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

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.

Microsurgical Anatomy of the Hypoglossal and C1 Nerves: Description of a Previously Undescribed Branch to the Atlanto-Occipital Joint.

OBJECTIVE: Distal branches of the C1 nerve that travel with the hypoglossal nerve have been well investigated but relationships of C1 and the hypoglossal nerve near the skull base have not been described in detail. Therefore, the aim of this study was to investigate these small branches of the hypoglossal and first cervical nerves by anatomic dissection. METHODS: Twelve sides from 6 cadaveric specimens were used in this study. To elucidate the relationship among the hypoglossal, vagus, and first and cervical nerve, the mandible was removed and these nerves were dissected under the surgical microscope. RESULTS: A small branch was found to always arise from the dorsal aspect of the hypoglossal nerve at the level of the transverse process of the atlas and joined small branches from the first and second cervical nerves. The hypoglossal and C1 nerves formed a nerve plexus, which gave rise to branches to the rectus capitis anterior and rectus capitis lateralis muscles and the atlanto-occipital joint. CONCLUSIONS: Improved knowledge of such articular branches might aid in the diagnosis and treatment of patients with pain derived from the atlanto-occipital joint. We believe this to be the first description of a branch of the hypoglossal nerve being involved in the innervation of this joint.

Treatment of hemicrania continua with radiofrequency ablation and long-term follow-up.

OBJECTIVE: The objective of this research is to describe novel procedural treatments for hemicrania continua that allow patients to remain off indomethacin. METHODS: Case reports are presented. RESULTS: We describe four distinct patients with indomethacin-responsive hemicrania continua who were unable to discontinue the use of indomethacin without headache recurrence. No other medications were effective for their syndrome. Secondary causes of headache were ruled out in each case. Each patient underwent diagnostic blockade of either the atlanto-axial joint, C2 dorsal root ganglion or sphenopalantine ganglion depending on their clinical examination and presence of cranial autonomic symptoms. A positive response led to a radiofrequency ablation of the C2 ventral ramus, C2 dorsal root ganglion or sphenopalantine ganglion, which provided headache relief in all case patients as complete as indomethacin. Long-term follow-up of these patients has shown that all have remained essentially headache free without the need for indomethacin. One patient has needed repeat radiofrequency procedures with consistent response. CONCLUSION: Hemicrania continua is defined by its sensitivity to indomethacin but very few patients are able to discontinue the medication without headache recurrence. As the risks of chronic indomethacin use are substantial, alternative treatments are necessary to protect patient health. We are now able to suggest several radiofrequency ablation procedures as effective as indomethacin with long-term follow-up.