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Sudden-Onset Headache and Binocular Vertical Diplopia in a Young Patient

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 24-year-old woman presented with a 3-week history of sudden onset of headache, nausea, and binocular vertical diplopia. She denied any other neurologic symptoms. Her medical history included untreated hyperprolactinemia manifested by galactorrhea and oligomenorrhea. She did not take any medications, smoke, or drink alcohol.

On examination, visual acuity was 20/20 in each eye. There was no relative afferent pupillary defect. There was minimal limitation in infraduction in the left eye with corresponding left hypertropia (Video), which was worse in left gaze and right head tilt. Ophthalmoscopy findings were normal in each eye. Visual field findings (Humphrey 24-2 algorithm) were normal in each eye as well.

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Pituitary apoplexy

C. Order urgent computed tomography scan and computed tomography angiography of the brain

The differential diagnosis for sudden onset of headache, nausea, and binocular vertical diplopia in a young patient is limited. The presence of left infraduction limitation and corresponding left hypertropia that does not fit the pattern for fourth nerve palsy could be because of partial third cranial nerve palsy. The relevant medical history of hyperprolactinemia makes pituitary apoplexy (ie, sudden hemorrhage within the pituitary gland, often within preexisting pituitary adenoma) and aneurysmal compression of the third nerve the most critical diagnoses to be ruled out. Thus, ordering an urgent computed tomography scan and computed tomography angiography scan of the brain with attention to the sellar region (choice C) would be the most appropriate course of action. Checking thyrotropin and thyroid autoantibody levels and ordering orbital imaging (choice A) would be correct if thyroid eye disease was the suspected culprit. However, sudden onset of headache and nausea is not a typical presentation of thyroid eye disease, making this diagnosis unlikely. While myasthenia gravis can present with vertical binocular diplopia, it would also not explain the sudden onset of headaches and nausea, and other likely signs of myasthenia gravis (eg, orbicularis oculi weakness and variable ptosis fatigable in sustained upgaze) were not present. Thus, ordering acetylcholine receptor antibody level titers (choice B) would not be the most important investigation. Lumbar puncture (choice D) would be the best test to order if subarachnoid hemorrhage were suspected as causing sudden onset of headache and nausea. However, computed tomography scan and computed tomography angiogram imaging are sensitive for identifying aneurysms and detecting blood in the subarachnoid space; thus it should be performed first.

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Article Information

Published Online: April 7, 2022. doi:10.1001/jamaophthalmol.2021.6165

Correction: This article was corrected on June 1, 2022, to fix the spelling of the second author’s surname.

Corresponding Author: Edward Margolin, MD, Department of Ophthalmology and Visual Sciences, Division of Neurology, Department of Medicine, University of Toronto, 801 Eglinton Ave West, Ste 301, Toronto, ON M5N 1E3, Canada (edmargolin@gmail.com).

Conflict of Interest Disclosures: None reported.

Reference
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Briet  C , Salenave  S , Bonneville  JF , Laws  ER , Chanson  P .  Pituitary apoplexy.   Endocr Rev. 2015;36(6):622-645. doi:10.1210/er.2015-1042 PubMedGoogle ScholarCrossref
2.
Rajasekaran  S , Vanderpump  M , Baldeweg  S ,  et al.  UK guidelines for the management of pituitary apoplexy.   Clin Endocrinol (Oxf). 2011;74(1):9-20. doi:10.1111/j.1365-2265.2010.03913.x PubMedGoogle ScholarCrossref
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Jenkins  TM , Toosy  AT . Visual acuity, eye movements and visual fields. In Turgut  M , Mahapatra  AK , Powell  M , Muthukumar  N , eds.  Pituitary Apoplexy. Springer, 2014: 75-88.
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Marx  C , Rabilloud  M , Borson Chazot  F , Tilikete  C , Jouanneau  E , Raverot  G .  A key role for conservative treatment in the management of pituitary apoplexy.   Endocrine. 2021;71(1):168-177. doi:10.1007/s12020-020-02499-8 PubMedGoogle ScholarCrossref
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Mou  C , Han  T , Zhao  H , Wang  S , Qu  Y .  Clinical features and immunohistochemical changes of pituitary apoplexy.   J Clin Neurosci. 2009;16(1):64-68. doi:10.1016/j.jocn.2008.02.012 PubMedGoogle ScholarCrossref
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Randeva  HS , Schoebel  J , Byrne  J , Esiri  M , Adams  CB , Wass  JA .  Classical pituitary apoplexy: clinical features, management and outcome.   Clin Endocrinol (Oxf). 1999;51(2):181-188. doi:10.1046/j.1365-2265.1999.00754.x PubMedGoogle ScholarCrossref
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