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N J Patronas

Publications and source records attributed to N J Patronas.

At least 19 recordsLinked to original sources
PubMed1992

Terminal transverse limb defects associated with familial cavernous angiomatosis.

Terminal transverse limb defects rarely are reported as familial. Multiple pathogenetic mechanisms, including vascular disruption, have been proposed to account for these defects. We report on a family followed over the past 6 years known to have familial cavernous angiomatosis in which 2 relatives have similar terminal transverse defects at the mid-forearm. Multiple relatives have had episodic bleeding from intracranial cavernous angiomas, a distinct finding in this disorder. Other findings in this family include retinal cavernous angiomas (2 patients), a high incidence of skin angiomas (12 patients), cavernous angiomas of the soft tissue (2 patients), and a hepatic angioma (one patient). One of the 2 individuals with the limb defect was evaluated extensively. Magnetic resonance imaging of the forearm with the terminal transverse defect using gadolinium-DTPA enhancement showed abrupt termination of all structures distal to the normal radial and ulnar heads. We propose that familial cavernous angiomatosis may be a new cause of vascular disruption resulting in terminal transverse limb defects.

Adolescent↗
PubMed1991

Magnetic resonance imaging of canine salivary glands after ductal ligation and stimulation by pilocarpine.

We performed magnetic resonance imaging in six dogs after submandibular ductal ligation on one side, followed by secretory stimulation with intraperitoneal injection of pilocarpine (5 mg/kg). On the images obtained after ductal ligation but before stimulation, there was no significant change in signal intensity on either side. After injection of pilocarpine the signal intensity of the ligated gland increased significantly (p less than 0.01) in T2-weighted images and nonsignificantly in inversion recovery images, and remained constant in T1-weighted images. Significant (p less than 0.01) volume increases of the glands after ligation and stimulation were measured for the T1, T2, and inversion recovery protocols. These findings likely reflect the retention of saliva caused by pilocarpine stimulation and the absence of drainage through the obstructed duct. The results indicate that the T2-weighted images obtained after pilocarpine stimulation may be useful for studying patients with disturbance of major salivary gland function and may provide an objective basis for the noninvasive diagnosis of unilateral stenosis.

Animals↗
PubMed1991

Pulse cyclophosphamide for severe neuropsychiatric lupus.

We studied the effect of parenteral pulse cyclophosphamide therapy in nine patients with active systemic lupus erythematosus and severe central nervous system involvement. Seven patients had focal neurological deficits and/or seizures associated with abnormalities on cerebrospinal fluid analysis and/or magnetic resonance imaging. Two patients had organic brain syndrome with psychosis and normal cerebrospinal fluid and/or magnetic resonance imaging analysis. Six patients were unresponsive to treatment with high dose corticosteroid. Cyclophosphamide, 0.75-1.0 g/m2 body surface area, was administered intravenously every month for at least 2 months. Eight patients had a complete recovery or recovered with minor residuals. Cyclophosphamide was well tolerated with few side effects. We conclude that parenteral pulse cyclophosphamide is an effective adjunctive therapy for the management of patients with active systemic lupus erythematosus and central nervous system symptoms.

Adolescent↗
PubMed1991

Positron emission tomography of pituitary macroadenomas: hormone production and effects of therapies.

