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A R Rezai

Publications and source records attributed to A R Rezai.

35 records · Page 2Linked to original sources

Contrast-enhancing progressive multifocal leukoencephalopathy: radiological and pathological correlations: case report.

OBJECTIVE AND IMPORTANCE: Progressive multifocal leukoencephalopathy (PML), a demyelinating disease caused by the JC papovavirus, is an opportunistic infection afflicting patients with impaired cellular immunity. Although initially described in patients with hematological malignancies, PML has become associated with several other immunocompromised states, particularly human immunodeficiency virus (HIV) infection. There are numerous central nervous system manifestations in patients with acquired immunodeficiency syndrome. A major characteristic that distinguishes PML from other more common lesions, such as toxoplasmosis or non-Hodgkin's lymphoma, is the lack of contrast enhancement. We describe a case of PML that exhibits contrast enhancement, and we conclude that the diagnosis of PML must be considered in patients with HIV who have contrast-enhancing lesions. CLINICAL PRESENTATION: A 40-year-old woman presented with progressive hemiparesis, blurred vision, and ataxia. Magnetic resonance imaging revealed a contrast-enhancing lesion involving the left middle cerebellar peduncle, causing mild compression of the fourth ventricle. INTERVENTION: The patient underwent a stereotactic serial biopsy with the presumptive diagnosis of moderate- to high-grade glioma. Histological examination of the biopsy specimen revealed early PML. Subsequently, a test for HIV was obtained and the results were positive. CONCLUSION: We have reported another atypical radiographic characteristic of PML associated with HIV. We conclude that PML lesions can enhance after the administration of gadolinium. Therefore, the diagnosis of PML must be entertained in patients whose test results were positive for HIV with contrast-enhancing lesions and that a stereotactic serial biopsy may be necessary to provide a definitive diagnosis.

Adult↗

Disseminated ependymomas of the central nervous system.

Ependymomas are rare central nervous system (CNS) neoplasms that occasionally disseminate along the neuraxis or to extraneural sites. Definitive criteria predictive of dissemination have yet to be determined. One hundred forty patients with CNS ependymoma (88 primary spinal and 52 primary intracranial tumors) were surgically treated by the senior author (F.J.E.) between 1986 and 1994. Sixteen patients (11.4%) demonstrated tumor dissemination. The disseminated group consisted of 11 (12.5%) of 88 primary spinal and five (9.6%) of 52 primary intracranial ependymomas. The authors retrospectively reviewed the patients with CNS ependymoma and have identified several characteristics associated with dissemination from the primary tumor site. The mean time from diagnosis to dissemination was 6.8 years. The patients with disseminated disease were younger (16.8 vs. 28.3 years old, p = 0.02), had fewer gross-total resections (29% vs. 68%, p = 0.015), and had tumors with higher proliferative indices (MIB-1 staining, 13.14% vs. 2.06%, p = 0.02). High-grade tumors had a mean proliferation index of 21%, versus 2.4% and 1.6% for myxopapillary and low-grade tumors, respectively (p = 0.0003). In contrast to previous studies, tumor histology was the most significant variable for time to dissemination as determined by multivariate analysis (p = 0.008). Myxopapillary and high-grade tumors were 3.6 and 5.6 times more likely to have a shorter time to dissemination than low-grade tumors. In addition, dissemination is associated with a worse prognosis. At follow-up review, 31% of patients with disseminated disease had died compared to 7% of patients without dissemination (p = 0.04). It is concluded that younger patients with subtotal resections, myxopapillary or high-grade histology, and tumors with high proliferative indices are at substantial risk for the development of disseminated disease during their clinical course.

Adolescent↗

Introduction of magnetoencephalography to stereotactic techniques.

