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Biomedical subjects

J A Jane

Publications and source records attributed to J A Jane.

At least 19 recordsLinked to original sources

The management of non-functioning pituitary adenomas.

Non-functioning pituitary adenomas most commonly present secondary to mass effect and are classified according to their size and immunohistochemical staining. Local intrasellar mass effect may cause varying degrees of hypopituitarism. With extrasellar growth, neurological signs and symptoms develop. Appropriate therapy for these tumors requires close interaction across multiple disciplines. Trans-sphenoidal surgery offers safe and effective treatment in the overwhelming majority of patients with relatively low risk of new neurological and endocrinologic deficits. The multidisciplinary management of non-functioning adenomas, their diagnosis and therapeutic outcomes, is discussed.

Adenoma↗

Surgical management of pituitary adenomas.

Pituitary adenomas are a diverse group of benign neoplasms. The hormonally active tumours present with well-recognised syndromes and include acromegaly (growth hormone adenoma), Cushing's disease (corticotropin adenoma), and amenorrhea-galactorrhea (prolactin adenoma or prolactinomas). The hormonally inactive, or clinically non-secreting, adenomas generally come to clinical attention secondary to local mass effect or pituitary deficiency. With the exception of the prolactinomas, transsphenoidal surgery remains the first-line therapy for most pituitary adenomas. The current diagnosis and surgical management of pituitary adenomas is discussed.

Adenoma↗

Acromegaly: historical perspectives and current therapy.

Soon after the initial description of acromegaly in the late 19th century, neurosurgeons performed the first operative procedures for the disease. Transcranial procedures eventually yielded to the transsphenoidal approach. Reasonably effective medical therapy was introduced in the 1970s and pharmacological progress continues to be realized. It is now recognized that excess growth hormone is associated with significant morbidity and mortality and that biochemical remission improves outcome. Although medical and radiation treatments offer useful adjuncts, surgery provides optimal results.

Acromegaly↗

Pilot study of electrical stimulation on median nerve in comatose severe brain injured patients: 3-month outcome.

PRIMARY OBJECTIVE: To determine if electrical stimulation (ES) benefits (waking time, 3-month outcomes) treated coma patients. RESEARCH DESIGN: Double blind randomized-controlled study. METHODS AND PROCEDURES: Ten coma patients; six treatment and four controls, using the 'Respond Select' by EMPI. EXPERIMENTAL INTERVENTIONS: Treatment group received radial nerve ES applied in 300 ms intermittent pulses at 40 Hz, 15-20m A 8 hours a day up to 14 days of coma; control group received sham stimulation. MAIN OUTCOMES AND RESULTS: ES group emerged from coma mean 2 days earlier than controls, although this result was not statistically significant. At 3 months post-injury, there was no group difference in Glasgow Outcome Scale, although the ES group had improved function over controls as measured by the FIM/FAM (mean of 114 and 64.5, respectively, n.s.). CONCLUSIONS: These data show an interesting trend, although statistical power was limited in this small pilot study, suggesting the need for a larger trial.

Adult↗

Fluoroscopic frameless stereotaxy for transsphenoidal surgery.

OBJECTIVE: To assess the value of frameless fluoroscopy-guided stereotactic transsphenoidal surgery using the FluoroNav Virtual Fluoroscopy System (Medtronic Sofamor Danek, Inc., Memphis, TN). METHODS: Twenty consecutive patients undergoing transsphenoidal surgery for sellar lesions were assigned to transsphenoidal surgery with or without computer-assisted fluoroscopic image guidance using the FluoroNav system. Prospective data regarding patient age, sex, lesion characteristics, operative time, and treatment cost were obtained. RESULTS: Although patients in the FluoroNav group were, on average, 17 years younger than the patients in the control group, more patients with recurrent adenomas were treated in the image guidance group. No other significant differences between the groups were found. FluoroNav provided accurate, continuous information regarding the anatomic midline trajectory to the sella turcica as well as anatomic structures (e.g., sella, sphenoid sinus) in the lateral view. No patient required reversion to intraoperative videofluoroscopy. No statistically significant differences were found with regard to preincision setup time, operative time, or cost. FluoroNav allowed procedures to be performed with significantly fewer x-rays being taken. CONCLUSION: Fluoroscopic computer-assisted frameless stereotaxy furnishes accurate real-time information with regard to midline structures and operative trajectory. Although it is useful in first-time transseptal transsphenoidal surgery, its primary benefit is realized in recurrent surgery.

Adenoma↗

Trilateral retinoblastoma variant indicative of the relevance of the retinoblastoma tumor-suppressor pathway to medulloblastomas in humans.

