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Richard Cowie and Bernard Williams

✓ A survey of 89 patients with subdural empyema was conducted to assess the incidence of late seizures and morbidity in this disease. Twenty-four patients died during the acute stage of the illness and, of the 65 survivors, 13 were lost or had incomplete follow-up review. Hemiparesis occurred in 48 of the survivors during the acute stage, and all but nine recovered completely. Thirteen patients had a visual field deficit and all recovered; in three of these 13 who had speech disorders the deficits persisted. Recovery from neurological morbidity was not related to the type of surgical treatment; however, the mortality rate was improved by craniotomy.

The same incidence of early seizures occurred in those who died (62%) as in those who survived (63%). Of those who had no early seizures, 42% had late seizures, the majority appearing within 16 months. Of those who had early seizures, 71% did not have subsequent attacks. The highest incidence of seizures occurred in patients who had their empyema in the second and third decades of life. The incidence of late seizures was not influenced by the method of surgical treatment, the degree of deterioration of consciousness during the acute stage of the illness, nor by occurrence of early seizures. A significantly increased incidence of early seizures was associated with paranasal sepsis, but not with late seizures.

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Man-Kai Cheng, Claudia Robertson, Robert G. Grossman, Richard Foltz and Vick Williams

✓ Occlusion of the abdominal aorta of the rabbit by inflating the balloon of a Swan-Ganz catheter positioned in the aorta is a simple and reliable method of producing spinal cord ischemia. The electrophysiological, neurological, and neuropathological correlates of ischemia with progressively longer durations and of ischemia after drug interventions were studied with the goal of developing an easily monitored, reproducible model for central nervous system ischemia. The percentage of animals developing paraplegia after varying periods of ischemia was zero after 15 minutes, 30% after 17 minutes, 33% after 20 minutes, 38% after 25 minutes, and 100% after 60 minutes of ischemia. After 25 minutes of ischemia the percentage of animals developing paraplegia was 87% when they were awake and not ventilated during ischemia and reperfusion, but dropped to 38% in animals that were paralyzed, sedated with ketamine, and ventilated, and when the metabolic acidosis that follows aortic occlusion was corrected during reperfusion. Pretreatment with thiopental, hypothermia, naloxone, methylprednisolone, and verapamil changed the percentage of animals developing paraplegia after 25 minutes of ischemia to 0%, 0%, 25%, 40%, and 100%, respectively. The component waves of the spinal somatosensory evoked potential (SSEP) disappeared sequentially during ischemia in the following order: P2, N4, N3, N2, and N1. After reperfusion, the SSEP components returned in reverse order of their disappearance. In the untreated animals, absence of the N3 wave for more than 10 minutes during ischemia was always followed by a neurological deficit. Pretreatment with thiopental or hypothermia permitted longer periods of electrophysiological silence without permanent neurological deficit. The ratio of the amplitude of N3 to N1 (N3/N1) was at least 70% of the control level, and N4 and P2 amplitudes were at least 30% of the control level at 120 minutes after reperfusion in all animals that had a normal outcome. Return of the N3/N1 amplitude to at least 90% of the control level or return of N3/N1 to 70% to 89% of control and P2 to at least 60% of control at 120 minutes after reperfusion reliably correlated with a normal 48-hour motor examination in animals with and without drug interventions.

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Harvey S. Levin, Eugenio Amparo, Howard M. Eisenberg, David H. Williams, Walter M. High Jr., Craig B. McArdle and Richard L. Weiner

✓ Twenty patients admitted for minor or moderate closed-head injury were studied to investigate the relationship between magnetic resonance imaging (MRI) and neurobehavioral sequelae. The MRI scans demonstrated 44 more intracranial lesions than did concurrent computerized tomography (CT) scans in 17 patients (85%); most of these lesions were located in the frontal and temporal regions. Estimates of lesion volume based on MRI were frequently greater than with CT; however, MRI disclosed no additional lesions that required surgical evacuation. Neuropsychological assessment during the initial hospitalization revealed deficits in frontal lobe functioning and memory that were related to the size and localization of the lesions as defined by MRI. Follow-up MRI and neuropsychological testing at 1 month (13 cases) and 3 months (six cases) disclosed marked reduction of lesion size paralleled by improvement in cognition and memory. These findings encourage further investigation of the prognostic utility of MRI for the clinical management and rehabilitation of mild or moderate head injury.