Magnetoencephalography (MEG), a noninvasive functional brain mapping technique, was used for preoperative localization of the sensorimotor cortex in patients harboring lesions involving these eloquent regions. Prior to surgery, MEG source locations were transferred onto high-resolution MRI pictures which were then used for preoperative evaluation, risk analysis, and planning. We have developed a process to transform the MEG-derived sensorimotor localization coordinates into the COMPASS stereotactic coordinate system. Thus the MEG-derived functional information is incorporated into the stereotactic database, enabling the simultaneous visualization of functional and anatomical data. This information can be used for the selection of cases and in planning safe approaches for computer-assisted volumetric resections. The integration of MEG and stereotactic neurosurgery also allows a more precise comparison between MEG and intraoperative direct electrocorticographic mapping (ECoG). Seven patients were studied with good correlation between MEG and intraoperative mapping. In 4, the correlation was only based on gross visual comparison between intraoperative identification of the gyrus pattern and MEG photographs. The availability of the MEG coordinates in the stereotactic system, however, allows a more precise correlation between MEG and ECoG. In all 3 patients studied in this manner, the MEG coordinates (pinpointed to a precise cortical representation of a few millimeters) overlapped with ECoG results. In summary, we compared functional MEG data to intraoperative ECoG and conclude that the introduction of MEG into stereotactic neurosurgery can provide precise functional and anatomic information for image-guided surgical planning and resection.

Brain Mapping↗

Acquired Chiari-I malformation and hydromyelia secondary to a giant craniopharyngioma.

Hydromyelia, or hydrosyringomyelia is frequently associated with the Chiari I malformation of the cerebellar tonsils. Descent of the cerebellar tonsils is considered a congenital anomaly with a few reports of 'acquired' Chiari I malformation. We report a patient with a giant craniopharyngioma and hydrocephalus who at presentation had a concomitant Chiari I malformation and hydromyelia. The patient underwent gross total resection of the tumor and, with no further treatment, demonstrated spontaneous resolution of the Chiari I malformation and hydromyelia during the postoperative period. This suggests that the Chiari I malformation and the resulting hydromyelia were 'acquired', and were caused by an intracranial mass effect. This provides further evidence for an associative mechanism of cerebellar tonsillar descent and the development of hydromyelia.

Arnold-Chiari Malformation↗

Modern management of spinal tuberculosis.

The resurgence of pulmonary tuberculosis in the United States has been paralleled by a concomitant rise in tuberculosis of the spine (Pott's disease). The appearance of drug-resistant strains of tuberculosis, infection in large numbers of immunocompromised hosts, newer imaging modalities, and the development of more effective spinal reconstruction techniques have raised important issues regarding the management of Pott's disease. In spite of this, there has been little published recently on the modern management of Pott's disease in developed countries. We report our experience with the management of 20 patients with Pott's disease in the past 5 years, 16 of whom were admitted during the last 18 months of this retrospective study. The mean patient age was 49 years. Sixteen (80%) were men. Nineteen (95%) had a positive tuberculin skin test, and 13 (65%) had pulmonary tuberculosis. Symptoms consisted of spinal pain, weakness, sensory complaints, and flank mass in order of decreasing frequency. Ten patients were neurologically intact; the remainder had motor deficits of variable severity. The thoracic spine was involved in 13 patients, the lumbar spine was involved in 4, the cervical spine was involved in 2, and the thoracolumbar spine was involved in 1. Spinal deformity was present in 11 patients, spinal epidural compression was present in 13, and a paraspinal mass was present in 18. Operative indications included motor deficits, spinal deformity, nondiagnostic computer tomographic-guided needle biopsy, and noncompliance with, or lack of, response to medical therapy. Eleven patients underwent operations. Six patients had vertebrectomy and bone grafting with posterior instrumentation when indicated; three had laminectomy, debridement, and abscess drainage; one had laminectomy and posterior instrumentation; and one had paraspinal abscess drainage. Two patients have died; the remainder have been monitored for at least 1 year and are neurologically improved or normal without residual infection. The average angulation decreased from 31 to 24 degrees by the follow-up examination. In selected patients, early operative treatment with instrumentation, when indicated, minimizes neurological deterioration and spinal deformity, allows early ambulation, and results in excellent neurological outcome.

Adult↗

Intramedullary spinal cord lipomas.

Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.

Adolescent↗

Interleukin-6 and interleukin-6 receptor gene expression in pituitary tumors.

Interleukin-6 (IL-6) is a multifunctional cytokine which plays a role in the stimulation, inhibition, differentiation, and regulation of cell growth. IL-6 has been shown to act as an autocrine growth factor in several tumors, and is expressed by a variety of tumors. IL-6 also exhibits a regulatory role in the hypothalamo-pituitary axis. It is produced by both the hypothalamus and the pituitary, and it induces the secretion of anterior pituitary hormones. Because of the regulatory role of IL-6 in tumor growth and its involvement in the pituitary, we decided to evaluate IL-6 and IL-6 receptor expression in pituitary tumors. For this purpose we utilized complimentary cDNA probes specific for the IL-6 and IL-6 receptor mRNA, as well as monoclonal anti-IL-6 antibodies for immunohistochemical analysis. Our results show that the IL-6 gene is expressed in the normal pituitary tissue. However, the non-functioning and functioning pituitary tumors such as the prolactin and growth hormone secreting tumors express increased levels of the IL-6 gene. The IL-6 receptor gene was only expressed in the prolactin secreting and non-functioning pituitary tumors. These results show that the IL-6 and IL-6 receptor gene expression is enhanced in pituitary tumors, thus suggesting a possible role of IL-6 in the pathogenesis of these tumors.

Adenoma↗

Traumatic posterior cerebral artery aneurysm secondary to an intracranial nail: case report.

We present the case of a traumatic posterior cerebral artery aneurysm from a self-inflicted pneumatic nail-gun missile injury through the roof of the mouth. The patient presented to us in a coma with subarachanoid and intraventricular hemorrhage. Cerebral angiography revealed an aneurysm of the left posterior cerebral artery with no distal filling. The patient died 6 days after admission. At autopsy, a pseudoaneurysm of the posterior cerebral artery was seen. This aneurysm resulted from direct disruption of the arterial wall by the intracranial nail.

Adult↗

Depressed skull fractures in children secondary to skull clamp fixation devices.

The use of external skull fixation devices (skull clamps) is a common practice in neurosurgery. The insertion of pins into the skull is usually routine and uneventful in adult patients. However, the safety of skull clamp fixation devices in children is not reported. We have examined our complications over the past 6 years, and have encountered 5 children with depressed skull fractures secondary to application of a skull clamp fixation device. There were 3 boys and 2 girls with ages ranging from 3 to 8 years (mean 5.8 years). Two patients had brainstem gliomas, 2 patients had hypothalamic gliomas and 1 patient had a medulloblastoma. Four of the children required separate cranial procedures for exploration and elevation of the depressed fractures. There were no sequelae associated with the depressed fractures. We conclude that skull clamp fixation devices are safe, but should be used with caution in the pediatric patient. In addition, we present several modifications of existing skull clamps which may decrease the risk of depressed skull fractures.

Brain↗

Acute surgical management of intracranial arteriovenous malformations.

The majority of intracranial arteriovenous malformations (AVMs) do not require acute surgical intervention. Some patients, however, require emergent surgical treatment because of a profound neurological deterioration from a mass effect. We report 10 patients who underwent emergency AVM surgery after experiencing neurological deterioration from an intracranial hemorrhage. Two patients bled spontaneously, whereas eight had an intracranial hemorrhage secondary to an embolization procedure. When the patients demonstrated neurological deterioration, they were intubated, hyperventilated, and underwent osmotic diuresis. Barbiturate anesthesia was initiated, and surgery was performed within 30 minutes in most cases. The hematomas were evacuated, and an attempt was made to excise the AVMs at the same time. Postoperatively, intracranial pressure was monitored, and barbiturate coma was maintained until the intracranial pressure returned to normal. Cerebral perfusion pressure was maintained above 55 mm Hg. The operation was confined to evacuating the hematoma in two patients with inoperable AVMs. The other eight patients underwent concomitant total AVM resection. Because of the severity of neurological deterioration, one patient who bled spontaneously underwent surgery based only on a computed tomographic scan of the brain. Nine patients made a good-to-excellent recovery. One patient with a large motor-strip AVM remained hemiplegic. We conclude that in patients presenting with profound neurological deterioration after a spontaneous intracranial hemorrhage or one associated with an embolization procedure, prompt hematoma evacuation with simultaneous AVM excision as well as perioperative intracranial pressure control with mannitol and barbiturates can yield a good-to-excellent outcome.

