Chronic persistent Horner’s syndrome in trigeminal autonomic cephalalgia subtypes and alleviation with treatment: two case reports

• Main Authors: Todd D. Rozen, Matthew T. Kline
• Format: Article
• Language: English
• Published: BMC 2019-03-01
• Series: Journal of Medical Case Reports
• Subjects: Trigeminal autonomic cephalalgia; Horner’s syndrome; Ptosis; Hemicrania continua; LASH syndrome
• Online Access: http://link.springer.com/article/10.1186/s13256-019-1986-y

Abstract Background The trigeminal autonomic cephalalgias are a group of primary headache syndromes marked by severe head pain and associated cranial autonomic symptoms which can include a full or partial Horner’s syndrome. Rarely, the eye-related symptoms will become fixed even between headache attacks. There is minimal documentation that the Horner’s syndrome can be reversed if successful treatment of the underlying headache disorder is initiated. Case reports Two cases are presented of trigeminal autonomic cephalalgia subtypes with chronic persistent Horner’s syndromes that alleviated with treatment of the underlying primary headache disorder. Patient 1, an 82-year-old Caucasian woman, presented with hemicrania continua with a partial Horner’s syndrome that was present for 2 years. She was unable to take indomethacin as she was on anticoagulation. After a C2–3 diagnostic facet injection, not only did she become pain free but her ptosis completely resolved. She then underwent a radiofrequency facet neurotomy with complete alleviation of head pain and complete resolution of her ptosis. Patient 2, a 21-year-old Caucasian woman, presented with long-lasting autonomic symptoms with hemicrania syndrome and a fixed miosis and ptosis of 6 months’ duration. After achieving 2 months of pain freedom on indomethacin her Horner’s syndrome completely resolved. Conclusion A chronic fixed partial or full Horner’s syndrome can occur in trigeminal autonomic cephalalgia subtypes, but it can also be reversed in patients with treatment even after months to years of duration. This would suggest that the sympathetic dysfunction leading to the eye-related symptoms is from irritation of the sympathetic chain rather than permanent injury as the result of vasodilatory trauma after trigeminal autonomic reflex activation.