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PubMed2001

Neoplastic growth of cerebral cavernous malformation presenting with impending cerebral herniation: a case report and review of the literature on de novo growth of cavernomas.

BACKGROUND: Cerebral cavernomas are rare vascular anomalies and their expansile growth has been considered to be mainly due to recurrent hemorrhages. They are not generally reported to show aggressive behavior. CASE DESCRIPTION: A 27-year-old male presented with headache, visual disturbances, and a 17-year history of seizures. He was known to have a temporal lobe lesion on CT scan, consistent with a diagnosis of cavernous malformation but with no relevant family history. Serial clinical and radiological follow-up revealed a progressive increase in the size of the lesion with formation of a growing cyst of 7 cm in diameter, which produced mass effect, resulting in the clinical picture of cerebral herniation. The patient underwent emergency surgical resection with symptomatic relief. There was no evidence of significant hemorrhage at surgery. CONCLUSION: Cavernous malformations can show expansile growth without any evidence of a presaging hemorrhagic event and, in the manner of a neoplastic lesion, present with raised intracranial pressure and cerebral herniation.

Adolescent↗
PubMed1999

Histological heterogeneity of dysembryoplastic neuroepithelial tumour: identification and differential diagnosis in a series of 74 cases.

AIMS: In a retrospective study of resected specimens from 416 patients being treated for long-standing epilepsy, 74 cases of dysembryoplastic neuroepithelial tumour (DNT) were encountered that were all characteristically composed of small round oligodendroglia-like cells (OLC), astrocytes and mature neurones in varying proportions. The architectural patterns, histological, immunohistochemical and ultrastructural features and results of cell proliferation studies and postoperative follow-up are described to facilitate the identification of DNT and to differentiate it from other intrinsic neoplasms that commonly present with seizures. METHODS AND RESULTS: The tumours presented with early onset of seizures, at a median age of 7 years, without the signs of raised intracranial pressure. A majority of the lesions were located in the temporal lobe (n = 59), with fewer cases in the frontal (n = 8), parietal (n = 6) and occipital lobes (n = 1), and ranged in size from 10 to 70 mm; 33 were cystic. Histologically three types could be distinguished, multinodular, solitary nodular and diffuse. The first type (37.8%) had the features of a typical DNT with multinodular architecture and mixed cellular composition. The second type (33.8%) consisted of a solitary nodule, while the third (28.4%) was a diffuse tumour, both composed of a similar mixture of cells as the multinodular DNT. The lesions were seen in the neocortex and white matter and tumours in the temporal lobe often involved the amygdala and hippocampus. The presence of myxoid matrix, microcystic change, calcification and leptomeningeal involvement were common. Dysplastic neurones at the periphery of the tumour and abnormalities in cortical lamination in the adjacent neocortex were found in about one-third of the resections. Rare mitotic figures were encountered in eight of the tumours and necrosis was found in two. Immunocytochemistry for glial fibrillary acidic protein (GFAP) and neuronal markers neuron-specific enolase, synaptophysin and neurofilament (RT 97) assists in establishing the diagnosis, highlighting the astrocytic and neuronal components, and the OLC, by the absence of expression of GFAP. Electron microscopy showed that in some cases OLC show neuronal differentiation. Although the proliferating cell nuclear antigen labelling index varied between 0 and 45.5%, 20 of the 51 tumours stained failed to express the antigen, in keeping with the indolent nature of this neoplasm. The response to surgery was excellent; none of the tumours have recurred, and the control of seizures remained good. CONCLUSIONS: Despite some histological heterogeneity, the clinical and pathological features and indolent biological behaviour indicate that these tumours constitute a single distinct entity. The spectrum of morphological appearances of DNT is broader than has been previously reported, the recognition of which is needed to avoid unnecessary neoadjuvant therapy.

Adolescent↗
PubMed2002

Postoperative radiotherapy for ganglioglioma; report of three cases and review of the literature.

Gangliogliomas are rare tumors of the central nervous system. The definite role of postoperative radiotherapy has not been established. In studying three cases who underwent radiotherapy after subtotal resection, we evaluated the clinical, pathological, radiological features with follow-up of mean 8.1 years. All of three patients were male, and mean age was 22.3 years. All of the cases have temporal lobe lesions and, except for one, they all have seizures. Radiotherapy was given to all patients after surgery. Parallely opposed local fields were used. A total of 54 Gy in 5.5 weeks was given to the mid-plane using a Co-60 tele-therapy device. No recurrence, no new symptoms, and no new neurological deficiency were detected during follow-up.