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W. Lee Titsworth, Jeannette Hester, Tom Correia, Richard Reed, Miranda Williams, Peggy Guin, A. Joseph Layon, Lennox K. Archibald and J Mocco

Object

To date, there has been a shortage of evidence-based quality improvement initiatives that have shown positive outcomes in the neurosurgical patient population. A single-institution prospective intervention trial with continuous feedback was conducted to investigate the implementation of a urinary tract infection (UTI) prevention bundle to decrease the catheter-associated UTI rate.

Methods

All patients admitted to the adult neurological intensive care unit (neuro ICU) during a 30-month period were included. The study consisted of two 1-month preintervention observation periods (approximately 1200 catheter days) followed by a 30-month intervention phase (20,394 catheter days). A comprehensive evidence-based UTI bundle encompassing avoidance of catheter insertion, maintenance of sterility, product standardization, and early catheter removal was enacted.

Results

The urinary catheter utilization rate dropped from 100% to 73.3% during the intervention phase (p < 0.0001) without any increase in the rate of sacral decubitus ulcers or other skin breakdown. The rate of catheter-associated UTI was also significantly reduced from 13.3 to 4.0 infections per 1000 catheter days (p < 0.001). There was a linear relationship between the decreased quarterly catheter utilization rate and the decreased catheter-associated UTI rate (r2 = 0.79, p < 0.0001).

Conclusions

This single-center prospective study demonstrated that a comprehensive UTI prevention bundle along with a continuous quality improvement program can significantly reduce the duration of urinary catheterization and rate of catheter-associated UTI in a neuro ICU.

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Michael A. Williams, Sean J. Nagel, Mark G. Luciano, Norman Relkin, Thomas J. Zwimpfer, Heather Katzen, Richard Holubkov, Abhay Moghekar, Jeffrey H. Wisoff, Guy M. McKhann II, James Golomb, Richard J. Edwards and Mark G. Hamilton

OBJECTIVE

The authors describe the demographics and clinical characteristics of the first 517 patients enrolled in the Adult Hydrocephalus Clinical Research Network (AHCRN) during its first 2 years.

METHODS

Adults ≥ 18 years were nonconsecutively enrolled in a registry at 6 centers. Four categories of adult hydrocephalus were defined: transition (treated before age 18 years), unrecognized congenital (congenital pattern, not treated before age 18 years), acquired (secondary to known risk factors, treated or untreated), and suspected idiopathic normal pressure hydrocephalus (iNPH) (≥ age 65 years, not previously treated). Data include etiology, symptoms, examination findings, neuropsychology screening, comorbidities, treatment, complications, and outcomes. Standard evaluations were administered to all patients by trained examiners, including the Montreal Cognitive Assessment, the Symbol Digit Modalities Test, the Beck Depression Inventory–II, the Overactive Bladder Questionnaire Short Form symptom bother, the 10-Meter Walk Test, the Boon iNPH gait scale, the Lawton Activities of Daily Living/Instrumental Activities of Daily Living (ADL/IADL) questionnaire, the iNPH grading scale, and the modified Rankin Scale.

RESULTS

Overall, 517 individuals were enrolled. Age ranged from 18.1 to 90.7 years, with patients in the transition group (32.7 ± 10.0 years) being the youngest and those in the suspected iNPH group (76.5 ± 5.2 years) being the oldest. The proportion of patients in each group was as follows: 16.6% transition, 26.5% unrecognized congenital, 18.2% acquired, and 38.7% suspected iNPH. Excluding the 86 patients in the transition group, who all had received treatment, 79.4% of adults in the remaining 3 groups had not been treated at the time of enrollment. Patients in the suspected iNPH group had the poorest performance in cognitive evaluations, and those in the unrecognized congenital group had the best performance. The same pattern was seen in the Lawton ADL/IADL scores. Gait velocity was lowest in patients in the suspected iNPH group. Categories that had the most comorbidities (suspected iNPH) or etiologies of hydrocephalus that directly cause neurological injury (transition, acquired) had greater degrees of impairment compared to unrecognized congenital, which had the fewest comorbidities or etiologies associated with neurological injury.