Positron emission tomography with [18F]fluorodeoxyglucose (FDG) was carried out in 24 patients with pituitary macroadenomas (32 studies) to assess the glucose utilization of these tumors in vivo. The adenoma metabolic index, which is the ratio of FDG uptake of tumor to a whole brain slice, was calculated. Comparisons were made between tumor uptake of FDG and hormone secretion and response to therapies. In each positron emission tomography study, the macroadenoma could be easily identified visually as an area of increased FDG uptake near the region of the sella. FDG uptakes were highest for nonfunctional adenomas, and the prolactin, growth hormone, and thyroid-stimulating hormone-producing groups displayed similar levels of glucose metabolism. The adenoma metabolic index for all tumors averaged 1.3, ranging from 0.3 for a thyroid-stimulating hormone adenoma to 3.5 for a nonfunctional tumor. Tumors did not exhibit metabolic rates that could characterize the type of hormone produced. Recurrent macroadenomas displayed metabolism similar to tumors not operated on, whereas irradiated adenomas showed lower glucose uptake than nonirradiated tumors. Drug therapy with bromocriptine or the long-acting somatostatin analogue octreotide also decreased the glucose utilization of the tumor. There was no correlation between the amount of hormone produced and the adenoma metabolic index when a group of tumors was analyzed. Patients scanned more than once, however, demonstrated changes in hormone levels that changed or did not change in parallel with tumor metabolism. Thus, positron emission tomography offers the potential capability for predicting and defining the growth of pituitary adenomas. This may be of particular value when plasma hormone assays and conventional imaging techniques prove inadequate for monitoring patient response to therapy.

Adenoma↗
PubMed1991

Central nervous system involvement in Von Hippel-Lindau disease.

Fifty individuals with Von Hippel-Lindau disease (VHL) were studied with gadolinium-enhanced magnetic resonance imaging (MRI) to determine the frequency and distribution of CNS lesions. The associated clinical features were also reviewed. Thirty-six (72%) of the 50 had 1 or more CNS tumors. The most frequently affected sites in the CNS excluding the retina were the cerebellum (52%), spinal cord (44%), and brainstem (18%). New regional predilections for the craniocervical junction and conus medullaris were demonstrated by this study. Forty-one percent of all VHL patients with CNS tumors were neurologically asymptomatic: cerebellar tumors (50%), spinal cord tumors (50%), and brainstem tumors (44%) were often without clinical signs or symptoms. Multiple lesions were common. The mean age of all VHL patients (34.5 years) was similar to the mean age of all CNS VHL patients (34.4 years), suggesting a lack of age association. CNS lesions commonly occurred in the 2nd decade of life. All patients at risk for VHL should be evaluated using gadolinium-enhanced MRI after 10 years of age, although ophthalmic examination should be initiated within the 1st 2 years of life. Enhanced MRI is particularly useful in the detection of CNS tumors in patients with the VHL gene.

Adolescent↗
PubMed1990

Disk fragment migration.

This report analyzes the most frequently observed migration paths of disk fragments in 47 patients who had extruded or sequestered disks. Observations are based principally on magnetic resonance (MR) images. When disk fragments moved in a superior (42%) or inferior (40%) direction from the donor disk, the displaced disk components were most frequently (94%) dislodged into the right or left half of the anterior epidural space (AES) and rarely straddled the midline. To explain this phenomenon, the authors investigated the anatomy of the AES by dissecting four cadaver specimens and reviewing 300 MR images of the spine. They conclude that the migrating path of a disk fragment is determined by the anatomy of the AES, a fairly well-defined space delimited posteriorly by the posterior longitudinal ligament and by membranes laterally attached to it. It consists of two compartments separated by a sagitally aligned septum. During migration, sequestered disk fragments usually stay in these compartments.

Cervical Vertebrae↗
PubMed1990

The diagnosis of acromegaly: value of inferior petrosal sinus sampling.

The early diagnosis of acromegaly may be difficult when serum levels of growth hormone are minimally elevated and imaging of the pituitary gland fails to show an adenoma. However, transsphenoidal surgery has the greatest chance of cure at this stage. We therefore investigated the value of sampling petrosal sinuses for measurement of growth hormone in this group of patients. Simultaneous bilateral sampling of the inferior petrosal sinuses to measure serum concentrations of growth hormone was performed in five patients suspected of having acromegaly but with nondiagnostic CT scans (n = 5) and MR images (n = 3) of the pituitary gland. Levels of growth hormone from the petrosal sinuses were five to 36 times greater than levels in the peripheral veins in all five patients, and three of four showed a marked response to growth hormone-releasing hormone. During transsphenoidal surgery, growth hormone-producing microadenomas were resected completely in four patients. In the fifth patient, a left-sided microadenoma had invaded the cavernous sinus and could not be resected completely. Lateralization of the adenomas within the pituitary gland on the basis of differences in levels of growth hormone between the two petrosal sinuses was not completely reliable. Elevated levels of growth hormone in selective samples from the inferior petrosal sinuses can help support an early diagnosis of acromegaly when peripheral growth hormone levels and imaging are not diagnostic.

