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Biomedical subjects

J M Provenzale

Publications and source records attributed to J M Provenzale.

At least 55 records · Page 3Linked to original sources
PubMed1999

Anti-phospholipid antibodies in patients with multiple sclerosis and MS-like illnesses: MS or APS?

OBJECTIVE: To describe the frequency, clinical, and laboratory features of patients diagnosed with multiple sclerosis (MS) or MS-like illnesses (MSL) among a large, prospectively followed cohort of anti-phospholipid antibody (aPL)-positive patients. METHODS: Between 1990 and 1995 patients referred to a university-affiliated rheumatology clinic were prospectively evaluated for aPL based on questionnaires designed to detect aPL-related symptoms and/or a family history of aPL-related illnesses. Magnetic resonance imaging (MRI) was performed when significant neurological features were present. A subgroup of all patients diagnosed with MS or MSL was identified and their clinical, laboratory, and imaging findings were reviewed. RESULTS: Of 322 patients evaluated for aPL-related symptoms or events, 189 (59%) were positive for at least one class of aPL. Twenty-six of 322 patients (8%) carried a diagnosis of MS or MSL, either at the initial evaluation or during the study period. Twenty-three of the 26 individuals (88%) tested positive for aPL, while the remaining 3 (11%) tested repeatedly negative. Eighteen of the 23 patients (78%) had either more than one class of aPL or had multiple positive titers. IgM aCL was noted in 18 of the 23 patients (78%). Oligoclonal bands were noted in five patients. Antinuclear antibodies (ANA) and low complement levels were frequently observed. Blinded MRI readings showed lesions consistent with MS in the majority of cases. Clinically, 7 patients had transverse myelitis (TM), while optic neuritis (ON) was present in 8 patients. Most patients had either occult symptoms of rheumatic disease or contributory family histories. None had a defined underlying connective-tissue disease. CONCLUSION: A substantial number of aPL-positive patients have a concurrent diagnosis of MS or MSL, frequently presenting with elevated IgM aCL, optic neuritis, and transverse myelitis. The anti-phospholipid syndrome (APS) should be strongly considered as an alternative diagnosis to MS in these patients.

Adolescent↗
PubMed1999

Irinotecan therapy in adults with recurrent or progressive malignant glioma.

PURPOSE: To determine the activity, toxicity, and pharmacokinetics of irinotecan (CPT-11, Camptosar; Pharmacia & Upjohn, Kalamazoo, MI) in the treatment of adults with progressive, persistent, or recurrent malignant glioma. PATIENTS AND METHODS: Patients with progressive or recurrent malignant gliomas were enrolled onto this study between October 1996 and August 1997. CPT-11 was given as a 90-minute intravenous (i.v.) infusion at a dose of 125 mg/m2 once weekly for 4 weeks followed by a 2-week rest, which comprised one course. Plasma concentrations of CPT-11 and its metabolites, SN-38 and SN-38 glucuronide (SN-38G), were determined in a subset of patients. RESULTS: All 60 patients who enrolled (36 males and 24 females) were treated with CPT-11 and all were assessable for toxicity, response, and survival. Pharmacokinetic data were available in 32 patients. Nine patients (15%; 95% confidence interval, 6% to 24%) had a confirmed partial response, and 33 patients (55%) achieved stable disease lasting more than two courses (12 weeks). Toxicity observed during the study was limited to infrequent neutropenia, nausea, vomiting, and diarrhea. CPT-11, SN-38, and SN-38G area under the plasma concentration-time curves through infinite time values in these patients were approximately 40%, 25%, and 25%, respectively, of those determined previously in patients with metastatic colorectal cancer not receiving antiepileptics or chronic dexamethasone treatment. CONCLUSION: Response results document that CPT-11, given with a standard starting dose and treatment schedule, has activity in patients with recurrent malignant glioma. However, the low incidence of severe toxicity and low plasma concentrations of CPT-11 and SN-38 achieved in this patient population suggest that concurrent treatment with anticonvulsants and dexamethasone enhances drug clearance.

Adult↗
PubMed1999

Gangliogliomas: characterization by registered positron emission tomography-MR images.

