Synchronous meningioma and anaplastic large cell lymphoma.

Synchronous primary brain tumors are exceedingly rare. When they occur, most cases are associated with metastatic disease. To the best of our knowledge, we report the first case of an atypical meningioma infiltrated by a T-cell-primary central nervous system lymphoma (PCNSL), specifically anaplastic large cell lymphoma (ALCL). We present a novel, unifying, plausible mechanism for its origin based on theories in the current literature. A 65-year-old man with a history of near-total resection of atypical meningioma presented with a complaint of progressive headaches. Imaging revealed recurrent tumor. Left frontal-temporal craniotomy with near-total tumor resection followed by radiation was performed. Recurrent symptomatic tumor led to repeat left frontotemporal craniotomy with tumor resection and partial anterior temporal lobectomy. Part of the specimen showed predominantly fibrotic neoplasm composed of nests and whorls of meningothelial cells, highlighted by epithelial membrane antigen (EMA) staining. The remainder of the specimen consisted of densely cellular neoplasm centered in connective tissue, including areas involved by meningioma. This tumor was composed of moderately large lymphoid cells with large nuclei, prominent nucleoli, and amphophilic cytoplasm. These cells were strongly immunoreactive for CD3 and CD30 but remained unstained with EMA, anaplastic lymphoma kinase-1 (ALK-1), CD15 or cytotoxic associated antigen TIA-1. Smaller mature lymphocytes, chiefly T-cells, were intermixed. The morphologic and immunohistochemical features were considered typical of anaplastic large T-cell lymphoma. The pathogenesis of this association may have been due to radiation-mediated breakdown of the blood-brain barrier with subsequent T-cell infiltration and proliferation. We advocate aggressive resection and long-term surveillance for individuals with metastasis, especially higher-grade neoplasms that receive radiotherapy.

Periodic alternating nystagmus and periodic alternating skew deviation in spinocerebellar ataxia type 6.

The combination of periodic alternating nystagmus (PAN) and periodic alternating skew deviation (PASD) is rare. We report a case of PAN and PASD in a patient with spinocerebellar ataxia type 6 (SCA-6) and discuss the role of the cerebellum as a plausible mechanism for this combined pathologic condition.

Delayed lateral rectus palsy following resection of a pineal cyst in sitting position: direct or indirect compressive phenomenon?

A rare case of delayed lateral rectus palsy in a patient following resection of a pineal lesion in the sitting position is presented. Postoperative pneumocephalus is common following craniospinal surgical intervention in the sitting position. The sixth cranial nerve is frequently injured because of its prolonged intracranial course. A 13-year-old girl was evaluated for unremitting headaches. No focal deficits were demonstrated on neurological examination. Magnetic resonance imaging revealed a cystlike pineal region mass with peripheral enhancement following intravenous contrast administration. A supracerebellar infratentorial craniotomy was performed in the sitting position, and complete resection of the lesion was achieved. Her postoperative course was complicated by sixth nerve palsy on the third postoperative day. Her symptoms improved with conservative management. The occurrence of sixth cranial nerve palsy secondary to pneumocephalus is a rare entity. Even rarer is the report of this anomaly following craniotomy in the sitting position. This patient's symptoms manifested in a delayed fashion. Although uncommon, this complication should be considered in patients undergoing cranial or spinal surgical interventions in this position.

A History of the Council of State Neurosurgical Societies.

As neurological surgery evolved into its own subspecialty early in the 20th century, a need arose to create an environment for communication and education among those surgeons working in this burgeoning surgical discipline. As the socioeconomic climate in health care began to change in the United States, an unforeseen need arose that was outside the scope of the American Association of Neurological Surgeons, Congress of Neurological Surgeons, and Society of Neurological Surgeons. The capacity to understand and address the evolving socioeconomic landscape and to offer a platform for advocacy required a new entity. Grassroots efforts of neurosurgeons at the state level ultimately yielded a formal organization of state neurosurgical societies to fill this void by recognizing, understanding, and addressing socioeconomic factors affecting the practice of neurological surgery. This formal organization became the Council of State Neurosurgical Societies (CSNS). The CSNS provides a forum in which state societies can meet to identify, understand, and advocate for policies on behalf of organized neurosurgery. The purpose of this paper is to detail the history of the formation of the CSNS. By understanding this history and the need for the development of the CSNS, it is hoped that its evolving role as a voice for neurological surgeons in the modern era of health care will be made clear.

