Valsalva-induced Headaches Research Articles

Cerebral Venous Sinus Pressure and Head Pain: A Case of Cough Headache Secondary to Superior Sagittal Sinus Stenosis (P3-12.009)

<h3>Objective:</h3> Headache triggered by cough is an uncommon disorder that can be primary or secondary in etiology. While the pathophysiology of cough headache is poorly described, there are several theories that suggest that an interplay of elevated intracranial pressure (ICP) and increased central venous pressure results in nociceptor activation with cough. We present a case of secondary cough headache resulting from sagittal sinus stenosis. <h3>Background:</h3> A 31-year old female with a history of migraine presented with a new headache described as 2–5 minute spikes of severe head pain triggered by cough or Valsalva. MRI brain imaging showed a 1.5 cm left para-falcine extra-axial mass involving the lateral wall of the sagittal sinus, most consistent with a meningioma. This finding was initially thought to be unrelated to her head pain. The patient’s head pain remained refractory to typical migraine treatments; however, it responded well to indomethacin and acetazolamide. Due to intolerable side effects, acetazolamide was tapered off and cough-induced head pain recurred. Venous manometry revealed a significant pressure gradient across the sagittal sinus stenosis. Her symptoms significantly improved with meningioma resection and superior sagittal venous stenting. <h3>Design/Methods:</h3> NA <h3>Results:</h3> NA <h3>Conclusions:</h3> This a rare presentation of cough headache secondary to superior sinus stenosis, which significantly improved on correction of increased venous pressure. This case highlights the role of pressure dynamics within the cerebral venous sinus system not only in the etiology of primary and secondary cough and other Valsalva-induced headaches, but also as a potential etiology in patients with idiopathic interracial hypertension with venous sinus stenosis. This case also suggests that venous stenting can be an alternative to acetazolamide. Prompt recognition of secondary causes of cough headache is essential for early intervention. <b>Disclosure:</b> Ms. Haddad has nothing to disclose. Dr. Wang has nothing to disclose. Dr. Parikh has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biohaven. Dr. Parikh has received personal compensation in the range of $500-$4,999 for serving as a Author with Medlink.

Acquired Chiari Type I Malformation Associated with Type IV Dural Arteriovenous Fistula: Case Report.

Chiari malformations, usually congenital, can rarely be associated with arteriovenous (AV) fistulas. We present the first case involving a type IV dural AV fistula with a Chiari type I malformation. Retrospective chart review was performed to obtain pertinent details regarding history and examination, pathologic findings, and treatment course. A 63-year-old woman with a 2-year history of migraines presented with 5 months of occipital, right-sided headaches and neck pain exacerbated by Valsalva maneuvers. Computed tomography (CT) and magnetic resonance imaging (MRI) of the head showed a possible right occipital AV malformation, bilateral cerebellar subdural hygromas, and tonsillar crowding at the foramen magnum indicating an acquired Chiari type I malformation. Angiography demonstrated a Cognardtype IV right posterior occipital dural AV fistula supplied by bilateral middle meningeal and posterior meningeal arteries. After treatment of the dural AV fistula, hygroma evacuation, and decompression of the acquired Chiari malformation, the patient's Valsalva-induced headaches abated.

Cervical cerebrospinal fluid venous fistula with syringomyelia treated with suboccipital decompression: illustrative case.

BACKGROUNDCerebrospinal fluid (CSF) venous fistulas are a recently discovered and underdiagnosed cause of spontaneous spinal CSF leak, which may lead to spontaneous intracranial hypotension. Most cases occur in the thoracic spine, and only 2 cases were reported in the cervical spine. Treatments include the epidural blood patch, fibrin glue injection, and surgical ligation of the fistula.OBSERVATIONSThe authors report the treatment of a C6–7 CSF venous fistula, for which direct ligation was not feasible, with suboccipital decompression, leading to the complete resolution of the symptoms. Based on the clinical course and outcome in our patient, the authors summarize the previous theory and propose a hypothesis for the pathophysiology of headache and other symptoms in patients with CSF venous fistulas.LESSONSThe symptoms of CSF venous fistulas may be linked not only to intracranial hypotension but also to the altered CSF dynamics induced by tonsillar herniation. Suboccipital decompression should be considered as a potential treatment option, especially in patients with Valsalva-induced headache who show a poor response to surgical ligation, patients in whom surgical ligation is not feasible, and patients with foramen magnum obstruction. Further investigation of the pathophysiology of CSF venous fistulas is warranted and should be performed in the future.

