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REVIEWER'S COMMENTS ON ARTICLE 1

Continuous intraoperative electromyographic monitoring of cranial nerves during resection of fourth ventricular tumors in children. Paul A. Grabb, A. Leland Albright, Robert J. Sclabassi, and Ian F. Pollack

Fred J. Epstein

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Fred J. Epstein and with Joshua Horwitz

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Fred J. Epstein and Memet Ozek

✓ A new instrument for use in surgically treating intra-axial neoplasms of the spinal cord and brain stem is described. The plated bayonet allows neoplastic tissue in the spinal cord to be separated from functioning neural elements without perforating the adjacent spinal cord. In addition, the plated bayonet facilitates exposure through the very small incision necessary to remove tumors of the brain stem without damaging cranial nerves or other vital structures.

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Karl F. Kothbauer, Vedran Deletis, and Fred J. Epstein

Resection of intramedullary spinal cord tumors carries a high risk for surgical damage to the motor pathways. This surgery is therefore optimal for testing the performance of intraoperative motor evoked potential (MEP) monitoring. This report attempts to provide evidence for the accurate representation of patients' pre- and postoperative motor status by combined epidural and muscle MEP monitoring during intramedullary surgery.

The authors used transcranial electrical motor cortex stimulation to elicit MEPs, which were recorded from the spinal cord (with an epidural electrode) and from limb target muscles (thenar, anterior tibial) with needle electrodes. The amplitude of the epidural MEPs and the presence or absence of muscle MEPs were the parameters for MEP interpretation. A retrospective analysis was performed on data from the resection of 100 consecutive intramedullary tumors and MEP data were compared with the pre- and postoperative motor status.

Intraoperative monitoring was feasible in all patients without severe preoperative motor deficits. Preoperatively paraplegic patients had no recordable MEPs. The sensitivity of muscle MEPs to detect postoperative motor deficits was 100% and its specificity was 91%. There was no instance in which a patient with stable MEPs developed a motor deficit postoperatively. Intraoperative MEPs adequately represented the motor status of patients undergoing surgery for intramedullary tumors. Because deterioration of the motor status was transient in all cases, it can be considered that impairment of the functional integrity of the motor pathways was detected before permanent deficits occurred.

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Fred J. Epstein and Jean-Pierre Farmer

✓ During the last decade, several authors have reported that certain brain-stem gliomas may be associated with a better prognosis than others. In this paper, retrospective correlations between the pathological findings and the magnetic resonance (MR) imaging appearance of 88 brain-stem gliomas are established. The authors propose an anatomical hypothesis that helps identify glioma growth patterns in general and that clarifies why cervicomedullary, dorsally exophytic, and focal tumors have a more favorable prognosis. According to this hypothesis, growth of benign gliomas of the brain stem is guided by secondary structures such as the pia, fiber tracts, and the ependyma, which in turn leads to stereotypical growth patterns that are clearly identified on MR images. The authors believe that this hypothesis, in conjunction with clinical data, may help establish selection criteria for the surgical treatment of specific brain-stem lesions.

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Fred J. Epstein, Jean-Pierre Farmer, and Diana Freed

✓ In this series, 25 adult patients with intramedullary astrocytomas were treated by radical excision alone. Six patients proved to have anaplastic astrocytoma; five of them died within approximately 2 years and the sixth has demonstrated disease progression. The other 19 patients were diagnosed as having low-grade astrocytoma (16 cases) or ganglioglioma (three cases); two of these had advanced preoperative neurological disability and died of medical complications. Fifteen of the remaining 17 patients have no clinical evidence of tumor recurrence after a mean follow-up period of 50.2 months; the other two have a small residual neoplasm that demonstrates no progression. Of these 17 patients, seven had previously received radiation therapy, but had clear evidence of tumor growth subsequently.

This experience suggests that surgery is not beneficial for anaplastic spinal astrocytoma. However, in cases of low-grade tumor, radical excision is associated with minimal morbidity and an excellent long-term prognosis when carried out before significant disability occurs.

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Fred J. Epstein, Jean-Pierre Farmer, and Diana Freed

✓ Thirty-eight patients underwent surgery for an intramedullary spinal cord ependymoma. In 37 patients, postoperative magnetic resonance imaging confirmed that the tumor was totally removed. The morbidity of surgery was directly related to the preoperative neurological condition. Patients who were normal or nearly normal preoperatively were rarely worse after surgery, and those who had significant disability preoperatively were at greatest risk of being more impaired after surgery. There has been no tumor recurrence in any patient after a mean follow-up period of 24 months, and radiation therapy has not been employed as a surgical adjunct.

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Fred J. Epstein, Jean-Pierre Farmer, and Steven J. Schneider

✓ The echographic characteristics of 186 suspected intramedullary spinal cord tumors were reviewed. Ultrasonography was found to be specific in distinguishing the tumor type, the extent of the lesion, and the presence and type of associated cysts. Ultrasonography greatly facilitates the selection of respective sites for the placement of a myelotomy, and for initiation of the resection. Additionally, this indispensable adjunct provides ongoing images that allow the preoperative plan to be precisely followed in a surgical field where anatomical landmarks are limited and the margin for error is minimal.

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Douglas C. Miller, Frederick F. Lang, and Fred J. Epstein

Histopathological features that suggest the diagnosis of ganglioglioma require, in most cases, confirmation by special stains to distinguish these tumors from other gliomas. For this purpose, immunostaining for synaptophysin, which has previously been shown to selectively label the cell surface of neoplastic ganglion cells, was used to retrospectively examine glioma tumor specimens. Sixty-three cases of ganglioglioma were identified. The files of the Division of Neuropathology of New York University Medical Center contained 45 tumors that had been diagnosed as ganglioglioma, of which 42 were verified by synaptophysin; three cases were reclassified, two as astrocytomas and one as a gangliocytic paraganglioma. Thus, a tumor identified as ganglioglioma based on other criteria was likely to be a ganglioglioma. The other 21 cases of gangliogliomas were originally diagnosed as astrocytoma or mixed glioma, but were shown by synaptophysin staining to be gangliogliomas. In some cases the ultimate diagnosis was obtained after radical surgery provided relatively abundant amounts of tissue, thereby limiting sampling errors, in contrast to the biopsies from which the original diagnoses were made.

Histopathological review of these cases demonstrated that four features represent important clues to the correct diagnosis: 1) clusters of large cells potentially representing neurons (without such cells the tumor cannot be classified as a ganglioglioma); 2) no perineuronal clustering of the glial cells around the alleged neoplastic neurons; 3) fibrosis (desmoplasia); and 4) calcification. Binucleate neurons, previously suggested to be common in gangliogliomas, were not frequently found in this series, and lymphocytic infiltrates, while common, are so often found in other tumors that they gave no specific hint that any single neoplasm was a ganglioglioma. The glial elements were astrocytic in all cases, except that one tumor also had oligodendroglial and ependymal patterns. Four tumors also had small mature neurons, as seen in neurocytomas. Cells from one tumor were successfully grown in short-term tissue culture; the culture contained large dividing neurons with synaptophysin immunoreactivity as well as smaller dividing cells, demonstrating that the neuronal cells are a proliferating element in gangliogliomas.