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PubMedDate not supplied

Isolated intracranial Rosai-Dorfman disease mimicking meningioma.

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.

Adult↗

PubMed1994

[Basic and clinical approaches for surgical treatment of intractable epilepsies].

Recent reports demonstrated that patients with medically intractable epilepsy were benefited with surgical treatments. Even in pediatric patients, early surgery is effective not only in seizure control but also in verbal, intelligent and social outcomes. In the present study, basic and clinical approaches in the treatment of intractable epilepsies were studied. In chronic cat model of intractable temporal lobe epilepsy induced by a microinjection of kainic acid, temporal cortical excision with amygdala focus resection or focus and hippocampal resections were examined by means of microsurgical technics. The cats with amygdala focus resection demonstrated spontaneous hippocampal seizures while cats with focus and hippocampal resection did not show seizures in the long time course after the surgery. In a cat model of cortical focal seizures, multiple subpial transection(MST) was examined. The cortical seizures, induced by a microinjection of kainic acid into unilateral sensori-motor cortex, were suppressed immediately after the MST. Postoperative course was uneventful and seizure was not recurred. However, pathological study demonstrated subpial scar formation at the site of MST in the sensori-motor cortex. In 38 patients with medically intractable epilepsies, surgical treatments were performed in the Department of Neurosurgery. Asahikawa Medical College. The site of the epileptogenic lesion was studied with Video-EEG longterm monitorings, Neuro-imagings with CT, MRI, interictal SPECT and/or ictal SPECT. At the surgery, intraoperative electrocorticograms(ECoGs) were monitored under light neuroleptanalgesia in order to localize epileptic focus. Seizure free rate with or without postoperative medication was 72%. Remarkable improvement was observed in 7 patients (18%), but, 4 patients (10%) did not show any improvement.

Adolescent↗

PubMed1995

Protoplasmic astrocytoma. A clinicopathologic study of 16 tumors.

UNLABELLED: Protoplasmic astrocytomas, composed of process-poor astrocytic cells and a microcystic background, are rare variants of low grade astrocytoma. Three hundred eight low grade astrocytomas over a 23-year period were reviewed. Sixteen (5.2%) were classified as pure protoplasmic astrocytoma. Patients (12 males, 4 females) ranged in age from 2.5 to 41 years (mean, 20.7 years). All patients presented with seizures and three had headaches. Duration of symptoms ranged from 7 months to 28 years (mean, 6.6 years). Nine tumors (56%) were left-sided and seven right-sided (44%). Seven (44%) occurred in the temporal lobe, six (37%) in the frontal lobe, two (13%) in the parietal lobe, and one (6%) in the thalamus. Surgery consisted of partial lobectomy with total tumor resection in nine and biopsy alone in seven. Five patients received adjuvant therapy with no apparent effect on survival. At frozen section, protoplasmic astrocytoma was most confused with fibrillary low grade astrocytoma (n = 6). Follow-up revealed 10 patients with no evidence of disease 2 to 108 months postoperatively (mean, 41 months), 5 patients were alive with disease 10 to 84 months postoperatively (mean, 56 months) and 1 patient died with disease at 36 months. Of patients with total tumor resection, eight had no evidence of disease and one died with disease. IN CONCLUSION: (1) protoplasmic astrocytomas in this study were more frequently observed in males at a younger mean age than fibrillary low grade astrocytomas as reported in literature; (2) temporal and frontal lobes were the most likely site of origin; and (3) complete excision may be beneficial, whereas adjuvant therapy appeared to have no effect on outcome.

Adolescent↗

PubMed1992

Does extent of surgery influence outcome for astrocytoma with atypical or anaplastic foci (AAF)? A report from three Radiation Therapy Oncology Group (RTOG) trials.

