Carotid-cavernous fistula was one of the first intracranial vascular lesions to be recognized. This paper focuses on the historical progression of our understanding of the condition and its symptomatology—from the initial hypothesis of ophthalmic artery aneurysm as the cause of pulsating exophthalmos to the recognition and acceptance of fistulas between the carotid arterial system and cavernous sinus as the true etiology. The authors also discuss the advancements in treatment from Benjamin Travers' early common carotid ligation and wooden compression methods to today's endovascular approaches.
Min Lang, Ghaith Habboub, Jeffrey P. Mullin, and Peter A. Rasmussen
Shahed Tish, Ghaith Habboub, Min Lang, Quinn T. Ostrom, Carol Kruchko, Jill S. Barnholtz-Sloan, Pablo F. Recinos, and Varun R. Kshettry
Spinal schwannoma remains the third most common intradural spinal tumor following spinal meningioma and ependymoma. The available literature is generally limited to single-institution reports rather than epidemiological investigations. As of 1/1/2004, registration of all benign central nervous system tumors in the United States became mandatory after the Benign Brain Tumor Cancer Registries Amendment Act took action, which provided massive resources for United States population-based epidemiological studies. This article describes the epidemiology of spinal schwannoma in the United States from January 1, 2006, through December 31, 2014.
In this study, the authors utilized the Central Brain Tumor Registry of the United States, which corresponds to 100% of the American population. The Centers for Disease Control and Prevention’s National Program of Cancer Registries and the National Cancer Institute’s Surveillance Epidemiology and End Results program provide the resource for this data registry. The authors included diagnosis years 2006 to 2014. They used the codes per the International Coding of Diseases for Oncology, 3rd Edition: histology code 9560/0 and site codes C72.0 (spinal cord), C70.1 (spinal meninges), and C72.1 (cauda equina). Rates are per 100,000 persons and are age-adjusted to the 2000 United States standard population. The age-adjusted incidence rates and 95% confidence intervals are calculated by age, sex, race, and ethnicity.
There were 6989 spinal schwannoma cases between the years 2006 and 2014. The yearly incidence eminently increased between 2010 and 2014. Total incidence rate was 0.24 (95% CI 0.23–0.24) per 100,000 persons. The peak adjusted incidence rate was seen in patients who ranged in age from 65 to 74 years. Spinal schwannomas were less common in females than they were in males (incidence rate ratio = 0.85; p < 0.001), and they were less common in blacks than they were in whites (IRR = 0.52; p < 0.001) and American Indians/Alaska Natives (IRR = 0.50; p < 0.001) compared to whites. There was no statistically significant difference in incidence rate between whites and Asian or Pacific Islanders (IRR = 0.92; p = 0.16).
The authors’ study results demonstrated a steady increase in the incidence of spinal schwannomas between 2010 and 2014. Male sex and the age range 65–74 years were associated with higher incidence rates of spinal schwannomas, whereas black and American Indian/Alaska Native races were associated with lower incidence rates. The present study represents the most thorough assessment of spinal schwannoma epidemiology in the American population.
Bryan S. Lee, Jaes Jones, Min Lang, Rebecca Achey, Lu Dai, Darlene A. Lobel, Sean J. Nagel, Andre G. Machado, and Francois Bethoux
Multiple sclerosis (MS) is a chronic autoimmune disease that causes demyelination and axonal loss. Walking difficulties are a common and debilitating symptom of MS; they are usually caused by spastic paresis of the lower extremities. Although intrathecal baclofen (ITB) therapy has been reported to be an effective treatment for spasticity in MS, there is limited published evidence regarding its effects on ambulation. The goal of this study was to characterize ITB therapy outcomes in ambulatory patients with MS.