Adult↗
PubMed1992

Pathological left-handedness and preserved function associated with a slowly evolving brain tumor.

The authors report the serial neuropsychological evaluations of a patient with acquired left-handedness who had a massive brain tumor that infiltrated the entire temporal and posterior parietal lobes of the left hemisphere. Although the patient had pre-operative impairment of non-verbal memory, follow-up assessment 31 and 66 months after the tumor was resected revealed cognitive functions to be in the high-average to superior range. This case demonstrates the sparing of neuropsychological functions that can be seen with a slowly evolving lesion. The authors suggest that such functional sparing may be due to transfer of function rather than to the residual function of tumor-infiltrated neuronal tissue. Possible mediators of functional preservation include slow lesion growth, the patient's youth at disease onset and the large size of the lesion.

Adult↗
PubMed2000

Successful epilepsy surgery in catastrophic postencephalitic epilepsy.

The authors studied prognostic factors for surgical treatment in 22 patients with intractable postencephalitic epilepsy. A small subgroup of patients (9/22) had a positive outcome after resective surgery. They had a higher functional level after encephalitis as measured by Glasgow Outcome Score, a longer time interval between encephalitis and onset of seizures, and localization of ictal EEG to one temporal lobe. The other patients had devastating seizures with poor outcome after surgery.

Adolescent↗
PubMed1997

Cerebral vasospasm after tumor resection. A case report.

OBJECTIVE AND IMPORTANCE: Symptomatic cerebral vasospasm can occur after resection of tumors in or adjacent to the basal cisterns, causing delayed neurological deterioration. This potentially treatable condition may go unrecognized. Delay in its recognition will adversely affect the outcome of the patients. There has been a few cases of vasospasm after tumoral resection reported in the literature, mostly in adults. We report a case of vasospasm after resection of a third nerve schwannoma in a pediatric patient. This is the youngest patient reported to date with vasospasm after resection of a brain tumor. CLINICAL PRESENTATION: A six years old girl presented with sudden onset diplopia. Radiological work-up revealed a third nerve mass. She underwent a craniotomy for resection of her mass. Pathological findings were consistent with a third nerve schwannoma. One week postoperatively, her mental status deteriorated. A CT scan revealed a diffuse hypodense area involving the right frontal and temporal lobes in the middle cerebral artery distribution as well as the midbrain. The absence of these findings on the MRI imaging performed on the first postoperative day made us evoke a vascular etiology. A cerebral angiogram was performed and revealed vasospasm in the right internal carotid artery and in the right middle and posterior cerebral arteries. Hyperdynamic hypervolemic hemodilutional therapy was instituted. CONCLUSION: Delayed clinical deterioration from vasospasm is a potentially reversible condition, if recognized early. A high index of suspicion should be maintained in case delayed clinical deterioration occurs after surgery of tumors in the basal cisterns. Cerebral angiography will confirm the diagnosis. Early institution of hyperdynamic hypervolemic hemodilutional therapy and angioplasty may reverse the deficit and improve outcome.

Child↗
PubMed2006

Endoplasmic reticulum stress and apoptosis signaling in human temporal lobe epilepsy.

Apoptosis signaling pathways are implicated in the pathogenesis of temporal lobe epilepsy (TLE), but the role of endoplasmic reticulum (ER) stress and ER-localized apoptosis signaling components remains largely unexplored. Presently, we investigated ER stress and ER localization of proapoptotic Bcl-2 family members and initiator and effector caspases in resected hippocampus from patients with intractable TLE and compared findings with autopsy controls. Hippocampal immunoreactivity for KDEL (Lys-Asp-Glu-Leu), a motif in ER stress chaperones glucose-regulated proteins 78 and 94, and calnexin, was significantly higher in TLE hippocampus compared with controls. The ER-containing microsomal fraction in control brain contained Bid, Bim, and caspase 3, whereas Bad and caspases 6, 7, and 9 were very low or absent. In contrast, caspases 6, 7, and 9 were present within the microsomal fraction of TLE brain. Furthermore, cleaved caspases 7 and 9 were detected in TLE samples but not controls, and KDEL-expressing neurons coexpressed cleaved caspase 9. Potentially adaptive changes were also detected, including lowered Bim levels in this fraction, and binding of caspase 7 to the X-linked inhibitor of apoptosis protein. These data suggest seizures may induce ER stress and trigger proapoptotic signaling pathways in the ER that are counteracted by antiapoptotic signals in chronic human TLE.