103 patients with the diagnosis of AAF were identified from the RT/BCNU arms of 3 RTOG malignant glioma trials. Pre-treatment tumor size was less than 5 cm for 48% and greater than or equal to 5 cm for 52%, and tumor sites were frontal lobe in 55%, temporal in 25%, and parietal in 16%. Surgery consisted of biopsy for 30%, partial resection for 56%, and total resection for 14%. Extent of surgery correlated with age, with 81% of patients less than 40 undergoing partial/total resection vs. 60% of those over 40 (P = 0.019). The median survival time (MST) of patients undergoing partial/total resection was 49 mo., vs. 18 mo. for those biopsied only (P = 0.002). Patients with frontal location had longer MST than those with non-frontal lesions (MST: 49 vs. 25 mo., P = 0.047), while no survival difference was apparent by univariate analysis of tumor size. Multivariate analysis demonstrated that only younger age, frontal location, and smaller tumor size correlated significantly with extended survival. Extent of surgery was not predictive. The close correlation between young age and extensive surgery obscures the survival advantage for greater surgery seen with univariate analysis. Smaller tumor size and frontal location favorably influence outcome even when adjusted by age.

Adult↗

PubMed1993

Functional neurosurgery in the treatment of epilepsy in The Netherlands. Aspects of presurgical evaluation and the contribution of subdural and stereotactically implanted depth electrodes in the Dutch Workgroup for Functional Surgery.

In the Netherlands all patients who are considered candidates for surgical treatment of their epilepsy are referred to the Dutch Workgroup for Functional Neurosurgery. Resective neurosurgery in partial epilepsy is a focus of special interest of national health authorities. Annually 15-20 patients can now be operated upon. Presurgical evaluation includes a.o. 18/FDG PET. For depth-EEG recording a specially designed method of "Combined Subdural and Depth-EEG Recording" is used which has been described previously. 70 patients underwent depth-EEG recording with the combined method. In 4.2% of the patients there were transient complications, in 1.4% there was a possibly permanent slight neurological deficit caused by the method. Fifty-six patients were evaluated at least one year after their surgical treatment (median follow-up 3 years). In the frontal lobe group comprising 9 patients 56% were seizure-free, 76% benefited from the treatment. In the temporal lobe group with 47 patients 68% were seizure-free and 92% benefited. No patient deteriorated from the resection. Permanent morbidity was nil.

Brain Mapping↗

PubMed1993

Cortical resections for intractable epilepsy of extratemporal origin: experience with seventeen cases over eleven years.

Seventeen patients underwent surgery between 1981 and 1990 for intractable partial epilepsy arising outside the temporal lobe. Twelve had frontal seizure onset, two parietal, two occipital and one diffusely in the hemisphere. Localization was achieved using extraoperative electrocorticography (ECoG) in five cases and intraoperative ECoG was employed in 12. Fifteen patients underwent cortical resections, but two did not subsequently have a resection. Both of these had porencephalic cysts. Of the 15 who had resections six (40%) were seizure free after a mean of 7.3 years. One (7%) was almost seizure free, six (40%) had worthwhile improvement. Pathological examination revealed oligodendroglioma in three, recurrent meningioma in one, vascular malformations in two, glial hamartoma in one and gliosis in six. One case with gliosis initially was shown to have an underlying malignant astrocytoma 2 years later. All these patients had CT abnormalities prior to surgery. Two patients (13%) had no worthwhile improvement. Pathology in these two was ischaemic neurons and arachnoid thickening. Both had normal CT findings preoperatively. One patient had an increased hemiparesis postoperatively. There were three cases of postoperative infection. It is concluded that extratemporal resection can achieve good results for seizure control and intraoperative ECoG is an effective technique for localizing the epileptogenic area. The presence of a structural lesion carries a particularly favourable prognosis for seizure outcome and surgery should, therefore, be strongly considered in patients with intractable partial epilepsy who have evidence of underlying structural pathology.

Adolescent↗

PubMed1992

Temporal lobectomy for seizures associated with unilateral schizencephaly.