Data from 47 ambulatory patients with MS who received ITB therapy were analyzed retrospectively. Outcome measures included Modified Ashworth Scale, Spasm Frequency Scale, Numeric Pain Rating Scale, and the Timed 25-Foot Walk. Repeated-measures ANOVA was used to test for changes in outcome measures between baseline and posttreatment (6 months and 1 year). Significance was set at p < 0.05. Descriptive data are expressed as the mean ± SD, and results of the repeated-measures ANOVA tests and the Wilcoxon rank-sum test are expressed as the mean ± SEM.
There was a statistically significant reduction in the following variables: 1) aggregate lower-extremity Modified Ashworth Scale scores (from 14.8 ± 1.0 before ITB therapy to 5.8 ± 0.8 at 6 months posttreatment and 6.4 ± 0.9 at 1 year [p < 0.05]); 2) Numeric Pain Rating Scale scores (4.4 ± 0.5 before ITB, 2.8 ± 0.5 at 6 months, and 2.4 ± 0.4 at 1 year [p < 0.05]); 3) spasm frequency (45.7% of the patients reported a spasm frequency of ≥ 1 event per hour before ITB therapy, whereas 15.6% and 4.3% of the patients reported the same at 6 months and 1 year posttreatment, respectively [p < 0.05]); and 4) the number of oral medications taken for spasticity (p < 0.05). Of the 47 patients, 34 remained ambulatory at 6 months, and 32 at 1 year posttreatment. There was no statistically significant change in performance on the Timed 25-Foot Walk test over time for those patients who remained ambulatory.
In this retrospective study, the authors found that ITB therapy is effective in reducing spasticity and related symptoms in ambulatory patients with MS. Because the use of ITB therapy is increasing in ambulatory patients with MS, randomized, prospective studies are important to help provide a more useful characterization of the effects of ITB therapy on ambulation.
Min Lang, Danilo Silva, Lu Dai, Varun R. Kshettry, Troy D. Woodard, Raj Sindwani, and Pablo F. Recinos
Preoperatively determining the extent of parasellar invasion of pituitary macroadenomas is useful for surgical planning and patient counseling. Here, the authors compared constructive interference in steady state (CISS), a T2-weighted gradient-echo MRI sequence, to volume-interpolated breath-hold examination (VIBE), a T1-weighted gradient-echo MRI sequence, for evaluation of cavernous sinus invasion (CSI) by pituitary macroadenomas.
VIBE and CISS images of 98 patients with pituitary macroadenoma were retrospectively analyzed and graded using the modified Knosp classification. The Knosp grades were correlated to surgical findings of CSI, which were determined intraoperatively using 0° and 30° endoscopes. The predictive accuracies for CSI according to the Knosp grades derived from the CISS and VIBE images were compared using receiver operating characteristic (ROC) curves. Postoperative MRI was used to evaluate the gross-total resection (GTR) rates.
The CSI rate by pituitary macroadenomas was 27.6% (27 of 98 cases). Of 196 assessments (left and right sides of 98 macroadenomas), 45 (23.0%) had different Knosp grades when scored using VIBE versus CISS images. For the VIBE images, 0% of Knosp grade 0, 4.5% of grade 1, 23.8% of grade 2, 42.1% of grade 3A, 100% of grade 3B, and 83.3% of grade 4 macroadenomas were found to have CSI intraoperatively. For the CISS images, 0% of Knosp grade 0, 2.1% of grade 1, 31.3% of grade 2, 56.3% of grade 3A, 100% of grade 3B, and 100% of grade 4 macroadenomas were found to have CSI intraoperatively. Two pituitary macroadenomas were classified as grade 4 on VIBE sequences but grades 3A and 2 on CISS sequences; CSI was not observed intraoperatively in both cases. The GTR rate was 64.3% and 60.0% for high-grade (3A, 3B, and 4) macroadenomas classified using VIBE and CISS sequences, respectively. The areas under the ROC curves were 0.94 and 0.97 for VIBE- and CISS-derived Knosp grades (p = 0.007), respectively.
Knosp grades determined using CISS sequence images are better correlated with intraoperative CSI than those determined using VIBE sequence images. CISS sequences may be valuable for the preoperative assessment of pituitary macroadenomas.