OBJECTIVE: The purpose of this study was to correlate 18F-fluorodeoxyglucose positron emission tomography (PET) and MR imaging features of cerebral gangliogliomas before and after PET-MR image registration. CONCLUSION: After registration of PET and MR images, all six gangliogliomas in our series were shown to have heterogeneous metabolic activity. Areas of hypermetabolic activity were seen in all lesions. In five of the six cases, PET-MR image registration provided information regarding tumor metabolism that was not available on nonregistered hard-copy examinations.

Adolescent↗
PubMed1999

White matter lesion contrast in fast spin-echo fluid-attenuated inversion recovery imaging: effect of varying effective echo time and echo train length.

OBJECTIVE: Our aim was to determine whether the contrast between white matter lesions and normal-appearing white matter in fast spin-echo fluid-attenuated inversion recovery (FLAIR) images can be improved by lengthening the effective TE and the echo train length. SUBJECTS AND METHODS: Thirty patients with various white matter lesions were imaged using fast spin-echo FLAIR sequences (TR = 10,002 msec; inversion time = 2200) on a 1.5-T MR imaging system. For 14 patients, fast spin-echo FLAIR sequences with a TE of 165 msec and echo train length of 32 (fast spin-echo FLAIR 165/32) were compared with fast spin-echo FLAIR sequences with a TE of 125 msec and echo train length of 24 (fast spin-echo FLAIR 125/24). For 16 other patients, fast spin-echo FLAIR 165/32 sequences were compared with fast spin-echo FLAIR sequences with a TE of 145 msec and echo train length of 28 (fast spin-echo FLAIR 145/28). Signal difference-to-noise ratios were calculated between the lesions and normal-appearing white matter for a typical lesion in each patient. RESULTS: In both groups, a small but statistically significant increase in the signal difference-to-noise ratio was found on the fast spin-echo FLAIR sequences using the longer TE and echo train length. In the first group, signal difference-to-noise ratio increased from 18.7 +/- 4.7 (mean +/- SD) for fast spin-echo FLAIR 125/24 to 20.1 +/- 4.5 for fast spin-echo FLAIR 165/32 (p < .05). In the second group, the signal difference-to-noise ratio increased from 15.4 +/- 4.0 for fast spin-echo FLAIR 145/28 to 16.8 +/- 4.6 for fast spin-echo FLAIR 165/32 (p <.01). In addition, fast spin-echo FLAIR sequences with a longer TE and echo train length were obtained more rapidly (6 min for fast spin-echo FLAIR 125/24, 5 min 20 sec for fast spin-echo FLAIR 145/28, and 4 min 41 sec for fast spin-echo FLAIR 165/32). CONCLUSION: Lengthening the TE to 165 msec and echo train length to 32 in fast spin-echo FLAIR imaging allows both a mild improvement in the contrast between white matter lesions and normal-appearing white matter and shorter imaging times.

Brain↗
PubMed1999

Diffusion-weighted MR imaging in acute stroke: theoretic considerations and clinical applications.

The major clinical use of diffusion-weighted imaging to date has been in evaluation of cerebral infarction, at which it excels. However, diffusion-weighted imaging has also shown promise for other applications, ranging from quantitative analysis of biologic changes that are not apparent from simple visual inspection of images (but are detectable after using regions of interest on apparent diffusion coefficient maps) to better characterization of other intracranial abnormalities (e.g., abscess and tumor). Both the clinical and research applications of diffusion-weighted imaging can be expected to increase, providing fresh insights into physiologic characteristics of both normal and abnormal tissue.

Acute Disease↗
PubMed1999

Malignant supratentorial ganglioglioma (ganglion cell-giant cell glioblastoma): a case report and review of the literature.

BACKGROUND: From both epidemiologic and pathologic viewpoints, gangliogliomas exhibiting components of giant cell glioblastomas are extraordinary neoplasms. We report herein the case of a 6-year-old girl who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell tumor-giant cell glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. METHODS: Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 microm for histochemical and immunohistochemical analyses. Hematoxylin-eosin-stained sections and immunohistochemically stained sections from the primary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear antigen was evaluated. RESULTS: Histologically, 2 distinct cell populations were noted on both the primary and secondary resections. The primary resection revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a diagnosis of ganglioglioma. The second resection (following therapy) demonstrated a much more prominent dysmorphic ganglion cell component and a subdued glial component. CONCLUSION: Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogliomas and rare cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histologic findings in a giant cell glioblastoma and a ganglioglioma and documents the aggressive biologic behavior of this complex neoplasm.

