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Biomedical subjects

A H Friedman

Publications and source records attributed to A H Friedman.

At least 19 recordsLinked to original sources
PubMed1995

Thromboembolic complications after Fontan operations.

BACKGROUND: Despite the increasing recognition of thromboembolic complications of the Fontan procedure, data characterizing such events are limited. The total cavopulmonary connection is believed to be less prone to this complication than other modifications of Fontan operations. We examined our experience with thromboembolism after Fontan operations to better characterize these events and their relation to the type of Fontan operation performed. METHODS AND RESULTS: We retrospectively identified 70 patients who underwent a Fontan operation between January 1978 and March 1994. Patients were divided into three groups: (1) total cavopulmonary connection, (2) atriopulmonary connection, and (3) conduit interposition. Fourteen patients (20%) developed a thromboembolic complication during a mean (+/- SD) follow-up of 5.2 +/- 4.7 years. The rate of thrombosis was similar in each group. The time from Fontan operation to thrombosis averaged 6.1 +/- 5.0 years. The overall rate of thromboembolic events was 3.9 per 100 patient-years. Twelve of the 14 thrombi were located within the venous circulation, 1 was in the left ventricle, and the location of 1 was undetermined. Six of the patients (43%) were asymptomatic, 3 (21%) presented with cerebrovascular events, and 5 (36%) presented with other symptoms. Thromboembolic events occurred from the perioperative period to 15 years after surgery. CONCLUSIONS: Thromboembolic complications occur frequently after the Fontan operation and its modifications and are a cause of significant morbidity. The time of presentation varies greatly. The rate of thrombosis appears to be similar in all modifications of the Fontan operation.

Adolescent↗
PubMed1995

Chromosome 10 deletion mapping in human gliomas: a common deletion region in 10q25.

The high incidence of loss of chromosome 10 alleles in glioblastoma multiforme suggests the presence on this chromosome of a tumor suppressor gene that is important in glioma tumorigenesis and progression. Our initial deletion mapping studies using restriction fragment length polymorphism markers indicated a common deletion region in 10q24-qter. In an attempt to localize the deleted region further, we screened a panel of 117 gliomas for loss of heterozygosity for chromosome 10 loci using 10 microsatellite markers. Seventeen tumors showed partial loss of a copy of chromosome 10 and were further analysed using 28 additional microsatellite markers. Of these, 10 had terminal deletion in the q arm, three had deletions in both p and q arms, two contained interstitial deletion in 10q and two carried deletions in 10p. In the 15 tumors with deletions in 10q, the minimal overlapping deletion region was in distal 10q between markers D10S587 and D10S216. Loci D10S587 and D10S216 are approximately mapped to a 5 cM region in 10q25.1.

Brain Neoplasms↗
PubMed1995

Fast spin-echo, magnetic resonance imaging-measured hippocampal volume: correlation with neuronal density in anterior temporal lobectomy patients.

To assess the value of magnetic resonance imaging (MRI)-measured hippocampal volume in the detection of hippocampal sclerosis, we studied 28 patients undergoing anterior temporal lobectomy for medically intractable mesial temporal lobe epilepsy. Hippocampal volumetry and visual analysis of T2 signal change were performed using fast spin-echo T2-weighed MRI. Quantitative neuronal density measurements were performed in the resected hippocampal specimens. There was a significant correlation between MRI-measured absolute hippocampal volume (AHV) and neuronal density in CA1, CA2, and CA3 subfields (p < 0.0001, p < 0.01, and p < 0.05, respectively). Differential hippocampal volume (side-to-side volume difference) failed to detect bilateral atrophy in three patients, but the bilateral hippocampal atrophy was recognized by considering AHV in these patients. This study suggests that MRI-measured AHV can be of value in elevating patients with mesial temporal lobe epilepsy, especially when there is no side-to-side difference in hippocampal volumetry.

Adolescent↗
PubMed1995

Neuro-Behçet's disease: factors hampering proper diagnosis.

We reviewed the clinical course of nine patients with neuro-Behçet's disease to assess difficulties in making this diagnosis. Factors delaying proper diagnosis included lack of accurate history and physical examination, lack of recognition of an underlying systemic syndrome and its relationship to the neurologic symptoms, presence of intermittently normal CSF studies, and use of noncontrasted neuroimaging techniques.

Adolescent↗
PubMed1995

Vascular malformations presenting as spinal cord neoplasms: case report.

Three cases of adult patients with subacute courses of progressive caudal spinal cord disease are presented. Computed tomography, magnetic resonance imaging, and myelographic studies were interpreted preoperatively as representing a spinal cord neoplasm in each case. No evidence of enlarged or abnormal surface vessels was observed by neuroimaging or intraoperatively. Biopsy specimens from each spinal cord lesion showed the typical histopathological features of a spinal vascular malformation. We conclude that vascular malformations of the caudal spinal cord can appear as isolated intramedullary lesions with apparently normal surface vessels and that these lesions may be difficult to distinguish from spinal cord neoplasms.