Cognitive and Psychological Functioning in Chiari Malformation Type I Before and After Surgical Decompression - A Prospective Cohort Study.

Chiari Malformation Type I (CM-I) is defined as cerebellar tonsil displacement more than 5mm below the foramen magnum. This displacement can alter cerebrospinal fluid flow at the cervicomedullary junction resulting in Valsalva-induced headaches and syringomyelia and compress the brainstem resulting in bulbar symptoms. However, little is known about cognitive and psychological changes in CM-I. To prospectively assess cognitive and psychological performance in CM-I and determine whether changes occur after surgical decompression. Blinded evaluators assessed symptomatic CM-I patients ages ≥18 with a battery of neuropsychological and psychological tests. Testing was conducted preoperatively and 6 to 18 mo postoperatively. Data were converted to Z-scores based on normative data, and t-tests were used to analyze pre-post changes. A total of 26 patients were included, with 19 completing both pre- and post-op cognitive assessments. All patients had resolution of Valsalva-induced headaches and there was improvement in swallowing dysfunction (P<.0001), ataxia (P=.008), and sleep apnea (P=.021). Baseline performances in visual perception and construction (z = -1.11, P=.001) and visuospatial memory (z = -0.93, P=.002) were below average. Pre-post comparisons showed that CM-I patients had stable cognitive and psychological functioning after surgery, without significant changes from preoperative levels. CM-I patients had below average performance in visuospatial and visuoconstructional abilities preoperatively. Prospective longitudinal data following surgery demonstrated improved neurologic status without any decline in cognition or psychological functioning. Routine pre- and postoperative formal neuropsychological assessment in CM-I patients help quantify cognitive and behavioral changes associated with surgical decompression.

Symptom outcome after craniovertebral decompression for Chiari type 1 malformation without syringomyelia.

Specific symptom outcomes after craniovertebral decompression for patients with Chiari type 1 malformation, without accompanying syringomyelia, are not well characterised and poorly reported. We performed a retrospective review of all patients diagnosed with Chiari type 1, without syringomyelia, who underwent craniovertebral decompression in our unit. We identified 129 individuals with a minimum of 2 years' follow-up. The most common pre-operative symptoms were pressure dissociation headaches (78%), visual disturbances (33%), dizziness/balance disturbances (24%) and blackouts (17%). The symptoms most likely to respond to surgery included Valsalva-induced headache (74% response, p < 0.0001) and blackouts (86% response, p < 0.001). After successful craniovertebral decompression, most patients presenting with pressure dissociation headaches and blackouts will improve. However, the large variety of other symptoms patients often present with may not improve after surgery.

Endoscopic Endonasal Odontoidectomy Preserving Atlantoaxial Stability: a Pediatric Case.