Brain Neoplasms↗
PubMed1998

Phase II study of carboplatin (CBDCA) in progressive low-grade gliomas.

In this study, the authors sought to investigate the response rate and toxicity of carboplatin in patients with progressive low-grade glioma (LGG). Thirty-two patients with progressive LGG were treated with carboplatin at a dosage of 560 mg/m(2). Treatment was given at 4-week intervals and continued until the disease progressed, unacceptable toxicity supervened, or for 12 additional courses after achieving maximal response. Patients with stable disease were treated with a total of 12 cycles. All patients were treated as outpatients. Patients were evaluated for response to treatment and toxicity. All patients received a minimum of two cycles of carboplatin, and were examined for response. A partial response was achieved in nine patients (28%) and a minimal response in two (6%), for an overall response rate of 34% (11 of 32 patients). Eighteen patients (56%) had stable disease. A partial response was achieved in the nine patients after a median of six cycles (range 4-11 cycles), a minimal response was achieved in the two patients after five cycles. Glioma progression was noted in three patients after three, five, and five cycles, respectively. The 11 patients in whom some response was achieved had either an optic pathway tumor or a juvenile pilocytic astrocytoma. Twenty-six of the 32 patients had those characteristics, making the response rate in that group 42% (11 of 26 patients). Thirty-two patients received a total of 387 cycles of chemotherapy. Hematological toxicity was moderate. Twenty-one patients developed thrombocytopenia (platelet count < 50,000/microl); three patients required one platelet transfusion each. Nine patients developed neutropenia (absolute neutrophil count < 500/microl); one developed fever and required administration of antibiotic agents. One dose adjustment in each of the patients prevented further thrombocytopenia and neutropenia. Two patients with stable disease died of respiratory complications. One patient developed Grade III ototoxicity after receiving five cycles, one patient developed hypersensitivity to carboplatin, and none developed nephrotoxicity. Carboplatin given at a dosage of 560 mg/m(2) every 4 weeks has activity in patients with progressive LGG. This drug regimen is relatively simple and well tolerated. Further investigation and longer follow-up study are warranted.

Journal Article↗
PubMed1998

Recurrent thrombosis of the superior vena cava associated with activated protein C resistance: imaging findings.

The purpose of this report is to describe imaging findings in activated protein C resistance, a hereditary cause of recurrent thrombosis. The case described was unusual in that a neonate was affected, whereas the vast majority of cases occur in adulthood. This entity is important to diagnose because of the recurrent nature of thromboses and the fact that relatives are often affected.

Blood Coagulation Disorders↗
PubMed1998

Disseminated thrombosis in primary antiphospholipid syndrome: MR findings.

We report the MR imaging findings in a patient with primary antiphospholipid syndrome, adrenal infarction and widespread thrombosis involving abdominal, pelvic, and pulmonary vessels. This syndrome should be suspected in patients with thromboses and organ infarctions of otherwise undetermined etiology.

Adrenal Glands↗
PubMed1998

Metastatic rhabdomyosarcoma presenting as intracranial hemorrhage: imaging findings.

The CNS is rarely the first site of metastasis for rhabdomyosarcoma. CNS involvement is uncommon, and usually seen as leptomeningeal spread after development of pulmonary metastases. We present the imaging findings in a 13-year-old boy in whom a large intracranial hemorrhage was the initial presentation of surgically documented metastatic prostatic rhabdomyosarcoma. Multiple chest CTs and radionuclide bone scans had previously shown no evidence of pulmonary or osseous metastases. The significance of this case in relation to the possible role of the need for use of chemotherapeutic agents that cross the blood-brain barriers to prevent brain metastasis is discussed.

Brain Neoplasms↗
PubMed1998

Schistosomal myelitis: findings at MR imaging.

Schistosomal spinal cord involvement is a rare complication of infection by Schistosoma mansoni. This work reports the MR imaging findings in a patient with schistosomal myelitis involving the lower spinal cord and conus medullaris. MR imaging showed a focal mass with a slightly nodular surface and which had peripheral enhancement after contrast administration. The MR appearance of this complication of schistosomal infection is important to recognize so that early medical therapy can be started in an attempt to maximize neurologic recovery.

