral total ophthalmoplegia. He had bilateral exophthalmos and dilated pupils (5 mm) with sluggish pupillary reflexes. The bilateral near reflex and corneal reflex disappeared. The findings of other neurological examinations were normal. Laboratory tests revealed elevated C-reactive protein (60.95 mg/L reference range, 0-5 mg/L), erythrocyte sedimentation rate (37 mm/h reference range, 0-15 mm/h), and reduced luteinizing hormone (1.12mIU/mL reference range, 1.7-8.6 mIU/mL), prolactin (2 ng/mL reference range, 4.04-15.2 ng/mL), testosterone T (0.365 ng/mL reference range, 1.93-7.4 ng/mL), and thyrotropin (0.13 μIU/mL reference range, 0.56-5.91 μIU/mL). The serum myocardial enzyme levels; liver function results; renal function results; D-dimer and homocysteine levels; and autoimmunity markers including antinuclear antibody, anti-neutrophil, cytoplasmic, and rheumatoid factor were within the normal range, and the screening results for syphilis, hepatitis B, hepatitis C, and acquired immunodeficiency syndrome were all negative. Contrast-enhanced MRI showed a mass lesion in the left cavernous sinus (Fig.1A). Pituitary MRI revealed pituitary compression and a right shift of the pituitary stalk under compression (Fig.1B). CTA showed a large aneurysm in the cavernous sinus segment of the left internal carotid artery (approximately 14.2 × 11.7 mm) (Fig. 1C) and no thrombus in the aneurysm cavity (Fig.1D). He was then referred to the Department of Interventional Neurology for endovascular coiling. The aneurysm was embolized using multiple detachable coils. Intraoperative cerebral angiography showed a strip aneurysm of the cavernous sinus segment of the left internal carotid artery with an irregular shape and a size of approximately 17.19 mm × 8.33 mm × 7.82 mm. (Fig. 2). One week after surgery, headache improved significantly, but eye movement was still severely limited, manifested by inability to move in all directions. He still has left ptosis. Four months after the operation, the patient's headache and ptosis disappeared, and eye movements have completely returned to normal (Fig. 3). Due to the limited cooperation of the patient, only part of the eye movement examination images are shown.
Discussion and conclusion
We describe the rare case of bilateral painful ophthalmoplegia possibly due to a unilateral aneurysm. The cavernous (C4) segment of the internal carotid artery is located medially within the cavernous sinus and is surrounded by a number of intracranial nerves, which includes the oculomotor nerve, trochlear nerve, abducent nerve, and ophthalmic and maxillary branches of the trigeminal nerve [7]. Therefore, an aneurysm of the cavernous segment of the internal carotid artery can cause retro-ocular pain, neuro-ophthalmic signs and symptoms which include diplopia, ophthalmoplegia, trigeminal neuropathy and so on. In our case, based on the patient's symptoms, findings of imaging studies, and failure of hormone therapy, it was likely that the patient's bilateral painful ophthalmoplegia was caused by the cavernous segment aneurysm of the left internal carotid artery.
The exact mechanism of painful ophthalmoplegia caused by an ipsilateral intracranial aneurysm is unclear. The following mechanisms may be speculated: (1) direct compression of the aneurysm, (2) pulsating effect of the aneurysm, (3) perianeurysmal edema, and (4) perianeurysmal inflammation, which was caused by the chronic stimulation of the surrounding tissue by the aneurysm. This stimulation could be compression or it could be blood leaking out of the aneurysm wall [8,9,10,11]. In this case, the aneurysm was located in the cavernous segment of the left internal carotid artery, but the patient presented with bilateral painful ophthalmoplegia. So far, the mechanism of action is not clear. In addition, the mechanism of hypopituitarism may be due to direct mechanical compression of the pituitary or aneurysm compression of the superior pituitary artery, leading to pituitary ischemia [12]. Pituitary MRI suggested that the pituitary gland was compressed by the aneurysm, and the pituitary stalk shifted to the right, which corroborates theory.
In conclusion, this case suggests that an internal carotid cavernous segment aneurysm can present with bilateral painful ophthalmoplegia. Given the potentially fatal outcome of aneurysmal rupture [13], any patient who presents with bilateral painful ophthalmoplegia should be promptly screened using CTA or MRA.
Availability of data and materials
There are no associated datasets for this manuscript. All data generated or analyzed during this study are included in this published article. Related queries can be directed to the corresponding author.