Schizencephaly is characterized by unilateral or bilateral cerebral clefts associated with neurologic deficits and epilepsy. Most commonly schizencephaly is attributed to abnormal neuronal migration, and these malformations are well visualized by current neuroimaging techniques. This report describes a patient with unilateral schizencephaly and poorly controlled complex partial seizures who was found to have a temporal lobe seizure focus; anterior temporal lobectomy produced nearly complete control of the seizures. Despite the extensive malformation, relatively restricted resection was of significant benefit. The principles of seizure focus localization and resection are applicable to the management of patients with schizencephaly.

Adult↗

PubMed1999

Isolated recurrence of granulocytic sarcoma of the brain: successful treatment with surgical resection, intrathecal injection, irradiation and prophylactic systemic chemotherapy.

We describe a 40-year-old male who developed an isolated recurrence of granulocytic sarcoma (GS) of the brain 2 years following successful treatment of acute myeloblastic leukemia (AML; M2). Computed tomography (CT) scans and magnetic resonance (MR) images demonstrated a homogeneously enhanced tumor mass in the left temporal lobe and massive peritumoral edema. There was no evidence of relapse in the bone marrow. The patient underwent an emergency surgical resection of the tumor. Five courses of injection with cytarabine and prednisolone through an Ommaya reservoir and whole brain irradiation (total 40 Gy) were performed. Furthermore, prophylactic systemic chemotherapy with cytarabine and etoposide was added. He has been in complete remission for 21 months. Our results, together with other reported cases, indicate that a favorable outcome could be obtained by intensive and combined treatment for an isolated recurrence of GS of the brain if the bone marrow remained in complete remission.

Adult↗

PubMed2005

Resection of arteriovenous malformation in the middle cerebellar peduncle by a posterior transpetrosal approach: illustration of a case and correlative microsurgical anatomy.

BACKGROUND: Lesions located in the posterolateral brain stem, particularly the middle cerebellar peduncle, have presented surgeons with a challenge associated with significant morbidity. METHODS: We present a case of a 20-year-old woman who had a hematoma in the right middle cerebellar peduncle with a ventricular rupture. Angiography revealed an arteriovenous malformation (AVM) located in the same portion, extending from the lateral surface near the entry zone of the trigeminal nerve to the paraventricular area of the fourth ventricle, which was completely resected through a posterior transpetrosal approach. We also describe the microsurgical anatomy of this region in a cadaveric specimen, presenting the anatomic landscape at the target when approaching through this route. RESULTS: The patient's postoperative course was uneventful and her neurologic deficits progressively but gradually improved. When a lesion exists at or close to the surface of the middle cerebellar peduncle, the best approach, in our estimation, is a transpetrosal approach because the lesion can be approached perpendicularly with a short working distance, various angles for dissection are available, and minimal retraction of the temporal lobe and cerebellum is required. CONCLUSION: An AVM embedded in the middle cerebellar peduncle can be successfully resected using a posterior transpetrosal approach, though we concede this approach is slightly labor-intensive and time-consuming.

Adult↗

PubMed2003

Pediatric epilepsy surgery at the University of Alberta: 1988-2000.

Epilepsy surgery is considered a treatment option for patients with intractable seizures. Relatively few studies of efficacy, safety, and long-term outcome are available for the pediatric age group. This study describes a 12-year experience with pediatric epilepsy surgery at the University of Alberta. Records of pediatric epilepsy surgery patients admitted to the Comprehensive Epilepsy Program at the University of Alberta between 1988 and 2000 were reviewed. All patients received preoperative and postoperative clinical evaluation, seizure charts, testing of drug levels, electroencephalogram, computed tomography/magnetic resonance imaging, neuropsychologic testing, and long-term video electroencephalogram monitoring. The patients were reassessed after surgery at 6 weeks, 6 months, and 1 year and then yearly. The duration of follow-up was 1 year to 12 years. Forty-two patients underwent temporal lobectomies; 35, extratemporal resection. The age at surgery ranged from 6 months to 16 years. Thirty-two (76%) of temporal lobe patients became seizure-free (Engel Class I) vs 24 (68%) for the extratemporal group (Engel Class I). One patient (2%) in the temporal group had an Engel Class II outcome and one patient (3%) in the extratemporal group had the same Engel Class II outcome. Three patients (4%) manifested postoperative complications, and there were no deaths. Patients reported improvement in cognitive abilities, behavior, and quality of life after the surgery. Epilepsy surgery in children is effective and safe. Many children are seizure-free after the operation and remain so, although the results of temporal lobectomy are better than for extratemporal resections. There are few complications, and children often have an improved quality of life.