Aged↗
PubMed1995

Dorsal root entry zone lesions for intractable pain after trauma to the conus medullaris and cauda equina.

This review was undertaken to determine the efficacy of using dorsal root entry zone (DREZ) lesions to treat intractable pain caused by trauma to the conus medullaris and cauda equina. Traumatic lesions of this area are unique in that both the spinal cord and the peripheral nerve roots are injured. Although DREZ lesions have been shown to relieve pain of spinal cord origin in many patients, they have been shown not to relieve pain of peripheral nerve origin. Therefore, 39 patients with trauma to the conus medullaris and cauda equina who underwent DREZ lesioning for intractable pain were reviewed retrospectively. The results of this review demonstrate the efficacy of DREZ lesions in these patients. At a mean follow-up period of 3.0 years, 54% of patients were pain-free without medications, and 20% required only nonnarcotic analgesic drugs for pain that no longer interfered with their daily activities. Better outcomes were noted in patients with an incomplete neurological deficit, with pain having an "electrical" character, and with injuries due to blunt trauma. Operative complications included weakness (four patients), bladder or sexual dysfunction (three), cerebrospinal fluid leak (two), and wound infection (two), but overall, 79.5% of patients (31 of 39) were without serious complications. Complications were limited to patients with prior tissue damage at the surgical exploration site and were most prevalent in patients who underwent bilateral DREZ lesions. In conclusion, this preliminary report suggests that DREZ lesions may be useful in combating intractable pain from traumatic injuries to the conus medullaris and cauda equina, with some risk to neurological function that may be acceptable in this group of patients.

Adult↗
PubMed1995

Fast spin-echo MR in hippocampal sclerosis: correlation with pathology and surgery.

PURPOSE: To identify the extent of hippocampal sclerosis in temporal lobe epilepsy with fast spin-echo MR and correlate it with histopathologic findings and surgical outcome. METHODS: MR images of 30 patients with temporal lobe epilepsy and pathologically proved hippocampal sclerosis and 30 control subjects were obtained using a fast spin-echo technique with 4000/100/4 (repetition time/echo time/excitations), 16 echo train, 2- to 3-mm section thickness with interleave, 256 x 256 matrix, and 18-cm field of view. Criteria for MR diagnosis of hippocampal sclerosis included hippocampal atrophy diagnosed with MR volumetry and/or T2-weighted signal change. Hippocampal sectional areas were plotted, and T2 signal changes were topographically evaluated to identify the extent of hippocampal sclerosis, which was subsequently correlated with histopathologic findings and surgical outcome. RESULTS: Hippocampal sclerosis was diffuse, involving both hippocampal head and body, in 96.7% of patients (29 of 30 patients). One patient had normal MR findings. Focal hippocampal sclerosis was not seen. Histopathologic findings of hippocampal sclerosis were present in all 29 patients who had abnormal MR findings. Eighty-six percent of patients (18 of 21 patients), who were followed for at least 1 year after temporal lobectomy, were seizure free (81%, 17 of 21 patients) or significantly improved (5%, 1 of 21 patients). CONCLUSION: Fast spin-echo MR enables accurate definition of the extent of hippocampal sclerosis in patients with temporal lobe epilepsy. All cases of hippocampal sclerosis identified in this study involved the hippocampus diffusely. However, leaving the posterior portion of the hippocampus during surgery does not seem to be a major factor influencing surgical outcome.

Adolescent↗
PubMed1995

Shadow cells in an intracranial dermoid cyst.

A case of an intracranial dermoid cyst containing shadow cells is presented. This case expands the group of lesions in which shadow cells, indicative of hair matrical differentiation, have been described.

Adult↗
PubMed1994

Association and chance occurrence of aniridia and retinoblastoma.

PURPOSE/METHODS: A 9-month-old infant had inherited aniridia and unilateral retinoblastoma. Family history disclosed three generations of aniridia; yet there were no instances of retinoblastoma. The coincidental occurrence of retinoblastoma and aniridia is rare. RESULTS/CONCLUSIONS: With DNA probes from within the retinoblastoma gene, we were able to determine that a retinoblastoma-predisposing mutation was not inherited with aniridia in this family. The occurrence of these two diseases in the same individual therefore represents a chance event.

Aniridia↗
PubMed1994

Alterations of the TP53 gene in human gliomas.