CONCLUSIONS

The clinical spectrum of hydrocephalus in adults comprises more than iNPH or acquired hydrocephalus. Only 39% of patients had suspected iNPH, whereas 43% had childhood onset (i.e., those in the transition and unrecognized congenital groups). The severity of symptoms and impairment was worsened when the etiology of the hydrocephalus or complications of treatment caused additional neurological injury or when multiple comorbidities were present. However, more than half of patients in the transition, unrecognized congenital, and acquired hydrocephalus groups had minimal or no impairment. Excluding the transition group, nearly 80% of patients in the AHCRN registry were untreated at the time of enrollment. A future goal for the AHCRN is to determine whether patients with unrecognized congenital and acquired hydrocephalus need treatment and which patients in the suspected iNPH cohort actually have possible hydrocephalus and should undergo further diagnostic testing. Future prospective research is needed in the diagnosis, treatment, outcomes, quality of life, and macroeconomics of all categories of adult hydrocephalus.

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Ziya L. Gokaslan, Patricia L. Zadnik, Daniel M. Sciubba, Niccole Germscheid, C. Rory Goodwin, Jean-Paul Wolinsky, Chetan Bettegowda, Mari L. Groves, Alessandro Luzzati, Laurence D. Rhines, Charles G. Fisher, Peter Pal Varga, Mark B. Dekutoski, Michelle J. Clarke, Michael G. Fehlings, Nasir A. Quraishi, Dean Chou, Jeremy J. Reynolds, Richard P. Williams, Norio Kawahara and Stefano Boriani

OBJECT

A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI.

METHODS

Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling.

RESULTS

A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p < 0.001), although no significant difference in survival was observed.

CONCLUSIONS

EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.

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Mark B. Dekutoski, Michelle J. Clarke, Peter Rose, Alessandro Luzzati, Laurence D. Rhines, Peter P. Varga, Charles G. Fisher, Dean Chou, Michael G. Fehlings, Jeremy J. Reynolds, Richard Williams, Nasir A. Quraishi, Niccole M. Germscheid, Daniel M. Sciubba, Ziya L. Gokaslan, Stefano Boriani and The AOSpine Knowledge Forum Tumor

OBJECTIVE

Primary spinal osteosarcomas are rare and aggressive neoplasms. Poor outcomes can occur, as obtaining marginal margins is technically demanding; further Enneking-appropriate en bloc resection can have significant morbidity. The goal of this study is to identify prognostic variables for local recurrence and mortality in surgically treated patients diagnosed with a primary osteosarcoma of the spine.

METHODS

A multicenter ambispective database of surgically treated patients with primary spine osteosarcomas was developed by AOSpine Knowledge Forum Tumor. Patient demographic, diagnosis, treatment, perioperative morbidity, local recurrence, and cross-sectional survival data were collected. Tumors were classified in 2 cohorts: Enneking appropriate (EA) and Enneking inappropriate (EI), as defined by pathology margin matching Enneking-recommended surgical margins. Prognostic variables were analyzed in reference to local recurrence and survival.

RESULTS

Between 1987 and 2012, 58 patients (32 female patients) underwent surgical treatment for primary spinal osteosarcoma. Patients were followed for a mean period of 3.5 ± 3.5 years (range 0.5 days to 14.3 years). The median survival for the entire cohort was 6.7 years postoperative. Twenty-four (41%) patients died, and 17 (30%) patients suffered a local recurrence, 10 (59%) of whom died. Twenty-nine (53%) patients underwent EA resection while 26 (47%) patients underwent EI resection with a postoperative median survival of 6.8 and 3.7 years, respectively (p = 0.048). EI patients had a higher rate of local recurrence than EA patients (p = 0.001). Patient age, previous surgery, biopsy type, tumor size, spine level, and chemotherapy timing did not significantly influence recurrence and survival.

CONCLUSIONS

Osteosarcoma of the spine presents a significant challenge, and most patients die in spite of aggressive surgery. There is a significant decrease in recurrence and an increase in survival with en bloc resection (EA) when compared with intralesional resection (EI). The effect of adjuvant and neoadjuvant chemotherapeutics, as well as method of biopsy, requires further exploration.