Neurosurgery concepts: Key perspectives on dendritic cell vaccines, metastatic tumor treatment, and radiosurgery.

BACKGROUND: This is a laboratory study to investigate the effect of adding brain-derived-neurotrophic factor (BDNF) in a poly (N-isopropylacrylamide-g-poly (ethylene glycol) scaffold and its effect on spinal cord injury in a rat model. METHODS: This is a laboratory investigation of a spinal cord injury in a rat model. A dorsolateral funiculotomy was used to disrupt the dorsolateral funiculus and rubrospinal tract. Animals were then injected with either the scaffold polymer or scaffold polymer with BDNF. Postoperatively, motor functions were assessed with single pellet reach to grasp task, stair case reaching task and cylinder task. Histological study was also performed to look at extent of glial scar and axonal growth. RESULTS: Animals received BDNF containing polymer had an increased recovery rate of fine motor function of forelimb, as assessed by stair case reaching task and single pellet reach to grasp task compared with control animals that received the polymer only. There is no significant difference in the glial scar formation. BDNF treated animals also had increased axon growth including increase in the number and length of the rubrospinal tract axons. CONCLUSION: BDNF delivered via a scaffold polymer results in increased recovery rate in forelimb motor function in an experimental model of spinal cord injury, possibly through a promotion of growth of axons of the rubrospinal tract.

Clinical outcomes in patients with Chiari I malformation: a review of 27 cases.

BACKGROUND: Chiari I malformation is a congenital maldevelopment defined as downward herniation of the cerebellar tonsils through the foramen magnum. It has been treated using a variety of surgical procedures. Surgical outcomes have been reported with varying results throughout the literature. METHODS: We retrospectively reviewed 27 patients with Chiari I malformation operated at the University Hospital during a 9-year period from 1988 to 1997. We assessed preoperative and postoperative signs and symptoms in all the patients including headache, neck pain, nystagmus, vertigo, weakness, spasticity, atrophy, numbness, pain and temperature dissociation, diplopia, dysphagia, and sphincter dysfunction. Each patient was analyzed to determine if there was an arrest in the progression of the disease after surgical intervention. RESULTS: Syringomyelia was present in 59% of the patients. Only 1 patient who presented with neck pain improved. One patient reported new onset headache, and one patient described his headache resolved. Vertigo resolved in three patients; two patients stated mild improvement, and one patient reported worsening. Nystagmus improved or resolved in six patients. Weakness improved in only two patients who did not have syringomyelia. Dysphagia improved in two patients, and in the others it remained unchanged. Diplopia, spasticity, atrophy, and numbness remained unchanged. CONCLUSIONS: Our study provides evidence that the main benefit of the surgical management in patients with Chiari I malformation with or without syringomyelia is to arrest the progression of the disease.

Quality of life and outcomes in glioblastoma management.

This article explores the effects of modern treatment on the health-related quality of life in patients who suffer from glioblastoma multiforme.

Metabolic targeting of lactate efflux by malignant glioma inhibits invasiveness and induces necrosis: an in vivo study.