Objectives We illustrate endoscopic endonasal odontoidectomy for the Chiari-I malformation respecting craniovertebral junction (CVJ) stability. Design Case report of a 12-year-old girl affected by the Chiari-I malformation. Magnetic resonance imaging (MRI) showed tonsillar herniation, basilar invagination, and dental retroversion, causing angulation and compression of the bulbomedullary junction. Patient underwent endoscopic third ventriculostomy (ETV) with reduction of ventricular size and resolution of gait disturbances, but she complained the Valsalva-induced headaches, hiccup, and dysesthesias in the lower limbs. Endoscopic endonasal odontoidectomy was chosen to decompress the cervicomedullary junction. Setting The research was conducted at University Hospital “Ospedale di Circolo,” Department of Neurosurgery at Varese in Italy. Participants Patients were from neurosurgical and ENT (ear, nose, and throat) skull base team. Main Outcome Measures A bilateral paraseptal approach was performed, using a four-hand technique. After resection of posterior edge of the nasal septum, the choana is entered and a rhinopharynx muscle–mucosal flap is dissected subperiosteal and transposed in oral cavity. The CVJ is exposed and, using neuronavigation and neuromonitoring, odontoidectomy is fulfilled until dura is reached, preserving the anterior arch of C1. Reconstruction is obtained suturing the flap previously harvested. Results Postoperative course was unremarkable and the patient experienced improvement of symptoms. Postoperative MRI documented the appearance of tight cerebrospinal fluid (CSF) film anterior to bulbomedullary junction and in retrotonsillar spaces, opening of the bulbomedullary angle, and slight tonsils reduction. No CVJ instability was occurred with any need of posterior fixation. Conclusion Endoscopic endonasal odontoidectomy is a feasible approach for CVJ malformation. In this case, bulbar decompression was achieved preserving CVJ stability and avoiding posterior fixation. The link to the video can be found at: https://youtu.be/VIobocHfCuc .

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

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. 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. 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. 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.

Are evoked potentials clinically useful in the study of patients with Chiari malformation Type 1?

OBJECTIVE In this study, the authors describe the brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SSEP) alterations found in a large cohort of patients with Chiari malformation Type 1 (CM-1), the relationship between the BAEPs/SSEPs and the clinical findings, the abnormalities in patients with associated syringomyelia, and the clinical and neuroradiological risk factors that are associated with abnormal evoked potentials (EPs). METHODS A prospectively collected database containing 545 patients with CM-1 was queried to search for patients satisfying the following criteria: 1) an age of at least 14 years, 2) neuroradiological criteria of CM-1, 3) no prior Chiari-related surgeries, and 4) preoperative EP studies conducted at the authors' institution. The 200 patients included in this cohort were classified into CM-0, CM-1, and CM-1.5 subtypes. Linear, planimetric, and angular measurements of the posterior fossa were conducted, as well as syringomyelia measurements. Two separate multiple logistic regression models were used, one to predict the covariates associated with abnormal BAEPs, and a second model to explore the variables associated with an abnormal SSEP. In these models, the BAEPs and SSEPs were dichotomized as being normal or abnormal. RESULTS Headaches were the main symptom in 70.5% of the patients, and Valsalva-induced headaches were most frequent in patients with CM-1 and CM-1.5 compared with patients with CM-0 (p = 0.031). BAEPs were abnormal in 38.5% of patients, and abnormal SSEPs were found in 43.5% of the entire cohort. Syringomyelia was most frequent in patients with CM-0 (64.3%) and CM-1 (51.1%) compared with those with CM-1.5 (34.7%; p = 0.03). Age (OR 1.03, 95% CI 1.00-1.06), the degree of tonsillar herniation (OR 1.08, 95% CI 1.01-1.16), and lower cranial nerve dysfunction (OR 3.99, 95% CI 1.29-14.01) had a statistically significant correlation with abnormal BAEPs. Only age (OR 1.07, 95% CI 1.04-1.10) and the degree of tonsillar herniation (OR 1.11, 95% CI 1.04-1.19) had a statistically significant correlation with abnormal SSEPs. CONCLUSIONS A high percentage of patients with CM-1 exhibited EP alterations regardless of their clinical or radiological findings. These findings suggest that EPs do not add any clinically relevant information nor are they helpful in establishing which symptomatic patients with CM should undergo surgical treatment. However, BAEP and SSEP studies clearly play an important role in incidentally detected patients with CM and may help to establish objective evidence of subclinical dysfunctions. In addition, neurophysiological studies may help to define subgroups of patients who require further testing and follow-up to personalize strategies for the management of incidental and oligosymptomatic patients.

Imaging-Based Features of Headaches in Chiari Malformation Type I.