Acromegaly↗
PubMed1990

Detection of malignant bone tumors: MR imaging vs scintigraphy.

One hundred six patients with a known or suspected diagnosis of bone cancer (11 patients with biopsy-proved primary tumors, 95 patients with metastatic disease) were evaluated with scintigraphy and MR imaging to determine the relative sensitivity of each technique in the detection of bone disease. MR imaging was performed at 0.5 T as part of the entry evaluation into Intramural Research Board protocols (30%), for evaluation of cord compression, or because of an equivocal scintigram. MR was performed with T1-weighted (e.g., 300-500/10-20 [TR/TE]), T2-weighted (e.g., 2000/80) spin-echo (SE), and a short-TI inversion recovery (STIR) pulse sequence. Scintigrams were performed with 99mTc-methylene diphosphonate. A retrospective analysis showed that in 30 (28%) of 106 patients, MR imaging performed over a limited region of interest revealed a focal abnormality consistent with tumor that was not observed on scintigraphy. Only one patient had an abnormality on scintigraphy, caused by a metastasis, that was not found on MR images. In 73 (69%) of the 106 patients, the results of MR imaging and scintigraphy were equivalent; in 41 cases results of both techniques were normal. A McNemar analysis of the discordant cases showed MR imaging to be more sensitive than scintigraphy was (p less than .001). Our results suggest that although MR imaging has a greater sensitivity in detecting focal disease, scintigraphy is still the most useful screening test for evaluating the entire skeleton. MR imaging should be reserved for clarification of scintigraphic findings when suspicion is high for tumor.

Adolescent↗
PubMed1990

Metalloporphyrin magnetic resonance contrast agents. Feasibility of tumor-specific magnetic resonance imaging.

The criteria for tumor-specific MRI contrast agents are considered. The metalloporphyrins have been shown to be potential tumor-specific contrast agents due to their selective retention by cancer cells. The Mn(III) water-soluble porphyrin complexes are preferred on the basis of relaxivity and stability. Protein binding in human plasma enhances the relaxivity of Mn(III)-tetraphenylsulfonato-porphyrin (MnTTPS). In preliminary work this agent was shown to enhance MRI contrast at 0.5 T in subcutaneous human colon carcinomas grown in nude mice. Here we report further evidence of enhanced contrast in the same system, and extend this work to human breast tumors in nude mice using a 2 T animal imager. Questions of metalloporphyrin stability, toxicity and dose-contrast relationship are discussed.

Animals↗
PubMed1990

Acute transverse myelitis in systemic lupus erythematosus: magnetic resonance imaging and review of the literature.

We describe a patient with systemic lupus erythematosus (SLE) who presented with acute transverse myelitis. Magnetic resonance imaging (MRI) of the cervical spine demonstrated increased signal intensity and diffuse edema in the cervical cord. The patient had low titer of IgG antiphospholipid antibodies and hypocomplementemia. Cerebrospinal fluid analysis showed pleocytosis, increased protein and decreased glucose. Clinical improvement of the neurologic impairment was noted after high dose corticosteroids and intravenous bolus cyclophosphamide. A repeat MRI 12 days after the treatment was normal. MRI of the spine can be useful in the diagnosis and followup of patients with SLE presenting with an acute myelopathic syndrome. Early aggressive treatment may contribute to complete recovery of these patients.