Adult↗

Sigmoid diverticular perforation in neurosurgical patients receiving high-dose corticosteroids.

Perforation of colonic diverticula is a complication of corticosteroid use that has not been described in the neurosurgical literature. Between 1987 and 1992, 719 patients who underwent surgery for primary and metastatic brain and spinal tumors of the central nervous system received 2246 to 4936 mg of methylprednisolone given over at least 7 days. Five patients in this group (all men, ages 50-69 yr) experienced a sigmoid diverticular perforation at a mean dose of 3947 mg of methylprednisolone (range, 2240-6160 mg). Of these five, two had a known history of diverticular disease. In contrast, during this same period, 3749 patients who underwent neurosurgical procedures for non-neoplastic conditions did not receive corticosteroids and experienced no colonic perforations. All five patients with colonic perforations presented with abdominal pain and had free intraperitoneal air that was revealed on radiographs of the abdomen. Perforation of a sigmoid diverticulum was confirmed in all five at exploratory laparotomy. Four patients had good outcomes, and one died. We conclude the following: 1) patients over age 50 who receive high-dose corticosteroids are at risk for sigmoid colonic perforation, and these medications should be used with caution in such patients; 2) if possible, lower total doses of perioperative corticosteroids should be used in patients with known diverticular disease; and 3) because corticosteroids mask many of the inflammatory signs of perforation, this diagnosis should be considered in any patient with abdominal discomfort, fever of unknown origin, or unexplained leukocytosis.

Abdomen, Acute↗

Vasopressin uptake by hypothalamopituitary axis and pineal gland in guinea pigs.

The uptake of circulating arginine vasopressin (AVP) by the pituitary gland (anterior lobe), pineal gland, and hypothalamus (ventromedial part) was investigated in an isolated in situ perused brain of anesthetized guinea pig. Kinetic experiments revealed saturable AVP uptake in all studied regions with Km values of 0.79, 0.19, and 0.76 microM and maximum velocity values of 22, 2.1, and 1.6 pmol.min-1.g-1 for the pituitary gland, pineal gland, and hypothalamus, respectively. The nonsaturable components (diffusion constants) were not significantly different from zero. Peptide fragments, L-phenylalanine, and Bestatin (an aminopeptidase inhibitor) did not interfere with AVP uptake. However, uptake of AVP was strongly inhibited in the presence of the V1 antagonist [1-(beta-mercapto-beta-beta-cyclopentamethylenepropionic acid), 2-(O-methyl)tyrosine]AVP at 2.7 microM, and regional Ki values, assuming that the observed inhibitions were purely competitive, ranged between 0.32 and 2.23 microM. The V2 agonist 1-desamino-8-D-AVP at 2.7 microM produced only a weak inhibition of AVP uptake, and regional Ki values ranged between 9.56 and 21.3 microM. It is concluded that specific uptake mechanisms in the hypothalamopituitary axis and pineal gland are sensitive enough to detect blood-borne AVP under the physiological hormonal state. It is suggested that AVP binding in situ is primarily related to V1 receptors, which may be involved in mediating the central effects of this circulating peptide.

Animals↗

Infection with HIV is associated with elevated IL-6 levels and production.

