V Jay

University of Toronto, Toronto, Ontario, Canada

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Publications (133)427.8 Total impact

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    ABSTRACT: In recent years, there is increasing recognition of polyphenotypic high-grade malignancies in the non-central nervous system (CNS) tumor literature. Some of these tumors have been regarded as variants of primitive neuroectodermal tumor (PNET) or as extrarenal malignant rhabdoid tumors (MRTs). This report concerns two posterior fossa neoplasms, both of which displayed a "polyphenotypic" expression of neural, epithelial, myogenic, and glial markers, including synaptophysin, neurofilament, vimentin, glial fibrillary acidic protein, S-100, neuron-specific enolase, desmin, S antigen, MIC2, cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. One tumor showed complex intercellular junctions, cytoplasmic intermediate filaments, well-developed rough and smooth endoplasmic reticulum and Golgi apparatus, cilia, and neurosecretory granules. The other neoplasm showed pools of glycogen, desmosomes, and tonofilaments. The histological and ultrastructural appearances were inconsistent with glioma, PNET, meningioma, ependymoma, choroid plexus carcinoma, sarcoma, germ cell tumor, and other tumors in the World Health Organization classification. Although the polyphenotype raises the issue that these may represent variants of MRT or the atypical teratoid-rhabdoid tumor, the morphologic findings in the two cases were very dissimilar. Our two cases underscore the problems in nosology and classification of polyphenotypic tumors of the CNS. This is particularly significant, as therapeutic protocols for PNET, MRT, and non-CNS polyphenotypic tumors are different. We review the literature on polyphenotypic tumors and reiterate the difficulties in precise classification of these complex tumors.
    Pediatric Pathology & Laboratory Medicine 04/2010; 17(3):369-89.
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    ABSTRACT: Prominent neuronal differentiation and MYCN amplification occur only in a small percentage of medulloblastomas (primitive neuroectodermal tumor of the cerebellum). In this article, we describe two medulloblastomas that showed a striking degree of neuronal differentiation with islands of mature neurons associated with abundant neuropil. In these differentiated foci, there were no mitoses or atypia, and the histology was reminiscent of a malformative or hamartomatous lesion. Both tumors were assessed for MYCN amplification by the novel technique of differential polymerase chain reaction (PCR) and fluorescent in situ hybridization (FISH). In case 1, MYCN amplification was in the 10-fold range determined by differential PCR, while in case 2 the level of amplification was marked, with 20-30 copies compared with diploid controls. FISH analysis also confirmed the presence of MYCN amplification in both tumors. These two cases are of great interest, especially as they show MYCN amplification in medulloblastomas with marked degree of neuronal differentiation. Furthermore, in both cases, there was evidence of a slower and more indolent clinical course, suggesting a more favorable outcome in medulloblastoma with this degree of neuronal differentiation.
    Fetal and Pediatric Pathology 04/2010; 18(2):127-142. · 0.40 Impact Factor
  • Journal of the Pakistan Medical Association 01/2004; 53(12):619-21. · 0.40 Impact Factor
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    ABSTRACT: We investigated aberrant cortical excitability in malformations of cortical development From subdural electrodes, we recorded afterdischarges lasting > or = 6 seconds in 12 of 13 patients with malformations of cortical development and 6 of 10 pediatric patients with nonmalformations of cortical development and reviewed amperage thresholds, distribution of afterdischarges, and motor responses. In patients with malformation of cortical development, motor response thresholds were high; afterdischarge and motor response thresholds, which essentially overlapped, inversely correlated with age (P < .01); afterdischarge thresholds declined with age; and 8 patients showed afterdischarges in remote sites. In nonmalformation of cortical development, afterdischarge thresholds did not significantly correlate with age; motor response thresholds tended to decline with age; and 2 patients had remote afterdischarges. Adolescent patients with malformations of cortical development had lower afterdischarge thresholds than adolescents with nonmalformation of cortical development (P < .05). From their high afterdischarge (and motor response) thresholds, we concluded that preadolescent patients with malformation of cortical development have less excitable, immature cortices, whereas adolescent patients with malformation of cortical development with low afterdischarge thresholds have hyperexcitable cortices. Remote afterdischarges over focal dysplastic cortex suggest aberrant cortical excitability and neural circuits.