Glial tumors of all grades and histological types from 72 adults and 48 children were analyzed for mutations of the TP53 gene, loss of heterozygosity (LOH) for 17p, and accumulation of TP53 protein to determine whether the incidence and type of TP53 alterations differ among tumors of different histological type and between tumors from adults and children. These tumors were also evaluated for LOH for chromosome 10 and for amplification of the epidermal growth factor receptor, C-MYC, N-MYC, GLI, platelet-derived growth factor receptor-alpha, and murine double minute 2 genes to determine the patterns of molecular alterations involved in the progression of these neoplasms. Seventeen of the 120 tumors contained mutations of the TP53 gene. One of the tumors with TP53 gene mutation was from one of the 48 patients less than 18 years of age. Twelve of the 17 tumors with mutations occurred among the 27 patients in the 18-45-year age group, while 4 tumors with mutations were among the 45 patients more than 45 years old. There was also an increased incidence of TP53 mutation in patients with anaplastic astrocytoma histology. However, no significant association between presence of TP53 mutation and patient survival was observed. These studies demonstrate that TP53 gene mutations are a common mechanism for glial cell neoplasms in the 18-45-year age group but are unrelated to progression and advanced histological grade. LOH for chromosome 10 and gene amplification, however, occurring in 82 and 40%, respectively, of glioblastoma multiforme, whether seen alone or along with TP53 gene alterations, are related to advanced histological grade of the tumor. In childhood gliomas, in contrast, TP53 gene alterations, LOH for 17p and 10q, and gene amplification are uncommon in tumors of all grades, suggesting that presently unknown mechanisms are responsible for the genesis and progression of these tumors.

Adolescent↗
PubMed1994

Neuronal migration disorders: positron emission tomography correlations.

We analyzed the interictal [18F]fluoro-2-deoxy-D-glucose positron emission tomography (PET) findings of 17 epileptic patients with neuronal migration disorders (NMDs). Fifteen patients had abnormal PET findings, i.e., focal hypometabolism in 9 patients and displaced metabolic activity of normal gray matter in 6. All 15 patients had magnetic resonance imaging (MRI) abnormalities; however, PET abnormality assisted in the identification of NMDs on MRI in 3 patients. Two patients with negative MRI also had negative PET studies. PET hypometabolism appeared to correlate with severity of neuronal dysgenesis or temporal lobe involvement, or both. Displaced metabolic activity of gray matter is regarded as a unique interictal [18F]fluoro-2-deoxy-D-glucose-PET finding in NMD. This study demonstrates variable metabolic patterns in NMD and that PET may be a useful complement to MRI in the evaluation of NMD.

Adult↗
PubMed1994

Ipsilateral subcortical atrophy associated with temporal lobectomy.

Stereologic methods for determining the volume of cerebral structures in vivo via magnetic resonance imaging have identified unilateral hippocampal atrophy among patients with complex partial epilepsy of temporal lobe origin. Metabolic imaging has also identified altered metabolism in temporal as well as extratemporal regions among these patients. As the temporal cortex and subcortical nuclei of the limbic system are reciprocally connected with striatal projection fields, we examined the putamen nuclei for evidence of associated extratemporal volume asymmetry in patients before and after temporal lobectomy. There was no evidence of preoperative putamen volume asymmetry, but a significant postoperative decrement in ipsilateral putamen volume was observed. The magnitude of postsurgical putamen volume asymmetry was correlated with the duration of time since resection. Progressive degeneration of extratemporal projections of the temporal lobe may occur in association with temporal lobectomy.

Adolescent↗
PubMed1994

Treatment of radiation-induced nervous system injury with heparin and warfarin.

When radiation is used to treat nervous system cancer, exposure of adjacent normal nervous system tissue is unavoidable, and radiation-induced injury may occur. Acute injury is usually mild and transient, but late forms of radiation-induced nervous system injury are usually progressive and debilitating. Treatment with corticosteroids, surgery, and antioxidants is often ineffective. We treated 11 patients with late radiation-induced nervous system injuries (eight with cerebral radionecrosis, one with a myelopathy, and two with plexopathies, all unresponsive to dexamethasone and prednisone) with full anticoagulation. Some recovery of function occurred in five of the eight patients with cerebral radionecrosis, and all the patients with myelopathy or plexopathy. Anticoagulation was continued for 3 to 6 months. In one patient with cerebral radionecrosis, symptoms recurred after discontinuation of anticoagulation and disappeared again after reinstitution of treatment. We hypothesize that anticoagulation may arrest and reverse small-vessel endothelial injury--the fundamental lesion of radiation necrosis--and produce clinical improvement in some patients.

Adult↗
PubMed1993

Complex partial seizures and mesial temporal sclerosis: evaluation with fast spin-echo MR imaging.

PURPOSE: This study was performed to determine the sensitivity and specificity of fast spin-echo (FSE) magnetic resonance (MR) imaging in detecting mesial temporal sclerosis. MATERIALS AND METHODS: Twenty-one patients with a diagnosis of complex partial seizures were studied. Criteria for abnormality included visual detection of hippocampal MR signal abnormality and atrophy or significantly decreased hippocampal volume determined by using MR morphometric techniques. RESULTS: The overall sensitivity and specificity of MR imaging in demonstrating correct lateralization of seizure focus were both 100%. The positive predictive value of FSE MR imaging was 100%. The negative predictive value was 50%. CONCLUSION: The authors recommend FSE MR imaging of the hippocampus as the imaging technique of choice in the evaluation of patients with complex partial seizures for mesial temporal sclerosis.

Adult↗