Adolescent↗
PubMed1990

Abnormal magnetic-resonance scans of the lumbar spine in asymptomatic subjects. A prospective investigation.

We performed magnetic resonance imaging on sixty-seven individuals who had never had low-back pain, sciatica, or neurogenic claudication. The scans were interpreted independently by three neuro-radiologists who had no knowledge about the presence or absence of clinical symptoms in the subjects. About one-third of the subjects were found to have a substantial abnormality. Of those who were less than sixty years old, 20 per cent had a herniated nucleus pulposus and one had spinal stenosis. In the group that was sixty years old or older, the findings were abnormal on about 57 per cent of the scans: 36 per cent of the subjects had a herniated nucleus pulposus and 21 per cent had spinal stenosis. There was degeneration or bulging of a disc at at least one lumbar level in 35 per cent of the subjects between twenty and thirty-nine years old and in all but one of the sixty to eighty-year-old subjects. In view of these findings in asymptomatic subjects, we concluded that abnormalities on magnetic resonance images must be strictly correlated with age and any clinical signs and symptoms before operative treatment is contemplated.

Adult↗
PubMed1989

Positron emission tomography in the detection of malignant degeneration of low-grade gliomas.

The management of low-grade gliomas represents a challenge to the physician as a significant proportion may undergo malignant degeneration to a high-grade tumor. We present the positron emission tomography (PET) scans, using [18F] fluorodeoxyglucose (FDG), of 12 patients who have histological and/or clinical evidence of malignant degeneration of a low-grade glioma. Each scan displays a focal area of hypermetabolism similar to that of malignant gliomas which arise de novo. Three patients also underwent PET scanning prior to malignant degeneration. When the initial scan is compared with the postmalignant degeneration study, a difference in tumoral glucose uptake can be recognized. A region previously shown to be hypometabolic develops focal hypermetabolism as malignant changes evolve. This study displays the utility of FDG-PET in the evaluation of malignant degeneration of low-grade gliomas. The knowledge that a neoplasm has altered its biological behavior may influence subsequent therapeutic options. If these findings can be confirmed in larger series and by other investigators, it is possible that FDG-PET may be adopted as one of the diagnostic tools for guiding the management of low-grade gliomas.

Adult↗
PubMed1989

The effect of intravenous interleukin-2 on brain water content.

Parenteral treatment with interleukin-2 (IL-2) is effective against certain advanced cancers outside the central nervous system. Prior to commencement of Phase II trials in patients with brain tumors, the neurological and neuroradiological features of 10 patients treated with intravenous administration of repeated doses of IL-2 were studied. Three patients had malignant gliomas, and seven patients had extracranial cancer without evidence of intracranial metastasis. All were treated with intravenous doses of 10(5) U/kg three times daily for up to 5 days. The patients with gliomas received cranial computerized axial tomography (CT) scans before IL-2 therapy was initiated and during the later stages of treatment. The patients with extracranial cancer underwent T2-weighted magnetic resonance (MR) imaging before and later during therapy. After two to 11 doses of IL-2, the patients with gliomas had marked neurological deterioration that was associated with a mild to marked increase in peritumoral edema and mass effect visible on CT scans. With cessation of treatment and appropriate supportive care, all returned to their pretreatment state. The patients with extracranial cancer were either neurologically unchanged or underwent minor transient changes in mental status (lethargy and confusion). In these patients, the MR signal intensity was quantified and compared in eight anatomic regions of interest. In six of the seven patients, there were increases in gray and white matter signal intensity consistent with increased cerebral water content. The percentage changes (means +/- standard error of the means) were 12.6% +/- 7.3% in the gray matter and 17.0% +/- 6.2% in the white matter. This study demonstrates that treatment with a high parenteral dose of IL-2 is not tolerated by patients with gliomas due to increased cerebral edema. In patients with extracranial cancer but no brain disease, parenteral IL-2 induces an increase in the cerebral water content of both gray and white matter.

Brain Chemistry↗