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Charles G. Fisher, Tony Goldschlager, Stefano Boriani, Peter Paul Varga, Laurence D. Rhines, Michael G. Fehlings, Alessandro Luzzati, Mark B. Dekutoski, Jeremy J. Reynolds, Dean Chou, Sigurd H. Berven, Richard P. Williams, Nasir A. Quraishi, Chetan Bettegowda and Ziya L. Gokaslan

Object

The National Institutes of Health recommends strategies to obtain evidence for the treatment of rare conditions such as primary tumors of the spine (PTSs). These tumors have a low incidence and are pathologically heterogeneous, and treatment approaches are diverse. Appropriate evidence-based care is imperative. Failure to follow validated oncological principles may lead to unnecessary mortality and profound morbidity. This paper outlines a scientific model that provides significant evidence guiding the treatment of PTSs.

Methods

A four-stage approach was used: 1) planning: data from large-volume centers were reviewed to provide insight; 2) recruitment: centers were enrolled and provided the necessary infrastructure; 3) retrospective stage: existing medical records were reviewed and completed with survival data; and 4) prospective stage: prospective data collection has been implemented. The AOSpine Knowledge Forum Tumor designed six modules: demographic, clinical, diagnostic, therapeutic, local recurrence, survival, and perioperative morbidity data fields and provided funding.

Results

It took 18 months to implement Stages 1–3, while Stage 4 is ongoing. A total of 1495 tumor cases were captured and diagnosed as one of 18 PTS histotypes. In addition, a PTS biobank network has been created to link clinical data with tumor pathology and molecular analysis.

Conclusions

This scientific model has not only aggregated a large amount of PTS data, but has also established an international collaborative network of spine oncology centers. Access to large volumes of data will generate further research to guide and enhance PTS clinical management. This model could be applied to other rare neoplastic conditions. Clinical trial registration no.: NCT01643174 (ClinicalTrials.gov).

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Maxwell S. H. Laurans, Michael L. DiLuna, Dana Shin, Faheem Niazi, Jennifer R. Voorhees, Carol Nelson-Williams, Eric W. Johnson, Adrian M. Siegel, Gary K. Steinberg, Michel J. Berg, R. Michael Scott, Gioacchino Tedeschi, T. Peter Enevoldson, John Anson, Guy A. Rouleau, Christopher Ogilvy, Issam A. Awad, Richard P. Lifton and Murat Gunel

Object. A gene contributing to the autosomal-dominant cerebral cavernous malformation (CCM) phenotype, KRIT1 (an acronym for Krev Interaction Trapped 1), has been identified through linkage analysis and mutation screening. The authors collected blood samples from 68 patients with familial CCM and 138 patients with apparently sporadic CCM as well as from their families, in an effort to characterize the prevalence and spectrum of disease-causing sequence variants in the KRIT1 gene.

Methods. The authors used single-strand conformational polymorphism analysis to identify genomic variants in KRIT1, which were sequenced to determine the specific mutation. Among 43 Hispanic-American kindreds who immigrated to the southwestern US from northern Mexico, 31 share an identical founder mutation. This Q455X mutation is found in 18 (86%) of 21 persons with a positive family history and in 13 (59%) of 22 persons with apparently sporadic CCM. This mutation was not found among 13 persons with CCM who were recruited from Mexico. These findings establish the key role of a recent founder mutation in Hispanic persons with CCM who live in the US.

Although nearly all Hispanic families in the US in which there are multiple CCM cases linked to the CCM1 locus, only 13 of 25 non-Hispanic CCM-carrying families have displayed evidence of linkage to the CCM1 locus. Among these 13 families, the authors identified eight independent mutations in nine kindreds. They identified four additional mutations among 22 familial CCM kindreds with no linkage information, bringing the total number of independent mutations to 12. Inherited KRIT1 mutations were not detected among 103 non-Hispanic persons in whom a family history of CCM was rigorously excluded.

Conclusions. All mutations were nonsense mutations, frame-shift mutations predicting premature termination, or splicesite mutations located throughout the KRIT1 gene, suggesting that these are genetic loss-of-function mutations. These genetic findings, in conjunction with the clinical phenotype, are consistent with a two-hit model for the occurrence of CCM.