Horner Syndrome as the Only Focal Neurologic Manifestation of Hypothalamic Hemorrhage

A 70-year-old woman suffered an anterior dorsal hypothalamic hemorrhage that caused an ipsilateral Horner syndrome (HS) as the only focal neurologic manifestation. This is only the second reported case of hypothalamic hemorrhage producing HS. Because HS was the sole focal neurologic manifestation, its confirmation with topical apraclonidine drops was a valuable clue toward prompt localization of the patient's confusional state.

Photo Essay Section Editor: Timothy J. McCulley, MD Horner Syndrome as the Only Focal Neurologic Manifestation of Hypothalamic Hemorrhage Cansu B. Sahin, MD, Neeraj Chaudhary, MD, Jonathan D. Trobe, MD FIG. 1. Coronal (A) and axial (B) precontrast CT, and axial fluid-attenuated inversion recovery MRI (C) performed on the day of the fall shows hemorrhage in the right anterior hypothalamus with blood products in the third ventricle. Abstract: A 70-year-old woman suffered an anterior dorsal hypothalamic hemorrhage that caused an ipsilateral Horner syndrome (HS) as the only focal neurologic manifestation. This is only the second reported case of hypothalamic hemorrhage producing HS. Because HS was the sole focal neurologic manifestation, its confirmation with topical apraclonidine drops was a valuable clue toward prompt localization of the patient's confusional state. Journal of Neuro-Ophthalmology 2018;38:192-194 doi: 10.1097/WNO.0000000000000604 © 2017 by North American Neuro-Ophthalmology Society A 70-year-old woman seemed confused to her husband and fell in the bathroom minutes later. Several weeks earlier, she had been diagnosed with a herpes zoster meningoencephalitis and treated with intravenous acyclovir with nearly complete recovery of mental status. Departments of Ophthalmology, Neurology, and Radiology (Neuroradiology), Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan. The authors report no conflicts of interest. Address correspondence to Jonathan D. Trobe, MD, University of Michigan, Ann Arbor, MI; E-mail: [e-mail] 192 Brain CT and MRI performed on the day of the fall revealed an acute intracranial hemorrhage centered in the right anterior hypothalamus with extension into the third ventricle (Fig. 1). Head and neck CT angiography was negative. The hemorrhage was attributed to to an underlying developmental venous anomaly identified on a brain MRI performed 1 month earlier (Fig. 2). The patient had been examined by an otolaryngologist for dizziness 3 days previously without any mention of ptosis or anisocoria. The patient's husband had noted right upper lid ptosis for the first time on the day of presentation. Twenty-four hours after the fall, the patient was confused and slightly somnolent with anisocoria and mild right upper lid ptosis. In darkness, pupils measured 2.5 mm in the right eye and 4 mm in the left eye, both constricting briskly and equally to the direct light of a flashlight held at about 1 foot from the eyes. All other aspects of the ophthalmic and neurologic examinations were normal. Thirty minutes after topical instillation of 2 drops of apraclonidine 0.5% in each eye, the right pupil measured 5.5 mm and the left pupil still measured 4 mm in darkness. Apraclonidine also elevated the right upper lid. It had no effect on the lid position of the left eye. Sahin et al: J Neuro-Ophthalmol 2018; 38: 192-194 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Axial fluid-attenuated inversion recovery MRI performed 1 month prior to the patient's presentation with Horner syndrome caused by thalamic hemorrhage. It shows an anterior thalamic developmental venous anomaly without hemorrhage. This is the second reported case of reversal of anisocoria after topical instillation of apraclonidine in a patient with a Horner syndrome (HS) caused by a hypothalamic hemorrhage. Kauh and Bursztyn (1) reported a patient with HS from a thalamic hemorrhage that was confirmed by a positive topical apraclonidine 0.5% test performed 96 hours after symptoms developed. Our case had a positive apraclonidine test within 24 hours after symptom onset. Until this report, the shortest reported latency between symptom onset in a first-order (brainstem) HS and a positive apraclonidine test was 3 days. It involved a patient with a dorsolateral pontomedullary infarction (2). We acknowledge that we cannot be certain about the latency from the onset of the HS to the positive apraclonidine test. However, we assume that the thalamic hemorrhage which caused the HS occurred at about the time of the patient's fall, which was when her husband noted right ptosis. The thalamic hemorrhage in our patient was located dorsally and anteriorly in the hypothalamus, corresponding to the anatomic location of the paraventricular nucleus, which controls sympathetic function (3). Although HS in thalamic hemorrhage has not been frequently described, HS with hypothalamic infarction has been well documented (4-8). In none of the reported cases was pharmacologic confirmation of HS provided. In those reported cases, the infarction also involved the Sahin et al: J Neuro-Ophthalmol 2018; 38: 192-194 overlying thalamus and extended ventrally to damage the paraventricular nucleus. Infarction was likely caused by occlusion of a proximal branch of the posterior cerebral artery. In previously reported cases of HS in hypothalamic infarction, the HS has never been the only (or even the most prominent) neurologic abnormality, usually overshadowed by lowered consciousness, ataxia, weakness, or aphasia. Our case, which was hemorrhagic rather than infarctive, is distinctive because HS, confirmed with topical apraclonidine drops, stood out as the only focal abnormality in a patient with a mild confusional and somnolent state. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: C. B. Sahin, N. Chaudhary, and J. D. Trobe; b. Acquisition of data: C. B. Sahin, N. Chaudhary, and J. D. Trobe; and c. Analysis and interpretation of data: C. B. Sahin, N. Chaudhary, and J. D. Trobe. Category 2: a. Drafting the manuscript: C. B. Sahin, N. Chaudhary, and J. D. Trobe and b. Revising it for intellectual content: J. D. Trobe. Category 3: a. Final approval of the completed manuscript: C. B. Sahin, N. Chaudhary, and J. D. Trobe. REFERENCES 1. Kauh CY, Bursztyn L. Positive apraclonidine test in Horner syndrome caused by thalamic hemorrhage. J Neuroophthalmol. 2015;35:287-288. 193 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay 2. Lebas M, Seror J, Debrouker T. Positive apraclonidine test 36 hours after acute onset of Horner syndrome in dorsolateral pontomedullary stroke. J Neuroophthalmol. 2010;30:12-17. 3. Martin JH. The Hypothalamus and Regulation of Bodily Functions. Neuroanatomy Text Book and Atlas. 4th edition. New York, NY: McGraw-Hill, 2012:365. 4. Rossetti AO, Reichhart MD, Bogousslavsky J. 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