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.
Background/ObjectiveThe 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.MethodsCase 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.ResultsOf 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.ConclusionGAN neuralgia should be considered in the differential for periauricular pain. GAN blocks or stimulators may be helpful for pain management.
A 47-year-old man presented with right-sided facial pain that started 2 years prior. He described the pain as extremely intense, stabbing along the right jaw, lasting 5-60 seconds. This pain was exacerbated by chewing, and to a lesser degree, by brushing his teeth. The pain was so intense that he avoided eating when possible, leading to a 20-pound weight loss. When he did eat, he would try to chew on the left side of his mouth. Around the onset of these symptoms, he also noticed a persistent numbness and burning extending from the right lower earlobe to the lateral angle of the jaw that was exacerbated by turning his head to the right. The patient was given a diagnosis of atypical trigeminal neuralgia (TN) and sent to our headache clinic for further management. Questions for consideration: 1. What features are typical and atypical for classical TN? 2. What is your differential diagnosis in this patient presenting with facial pain? GO TO SECTION 2
A 66-year-old man with no headache history presented with a new daily and persistent headache that began acutely 1 year ago without any clear provoking features. The pain was mild, constant holocephalic pressure with associated photophobia and phonophobia. He denied nausea/vomiting or migraine aura-like symptoms. Previously failed preventive migraine medications included onabotulinumtoxinA injections, nortriptyline, gabapentin, and metoprolol. The patient's neurologic examination including funduscopic evaluation was normal. Questions for consideration:1. What is your differential diagnosis of new daily and persistent headache? 2. What additional clinical or examination features would help narrow the differential?GO TO SECTION 2
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