Adolescent↗

PubMed2004

Collateral brain damage, a potential source of cognitive impairment after selective surgery for control of mesial temporal lobe epilepsy.

BACKGROUND: Highly selective epilepsy surgery in temporal lobe epilepsy is intended to achieve seizure freedom at a lower cognitive risk than standard en bloc resections, but bears the risk of collateral cortical damage resulting from the surgical approach. OBJECTIVE: To investigate cortical damage associated with selective amygdalo-hippocampectomy (SAH). METHODS: 34 epileptic patients were evaluated. They were randomly assigned to SAH using either a sylvian (9 left/10 right) or a transcortical surgical approach (5 left/10 right). Postoperative MRI signal intensity changes adjacent to the approach were correlated with performance changes in serial word and design list learning. RESULTS: Losses in verbal learning and recognition memory were positively related to signal intensity changes, independent of the side of the resection, the surgical approach, or the extent of the mesial resection. Losses in consolidation/retrieval (memory) were greater after left sided surgery. Losses in design learning were related to right sided surgery and signal intensity changes. Seizure outcome (85% seizure-free) did not differ depending on the side or type of surgery. CONCLUSIONS: Collateral damage to cortical tissues adjacent to the surgical approach contributes to postoperative verbal and figural memory outcome after SAH. Controlling for collateral damage may clarify the controversial memory outcomes after SAH reported by different surgical centres.

Amygdala↗

PubMed1999

Gangliocytoma masquerading as a prolactinoma. Case report.

The authors describe the case of a 36-year-old man who presented with bitemporal hemianopsia and a serum prolactin concentration of 1440 ng/ml. Magnetic resonance imaging of the pituitary revealed a presumed macroadenoma with suprasellar and temporal lobe extension. Although the patient's prolactin level was lowered to 55 ng/ml by bromocriptine therapy, no tumor shrinkage occurred. Fourteen months later, progression of visual field defects necessitated transsphenoidal resection, which was incomplete. Immunocytochemical analysis of the biopsy tissue was positive for prolactin and, in view of the clinical picture, more detailed analysis was not performed. External-beam radiotherapy was given 2 years later because of enlargement of residual tumor. Subsequently, despite a fall in the serum prolactin concentration to less than 20 ng/ml in response to the course of bromocriptine, the mass displayed further extension into the temporal lobe. Nine years after the patient's initial presentation, he underwent transfrontal craniotomy for sudden deterioration in visual acuity caused by hemorrhage into the mass. No adenohypophyseal tissue was identified in the resected tissue. The mass was composed of dysplastic neurons that were strongly immunoreactive for synaptophysin and neurofilament (indicating neural differentiation) and prolactin. Review of the original biopsy specimen indicated that the prolactin-positive cells had striking neuronal morphological characteristics. The final diagnosis in this case is prolactin-secreting gangliocytoma. Although exceedingly rare, this disease must be added to the differential diagnosis in cases of "prolactinoma" when bromocriptine therapy is followed by a marked decline in serum prolactin that is not accompanied by significant tumor shrinkage. Furthermore, in such instances, consideration should be given to "obtaining a biopsy sample prior to electing for radiotherapy.

Adult↗

PubMed1995

Brain tumors associated with infantile spasms.