Apoptosis↗
PubMed2003

Ictal magnetoencephalography in temporal and extratemporal lobe epilepsy.

PURPOSE: We evaluated visual patterns and source localization of ictal magnetoencephalography (MEG) in patients with intractable temporal lobe epilepsy (TLE) and extratemporal epilepsy (ETE). METHODS: We performed spike and seizure recording simultaneously with EEG and MEG on two patients with TLE and five patients with ETE. Scalp EEG was recorded from 21 channels (10-20 international system), whereas MEG was recorded from two 37-channel sensors. We compared ictal EEG and MEG onset, frequency, and evolution and performed MEG dipole source localization of interictal spikes and early ictal discharges and co-registered dipoles to brain magnetic resonance imaging (MRI). We correlated dipole characteristics with intracranial EEG, surgical resection, and outcome. RESULTS: Ictal MEG lateralized seizure onset in both TLE patients and demonstrated ictal onset, frequency, and evolution in accordance with EEG. Ictal MEG source analysis revealed tangential vertical dipoles in the anterolateral angle in one patient, and anterior dipoles with anteroposterior orientation in the other. Intracranial EEG revealed regional entorhinal seizure onset in the first patient. Both patients became seizure free after temporal lobectomy. In ETE, ictal MEG demonstrated visual patterns similar to ictal EEG and had concordant localization with interictal MEG in all five patients. Two patients underwent surgery. Ictal MEG localization was concordant with intracranial EEG in both cases. One patient had successful outcome after surgery. The second patient did not improve after limited resection and multiple subpial transections. CONCLUSIONS: Ictal MEG can demonstrate ictal onset frequency and evolution and provide useful localizing information before epilepsy surgery.

Electroencephalography↗
PubMed2003

Cellular pathology of amygdala neurons in human temporal lobe epilepsy.

The amygdala complex substantially contributes to the generation and propagation of focal seizures in patients suffering from temporal lobe epilepsy (TLE). A cellular substrate for increased excitability in the human amygdala, however, remains to be identified. Here, we analyzed the three-dimensional morphology of 264 neurons from different subregions of the amygdaloid complex obtained from 17 "en bloc" resected surgical specimens using intracellular Lucifer Yellow (LY) injection and confocal laser scanning microscopy. Autopsy samples from unaffected individuals ( n=3, 20 neurons) served as controls. We have identified spine-laden, spine-sparse and aspinous cells in the lateral, basal, accessory basal and granular nuclei. Semiquantitative analysis points to significant changes in neuronal soma size, number of dendrites and spine densities in specimens from epilepsy patients compared to controls. Neuronal somata in the epilepsy group were smaller compared to controls ( P<0.01), neurons had fewer first-order dendrites ( P<0.01), whereas the maximum density of spines per dendritic segment in these cells was increased in TLE patients ( P<0.01). There were also dendritic alterations such as focal constrictions or spine bifurcations. These changes were consistent between amygdaloid subregions. The dendritic morphology of amygdaloid neurons in TLE patients points to substantial changes in synaptic connectivity and would be compatible with altered neuronal circuitries operating in the epileptic human amygdala. Although the morphological alterations differ from those described in hippocampal subregions of a similar cohort of TLE patients, they appear to reflect a characteristic pathological substrate associated with seizure activity/propagation within the amygdaloid complex.

Adult↗
PubMedDate not supplied

Basal temporal subdural electrodes in the evaluation of patients with intractable epilepsy.

In evaluation of patients with complex partial seizures who are candidates for surgical treatment, exact definition of the epileptogenic focus is essential for a good surgical outcome. We report a new technique which permits detailed mapping of the epileptogenic activity in the basal temporal lobe and the convexity of the temporal lobe. The technique consists of placement of at least 16 basal temporal electrodes and an additional 64 electrodes covering the temporal convexity. This extensive coverage permits accurate definition of the limits of the epileptogenic focus and also of adjacent functional areas and therefore allows more significant determination than have previous techniques of the ideal extent of the surgical resection. This accuracy cannot be achieved with depth electrodes or the limited coverage provided by previously reported epidural or subdural electrode techniques.

Action Potentials↗
PubMed1998

Surgical techniques for arteriovenous malformations in functional areas: focus on the superior temporal gyrus.

