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Biomedical subjects

L Ginsberg

Publications and source records attributed to L Ginsberg.

At least 37 records · Page 2Linked to original sources
PubMed1997

Independent segregation of von Hippel-Lindau disease and cerebral cavernomas.

A probable diagnosis of von Hippel-Lindau disease was made in a two generation family in which the proband had a phaeochromocytoma, renal cysts, and multiple cerebral cavernomas. His sister had multiple similar cerebral vascular lesions and his father died from renal carcinoma aged 42. Although the family did not satisfy the conventional diagnostic criteria for von Hippel-Lindau disease, an underlying germline mutation in the von Hippel-Lindau disease tumour suppressor gene was identified in the proband. Molecular genetic analysis not only confirmed the putative diagnosis of the disease in the proband but also showed that the cerebral vascular lesions segregated independently from the von Hippel-Lindau disease mutation. This report exemplifies how molecular genetic investigations can enhance the diagnosis and management of families with suspected von Hippel-Lindau disease, particularly when the manifestations, as in this family, are not typical.

Adult↗
PubMed1997

Craniospinal dissemination of central neurocytoma. Report of two cases.

Central neurocytoma was first described in the literature in 1982 and has been noted to be a benign neuronal tumor usually located in the ventricular system. Of the more than 100 reported cases, only seven recurrences have been reported, all of which have been local. The authors report two cases of recurrent central neurocytoma that disseminated through the ventricular system with seeding to the spine, as evidenced by magnetic resonance images and positive cerebrospinal fluid cytology. The histological appearance of these two tumors was typical for the lesion and lacked evidence of malignant change. Central neurocytoma may not be as benign as previously thought, and the recognition of this more malignant behavior has implications for patient follow up and therapy.

Adult↗
PubMed1997

Recent advances in paclitaxel-containing chemotherapy for recurrent or metastatic squamous cell carcinoma of the head and neck.

Current chemotherapeutic approaches to recurrent or metastatic head and neck cancer have yielded response rates of 10% to 20% for single agents and 30% to 40% for combination chemotherapy. Median survival for patients with recurrent or metastatic disease treated with single agents or combination chemotherapy is between 4 and 6 months. Investigation of new drugs, therefore, has high priority among clinicians and researchers. One new agent that has been effective as single-agent therapy is paclitaxel (Taxol; Bristol-Myers Squibb Company, Princeton, NJ). We tested the combination of paclitaxel, ifosfamide, and cisplatin in recurrent or metastatic head and neck cancer. The starting dose of paclitaxel was 175 mg/m2 as a 3-hour infusion on day 1, ifosfamide 1 g/m2 as a 2-hour infusion on days 1 to 3, and cisplatin 60 mg/m2 via 2-hour infusion on day 1. This schedule was repeated every 3 weeks. Sixty-five patients were entered into the study and 62 patients are currently evaluable for response and toxicity in the phase I and phase II portions of this study. We observed 10 (16%) complete responses and 24 (39%) partial responses. The overall response rate was 55% in phases I/II of this interim analysis. In the phase II part alone, we have observed eight (16%) complete responses and 22 (44%) partial responses to date among 50 evaluable patients. Median survival times were 8.9 months for all patients and 9.7 months for patients in the phase II part of the study. Preliminary results demonstrate significant antitumor activity in patients with recurrent or metastatic head and neck cancer. The paclitaxel/ifosfamide/cisplatin regimen was well tolerated. Chemotherapy with paclitaxel/ifosfamide/cisplatin should be tested as an induction regimen in patients with locally advanced head and neck cancer. It also warrants testing in a randomized setting to compare it with a standard regimen, such as the combination of 5-fluorouracil and cisplatin.

Adult↗
PubMed1996

Corticosteroids and the prevention of adverse reactions to myelography.

Side-effects of iohexol myelography were compared after pre-treatment with oral dexamethasone (n = 42) or placebo (n = 44) in a prospective, randomized, double blind, controlled clinical trial. Although myelogram side-effects were commoner in the placebo group, the differences generally did not reach statistical significance. The routine prophylactic use of oral corticosteroids for myelogram side-effects therefore cannot be recommended and our results may cast doubt on their use in the treatment of these symptoms once they have developed. These findings also suggest that inflammatory processes (allergic or chemical irritant), relating to the contrast agent itself, against which corticosteroids might have been expected to act, have at most a minor pathogenetic role in postmyelogram symptomatology.