Two patients with brain tumors associated with infantile spasms are reported. Both infants displayed typical clinical features of infantile spasms, comprising tonic spasms manifesting in series and hypsarrythmia. In Patient 1, magnetic resonance imaging revealed a tumor in the hypothalamic region, suggestive of hypothalamic hamartoma. In Patient 2, cranial computed tomography and magnetic resonance imaging indicated the existence of a primary brain tumor with calcification in the right temporal lobe. Adrenocorticotropic hormone therapy combined with clonazepam relieved seizures in both infants. In Patient 1, resection of the hypothalamic tumor is impossible because the tumor lacks a stalk. In Patient 2, pathologic investigation of removed tumor tissue demonstrated mixed-oligoastrocytoma. It is suggested that focal lesions, like those in our patients, are involved in the development of infantile spasms.

Brain Neoplasms↗

PubMed1999

Acromegaly: report of two patients with an unusual presentation.

The presenting features of functionally active pituitary tumours depend on the specific hormone which is overproduced. Growth hormone (GH) producing tumours usually present with the clinical manifestations of acromegaly due to excessive GH secretion or symptoms resulting from mass effects of the enlarging tumour. The changes in physical features and the increase in tumour size are usually insidiously slow and therefore, recognition of the disease is delayed. In this report two patients with acromegaly are described with an atypical presentation due to acute onset of symptoms. The first patient presented with central diabetes insipidus. The diagnosis acromegaly was made on physical examination. The second patient presented with a generalized seizure during sleep. On CT-scanning a large tumour protruding into the left temporal lobe connected to the pituitary gland was seen. Immunohistochemistry of the tumour after partial transcranial resection confirmed the clinical diagnosis of acromegaly. At a later stage transsphenoidal resection of the pituitary tumour was performed with full recovery and without loss of pituitary function.

Acromegaly↗

PubMed1993

Impaired visual motion perception in the contralateral hemifield following unilateral posterior cerebral lesions in humans.

Contrast thresholds for a number of tasks were measured in the contralateral and ipsilateral upper quadrants of the visual field (eccentricity = 10 degrees) before and after an occipito-parietal surgical resection, in one patient, carried out for intractable epilepsy. Postoperatively the contrast thresholds for discriminating the speed of movement of drifting sine-wave gratings were elevated by greater than a log unit in the contralateral field with little or no change in the detection thresholds for the same stimuli. Contrast thresholds for opposite direction-of-motion (DOM) discrimination of a contrast modulated (CMod) grating (a 'non-Fourier' motion stimulus) were also elevated by about a log unit in the contralateral hemifield but thresholds for DOM discrimination of a sine-wave (luminance modulated, LMod) grating were unaffected. Contrast thresholds for orientation discrimination of stationary gratings (a non-motion task) were unaffected. This general pattern of results was found in two other patients following lateral occipital surgical resections. Eight other patients with occipito-temporal (two cases), parietal (three cases) and medial occipital lobe lesions (three cases) showed no difference between the two hemifields on any of the tasks. Comparison of the location of the lesions leads to the conclusion that damage to the lateral occipital gyri is responsible for the pattern of visual deficit observed. Damage to an extra-striate visual area concerned with motion perception (the human homologue of primate V5-MT) may have occurred. There has been no previous description of impairment of motion perception localized to a hemifield in humans. The characteristics of the residual motion perception in these cases is described further in the accompanying article [Plant and Nakayama (1993), Brain, 116, 1337-1353].

Adult↗

PubMed2004

Giant infiltrative cavernous malformation: clinical presentation, intervention, and genetic analysis: case report.

