Mark N. Hadley

Cerebral cavernous malformations. Incidence and familial occurrence.

We studied 24 patients with histologically verified cerebral cavernous malformations, reviewing the familial occurrence and presenting signs, symptoms, and radiographic features of the disorder. Eleven patients had no evidence of a heritable trait and had negative family histories. Thirteen patients were members of six unrelated Mexican-American families. Sixty-four first-degree and second-degree relatives were examined, and family pedigrees were established. Most relatives (83 percent) were asymptomatic; 11 percent had seizures. Magnetic resonance imaging was performed in 16 relatives (5 of whom were asymptomatic). Fourteen of the 16 studies revealed cavernous malformations; 11 studies identified multiple lesions. As compared with computerized tomography and angiography, magnetic resonance imaging was far more accurate in detecting cavernous malformations. We conclude that cavernous malformations are more prevalent than previously reported, and that a familial form of the disorder exists that is more common than expected, with a high incidence of multiple lesions and an increased frequency of occurrence among Mexican-American families. Magnetic resonance imaging is the radiographic technique of choice for the identification and follow-up of these lesions.

Recommendations for Comprehensive Stroke Centers: A Consensus Statement From the Brain Attack Coalition

Background and Purpose— To develop recommendations for the establishment of comprehensive stroke centers capable of delivering the full spectrum of care to seriously ill patients with stroke and cerebrovascular disease. Recommendations were developed by members of the Brain Attack Coalition (BAC), which is a multidisciplinary group of members from major professional organizations involved with the care of patients with stroke and cerebrovascular disease. Summary of Review— A comprehensive literature search was conducted from 1966 through December 2004 using Medline and Pub Med. Articles with information about clinical trials, meta-analyses, care guidelines, scientific guidelines, and other relevant clinical and research reports were examined and graded using established evidence-based medicine approaches for therapeutic and diagnostic modalities. Evidence was also obtained from a questionnaire survey sent to leaders in cerebrovascular disease. Members of BAC reviewed literature related to their field and graded the scientific evidence on the various diagnostic and treatment modalities for stroke. Input was obtained from the organizations represented by BAC. BAC met on several occasions to review each specific recommendation and reach a consensus about its importance in light of other medical, logistical, and financial factors. Conclusions— There are a number of key areas supported by evidence-based medicine that are important for a comprehensive stroke center and its ability to deliver the wide variety of specialized care needed by patients with serious cerebrovascular disease. These areas include: (1) health care personnel with specific expertise in a number of disciplines, including neurosurgery and vascular neurology; (2) advanced neuroimaging capabilities such as MRI and various types of cerebral angiography; (3) surgical and endovascular techniques, including clipping and coiling of intracranial aneurysms, carotid endarterectomy, and intra-arterial thrombolytic therapy; and (4) other specific infrastructure and programmatic elements such as an intensive care unit and a stroke registry. Integration of these elements into a coordinated hospital-based program or system is likely to improve outcomes of patients with strokes and complex cerebrovascular disease who require the services of a comprehensive stroke center.

Acute axis fractures. Analysis of management and outcome in 340 consecutive cases.

Study Design. Retrospective review of acute axis fractures treated at a tertiary referral center. Objective. To determine the optimal treatment of axis fractures based on 340 cases from a single institution. Summary of Background Data. Axis fractures account for almost 20% of acute cervical spine fractures. However, their management and the clinical criteria predictive of nonoperative failure remain unclear. Methods. Admission imaging studies and clinical variables were obtained for 340 consecutive axis fracture patients. Fractures were classified as odontoid Type I, II, or III with dens displacement on admission roentgenograms; hangmans fractures of Francis grade and Effendi type; and miscellaneous fractures. Treatment methods were documented, and outcomes were based on dynamic lateral roentgenograms, clinical examination, or telephone interviews at last follow‐up. Results. Follow‐up data were available in 92% of cases. Type II odontoid fractures comprised 35% of all axis fractures, were the most difficult to treat, and had the highest nonunion rate (28.4%). Odontoid displacement of 6 mm or more was associated with Type II nonunion (chi‐square = 33.74, P < 0.0001). Patients underwent surgical fusion if fracture alignment could not be maintained by an external orthosis, or if they had odontoid fractures with transverse ligament disruption, Type II odontoid fractures with dens displacement of at least 6 mm, or hangmans fractures of severe Francis grade or Effendi type. Conclusions. Type II odontoid fractures have the highest nonunion rate and were associated with dens displacement of 6 mm or greater. Early surgical fusion is recommended for acute fracture instability despite external immobilization, transverse ligament disruption, Type II odontoid fractures with dens displacement of at least 6 mm on admission, or severe Francis grade or Effendi‐type hangmans fractures. Otherwise, nonoperative management is sufficient.

Cavernous malformations and capillary telangiectasia: A spectrum within a single pathological entity

Cerebral vascular malformations have traditionally been divided into four categories: arteriovenous, venous, cavernous, and capillary telangiectases. A controversy exists about separating the latter two lesions into separate entities. Critics claim the distinction is arbitrary but have been unable to present convincing evidence linking the two types of lesions. We have reviewed the histories of 20 patients with cavernous malformations and have analyzed the clinical, radiographic, and surgical-autopsy data associated with these lesions. In some patients, multiple lesions, including cavernous malformations, capillary telangiectases, and transitional forms between the two, were identified. Based on this analysis, we conclude that capillary telangiectasia and cavernous malformations represent two pathological extremes within the same vascular malformation category and propose grouping them as a single cerebral entity called cerebral capillary malformations.