Administration, Oral↗
PubMed1996

Topographical analyses of attention disorders of childhood.

Cognitive ERPs and EEG spectral differences were compared in three groups of children: nonreferred controls, those with a dominant hyperactivity/impulsivity factor (ADHD-Im), and those with a dominant inattentive factor (ADHD-Ia). The results from the ERP analyses indicated that the P250, P350, and P500 components differed between the groups. The most marked differences were seen with respect to the amplitude of the P500 components. In addition, the topographic foci of the P500 components for the CON and ADHD-Im groups were symmetrical, but the ADHD-Ia group featured P250 and P350 components that were biased away from the right hemisphere. Nevertheless, the P500 was found to be an effective discriminator between the groups. The combined spectral and ERP results suggest that the attention disordered children have difficulty adjusting their level of physiological arousal, and are defective with respect to controlled (or effortful) processing.

Attention Deficit Disorder with Hyperactivity↗
PubMed1995

Disease and anatomic specificity of ethanolamine plasmalogen deficiency in Alzheimer's disease brain.

A significant and selective deficiency of ethanolamine plasmalogen (PPE) relative to phosphatidylethanolamine was identified in post mortem brain samples from patients with Alzheimer's disease (AD). This lipid defect showed anatomic specificity, being more marked at a site of neurodegeneration in AD brain than in a region relatively spared by the disease (mid-temporal cortex vs. cerebellum) and disease specificity for AD: it was not observed at the primary site of neurodegeneration in Huntington's disease (caudate nucleus) nor Parkinson's disease (substantia nigra). PPE deficiency parallels an inherent tendency towards membrane bilayer instability previously detected in AD brain which is necessarily due to a change in membrane lipid composition, and which may contribute to AD pathogenesis.

Aged↗
PubMed1995

Chronic inflammatory demyelinating polyneuropathy mimicking a lumbar spinal stenosis syndrome.

A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) established by biopsy developed cauda equina symptoms due to swelling of the nerve roots in the lumbar spinal canal. Magnetic resonance imaging of the lumbar spine showed profoundly thickened nerve roots from the level of the conus medullaris, filling the caudal thecal sac. Immunosuppressant treatment produced partial clinical and radiological resolution. This case shows that spinal compressive syndromes may occur in acquired hypertrophic neuropathies as well as in hereditary motor and sensory neuropathy and expands the range of the clinical presentation of CIDP.

Adult↗
PubMed1994

Analysis of HLA DQ alpha allele and genotype frequencies in populations from Florida.

HLA DQ alpha allele and genotype frequencies for Caucasian, African American, Haitian, and Hispanic populations in Florida have been estimated. The Florida laboratories involved in these studies collected donor samples from a variety of sites including clinical laboratories, victim and suspect standards, blood banks, county jail detainees, and laboratory personnel. We have determined that the Caucasian and African American DQ alpha genotype frequencies do not deviate significantly from Hardy-Weinberg expectations and as a result of this heterogeneity analyses, data from the four Florida Caucasian populations may be combined and data from the four Florida African American populations may be combined to form two large HLA DQ alpha genotype frequency databanks. Further, data from the Florida Haitian population may be combined with the Florida African American population. Comparison of the combined Florida Caucasian populations, combined Florida African American populations, the Palm Beach Sheriff's Office (PBSO) Hispanic, and PBSO Haitian population with other databases does not support combination because allele frequency distributions are heterogeneous.

Alleles↗
PubMed1994

Acute encephalopathy: diagnosis and outcome in patients at a regional neurological unit.