Glioblastoma multiforme (GBM) are the most malignant among brain tumors. They are frequently refractory to chemotherapy and radiotherapy with mean patient survival of approximately 6 months, despite surgical intervention. The highly glycolytic nature of glioblastomas describes their propensity to metabolize glucose to lactic acid at an elevated rate. To survive, GBMs efflux lactic acid to the tumor microenvironment through transmembrane transporters denoted monocarboxylate transporters (MCTs). We hypothesized that inhibition of MCT function would impair the glycolytic metabolism and affect both glioma invasiveness and survival. We examined the effect on invasiveness with α-cyano-4-hydroxy-cinnamic acid (ACCA, 4CIN, CHCA), a small-molecule inhibitor of lactate transport, through Matrigel-based and organotypic (brain) slice culture invasive assays using U87-MG and U251-MG glioma cells. We then conducted studies in immunodeficient rats by stereotaxic intracranial implantation of the glioma cells followed by programmed orthotopic application of ACCA through osmotic pumps. Effect on the implanted tumor was monitored by small-animal magnetic resonance imaging. Our assays indicated that glioma invasion was markedly impaired when lactate efflux was inhibited. Convection-enhanced delivery of inhibitor to the tumor bed caused tumor necrosis, with 50% of the animals surviving beyond the experimental end points (3 months after inhibitor exhaustion). Most importantly, control animals did not display any adverse neurologic effects during orthotopic administration of ACCA to brain through programmed delivery. These results indicate the clinical potential of targeting lactate efflux in glioma through delivery of small-molecule inhibitors of MCTs either to the tumor bed or to the postsurgical resection cavity.

Diagnostic and therapeutic strategy for confounding radiation myelitis.

We report a case of confounding radiation myelitis to demonstrate the usefulness of surgical biopsy in ensuring the correct diagnosis and to avoid unnecessary treatment. The patient was a 40-year-old man with a history of epiglottis carcinoma and sarcoidosis. Six months after radiation therapy and chemotherapy for epiglottis carcinoma, he noticed paresthesia and dysesthesia in the left arm and leg. Two months after that, he complained of severe neck pain and rapidly progressing weakness in all extremities. MRI showed an enhanced intramedullary lesion with extensive edema in the cervical spinal cord. Radiation myelitis, intramedullary spinal tumor, and neurosarcoidosis were considered as differential diagnoses. Spinal cord biopsy with laminectomy was performed and radiation myelitis was diagnosed. After the surgery, the lesion was significantly decreased in size even though corticosteroid therapy was rapidly tapered. We emphasize that a spinal cord biopsy is indicated to obtain a pathological diagnosis and to make a clear treatment strategy for patients with associated diseases causing lesions of the spinal cord.

Safety of instrumentation in patients with spinal infection.

OBJECT: Treatment of spine infection remains a challenge for spine surgeons, with the most effective method still being a matter of debate. Most surgeons agree that in early stages of infection, antibiotic treatment should be pursued; under certain circumstances, however, surgery is recommended. The goals of surgery include radical debridement of the infective focus. In some cases, when surgery causes mechanical spinal instability, the question arises whether the risk of recurrent infection outweighs the benefits of spinal instrumentation and stabilization. The authors report their series of cases in which instrumentation was placed in actively infected sites and review the relevant literature. METHODS: The authors performed a retrospective analysis of all cases of spinal infection that were surgically treated with debridement and placement of instrumentation at their institution between 2000 and 2006. Patient presentation, risk factor, infective organism, surgical indication, level of involvement, type of procedure, and ultimate outcome were reviewed. Improved outcome was based on improvement of initial American Spinal Injury Association Impairment Score. RESULTS: Forty-seven patients (32 men, 15 women) were treated with instrumented surgery for spinal infection. Their average age at presentation was 54 years (range 37-78 years). Indications for placement of instrumentation included instability, pain after failure of conservative therapy, or both. Patients underwent surgery within an average of 12 days (range 1 day to 5 months) after their presentation to the authors' institution. The average length of hospital stay was 25 days (range 9-78 days). Follow-up averaged 22 months (range 1-80 months). Eight patients died; causes of death included sepsis (4 patients), cardiac arrest (2), and malignancy (2). Only 3 patients were lost to follow-up. Using American Spinal Injury Association scoring as the criterion, the patients' conditions improved in 34 cases and remained the same in 5. Complications included hematoma (2 cases), the need for hardware revision (1), and recurrent infection (2). Hardware replacement was required in 1 of the 2 patients with recurrent infection. CONCLUSIONS: Instrumentation of the spine is safe and has an important role in stabilization of the infected spine. Despite the presence of active infection, we believe that instrumentation after radical debridement will not increase the risk of recurrent infection. In fact, greater benefit can be achieved through spinal stabilization, which can even promote accelerated healing.

