Great Auricular Neuralgia: Case Series.

BACKGROUND/OBJECTIVE: The great auricular nerve (GAN) arises from C2-C3 and provides innervation over the skin in the pre-auricular region, jaw angle, posteroinferior pinna, and mastoid. Although damage to the GAN has been reported following trauma or procedures nearby this nerve course, neuralgia of this nerve is uncommon with knowledge based on a handful of case reports in literature. The objective of this study is to describe the presentation, treatment, and outcome of 13 cases of GAN neuralgia. METHODS: Case series. Retrospecive review of charts from 1994 to 2018 with diagnoses: "auricular neuralgia," "auricular neuritis," or "auricular neuropathy." We included subjects with neuralgic pain within the distribution of the GAN, and excluded patients with atypical facial pain, GAN neuropathy, or unclear etiology. RESULTS: Of 79 charts, 13 patients met criteria (age at onset 11-59; 11 women, 2 men). Pain was most often described as paroxysmal stabbing provoked by: turning the head (n = 7), touching the neck (n = 5), neck position during sleep (n = 2), jaw movement (n = 2), and other (n = 2). Seven patients received GAN blocks: all noted dramatic improvement in pain, including 3 who continued to receive serial blocks at our institution successfully for the next 2 to 5 years. Two patients successfully transitioned from GAN blocks to GAN stimulators. One patient with GAN lymphoma had resolution of pain following GAN resection. CONCLUSION: GAN neuralgia should be considered in the differential for periauricular pain. GAN blocks or stimulators may be helpful for pain management.

Headache due to spontaneous spinal cerebrospinal fluid leak secondary to cerebrospinal fluid-venous fistula: Case series.

OBJECTIVE: Cerebrospinal fluid-venous fistula is an uncommon cause of spontaneous spinal cerebrospinal fluid leak (SSCSFL). We aim to describe the clinical presentation, imaging evaluation, treatment and outcome of SSCSFL secondary to cerebrospinal fluid-venous fistula. METHODS: A retrospective review was undertaken of SSCSFL cases secondary to cerebrospinal fluid-venous fistula confirmed radiologically or intraoperatively, seen at our institution from January 1994 to March 2019. Cases with undetermined SSCSFL etiology, alternative etiology or unconfirmed fistula were excluded. RESULTS: Forty-four of 156 patients met the inclusion criteria (31 women, 13 men). Mean age of symptom onset was 52.6 years (SD 8.7, range 33-71 years). Headache was the presenting symptom in almost all, typically daily (69%), and most often in occipital/suboccipital regions. Headache character was most commonly pressure (38%), followed by throbbing/pulsing (21.4%). Orthostatic headache worsening occurred in 69% and an even greater percentage of patients (88%) reported Valsalva-induced headache exacerbation or precipitation. Headache occurred in isolation to Valsalva maneuvers in 12%. Of 37 patients with documented cerebrospinal fluid opening pressure, 13% were <6 cmH2O; 84%, 7-20 cmH2O; and one, 25 cmH2O. Fistulas were almost exclusively thoracic (95.5%). Only one patient responded definitively to epidural blood patch (EBP). Forty-two patients underwent surgery. Most improved following surgery; 48.7% were completely headache free and 26.8% had at least 50% improvement. CONCLUSION: In our series, cerebrospinal fluid-venous fistula was associated with a greater occurrence of Valsalva-induced headache exacerbation or precipitation than orthostatic headache and did not respond to EBP. Surgery provided significant improvement. Cerebrospinal fluid-venous fistula should be considered early in the differential diagnosis of Valsalva-induced ("cough") headache.

Headache Attributed to Autonomic Dysreflexia: Clinical Presentation, Pathophysiology, and Treatment.

A patient presenting with marked elevation in blood pressure and concurrent headache often presents a diagnostic challenge for even the most seasoned clinician. When marked hypertension and headache occur in a patient with a history of upper spinal cord injury, the patient should be presumed to have autonomic dysreflexia until proven otherwise. Autonomic dysreflexia can at times trigger headaches, hypertension, and variations in pulse, as well other autonomic signs and symptoms. Autonomic dysreflexia is a medical emergency for which appropriate treatment may be life-saving. In this review, we address the historical origins, risk factors, pathophysiology, diagnostic criteria, clinical presentation, differential diagnosis, and treatment of headache attributed to autonomic dysreflexia. Included are two case presentations from the authors' clinic, which illustrate the diagnosis and treatment of headache attributed to autonomic dysreflexia.