Cases reported "Glioma"

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1/39. Transient paralytic attacks of obscure nature: the question of non-convulsive seizure paralysis.

    Eleven patients with transient paralytic attacks of obscure nature are described. paralysis could involve face or leg alone, face and hand, or face, arm and leg. The duration varied from two minutes to one day. Four patients had brain tumors, six probably had brain infarcts, and one a degenerative process. The differential diagnosis included TIAs, migraine accompaniments, and seizures. In the absence of good evidence for the first two, the cases are discussed from the standpoint of possibly representing nonconvulsive seizure paralysis (ictal paralysis, inhibitory seizure paralysis or somatic inhibitory seizure). Because of the difficulty in defining seizures as well as TIAs and migraine in their atypical variations, a firm conclusion concerning the mechanisms of the spells was not attained. Two cases of the hypertensive amaurosis-seizure syndrome have been added as further examples of ictal deficits.
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2/39. A case history of glioma progression.

    Low-grade diffuse astrocytomas have an intrinsic tendency for malignant progression but the factors determining the kinetics of this process are still poorly understood. We report here the case of a male patient who developed a fibrillary astrocytoma at the age of 33 years and who underwent six surgical interventions over a period of 17 years without radiotherapy or chemotherapy. The first three biopsies spanned a period of 11 years and led to the diagnosis of low-grade, diffuse astrocytoma (WHO grade II), with a growth fraction (MIB-1 labeling index) of 2.3-3.7%. The fourth to sixth biopsies showed histological features of anaplastic astrocytoma (WHO grade III), with growth fractions between 5.0 and 10.5%. The fraction of gemistocytic neoplastic astrocytes also increased, from 0.3% in the first biopsy to 17.5% in the last biopsy and preceded the increase in proliferative activity and transition to anaplastic astrocytoma. The fraction of tumor cells immunoreactive to BCL-2 increased from 0.3% to 8.2%. A p53 mutation in codon 273 (CGT-->TGT, Arg-->Cys) was identified in the first biopsy and persisted throughout the course of the disease. However, the fraction of cells with p53 protein accumulation increased significantly during progression, from 3.2% in the first biopsy to 13.7% in the last. The absence of additional genetic alterations (PTEN mutations, loss of chromosome 10 and 19q) may be responsible for the slow progression and lack of glioblastoma features even after a 17-year disease duration.
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3/39. Intraspinal extradural myxopapillary ependymoma of the sacrum arising from the filum terminale externa. Case report.

    Extradural ependymomas of the sacrococcygeal region are very rare, with most arising from the soft tissues of the presacral area or from the regions dorsal to the sacrum. In even rarer circumstances, the tumor may arise within the sacral canal, likely as a result of ependymal cells of the extradural filum terminale. Because of bone erosion caused by extension of the tumor into the pelvis or dorsal to the sacrum, a truly intraspinal extradural ependymoma in this region has until now never been clearly demonstrated. The authors present a patient with a myxopapillary ependymoma arising from the filum terminale externa in which there was no involvement of the intradural filum or extension outside the sacral canal. A review of the literature is presented, with emphasis on the pathogenesis and clinical management of these rare tumors.
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4/39. Fatal tumoural haemorrhage following decompressive craniectomy: a report of three cases.

    Three cases of large and deep seated anaplastic cerebral glioma were treated by bone and dural decompression. The patients worsened suddenly within 12 h of surgery and later died. Postmortem examination revealed a large intratumoural clot in each case. The effects of decompression and the probable causes of fatal bleeding are analysed in this report.
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5/39. optic nerve glioma in an 18-month-old child.

    An optic nerve glioma in an 18-month-old child was examined by both light and electron microscopy. The tumor revealed the characteristic features of uniform benign and fibrillary astrocytoma. Rosenthal fibers and calcium depostis were found within numerous intracellular glial processes. The above features indicated a slow-growing tumor of long duration, confirming the generally supported assumption of the congenital nature of optic nerve glioma. One unsuspected feature was the presence of fenestrated blood vessels.
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6/39. Complete response of a recurrent, multicentric malignant glioma in a patient treated with phenylbutyrate.

    sodium phenylbutyrate is a biological-response modifier that acts as a dose-dependent inhibitor of glioma cell proliferation, migration, and invasiveness in vitro, possibly by inhibition of urokinase and c-myc pathways. Despite its biological activity in vitro, there have not been any prior reports of efficacy in the treatment of human malignant gliomas. We report a 44-year-old female with a recurrent, multicentric, malignant glioma who experienced a durable remission lasting more than four years. The patient initially presented with seizures caused by a biopsy-proven anaplastic astrocytoma of the frontal lobe. The patient was treated with radiation therapy and procarbazine-CCNU-vincristine (PCV). However, the tumor progressed and extended to the corpus callosum with midline shift, refractory to four cycles of continuous 72-h infusion of BCNU/Cisplatinum. Additional enhancing lesions appeared in the left frontal and left temporal lobes. The patient was started on sodium phenylbutyrate, 18 g daily in three divided oral doses, and reduced to 9 g/day and eventually to 4.5 g/day to eliminate mild, reversible side effects. Four years later, the patient has a KPS functional score of 100%. Phenylbutyrate is a well-tolerated, oral agent that shows potential for the treatment of malignant gliomas. Further studies should be considered to identify a subset of patients that have tumors sensitive to phenylbutyrate, either as a single agent or in combination with radiation therapy or other chemotherapeutic agents.
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7/39. Occupational head injury and subsequent glioma.

