What is the significance of pain in the evaluation of Horner syndrome?

Updated: May 01, 2019
  • Author: Christopher M Bardorf, MD, MS; Chief Editor: Edsel Ing, MD, MPH, FRCSC  more...
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Horner syndrome in the presence of pain merits special consideration.

Horner syndrome in the presence of axial, shoulder, scapula, arm, or hand pain may be indicative of compression by an apical lung tumor (Pancoast tumor).

Horner syndrome in the presence of acute-onset, ipsilateral facial or neck pain may indicate carotid artery dissection, which may be caused by cardiovascular disease, arteriopathy (eg, fibromuscular dysplasia or collagen disorders), or trauma (even minor trauma, such as results from quick head turns). If carotid artery dissection is suspected, especially if there are signs or symptoms of retinal ischemia, urgent neuroimaging studies (magnetic resonance imaging or magnetic resonance angiography) should be obtained along with neurologic consultation.

Postganglionic Horner syndrome associated with ipsilateral headache has several causes. Patients with spontaneous carotid artery dissection may present with Horner syndrome and ipsilateral headache. Patients with cluster headaches may develop ipsilateral Horner syndrome during an acute attack.

The term Raeder paratrigeminal syndrome is applied to patients, usually middle-aged males, who have Horner syndrome and daily unilateral head pain. In the original Raeder syndrome, the pain is trigeminal pain associated with hypoesthesia or anesthesia in the distribution of the trigeminal nerve (cranial nerve [CN] V). Pain related to Raeder syndrome can be distinguished from that related to cluster headaches or carotid disease in that the latter conditions occur without impairment of trigeminal nerve function.

Horner syndrome may be the first manifestation of neuroblastoma.

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