OBJECTIVE AND IMPORTANCE: Cavernous malformations can present in children with a sporadic course of repeated hemorrhage and enlargement, but they are rarely aggressive, infiltrative, or multilobar. We present the case of a young boy with a complex cavernous malformation that evolved during the course of a decade to encompass the majority of his right cerebral hemisphere. CLINICAL PRESENTATION: A 16-month-old boy presented with seizures, and radiographic studies demonstrated a large cavernous malformation in his right frontal pole. During the next 10 years, his seizures became intractable, and he developed progressive left hand weakness and atrophy. His malformation infiltrated his entire right frontal lobe as well as portions of his right parietal lobe, temporal lobe, and deep gray matter structures. INTERVENTION: The patient underwent right hemicraniotomy and near total resection of the lesion. Pathological analysis revealed dilated, thin-walled vessels separated by small amounts of intervening astrogliotic brain consistent with cavernous malformation. The patient recovered to his baseline neurological condition and has had no seizure or hemorrhage since his operation. Genetic testing did not reveal mutations in either the CCM1 (KRIT1) or CCM2 (malcavernin) genes. CONCLUSION: This case may represent an atypical variant of cavernous malformation best termed giant infiltrative cavernous malformation. Despite its unusual size, multilobar location, and aggressive infiltration, it can be managed effectively with standard surgical resection.

Cavernous Sinus↗

PubMed2026

Leptomeningeal Dissemination in TERT Promoter-mutant Anaplastic Pleomorphic Xanthoastrocytoma Responding to BRAF-MEK Inhibition: A Case Report.

Pleomorphic xanthoastrocytoma is a rare brain tumor that frequently harbors the oncogenic BRAF V600E mutation. Approximately 28.6%-47% of high-grade pleomorphic xanthoastrocytomas are associated with TERT promoter mutation and leptomeningeal dissemination, for which no established treatment exists and the prognosis remains poor. Combination therapy with BRAF and MEK inhibitors has demonstrated efficacy in BRAF V600E-mutant brain tumors. We report a case of a 22-year-old man with a right temporal lobe tumor initially diagnosed as World Health Organization grade 2 pleomorphic xanthoastrocytoma after gross total resection. Two years later, the tumor recurred and underwent malignant transformation to World Health Organization grade 3 pleomorphic xanthoastrocytoma. At the third resection, pathological and genomic analyses confirmed BRAF V600E mutation together with TERT promoter mutation. Following chemoradiotherapy, spinal leptomeningeal dissemination developed. After spinal irradiation, dabrafenib plus trametinib was initiated, resulting in partial radiological response and symptomatic improvement. Although regrowth occurred 10 months after initiation of targeted therapy, the patient remains alive at the time of writing. Here, we report a case of recurrent anaplastic BRAF V600E-mutant pleomorphic xanthoastrocytoma with leptomeningeal dissemination that showed a transient but clinically meaningful response to combined BRAF-MEK inhibition and spinal radiation therapy. In addition, this case raises the possibility of an association between TERT promoter mutation and leptomeningeal dissemination, although further studies are required to clarify this relationship.

BRAF V600E↗

PubMed1995

Hippocampal adequacy versus functional reserve: predicting memory functions following temporal lobectomy.

While decrements in memory following temporal lobectomy for intractable epilepsy remain a major neurocognitive complication of surgical intervention, it has been difficult to identify patients who are most at risk for developing decrements in their memory functions following surgery. Two different models of hippocampal functioning have been posited to account for postsurgical memory losses: hippocampal reserve versus functional adequacy. This article briefly examines the basis of the traditional model of hippocampal reserve, which suggests that it is the reserve or capacity of the contralateral hippocampus to support memory after surgery that determines whether changes in memory function will be observed. A more in-depth review of emerging data from studies of function (baseline neuropsychological testing and intracarotid amytal procedure) and structure (histologic cell densities and MRI volumetrics) supporting a functional adequacy model of hippocampal functioning is then presented. Data from these latter studies provide strong convergent support for the view that the risk for memory decrements following mesial temporal lobe epilepsy surgery is inversely related to the functional adequacy of the tissue to be resected. Data reviewed here have broad implications for the field of neuropsychology. Not only do the data reported in this article challenge some of the basic tenets of our understanding of the role of the hippocampus in memory functioning, but they illustrate the importance of ongoing research, critical reevaluation of basic concepts, and the importance of self-scrutiny in light of new technologies.

Journal Article↗