Direct surgical intervention of arteriorvenous malformations (AVMs) in functional areas has been accepted as a standard mode of treatment. However, safe and successful intervention requires that such factors as exact location, size, vascular supply, and drainage be considered. Importantly, surgical techniques must be individualized to each patient, based on hemodynamic anatomy of the AVM. This paper discusses AVMs in the superior temporal lobe, which have a complex neuronal anatomy and circulatory system; the authors present 22 patients with AVMs of various sizes and describe the surgical techniques specific for the indicated location. Surgical procedures adhered to the following principles: 1) avoid brain tissue removal; 2) preserve microcirculation; 3) maintain circulation of the isolated major draining vein to access the AVM core; 4) compartmental isolation; and 5) preservation of functional area cortex covering the AVM. All patients underwent total resection except one, who had a subtotal resection. Neurological and occupational recovery was remarkable except for partial hemianesthesia in one patient; two patients are still in rehabilitation. This is the first description of a direct surgical approach to AVMs in the superior temporal gyrus, where management is challenging because the lesions may extend elsewhere, such as to Broca's and Wernicke's areas. The results suggest that the procedure is promising.

Adolescent↗
PubMed2000

Telomerase activity and the expression of telomerase components in pituitary adenoma with malignant transformation.

BACKGROUND: Telomerase activity responsible for cellular immortality may participate the development of human cancers. Telomerase is a multisubunit ribonucleoprotein composed of at least three components: hTERT, hTERC, and TEP1. This is the first report showing telomerase activity and telomerase component expression in pituitary adenoma with histological malignant transformation. DESCRIPTION: A 16-year-old male with a prolactin-producing pituitary adenoma with metastasis is presented. The patient underwent three partial resections of an intra- and suprasellar lesion over a 2-year period and received focal irradiation. Eight years after the first admission, a metastatic lesion to the subarachnoid space around the medulla oblongata was detected and the lesion was resected as the fourth operation. Furthermore, the suprasellar lesion showed regrowth and partial resection was performed as the fifth operation. The last two specimens were diagnosed as pituitary carcinoma. Radiotherapy with gamma knife was performed for the residual suprasellar lesion and a new lesion in the left temporal lobe after the fifth operation. Telomerase activity was examined by TRAP/TRAP-HPA methods, qualitatively and quantitatively. Telomere length was examined by Southern blot analysis, and the expression of telomerase components (hTERT, hTERC, and TEP1) was examined by reverse transcriptase-polymerase chain reaction (RT-PCR). The MIB1 index, telomerase activity, and hTERT expression increased according to histologic malignancy chronologically in this patient. None of the specimens showed immunoreactivity for p53, EGFR, or bc12. No telomerase activity was detected in pituitary adenomas without malignant transformation, other benign brain tumors, or normal brain tissues. CONCLUSION: We report a patient with pituitary adenoma transforming to carcinoma. The tumor cells acquired immortality and revealed malignant transformation during the course of the disease, that was proved by an increase of telomerase activity and hTERT expression.

Adenoma↗
PubMed2006

Morbidity associated with the use of intracranial electrodes for epilepsy surgery.

BACKGROUND: Invasive monitoring for the investigation of medically intractable epilepsy may be associated with undesirable morbidity. We performed a review of our recent experience to determine the incidence of major complications. METHODS: We reviewed the clinical records of all patients who underwent invasive EEG monitoring at our institution between 2000 and 2004. RESULTS: One-hundred and sixteen patients (57 males, 59 females) with a mean age of 32 years of age underwent intracranial placement of electrodes for epilepsy surgery investigation. Subdural strips were placed in 115 patients with a mean of eight strips per patient. Subdural grids were inserted in 11 patients and depth electrodes in five. Fourteen of the 15 patients with grids or depth electrodes also had strips. Coverage was unilateral in 37 patients and bilateral in 79 patients. Electrodes were placed over the frontal lobe in 78 cases, temporal in 93, parietal in 24, and occipital in 27 patients. The average duration of investigation was 12.3 days (range 3-29). The evaluation led to the performance of a surgical resection in 85 patients (74%). Complications were seen in four patients with subdural strips (3%), and in two patients with grids (13%), characterized by clinical infection, intracranial hemorrhage, aseptic meningitis, transient neurological deficits, and status epilepticus. Mortality was nil. CONCLUSIONS: In comparison with previously published literature on the topic, the major complication rate in this group of patients appears to be low.