Results of recent studies have led investigators to suggest that the retinoblastoma tumor-suppressor (rb) gene plays an underappreciated role in the genesis of brain tumors. Such tumors cause significant rates of mortality in children suffering from hereditary retinoblastoma. It has been assumed that the pineal gland, which is ontogenetically related to the retina, accounts for the intracranial origin of these trilateral neoplasms. To address this issue, the authors describe an unusual trilateral retinoblastoma variant. The authors provide a detailed clinicopathological correlation by describing the case of a child with bilateral retinoblastoma who died of a medulloblastoma. The intraocular and intracranial neoplasms were characterized by performing detailed imaging, histopathological, and postmortem studies. Karyotype analysis and fluorescence in situ hybridization were used to define the chromosomal defect carried by the patient and members of her family. An insertion of the q12.3q21.3 segment of chromosome 13 into chromosome 18 at band q23 was identified in members of the patient's family. This translocation was unbalanced in the proband. The intraocular and cerebellar neoplasms were found to be separate primary neoplasms. Furthermore, the pineal gland was normal and the cerebellar neoplasm arose within the vermis as a medulloblastoma. Finally, the two neoplasms had different and characteristically identifiable cytolological and immunohistochemical profiles. The findings of the present study, taken together with those of recent molecular and transgenic studies, support the emerging concept that rb inactivation is not restricted to central nervous system regions of photoreceptor lineage and that inactivation of this tumor suppressor pathway may be relevant to the determination of etiological factors leading to medulloblastoma in humans.

Cerebellar Neoplasms↗

Degenerative lumbar stenosis: the neurosurgical perspective.

Degenerative lumbar stenosis is a complex entity caused by predictable patterns of degenerative pathoanatomy. Patients with spinal stenosis are treated by orthopaedic surgeons and neurosurgeons, who often have slightly different ideas regarding treatment strategies. However, data exist to support several recommendations, regarding fusion with or without instrumentation, surgical versus conservative treatment, and limitation of procedures to symptomatic levels. In the current study, the typical patterns of degenerative disease and surgical treatment and a view of the neurosurgical perspective of the treatment of patients with lumbar stenosis are presented.

Decompression, Surgical↗

A novel technique for ventriculovesical shunting of congenital hydrocephalus.

PURPOSE: Hydrocephalus is typically treated with a ventriculoperitoneal or ventriculoatrial shunt. However, shunt malfunction, recurrent infection or other co-morbidities occasionally make these shunts inappropriate. As early as 1925 a ureterodural anastomosis was used to divert cerebrospinal fluid into the urinary system. Since then techniques for cerebrospinal fluid urinary diversion have improved. We designed a new technique to drain cerebrospinal fluid into the urinary system surgically while averting some of the problems encountered with previous methods. MATERIALS AND METHODS: We describe a new technique to perform a ventriculovesicular shunt, which we performed on a 17-year-old woman with congenital hydrocephalus. To put this unique operation into perspective we briefly review the history of cerebrospinal fluid urinary shunting. RESULTS: Our results were promising, with no evidence of distal shunt malfunction or infection postoperatively. CONCLUSIONS: Our novel technique of a ventriculovesical shunt with a polyester cuff that provides stabilization and infection control holds promise for patients with hydrocephalus who are not candidates for a ventriculoperitoneal or ventriculoatrial shunt.

Adolescent↗

Occipital bone graft for atlantoaxial fusion.

BACKGROUND: Instability of the atlantoaxial segment is frequently encountered in neurosurgical practice. Numerous fusion techniques have been employed at this level. Most commonly, arthrodesis is achieved through bone and wire techniques. We have employed the use of suboccipital bone in lieu of iliac crest autograft in order to avoid the significant morbidity associated with iliac crest graft harvest. METHODS: Twenty one patients suffering instability from various etiologies underwent C1-C2 fusion at our institution using occipital bone graft and wire fixation. A small craniectomy was performed near the foramen magnum, and the bone graft was notched and secured in place using wire fixation. Patients were placed in a Philadelphia or Aspen collar for 6-12 weeks postoperatively, and flexion/extension plain film of the cervical spine were used to evaluate fusion. RESULTS: Long term follow up was available on all patients (mean 32 months, range 12-48 months). Fusion was achieved in 81% of patients within 12 weeks. Specifically, 71% (5/7) of rheumatoid patients were successfully fused. All patients with traumatic C1-C2 instability were fused. No complications were associated with the harvest of the occipital bone. CONCLUSION: The results of fusion via this technique are comparable to other reported series of C1-C2 fusion. Additionally, the complications associated with iliac crest graft harvest were avoided by the use of occipital bone graft. Occipital bone appears to be a suitable bone graft substance for fusion of the C1-C2 level.

Arthritis, Rheumatoid↗

The use of bone morphogenetic protein gene therapy in craniofacial bone repair.