    Journal of Child Neurology 05/2003; 18(4):247-53. · 1.67 Impact Factor
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    ABSTRACT: Centronuclear myopathy has been extremely rarely associated with cardiomyopathy, which can lead to heart failure and premature death. We report the case of a 3.5-year-old girl with early-onset dilated cardiomyopathy, biventricular hypertrophy and histologic features suggestive of centronuclear myopathy. After unsuccessful medical treatment for heart failure, she underwent cardiac transplantation at the age of 4.5 years. Results of a skeletal muscle biopsy showed increased central nuclei and perinuclear vacuolations with aggregates of mitochondria. Examination of the heart at the time of transplantation confirmed a diagnosis of dilated cardiomyopathy. Histologic results revealed hypertrophic myocardiocytes, focal areas of infarction and endocardial fibroelastosis, most prominently in the left ventricle. Although cardiomyopathy is commonly associated with other childhood myopathies, to our knowledge, this is the youngest patient reported with centronuclear myopathy presenting with heart failure caused by cardiomyopathy, and the first patient to successfully undergo cardiac transplantation. One year after the heart transplant, there were no signs of rejection. We recommend detailed cardiac assessment with regular follow-up for children with histologic features consistent with centronuclear myopathy.
    Brain and Development 02/2003; 25(1):62-6. · 1.54 Impact Factor
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    ABSTRACT: To discover whether the spatial distribution of spike sources determined by magnetoencephalography (MEG) provides reliable information for planning surgery and predicting outcomes in pediatric patients with lesional extrahippocampal epilepsy. We retrospectively studied 12 children with extrahippocampal epilepsy secondary to cortical dysplasia (CD), tumor, or porencephalic cyst. We compared interictal MEG spike source locations and somatosensory evoked fields derived from equivalent-current dipole modeling with intraoperative or extraoperative electrocorticography (ECoG). MEG spike sources were found in proximity to the lesion in all patients and extended from lesions in five patients with CD. Marginal spike sources were noted in three patients with tumors, one patient with a cyst, and one with CD, and extramarginal sources in three patients with tumors. Three patients with tumors underwent lesionectomy only; two had further cortical excisions. One patient with CD underwent lesionectomy only, three had lesionectomy and cortical excisions, and two had lesionectomy and multiple subpial transection. Asymmetric MEG spike sources correlated with ECoG findings in all patients. Residual epileptiform discharges on postexcisional ECoG corresponded to spike sources in three patients with tumors and one patient with a cyst. Eleven patients have been seizure free for 1-6 years (mean, 4 years). One patient had residual seizures after incomplete excision of right temporal CD. MEG delineated asymmetric epileptogenicity surrounding lesions and the eloquent cortex. Complete tumor resection produced favorable outcomes despite residual postexcisional ECoG spikes and extramarginal MEG spike sources. CD characterized by clusters of MEG spike sources within and extending from lesions seen on magnetic resonance imaging (MRI) should be removed to prevent seizures.
    Epilepsia 12/2002; 42(12):1523-30. · 4.58 Impact Factor
  • Susan I Blaser, Venita Jay
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    ABSTRACT: The advent of newer imaging techniques, such as high-resolution MR imaging and surface reconstructions of three-dimensional data sets, has led to a greater in vivo understanding of cortical malformations of the brain. Disorders of cortical formation are illustrated with routine imaging, surface reconstruction, and pathogenic specimens.