Adult↗
PubMed1998

Cerebrovascular disease risk factors: neuroradiologic findings in patients with activated protein C resistance.

PURPOSE: To assess the patterns of abnormal neuroradiologic findings in patients with a hypercoagulable state related to activated protein C (APC) resistance. MATERIALS AND METHODS: Records in 23 patients with a hypercoagulable state related to APC resistance (18 women, five men; average age, 44.5 years) were reviewed for cerebrovascular disease risk factors and other causes of a hypercoagulable state. Computed tomographic scans, magnetic resonance (MR) images, angiograms, and transesophageal echocardiograms were also reviewed. RESULTS: Stroke risk factors or other causes of a hypercoagulable state were found in 12 patients. Arterial infarcts were seen in 18 patients. Hyperintense white matter foci were seen on MR images in six patients. Dural sinus thrombosis was found in four patients. Angiograms of intracranial circulation in six patients showed major artery occlusions in four. MR angiograms in four patients showed internal carotid artery occlusion in one. No major abnormalities were seen in extracranial cerebral vasculature in 15 patients. Transesophageal echocardiograms in 11 patients showed a patent foramen ovale in one patient but no systemic source of embolism. Seven patients had non-central nervous system thrombotic events. CONCLUSION: Patients with APC resistance and stroke appear to differ from the general stroke population in terms of age and frequency of extracranial sources of cerebrovascular disease.

Adolescent↗
PubMed1998

Familial leukoencephalopathy in bipolar disorder.

OBJECTIVE: Imaging studies of patients with bipolar disorder demonstrate changes in deep white matter and subcortical gray nuclei that are seen as focal hyperintensities on T2-weighted magnetic resonance imaging (MRI). The objective of this study was to examine MRIs in a family with a strong history of bipolar disorder to look for possible MRI abnormalities in members with and without affective illness. METHOD: The authors obtained MRIs of 21 members of a family with a strong history of bipolar disorder. Eight of the family members studied had bipolar illness, one had symptoms of bipolar disorder but did not meet full DSM-III-R criteria, two had unipolar disorder, and 10 did not have bipolar disorder. RESULTS: Fifteen of the 21 family members had MRI findings, including six of 10 family members who had no affective disorder and all of those with bipolar disorder. Lesions of both white matter and subcortical gray nuclei were found. CONCLUSIONS: Although the clinical significance of these MRI findings is unknown, the high prevalence of MRI findings in both affected and unaffected family members suggests that MRI findings may potentially serve as a biological marker for bipolar disorder. Recent genetic studies have established a link between familial leukoencephalopathy and chromosome 19. If leukoencephalopathy appears to be related to bipolar disorder, it may allow clearer characterization of the genetics of the disorder.

Adolescent↗
PubMed1998

DNA mismatch repair and O6-alkylguanine-DNA alkyltransferase analysis and response to Temodal in newly diagnosed malignant glioma.

PURPOSE: We evaluated the response to Temodal (Schering-Plough Research Institute, Kenilworth, NJ) of patients with newly diagnosed malignant glioma, as well as the predictive value of quantifying tumor DNA mismatch repair activity and O6-alkylguanine-DNA alkyltransferase (AGT). PATIENTS AND METHODS: Thirty-three patients with newly diagnosed glioblastoma multiforme (GBM) and five patients with newly diagnosed anaplastic astrocytoma (AA) were treated with Temodal at a starting dose of 200 mg/m2 daily for 5 consecutive days with repeat dosing every 28 days after the first daily dose. Immunochemistry for the detection of the human DNA mismatch repair proteins MSH2 and MLH1 and the DNA repair protein AGT was performed with monoclonal antibodies and characterized with respect to percent positive staining. RESULTS: Of the 33 patients with GBM, complete responses (CRs) occurred in three patients, partial responses (PRs) occurred in 14 patients, stable disease (SD) was seen in four patients, and 12 patients developed progressive disease (PD). Toxicity included infrequent grades 3 and 4 myelosuppression, constipation, nausea, and headache. Thirty tumors showed greater than 60% cells that stained for MSH2 and MLH1, with three CRs, 12 PRs, three SDs, and 12 PDs. Eight tumors showed 60% or less cells that stained with antibodies to MSH2 and/or MLH1, with 3 PRs, 3 SDs, and 2 PDs. Eleven tumors showed 20% or greater cells that stained with an antibody to AGT, with 1 PR, 2 SDs, and 8 PDs. Twenty-five tumors showed less than 20% cells that stained for AGT, with 3 CRs, 12 PRs, 4 SDs, and 6 PDs. CONCLUSION: These results suggest that Temodal has activity against newly diagnosed GBM and AA and warrants continued evaluation of this agent. Furthermore, pretherapy analysis of tumor DNA mismatch repair and, particularly, AGT protein expression may identify patients in whom tumors are resistant to Temodal.