Bone morphogenetic proteins (BMPs) are capable of inducing endochondral bone formation when applied on biologic carriers in numerous mammalian in vivo assay systems. Bone morphogenetic protein gene therapy is also currently being developed to promote osteogenesis for clinical indications such as spinal fusions, craniofacial bone loss, and osteoporosis. In this study, critical-sized mandibular defects were treated with a control adenoviral vector (Ad-beta-gal), a BMP-2 adenoviral vector (Ad-BMP-2), or a BMP-9 adenoviral vector (Ad-BMP-9). Gross tissue examination, radiographic analysis, and histologic analysis demonstrated significant bony healing in the BMP treated groups compared to controls. Osteogenesis was limited to the bony defect, without extension into the surrounding soft tissues. The study suggests that with further development, BMP gene therapy may be potentially useful for repair of bony defects in the craniofacial region.

Animals↗

Resolution of tonsillar herniation and syringomyelia after supratentorial tumor resection: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: The pathophysiological features of syringomyelia are not yet entirely understood. We present a case of a supratentorial mass causing tonsillar herniation and syringomyelia. CLINICAL PRESENTATION: A 51-year-old woman underwent magnetic resonance imaging for evaluation of progressive headaches. A large parieto-occipital mass was revealed. Herniation of the cerebellar tonsils and a cervical syrinx were also noted. INTERVENTION: A craniotomy was performed without incident. After tumor resection, the tonsils ascended and the syrinx resolved in a 1-year period. CONCLUSION: This case highlights the importance of tonsillar herniation in the pathogenesis of syringomyelia. "Acquired" Chiari malformations and syringomyelia attributable to supratentorial masses may be treated by mass resection alone, without the need for foramen magnum decompression.

Female↗

Chemotherapy for esthesioneuroblastomas.

Esthesioneuroblastoma can be regarded as a chemosensitive tumor based on multiple reports of response to treatment. Neoadjuvant therapy is seldom curative, however, and may be of no benefit in some patients. Individuals who respond to preoperative therapy have a greater chance of long-term disease-free survival. Platinum-based therapy has been the mainstay of treatment. Toxicity has been mild, and treatment failures have been treated with postoperative chemotherapy with or without bone marrow transplantation. Given the small number of cases of esthesioneuroblastoma diagnosed annually, it is unlikely that any consensus on this issue is forthcoming. Either post- or presurgical treatment of advanced stage or recurrent disease has become the standard of care, however.

Adult↗

Gene therapy for skull base tumors.

During the last 10 years, gene therapy has certainly risen to the forefront of basic science and clinical medicine. Designing more efficient gene delivery systems is currently the "holy grail" of genetic therapeutics, because even the most efficient viral vectors do not transfect all cells at the treatment site, which leads to local recurrence in the case of most brain tumors. The use of oncolytic viruses that propagate through tumors may have the best potential for treating skull base lesions in the future. Because many skull base tumors are histologically benign, gene therapy approaches for these tumors may be an excellent first step, as partial killing leading to local control with minimal morbidity and mortality may soon be possible. The accessibility of these tumors by endovascular approaches is also currently feasible, which could lead to high vector concentrations within the tumor bed, although limiting vector administration and gene expression in the adjacent brain. With advances in vector development, limiting gene expression to tumor cells with transcriptional or transductional targeting, and the application of more toxic gene therapy paradigms, the treatment of many skull base tumors may soon be possible.

Forecasting↗

Mechanisms of premature closure of cranial sutures.

Craniosynostosis is defined as premature closure of the sutures of the skull, resulting in cranial deformity. Since Virchow's original paper describing the relationship between premature suture closure and skull morphology, we have learned much about the underlying mechanisms and consequences of premature suture closure. In this paper we will describe the biology of suture closure, the rules governing the resulting phenotypes seen clinically, and a prospective study of skull growth during the 1st year of life.

Case-Control Studies↗

Solitary sarcoid granuloma of the cerebellopontine angle: a case report.

BACKGROUND: Sarcoidosis involves the nervous system about 5% of the time and usually manifests as a granulomatous inflammation of the basal meninges and hypothalamus. Cases which are strictly isolated to the central nervous system occur infrequently; rarely, they may present as an intracranial mass. METHODS: We present the case of a solitary sarcoid granuloma at the cerebellopontine angle in a 42-year-old female who presented with headache, facial numbness, and hearing loss. RESULTS: A suboccipital craniectomy was performed and the lesion was noted to be grossly adherent to the lower cranial nerves and skull base. The lesion was misdiagnosed as a meningioma with preoperative magnetic resonance imaging and intraoperative histology, and perhaps additional morbidity resulted. CONCLUSION: We present this case in order to demonstrate the importance of differentiating these dural-based lesions and propose that cases of neurosarcoidosis presenting as a solitary granuloma be treated with surgical debulking and immunosuppression.

Adult↗