Pediatric spinal clear cell meningioma. Case report.

In this report the authors describe a unique case of spinal clear cell meningioma in a 13-year-old girl. Clear cell meningiomas (CCMs) are not uncommon. To the authors' knowledge, 14 cases of pediatric CCM occurring in the spinal canal have been reported. Factors lending resistance to meningioma initiation and invasion are analyzed. This 13-year-old girl presented with pain radiating down her left leg. Admission MR imaging showed an inhomogeneous enhancing intradural-extramedullary mass at the L4-5 level. Resection revealed a CCM, and radiotherapy was subsequently administered. Postoperatively there has been no recurrence in > 2 years. In this paper the authors report a case of CCM and provide a comprehensive literature review on this disease. Current recommendations for its management are still debatable, especially in the pediatric population, and the authors propose an algorithm for its treatment and surveillance.

Outcome of brain abscess by Clostridium perfringens.

OBJECTIVE: Despite the aggressive infection of soft tissue caused by Clostridium perfringens (gas gangrene-necrotizing fasciitis), a brain abscess with this bacteria treated by early surgical excision, debridement of necrotic tissue, and antibiotic coverage may be expected to have a good recovery. Long-term follow-up has not been well established in this group of patients. We report this case to show the outcome at 3 years post surgical and antibiotic treatment for C. perfringens brain abscess and stress the need for urgent intervention to achieve good outcome. We also present a literature review of Clostridial brain abscesses since the 1960s. CLINICAL PRESENTATION: A 53-year-old man was brought to the emergency room after having a witnessed seizure status postassault 3 days before admission. On presentation, he was febrile, disoriented, lethargic, and demonstrated right upper extremity weakness. A computed tomographic scan of the head showed a left frontoparietal depressed cranial fracture complicated with gas and intraparenchymal air fluid level cavity. INTERVENTION: Emergent surgery for debridement and excision of necrotic tissue was performed. Empiric intravenous antibiotic therapy was started and penicillin G was added for 6 weeks after C. perfringens was demonstrated. CONCLUSION: Despite the severe infection and effect of C. perfringens in soft tissues in the brain, it appears that emergent surgical debridement and antibiotic coverage will yield an excellent outcome for these patients.

Lactate and malignant tumors: a therapeutic target at the end stage of glycolysis.

Metabolic aberrations in the form of altered flux through key metabolic pathways are primary hallmarks of many malignant tumors. Primarily the result of altered isozyme expression, these adaptations enhance the survival and proliferation of the tumor at the expense of surrounding normal tissue. Consequently, they also expose a unique set of targets for tumor destruction while sparing healthy tissues. Despite this fact, development of drugs to directly target such altered metabolic pathways of malignant tumors has been under-investigated until recently. One such target is the ultimate step of glycolysis, which, as expected, presents itself as a metabolic aberration in most malignant tumors. Termed "aerobic glycolysis" due to abnormal conversion of pyruvic acid to lactic acid even under normoxia, the altered metabolism requires these tumors to rapidly efflux lactic acid to the microenvironment in order to prevent poisoning themselves. Thus, exposed is a prime "choke-point" to target these highly malignant, frequently chemo- and radio- resistant tumors. This review will focus on current outcomes in targeting lactate efflux in such tumors using glioma as a model, an ongoing project in our laboratory for the past half-decade, as well as supporting evidence from recent studies by others on targeting this "tail-end" of glycolysis in other tumor models.