    Neurosurgery Clinics of North America 02/2002; 13(1):41-62, viii. · 1.54 Impact Factor
  • Susan I. Blaser, Venita Jay
    Neurosurgery Clinics of North America 01/2002; 13(1):41-62. · 1.54 Impact Factor
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    ABSTRACT: Patients with intractable epilepsy due to lesions may undergo surgical treatment of both the lesion and the seizures. The surgical plan which lesion may be completely resected and which seizures has to be controlled remains for the team of epilepsy surgery, including neurosurgeon, neurologist, and neuro- radiologist. In these cases, the extent of surgical resection is sometimes guided by spikes recorded on intraoperative electrocorticography (ECoG). Relationships between postresection spikes and seizure outcome has been reported that tendency of residual extramarginal spikes correlate to the seizure recurrence (1, 2). Whether spikes recorded on the intraoperative ECoG imply active epileptogenesis has been uncertain. Recent clinical application of MEG studies (3-7) have correlated the results of MEG epileptic spike localizations with conventional noninvasive and invasive localizing modalities in patients with refractory seizures who are candidates for epilepsy surgery. These studies have concluded that MEG, a noninvasive technique, may be useful for localizing the epileptogenic zone. In this study, we sought to determine whether the spatial extent of MEG spike sources in pediatric patients with extrahippocampal lesional epilepsy predicts surgical outcome. We investigate the relations among the location of interictal MEG spike sources, lesion on MRI, surgical procedures, ECoG results, pathological substrates, and follow-up seizure outcome. 2 Methods We studied MEG in 13 children with extrahippocampal lesional epilepsy including 5 patients with post-operative recurrent seizures. MSF were divided into four different groups; A) within the lesion; B) at the margin; C) on the remote area; D) on the contralateral homotopic region. MEG spike sources and somatosensory evoked magnetic magnetic fields
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    ABSTRACT: To describe the radiologic-pathologic correlation in children who underwent epilepsy surgery for medically intractable epilepsy with pathologically confirmed focal cortical dysplasia and hemimegalencephaly, we conducted a retrospective review on the magnetic resonance imaging and pathology of 18 children (10 boys and 8 girls). The preoperative MRIs were reviewed by one neuroradiologist who did not know the radiologic diagnosis and the pathology reports. MRI revealed focal cortical dysplasia (10), hemimegalencephaly (3), hamartomas (2), polymicrogyria (1), pial hemosiderosis (1), and no abnormality (1). Pathologic examination revealed focal cortical dysplasia (9), forme fruste of tuberous sclerosis (5), hemimegalencephaly (3), and focal cortical dysplasia with mesial temporal sclerosis (1). MRI was accurate in making the preoperative diagnosis in 16 out of 18 patients. On MRI, 12 patients had abnormal gyral formation and 12 had abnormal cortical thickness. Eleven patients manifested loss of gray-white differentiation, and 11 patients had abnormal signal on T(2)-weighted image. Pathologically, 15 patients had neuronal heterotopia, 12 had misalignment or disorientation of neurons, 11 had large neurons, and 10 had abnormal cortical lamination. The presence of ectopic and large neurons and abnormal cortical lamination may be responsible for the MRI characteristics.
    Pediatric Neurology 11/2001; 25(4):295-303. · 1.50 Impact Factor
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    ABSTRACT: To describe the diagnosis, treatment, and outcomes in children with malignant rolandic-sylvian epilepsy (MRSE), defined as a form of epilepsy characterized by sensorimotor seizures, medical refractoriness, normal MRI, frontocentrotemporal EEG spikes, rolandic-sylvian spike sources on magnetoencephalography (MEG), and cognitive problems. A retrospective chart analysis of seven patients who had shown these characteristics and undergone extensive diagnostic testing, including MEG and intracranial video-EEG was performed. Interictal scalp EEG spikes were seen over the frontocentrotemporal regions bilaterally (6) and unilaterally (1). MEG showed spike sources in the perisylvian region in two patients (both bilateral) and in the perirolandic fissure in five (two bilateral). Three patients required bilateral subdural strips to lateralize seizures before electrocorticography. Final electrocorticograms showed an ictal onset zone around the rolandic (four cases) and rolandic-sylvian regions (three cases). Six patients showed neuropsychological deficits. After cortical excision and multiple subpial transection, three were seizure free and four had seizures rarely (30 months' mean follow-up). No child had a permanent deficit in sensorimotor or cognitive functions, although two showed exacerbation of preexisting attentional deficits. Tissue analysis showed definite evidence of neuronal migration disorders (3) and gliosis (2). MEG was helpful for localizing both malignant rolandic-sylvian neuronal activities and functional cortex. Successive neuropsychological assessments are necessary to detect cognitive deterioration and indicate remedial programming. If, after careful observation over at least 5 years, surgery is considered to control refractory seizures, intracranial video-EEG is needed to localize the epileptogenic zone.