Abbreviations
- CTA:
-
Computed tomography angiography
- MRA:
-
Magnetic resonance angiography
- MRI:
-
Magnetic resonance imaging
References
Mendonça M, et al. Steroid responsive painful ophthalmoplegia: Tolosa Hunt syndrome, Eales disease, or both? Cephalalgia. 2017;37(2):191–4.
van Rooij WJ. Endovascular treatment of cavernous sinus aneurysms. AJNR Am J Neuroradiol. 2012;33(2):323–6.
Anton-Vazquez V, Dru R, Rich P, Arias M, Macallan D. Images of the month: Cavernous sinus venous thrombosis secondary to Streptococcus milleri maxillary sinusitis: An unusual cause of diplopia and headache. Clin Med (London, England). 2020;20(6):e271–2.
Numao A, Suzuki K, Watanabe Y, Izawa N, Nakamura T, Iwanami H, et al. [A 56-year-old woman with adult-onset ophthalmoplegic migraine presenting with recurrent bilateral abducens nerve palsy]. Rinsho shinkeigaku = Clin Neurol. 2012;52(4):239–44.
Takasuna H, Sasaki R, Shiraishi M, Doi M, Wakui D, Ito H, et al. Steroid-resistant Tolosa-Hunt syndrome with a de novo intracavernous aneurysm: A case report. Surg Neurol Int. 2016;7(Suppl 30):S779-s84.
Vahdani K, McVeigh K, Harrison R, Williams M, Garrott H. Intracranial hypotension mimicking chronic progressive external ophthalmoplegia. Orbit (Amsterdam, Netherlands). 2018;37(5):371–4.
Qu H, Li Y, Chen M, Sun H, Du S, Cheng Y, et al. Cavernous sinus thrombosis: An insidious and dangerous “do-not-miss” diagnosis. Headache. 2021;61(7):1144–9.
Puffer R, Piano M, Lanzino G, Valvassori L, Kallmes D, Quilici L, et al. Treatment of cavernous sinus aneurysms with flow diversion: results in 44 patients. AJNR Am J Neuroradiol. 2014;35(5):948–51.
Nam KH, Choi CH, Lee JI, Ko JG, Lee TH, Lee SW. Unruptured Intracranial Aneurysms with Oculomotor Nerve Palsy: Clinical Outcome between Surgical Clipping and Coil Embolization. J Korean Neurosurg Soc. 2010;48(2):109–14.
Raza H, Chen H, Chansysouphanthong T, Cui G. The aetiologies of the unilateral oculomotor nerve palsy: a review of the literature. Somatosens Motor Res. 2018;35:229–39.
Hyland HH, Barnett HJ. The pathogenesis of cranial nerve palsies associated with intracranial aneurysms. Proc R Soc Med. 1954;47(2):141–6.
Tan L, Sandler V, Todorova-Koteva K, Levine L, Lopes D, Moftakhar R. Recovery of pituitary function following treatment of an unruptured giant cavernous carotid aneurysm using Surpass flow-diverting stents. J Neurointerventional Surg. 2015;7(6):e20.
Khalsa SS, Hollon TC, Shastri R, Trobe JD, Gemmete JJ, Pandey AS. Spontaneous subarachnoid hemorrhage due to ruptured cavernous internal carotid artery aneurysm after medical prolactinoma treatment. J Neurointerventional Surg. 2017;9(3):e9.
Acknowledgements
The authors are grateful to the patient and his family for their support and cooperation.
Funding
This study was supported by the National Natural Science Foundation of China (81971214) and the Outstanding Young Talent Cultivation Project of Henan Science and Technology Innovation Talents (YXKC2022037).
Author information
Authors and Affiliations
Contributions
XNC contributed to the conception and design of the manuscript. ZWW and XYY collected the data and drafted the manuscript. ZJW, WC, LYJ and LHB reviewed and modified the manuscript. All authors contributed to manuscript revision, read, and approved the submitted version.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
Protocols were established, according to the ethical guidelines of the Helsinki Declaration and was approved by the Independent Ethics committee, The First Affiliated Hospital of Zhengzhou University. Written informed consent was obtained from the patient.
Consent to publication
Written informed consent regarding the submission and potential publication of this manuscript was obtained from the patient. Additionally, consent for treatment was likewise obtained in the usual fashion during the course of the patient’s hospitalization.
Competing interests
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Zhang, W., Xie, Y., Zhang, J. et al. Single internal carotid cavernous sinus aneurysm presented as bilateral painful ophthalmoplegia: a case report. BMC Neurol 23, 23 (2023). https://doi.org/10.1186/s12883-023-03066-0
Received:
Accepted:
Published:
DOI: https://doi.org/10.1186/s12883-023-03066-0