Sixty-five patients with a diagnosis of acute encephalitis or encephalopathy were discharged from a regional neurological unit over a 17-year period. Investigation during the acute illness, or subsequent clinical and laboratory observations, yielded a definite or probable diagnosis in 34 of these patients, including herpes simplex encephalitis (8 cases), encephalitis due to other identified viruses (7 cases), vascular disease (7 cases) and multiple sclerosis (4 cases). In these 34 patients, mortality relating to the presenting illness was 50% and a further 29% had significant long-term neurological morbidity. In the other 31 patients, no cause for the encephalopathy was identified, despite extensive investigation. These patients had an alteration in conscious state, often with recurrent seizures (45%), focal neurological signs (52%), pyrexia (65%), abnormal electroencephalogram (85%) and cerebrospinal fluid lymphocytosis (80%). During follow-up (6 months to 15 years) none had recurrent encephalopathy, and 65% eventually made a complete recovery, although delayed by seizures in 6% and psychiatric illness in 13%. The mortality in this group relating to the acute illness was 6%. Overall, nearly half the patients with a discharge diagnosis of acute encephalitis or encephalopathy had a good prognosis for recovery, following a monophasic illness of undetermined cause.

Acute Disease↗
PubMed1993

Regional specificity of membrane instability in Alzheimer's disease brain.

We report an inherent tendency towards the destabilisation of cellular membranes in Alzheimer's disease (AD) brain. This tendency is a natural consequence of abnormal membrane lipid composition, which has previously been documented in AD. Membrane destabilisation may contribute to AD pathogenesis in its own right and may also facilitate amyloid beta-protein deposition, which is potentially neurotoxic. The instability was found to co-localise selectively with areas of neurodegeneration in AD brain, thereby possibly accounting for the focal pathology observed in this disorder.

Aged↗
PubMed1991

Membrane bilayer instability and the pathogenesis of disorders of myelin.

We have previously shown that total lipid extracts from normal nervous tissues spontaneously form a structure in vitro resembling the cell membrane bilayer, but only at a critical temperature, T*, equal to the 'physiological' temperature of the original tissues. In the present study, we found T* for normal human myelin lipids was 37 degrees C, in agreement with the concept that lipid metabolic pools maintain a critical composition in vivo which permits spontaneous formation of the (myelin) membrane bilayer at normal body temperature. But T* for myelin lipids from a patient with metachromatic leukodystrophy was less than 30 degrees C. Thus, myelin lipid composition was inappropriate for normal bilayer stability at this patient's core temperature, suggesting a mechanism whereby defective lipid metabolism in this disease could produce pathological myelin. The shift in T* in this patient was unlikely to be simply secondary to myelin destruction, as myelin lipids from a patient with advanced multiple sclerosis yielded a normal value for T* of 37 degrees C, even when extracted from areas of extensive demyelination.

Adult↗
PubMed1991

Membrane bilayer assembly in neural tissue of rat and squid as a critical phenomenon: influence of temperature and membrane proteins.

Cell membrane bilayers have been reconstructed in vitro utilizing total lipid extracts from rat neural tissue (forebrain, cerebellum, brainstem and spinal cord) and from the optic lobe and fin nerve of the squid Loligo pealei. In agreement with the critical state theory of bilayer assembly (Gershfeld, N.L. 1986. Biophys. J. 50:457-461; Gershfeld, NL.L. 1989. J. Phys. Chem. 93:5256-5261), these lipid extracts spontaneously formed purely unilamellar structures in aqueous dispersion, but only at a critical temperature, T*, which was species dependent. For all the rat tissues T* = 37 +/- 1 degrees C; for squid neural extracts T* = 15.5 +/- 1.4 degrees C. These values correspond to 'physiological' temperatures for both organisms, implying that their lipid metabolism is geared to permit spontaneous assembly of unilamellar membranes at the ambient temperature in the tissues. Membrane protein composition had little or no effect on critical bilayer formation.

Animals↗
PubMed1989

Reversal of an abnormal free fatty acid response to emotional stress in a diabetic adolescent using a portable insulin-infusion pump.

Repeated episodes of ketoacidosis in some juvenile diabetics has been associated with an abnormal rise in circulating free fatty acids (FFA) following emotional stress. Reversal of this phenomenon occurred after treatment with a portable insulin infusion pump (PIIP). Following initiation of PIIP treatment, this abnormal rise in FFA secretion was eliminated, as were the patient's episodes of ketoacidosis. We believe that PIIP treatment should be considered in the poorly controlled compliant diabetic patient.

Adolescent↗