    Neurology 09/2001; 57(4):590-6. · 8.30 Impact Factor
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    ABSTRACT: The purpose of the study was to investigate factors altering the amperage threshold needed to provoke functional responses in children with epilepsy. Twenty patients (4-18 years of age) who underwent epilepsy surgery at our institution from 1996-2000 after insertion of subdural grid electrodes were reviewed retrospectively. Extraoperative electrical cortical stimulation was performed with 50-Hz biphasic pulses of 0.2 ms in duration using a "distance reference" technique. Amperage thresholds of primary motor responses and afterdischarges were evaluated. The patients were grouped according to underlying pathology: eight with neuronal migration disorders (group A) and 12 with other disorders (group B). The motor cortex was defined successfully in all children because the afterdischarges threshold was higher than the motor cortical threshold. Amperage thresholds ranged from 2-20 mA (mean = 7.7) for primary motor function. An inverse relationship was found between amperage threshold and age: the younger the patient, the higher the threshold (P = 0.0005). Patients in group A required a higher amperage (2-20 mA, mean = 8.6) for motor cortical mapping than those in group B (2-14 mA, mean = 6.4). Younger children with neuronal migration disorders require a higher amperage threshold to achieve adequate motor functional mapping with careful observation of afterdischarges.
    Pediatric Neurology 06/2001; 24(5):344-50. · 1.50 Impact Factor
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    ABSTRACT: To clarify the clinical features and pathologic manifestations of isolated angiitis of the CNS (IACNS) in children. The authors report two new cases and summarize the literature of childhood IACNS confirmed by pathology. IACNS affecting small vessels (n = 5). Neurologic manifestations included headaches, focal seizures, and progressive, behavioral, or multifocal neurologic impairment. MRI showed multifocal, T2-hyperintense, cerebral lesions without mass effect or tumor-like lesions. CSF, erythrocyte sedimentation rate, and cerebral angiograms were often normal.CNS biopsy disclosed a nongranulomatous vasculitis. Children were treated with prednisone alone or combined with cyclophosphamide. One child died. Four children had a favorable outcome. IACNS affecting large and medium arteries (n =5). Three children presented with acute ischemic stroke or TIA. Brain CT showed ischemic infarcts. Two children presented with subarachnoid hemorrhage. In this group, CSF, erythrocyte sedimentation rate, and angiograms were often abnormal. No patient received immunosuppressive therapy. Five children died. Autopsy showed granulomatous IACNS (n =5). Clinical and radiologic features correlate with the size of affected vessels. Prognosis differs between groups. Potential markers of poor outcome are acute stroke presentation secondary to large and medium-sized artery involvement, granulomatous angiitis, and delayed institution of immunosuppressive therapy.
    Neurology 05/2001; 56(7):837-42. · 8.30 Impact Factor
  • V Jay
    Archives of pathology & laboratory medicine 04/2001; 125(3):320-1. · 2.88 Impact Factor
  • Source
    Venita Jay, Jiri Vajsar
    The Lancet 03/2001; 357(9255):550-2. · 39.21 Impact Factor
  • V Jay
    Pediatric and Developmental Pathology 02/2001; 4(2):203-4. · 0.86 Impact Factor
  • V Jay
    Archives of pathology & laboratory medicine 01/2001; 124(12):1750-1. · 2.88 Impact Factor
  • V Jay
    Archives of pathology & laboratory medicine 01/2001; 124(12):1751. · 2.88 Impact Factor
  • V Jay
    Archives of pathology & laboratory medicine 12/2000; 124(11):1594-5. · 2.88 Impact Factor
  • V Jay
    Archives of pathology & laboratory medicine 11/2000; 124(10):1420-1. · 2.88 Impact Factor

Publication Stats

2k Citations
427.80 Total Impact Points


  • 1991–2010
    • University of Toronto
      • • Hospital for Sick Children
      • • Division of Neurosurgery
      • • Department of Radiation Oncology
      Toronto, Ontario, Canada
  • 1990–2010
    • SickKids
      • • Division of Pathology
      • • Division of Infectious Diseases
      Toronto, Ontario, Canada
  • 2004
    • Aga Khan University Hospital, Karachi
      Kurrachee, Sindh, Pakistan
  • 1991–1996
    • The Princess Margaret Hospital
      Toronto, Ontario, Canada