Adolescent↗
PubMed1997

Olfactory prodromal symptoms and unilateral olfactory dysfunction are associated in patients with right mesial temporal lobe epilepsy.

PURPOSE: We report a patient with mesial temporal lobe epilepsy (MTLE) with olfactory prodromal symptoms manifested as an unpleasant smell of onions, who was found to have an ipsilateral deficit of olfactory naming (olfactory agnosia). METHODS AND RESULTS: Preoperative olfactory testing revealed a selective right-sided olfactory deficit for naming of odors. Olfactory threshold was within the normal range. The patient has been seizure free after selective amygdalohippocampectomy for 4 months. No olfactory prodromal events have occurred since surgery. Olfactory testing 3 months after resection showed that right-sided odor naming was still impaired. CONCLUSIONS: We conclude that olfactory prodromal symptoms may be associated with unilateral olfactory dysfunction, and lateralization of seizure origin may be possible by unilateral olfactory testing.

Agnosia↗
PubMed2000

Transcortical selective amygdalohippocampectomy in temporal lobe epilepsy.

Numerous studies of the electrophysiology and neuropathology of temporal lobe epilepsy have demonstrated the mesial temporal structures to be the site of seizure origin in the majority of cases. This is the rationale for a transcortical selective approach, first introduced by Niemeyer, for removal of the hippocampus and amygdala. Series from a number of centers have demonstrated the efficacy of selective amygdalohippocampectomy compared to a more traditional resection. The technique described here and used at the Montreal Neurological Institute (MNI) utilizes a strictly endopial resection of the hippocampal formation and amygdala in addition to computer image guidance to perform the procedure. Ninety-five percent of patients at the MNI who underwent selective amygdalohippocampectomy realized a cessation of seizures, or greater than 90% reduction, with minimal risk of complications.

Amygdala↗
PubMed1995

Low-grade astrocytoma with neuraxis dissemination at diagnosis.

Little is known about low-grade astrocytoma with neuraxis dissemination at diagnosis. A review of medical records identified this phenomenon in eight of 150 pediatric patients evaluated between 1985 and 1994 for histologically confirmed low-grade astrocytoma. These patients (five male and three female) ranged in age from 5 months to 20 years (median 8 years). Symptoms of neuraxis disease were minimal or absent. Primary tumor sites were the hypothalamus in four cases, brainstem/spinal cord in three, and temporal lobe in one. Patterns of dissemination (evaluated by computerized tomography and/or magnetic resonance imaging techniques) appeared to be related to the primary site: hypothalamic tumors metastasized along the ventricular cerebrospinal fluid pathways, and tumors in other locations disseminated along subarachnoid pathways. Following initial treatment with chemotherapy (in three), partial resection (in one), radiation therapy (in three), and chemotherapy plus irradiation (in one), four patients required salvage therapy for progressive or recurrent disease. Seven of the eight patients are alive with stable or progressive disease 6 to 105 months postdiagnosis (median 15 months). Low-grade astrocytoma with initial neuraxis dissemination is responsive to chemotherapy and radiation, a proportion showing periods of stable disease. The optimum therapy or combination of therapies remains unclear.

Adolescent↗
PubMedDate not supplied

Focal cerebral magnetic resonance changes associated with partial status epilepticus.

We report 2 patients with transient abnormalities on magnetic resonance imaging (MRI) associated with partial status epilepticus (SE). A man with a 4-month history of partial seizures had complex partial SE for 9 days, with left temporal maximum on ictal EEG. Left temporal lobe T2 signal was increased on MRI during SE, but cerebral MRI was normal 9 weeks later. A woman with "cryptogenic" temporal lobe epilepsy for 16 years had complex partial SE for 1 week, with right temporal maximum on ictal EEG. T2 Signal was increased over the entire right temporal lobe, extending into the insula, without mass effect, on MRI 1 month after SE ended. Repeat MRI 1 month later showed marked decrease in volume of increased T2 intensity, without gadolinium enhancement, but with mild mass effect over the right anterioinferomesial temporal areas. A gemistocytic astrocytoma was resected. Focal cerebral MRI abnormalities consistent with cerebral edema may be due to partial SE but also may indicate underlying glioma, even in long-standing partial epilepsy. Focal structural imaging changes consistent with neoplasm should be followed to full resolution after partial SE.

Adult↗