Pharmacological Therapy for Acute Spinal Cord Injury

• Administration of methylprednisolone (MP) for the treatment of acute spinal cord injury (SCI) is not recommended. Clinicians considering MP therapy should bear in mind that the drug is not Food and Drug Administration (FDA) approved for this application. There is no Class I or Class II medical evidence supporting the clinical benefit of MP in the treatment of acute SCI. Scattered reports of Class III evidence claim inconsistent effects likely related to random chance or selection bias. However, Class I, II, and III evidence exists that high-dose steroids are associated with harmful side effects including death. • Administration of GM-1 ganglioside (Sygen) for the treatment of acute SCI is not recommended.

Acute traumatic atlas fractures: management and long term outcome.

Fractures of the 1st cervical vertebra (C1) represent 7% of all acute cervical spine fractures. Isolated atlas fractures are most commonly bilateral or multiple fractures through the ring of C1. Frequently (44% of cases), the atlas will be fractured in combination with the axis. Treatment of isolated C1 fractures should be governed by the rules of Spence. The treatment of combination C1-C2 fractures is dictated by the type and severity of the C2 fracture. Experience with 57 cases of acute atlas fractures is reviewed. Nonoperative external immobilization was used in 53 patients (with 1 failure), and early surgical wiring and fusion were performed in 4 patients. The long term outcome from an atlas fracture is good (median follow-up, 40 months).

Guidelines for the management of acute cervical spine and spinal cord injuries: 2013 update.

In 2002, an author group selected and sponsored by the Joint Section on Spine and Peripheral Nerves of the American Association of Neurological Surgeons and Congress of Neurological Surgeons published the first evidence-based guidelines for the management of patients with acute cervical spinal cord injuries (SCIs). In the spirit of keeping up with changes in information available in the medical literature that might provide more contemporary and more robust medical evidence, another author group was recruited to revise and update the guidelines. The review process has been completed and is published and can be once again found as a supplement to Neurosurgery. The purpose of this article is to provide an overview of the changes in the recommendations as a result of new evidence or broadened scope.

Pediatric spinal cord injury without radiographic abnormalities: report of 26 cases and review of the literature.

Spinal cord injury without radiographic abnormality (SCIWORA) occurs primarily in the pediatric population but is less common than other forms of spinal injury among children. Between 1972 and 1990, 159 pediatric patients were admitted to the Barrow Neurological Institute with acute traumatic spinal cord or vertebral column injuries. Of these, 26 children (16%) sustained SCIWORA. The mechanism of injury, its severity, and the prognosis for recovery were related to the patients age. In young children, SCIWORA accounted for 32% of all spinal injuries and tended to be severe; 70% were complete injuries. In older children, SCIWORA accounted for only 12% of the spinal injuries, was rarely associated with a complete injury, and had an excellent prognosis for complete recovery of neurologic function. As with other types of spinal cord injuries, the severity of neurological injury was the most important predictor of outcome. Patients with complete neurological deficits from SCIWORA had a poor prognosis for recovery of neurological function.

Smoking and the human vertebral column: a review of the impact of cigarette use on vertebral bone metabolism and spinal fusion.

Chronic cigarette consumption has significant adverse effects on the human spinal column. Multiple mechanisms induced by tobacco use lead to less strong, less healthy, mineral-deficient vertebrae with reduced bone blood supply and fewer and less functional bone-forming cells among chronic smokers. Compared to nonsmokers, chronic smokers develop advanced bony degradation, are more likely to suffer from spinal column degenerative disease, and seem more susceptible to traumatic vertebral injury. Spinal fusion procedures in chronic smokers are less often clinically and radiographically successful, compared to similar procedures performed among nonsmokers for definitive biological, physiological, and mechanical reasons.

Administration of Intraspinal Morphine Sulfate for the Treatment of Intractable Cancer Pain

A total of 24 patients with intractable cancer pain were evaluated as candidates for spinal morphine therapy. Temporary trials were carried out with bolus injections of preservative-free morphine sulfate via percutaneously inserted epidural catheters. Fourteen patients felt that pain relief was sufficient to warrant long term morphine application, and permanent drug delivery systems were implanted. These consisted of an Ommaya reservoir and an epidural spinal catheter in 6 patients and an Infusaid pump with either an epidural or subarachnoid spinal catheter in 8 patients. Pain relief with these systems was felt to be excellent in 7 patients, good in 4 patients, and fair in 3 patients. There was a statistically significant reduction in supplemental narcotic use between the pre- and postoperative periods (P less than 0.001). Median survival after operation was 3.0 months (mean, 5.0 months), with a range of 1 to 23 months. Tolerance was seen in all patients regardless of the mode of drug delivery, but it occurred more quickly with bolus injections than with continuous infusion (statistically significant difference, P less than 0.05). A persistent cerebrospinal fluid fistula developed in 1 patient; this required wound revision. No other serious complications or episodes of respiratory depression occurred. We conclude that intraspinal morphine sulfate is a beneficial treatment option for cancer patients in whom pain has become debilitating and unresponsive to oral or parenteral narcotic regimes.