Suboccipital cough-induced headaches are considered a hallmark symptom of Chiari malformation type I (CMI). However, non--Valsalva-related suboccipital headaches and headaches in other locations are also common in CMI. The diagnostic significance and the underlying factors associated with these different headaches types are not well understood. To compare cranial morphology and hydrodynamics in 3 types of headaches in CMI to better understand the pathophysiological basis for the different headache characteristics. Twenty-two cranial physiological and morphological measures were obtained with specialized magnetic resonance imaging scans from 63 symptomatic pretreated CMI patients, 40 with suboccipital headaches induced by Valsalva maneuvers (34 women; age, 36 ± 10 years), 15 with non--Valsalva-related suboccipital headaches (10 women; age, 33 ± 9 years), 8 with nonsuboccipital non--Valsalva-induced headaches (8 women; age, 39 ± 13 years), and 37 control subjects (24 women; age, 36 ± 12 years). Group differences were identified with the use of the 2-tailed Student t test. Posterior cranial fossa markers of CMI were similar among the 3 headache subtypes. However, the Valsalva-related suboccipital headaches cohort demonstrated a significantly lower intracranial compliance index than the non--Valsalva-related suboccipital headaches cohort (7.5 ± 3.4 vs 10.9 ± 4.9), lower intracranial volume change during the cardiac cycle (0.48 ± 0.19 vs 0.61 ± 0.16 mL), and higher magnetic resonance imaging--derived intracranial pressure (11.1 ± 4.3 vs 7.7 ± 2.8 mm Hg; P = .02). The Valsalva-related suboccipital headaches cohort had smaller intracranial and lateral ventricular volumes compared with the healthy cohort. The non--Valsalva-related suboccipital headaches cohort had reduced venous drainage through the jugular veins. Valsalva-induced worsening of occipital headaches appears to be related to a small intracranial volume rather than the smaller posterior cranial fossa. This explains the reduced intracranial compliance and corresponding higher pressure measured in CMI patients with headaches affected by Valsalva maneuvers.

Indomethacin-responsive headaches.

Indomethacin-responsive headaches are a heterogeneous group of primary headache disorders distinguished by their swift and often absolute response to indomethacin. The epidemiology of these conditions is incompletely defined. Traditionally, indomethacin-responsive headaches include a subset of trigeminal autonomic cephalalgias (paroxysmal hemicrania and hemicrania continua), Valsalva-induced headaches (cough headache, exercise headache, and sex headache), primary stabbing headache, and hypnic headache. These headache syndromes differ in extent of response to indomethacin, clinical features, and differential diagnoses. Neuroimaging is recommended to investigate for various organic causes that may mimic these headaches. Case reports of other primary headache disorders that also respond to indomethacin, such as cluster headache, nummular headache, and ophthalmoplegic migraine, have been described. These "novel" indomethacin-responsive headaches beg the question of what headache characteristics are required to qualify a headache as an indomethacin-responsive headache. Furthermore, they challenge the concept of using a therapeutic intervention as a diagnostic criterion.

Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I

The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation. The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010. The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and "return to school" time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed. The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.

Indomethacin-responsive headache syndromes.

Indomethacin-responsive headache syndromes represent a unique group of primary headache disorders characterized by a prompt and often complete response to indomethacin to the exclusion of other nonsteroidal anti-inflammatory drugs and medications usually effective in treating other primary headache disorders. Because these headache disorders can easily be overlooked in clinical practice, they likely are more common than previously recognized. Indomethacin-responsive headache syndromes can be divided into several distinct categories: a select group of trigeminal-autonomic cephalgias, valsalva-induced headaches, and primary stabbing headache (ice-pick headache or jabs and jolts syndrome). Each category can be differentiated clinically and by the extent to which the individual headache disorders respond to indomethacin. The paroxysmal and continuous hemicranias invariably respond in an absolute manner to indomethacin, whereas valsalva-induced and ice-pick headaches may respond in an equally dramatic, but somewhat less consistent fashion. Hypnic headache recently has been described as another primary headache disorder that may respond to indomethacin.