Polyclonal B cell activation is commonly observed in AIDS and in infection with HIV. Because IL-6 (B cell stimulatory factor 2) plays an essential role in the differentiation of activated B cells to Ig-secreting cells, and because IL-6 production is induced by exposure of human PBMC to HIV, we measured the level of circulating plasma IL-6, spontaneously-produced IL-6, and IL-6 mRNA in HIV-infected donors and in healthy control donors. Elevated levels of plasma IL-6 and IL-6 mRNA were detected in HIV-infected donors. PBMC isolated from the peripheral circulation of HIV-infected donors, and cultured without added exogenous activators of IL-6 production, produced markedly elevated amounts of IL-6 when compared with cells isolated from healthy donors. Interestingly, levels of an acute-phase protein, which is known to be induced by IL-6, was also increased in HIV-infected donors. These results demonstrate that elevated levels of IL-6 are associated with HIV-infection, and suggest that IL-6 over-production may contribute to the polyclonal B cell activation seen in AIDS and HIV infection.

C-Reactive Protein↗

AIDS Kaposi sarcoma-derived cells produce and respond to interleukin 6.

Cell lines derived from Kaposi sarcoma lesions of patients with AIDS (AIDS-KS cells) produce several cytokines, including an endothelial cell growth factor, interleukin 1 beta, and basic fibroblast growth factor. Since exposure to human immunodeficiency virus increases interleukin 6 (IL-6) production in monocytes and endothelial cells produce IL-6, we examined IL-6 expression and response in AIDS-KS cell lines and IL-6 expression in AIDS Kaposi sarcoma tissue. The AIDS-KS cell lines (N521J and EKS3) secreted large amounts of immunoreactive and biologically active IL-6. We found both IL-6 and IL-6 receptor (IL-6-R) RNA by slot blot hybridization analysis of AIDS-KS cells. The IL-6-R was functional, as [3H]thymidine incorporation by AIDS-KS cells increased significantly after exposure to human recombinant IL-6 (hrIL-6) at greater than 10 units/ml. When AIDS-KS cells (EKS3) were exposed to IL-6 antisense oligonucleotide, cellular proliferation decreased by nearly two-thirds, with a corresponding decrease in the production of IL-6. The decrease from IL-6 antisense in AIDS-KS cell proliferation was reversed by the addition of hrIL-6. We confirmed that AIDS-KS cells produced IL-6 in vivo by preparing RNA and tissue sections from involved and uninvolved skin from a patient with AIDS Kaposi sarcoma. We detected immunoreactive IL-6 in the involved tumor areas and to a lesser extent in the surrounding normal epidermis. Slot blot hybridization showed a great excess of IL-6 and IL-6-R RNA in involved skin compared to uninvolved skin. These results show that both IL-6 and IL-6-R are produced by AIDS-KS cells and that IL-6 is required for optimal AIDS-KS cell proliferation, and they suggest that IL-6 is an autocrine growth factor for AIDS-KS cells.

Acquired Immunodeficiency Syndrome↗

Histamine blocks interleukin 2 (IL-2) gene expression and regulates IL-2 receptor expression.

Histamine inhibited the proliferative response of human peripheral blood mononuclear cells (PBMC) to the T cell mitogen Phytohemagglutinin-P (PHA-P) in a dose-dependent fashion. This inhibition was mediated via the H2 receptor since cimetidine, a known H2 antagonist, removed the inhibition, whereas the addition of the H1 antagonist Diphenhydramine did not. Inhibition occurred during the inductive phase of the cell cycle, since histamine added 24 hours after PHA-P stimulation had no effect on subsequent T cell proliferation, and was attributable to inhibition of interleukin-2 (IL-2) gene expression. Both secreted IL-2 and messenger RNA coding for IL-2 were inhibited by histamine. In contrast, histamine exerted no inhibitory effect on the expression of cell surface receptors for IL-2 as determined by flow cytometry. Furthermore, histamine-treated cells retained full responsiveness to exogenously administered IL-2, which completely reversed the anti-proliferative effect of histamine. In some donors, histamine enhanced the percentage of IL-2 receptor positive cells. Stimulated PBMC from AIDS KS patients as a group, displayed a lower percentage of IL-2 receptor bearing cells, which was significantly increased by the addition of histamine even at concentrations as low as 10(-6) M and peaking at 10(-3) M. These findings indicate that histamine exerts its anti-proliferative effects on T cells by inhibiting IL-2 production, via blockade of IL-2 gene expression. In addition, histamine seems to exert immunomodulating effects on IL-2 receptor expression, particularly in those individuals with AIDS-KS.