    We report the case of a policeman who suffered a severe head injury to the right temporoparietal lobe while driving a police car. Four years later, the patient developed a neoplasm at the precise site of the meningocerebral scar. Histological examination confirmed a glioblastoma multiforme adjacent to the dural scar. Radiological documentation of the absence of tumor at the time of injury, exact localization of the neoplasm in the injured cerebral area, and latency of the cancer supported the hypothesis of a causal relationship with brain trauma. physicians faced with brain neoplasms in adults should carefully investigate the patient's personal history of head trauma. When a relationship with occupational head injury is probable, reporting of suspect occupational illness is compelling.
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8/39. Brain surgery in motor areas: the invaluable assistance of intraoperative neurophysiological monitoring.

    AIM: Surgery for tumors in the central and precentral region, as much as for insular tumors, places at risk the functional integrity of the motor cortex and the subcortical motor pathways. These procedures may therefore benefit from the assistance of intraoperative neurophysiological monitoring (INM). INM consists of "mapping" and true "monitoring" (the continuous "on-line" assessment of the functional integrity of neural pathways) techniques. In spite of the large interest in mapping techniques, monitoring techniques have received less attention. We describe our experience with intraoperative neurophysiological mapping and monitoring of motor tracts during surgery for brain gliomas in or near motor areas, in order to support the feasibility and reliability of monitoring as an essential adjunct to mapping during surgery in these areas. methods: Between September 2000 and January 2002, 51 patients were surgically treated for brain gliomas located in the precentral gyrus (45.1%), the postcentral gyrus (23.5%), anterior to the precentral gyrus (15.6%), or in the insula (15.6%). INM of the motor system consisted of monitoring muscle motor evoked potentials (mMEPs) recorded via needle electrodes inserted into the controlateral upper and lower extremity muscles and elicited by transcranial multipulse electrical stimulation (TES). Once the dura was open and the central sulcus was identified using the phase reversal technique, mMEPs were elicited by direct stimulation of the motor cortex (DCS). Motor mapping was performed with a monopolar electrode using the same stimulation parameters as used for monitoring except for much lower intensity (up to 20 mA). RESULTS: Ninety-eight percent of the patients exhibited recordable baseline mMEPs. The success rate of the phase reversal technique was 95.8%. Eight patients presented disappearance of mMEPs during tumor removal. Using corrective measures, all intraoperative changes in mMEPs were reversed in time to prevent an irreversible complete injury to the motor system and no patient lost mMEPs at the end of the operation. At discharge, 66% of the patients remained at their preoperative status, 4% improved, and 24% had a mild worsening as compared to the preoperative status assessed using the Medical research Council scale; 6% of the patients presented a moderate to severe supplementary motor area syndrome. CONCLUSION: Monitoring techniques significantly implement the reliability and effectiveness of INM since these provide: 1) continuous "on-line" assessment of the functional integrity of motor pathways with higher chance to early detect a progressive mechanical or vascular injury to the neural tissue, as compared to mapping techniques; 2) lower risk to induce intraoperative seizures and strong muscular twitches as compared to the single pulse mapping technique; 3) possibility to monitor motor pathways using TES also when there is no direct access to the motor cortex.
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9/39. Long term survival in a patient with recurrent malignant glioma treated with intratumoral infusion of an IL4-targeted toxin (NBI-3001).

    Intratumoral infusion of a recombinant targeted toxin (NBI-3001) consisting of the receptor binding domain of human interleukin 4 (IL-4) and pseudomonas aeruginosa exotoxin A is an investigational treatment for malignant brain tumors. This 27-year-old male patient presented with a recurrent malignant glioma WHO grade IV after surgery and adjuvant radiation and chemotherapy. The recurrence was treated with intratumoral infusion of NBI-3001 at a dose of 9 microg/ml in 66 ml of infusate. Treatment resulted in long-term survival for 3 years after toxin infusion with a durable tumor response. There were some permanent neurological side effects resulting from toxin infusion. The patient eventually died after a late local recurrence of the known brain tumor. Such clinical evolution of a malignant glioma after a single round of immunotoxin infusion is rather unusual. The late local recurrence may suggest that repeated courses rather than a single infusion of intratumoral toxin are possibly needed for successful long-term tumor control.
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10/39. "Shave" biopsy of the optic nerve in isolated neurosarcoidosis.

    We report a case of isolated central nervous system sarcoidosis, limited to the optic nerve, characterized by negative systemic work up and neuroradiological features consistent with both optic sheath meningioma and optic nerve glioma. A magnetic resonance imaging examination revealed a dural tail sign associated with a diffuse enhancement and enlargement of the optic nerve. Both positive laboratory findings and systemic manifestation of sarcoidosis or autoimmune diseases were absent. Because of progressive visual loss, tissue confirmation was sought and the optic nerve itself biopsied after non-diagnostic dural samplings. The approach to this was to perform a "shave" biopsy of the optic nerve within its intracranial compartment. This procedure spared the central macular fibers and was not associated with loss of vision. The use of the fiber-sparing "shave" biopsy provided diagnostic samples containing non-caseating granulomas, without compromising the patient's preoperative central visual field. The authors advocate the use of this simple and safe technique when a definitive diagnosis among optic nerve meningioma, optic nerve glioma and isolated neurosarcoidosis cannot be reached by means of less invasive procedures.
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