Adult↗
PubMed1998

Iodine-131-labeled antitenascin monoclonal antibody 81C6 treatment of patients with recurrent malignant gliomas: phase I trial results.

PURPOSE: To determine the maximum-tolerated dose (MTD) of iodine 131 (131I)-labeled 81C6 monoclonal antibody (mAb) in brain tumor patients with surgically created resection cavities (SCRCs) and to identify any objective responses to this treatment. METHODS: In this phase I trial, eligible patients were treated with a single injection of 131I-labeled 81C6. Cohorts of three to six patients were treated with escalating dosages of 131I (starting dose of 20 mCi with a 20-mCi escalation in subsequent cohorts) administered through an Ommaya reservoir in the SCRC. Patients were followed up for toxicity and response until death or for a minimum of 1 year after treatment. The SCRC patients, who were previously irradiated, were followed up without additional treatment unless progressive disease was identified. RESULTS: We administered 36 treatments of 131I doses up to 120 mCi to 34 previously irradiated patients with recurrent or metastatic brain tumors. Dose-limiting toxicity was reached at 120 mCi and was limited to neurologic or hematologic toxicity. None of the patients treated with less than 120 mCi developed significant neurologic toxicity; one patient developed major hematologic toxicity (MHT). The estimated median survival for patients with glioblastoma multiforme (GBM) and for all patients was 56 and 60 weeks, respectively. CONCLUSION: The MTD for administration of 131I-labeled 81C6 into the SCRCs of previously irradiated patients with recurrent primary or metastatic brain tumors was 100 mCi. The dose-limiting toxicity was neurologic toxicity. We are encouraged by the minimal toxicity and survival in this phase I trial. Radiolabeled mAbs may improve the current therapy for brain tumor patients.

Adolescent↗
PubMed1998

Systemic thrombosis in patients with antiphospholipid antibodies: lesion distribution and imaging findings.

OBJECTIVE: The purpose of this study was to determine the patterns of non-CNS thromboses in patients with a hypercoagulable state associated with antiphospholipid antibodies (APA). MATERIALS AND METHODS: A search of our institution's clinical coagulation and immunology laboratories' records of patients examined from January 1993 to January 1996 revealed 1290 patients with APA. Computerized records of radiologic studies were reviewed for evidence of thrombotic events, which were found in 93 patients (49 males and 44 females; average age, 40 years). The anatomic distribution of thrombotic events was recorded. RESULTS: Fifty-five patients (59%; 29 males and 26 females; average age, 44.2 years) had solely venous thromboses, 26 patients (28%; 15 males and 11 females; average age, 33.1 years) had solely arterial thromboses, and 12 (13%; 5 males and 7 females; average age, 35.4 years) had both types of events. Deep vein thrombosis (DVT) of the legs was the most common finding, occurring in 45 patients (48%). Six patients had recurrent DVT. Other sites of venous thrombotic events included pulmonary embolism, 30 patients (32%); thoracic veins (superior vena cava, subclavian vein, or jugular vein), 10 patients (11%); and abdominal or pelvic veins, 18 events in 11 patients (12%). Sites of arterial thromboses included arteries supplying the upper limbs (great vessels arising from the aorta or the brachial, radial, ulnar, or digital arteries), 15 events in 12 patients (13%); aorta, one patient (1%); abdominal or pelvic arteries, 11 events in eight patients (9%); and arteries supplying the lower limbs (femoral or popliteal arteries), seven patients (8%). CONCLUSION: Venous thromboses were more common than arterial thromboses in our patient group, with DVT being the most common. However, thromboses in sites that are unusual for the general population were also relatively common. APA should be suspected in patients with thromboses in unusual sites or recurrent thromboses of an otherwise unexplained cause.

Adult↗