Adult↗

Immunomodulating effects in vitro of interleukin-2 and interferon-gamma on human blood and bone marrow mononuclear cells.

Mononuclear cells (MNC) derived from peripheral blood (PBMNC) of 23 normal donors and 4 AIDS patients, and from bone marrow (BMMNC) of 15 normal donors were incubated at 37 degrees C in culture medium alone or in the presence of either natural or recombinant human interleukin-2 (IL-2) or recombinant human interferon-gamma (IFN-gamma; 1-1,000 U/ml). The cultured cells were washed on days 1, 4 or 7 and tested for various immune functions in vitro and for cell surface phenotype. IL-2, but not IFN-gamma, was found mitogenic for both PBMNC and BMMNC. The natural killer (NK) activity of both PBMNC and BMMNC was the only function tested that was markedly augmented (over 100-fold compared to medium control) by both lymphokines. Pretreatment of PBMNC with IL-2 at greater than or equal to 10 U/ml profoundly suppressed (up to 90%) various functions, such as mitogenic responses (phytohemmagglutinin, concanavalin A, pokeweed mitogen), allogeneic mixed leukocyte reaction, antibody production and T cell colony formation in agar. In contrast, some BMMNC functions were elevated at low doses of IL-2 and IFN-gamma, and significant suppression of BMMNC was seen only with high doses of IL-2 (greater than or equal to 100 U/ml) and IFN-gamma (1,000 U/ml). IL-2 was by far more effective than IFN-gamma in both the amplification of NK activity and the suppression of most of the other functions. IL-2, but not IFN-gamma, was found to activate/induce suppressor cells and increased the proportion of Leu-2+ (CD8) cells in PBMNC; the suppressive effect was time- and dose-dependent. The IL-2-induced suppression could be diminished by inclusion of anti-IL-2 antibody during the pretreatment phase. Similar suppressive effects were noted in PBMNC from AIDS patients. These findings suggest that: (a) high-dose IL-2 may elicit immunosuppression which can be mediated by nondiscriminative highly cytotoxic cells (i.e. lymphokine-activated killer cells) and/or by noncytotoxic, nonspecific suppressor cells, and (b) that PBMNC respond differently to the lymphokines than do BMMNC.

Acquired Immunodeficiency Syndrome↗

Increased spontaneous immunoglobulin secretion associated with cyclophosphamide-induced immune suppression.

Spontaneous immunoglobulin (Ig) secretion by cells from multiple sclerosis (MS) patients (in the progressive phase) treated with monthly pulse doses of cyclophosphamide (CY) (1000-1600 mg/M2) was measured using the protein A plaque assay, to evaluate the effect of CY treatment on B-cell function. Surprisingly, an increase, rather than a decrease, in Ig-secreting cells was seen following CY treatment. CY-treated MS patients averaged 1380 +/- 535 spontaneous total (IgM + G + A) Ig plaque-forming cells (PFC) per 1 X 10(6) peripheral blood mononuclear cells (MNC), measured at 15-22 days after monthly CY administration, while healthy adults had 280 +/- 47 Ig PFC/10(6) MNC, and MS patients not treated with CY had 300 +/- 43 Ig PFC/10(6) MNC. The observed increase was due to an increase in IgG and IgA PFC. PFC levels remained elevated for 4 weeks following CY treatment, decreasing to control levels by 7-8 weeks post-CY. A small increase in serum IgG level was noted after greater than 12 months of pulse CY therapy; no increase was seen in CSF IgG levels. A preferential decrease in the number of CD4+ T cells was also seen in the CY-treated MS patients. We propose that the observed increase in the number of spontaneous Ig PFC was due to the CY-induced disruption of the CD4+ T cell-mediated control of in vivo activated B cells.

Antibody-Producing Cells↗