PubMed Health⌕ Search

Biomedical subjects

C M Hulette

Publications and source records attributed to C M Hulette.

At least 37 records · Page 2Linked to original sources
PubMed1996

Microglioma, a histiocytic neoplasm of the central nervous system.

Neuropathologists have long suspected the existence of a tumor derived from the microglia, which are the resident immunocompetent cells of the central nervous system. Previously, definitive characterization of this rare putative tumor was hampered by the lack of precise immunohistochemical reagents. We herein report on a patient with microglioma, and we define the immunohistochemical characteristics of the tumor. The patient was a 50-year-old white woman who presented with a 1-year history of progressive paresthesia, visual difficulties, and cranial nerve abnormalities. The patient died in June 1972. At autopsy, the brain weighed 1540 grams and was remarkable for a diffusely infiltrating periventricular tumor, which extended from the rostral tip of the lateral ventricles through the spinal cord. Microscopically, the tumor cells had extremely long, slender, twisted nuclei, and the cells diffusely infiltrated the brain parenchyma so that the extent of the tumor was difficult to determine. Formalin-fixed, paraffin-embedded tissue blocks from the neuropathology archives were studied. The neoplastic cells stained intensely with CD68 (KP1) and Ricinus communis agglutinin-120 markers for microglia and also with HAM-56, a marker for macrophages. The tumor cells stained negative for glial fibrillary acidic protein. The recent availability of precise immunohistochemical reagents has clearly defined this rare neoplasm and has facilitated reliable distinction from lymphoma and gliomatosis cerebri.

Antigens, CD↗
PubMed1996

Linkage of frontotemporal dementia to chromosome 17: clinical and neuropathological characterization of phenotype.

Frontotemporal dementia is a behavioral disorder of insidious onset and variable progression. Clinically, its early features reflect frontal lobe dysfunction characterized by personality change, deterioration in memory and executive functions, and stereotypical and perseverative behaviors. Pathologically, there is degeneration of the neocortex and subcortical nuclei, without distinctive features such as plaques, neurofibrillary tangles, or Pick or Lewy bodies. Within-family variation in neuropathology and clinical phenotype is observed. In cases where family aggregation is observed, it is inherited as an autosomal dominant, age-dependent disorder. Family studies recently have identified two dementia loci: chromosome 17 for disinhibition-dementia-parkinsonism-amyotrophic complex and pallido-ponto-nigral degeneration and chromosome 3 for familial nonspecific dementia. We describe a family (DUK1684) with clinically and neuropathologically confirmed, autosomal dominant, non-Alzheimer disease dementia. Linkage analysis of this family showed evidence for linkage to chromosome 17q21, with a multipoint location score (log10) of 5.52. A comparison of the clinical and pathological features in DUK1684 with those of the other chromosome 17-linked families, together with the linkage data, suggests that these families are allelic. These studies emphasize that genetic linkage analysis remains a useful tool for differentiating disease loci in clinically complex traits.

Age of Onset↗
PubMed1995

Argyrophilic plaque-like deposits in children.

We recently examined the brain from an 8-month-old infant with Down's syndrome and found argyrophilic plaque-like deposits throughout the neocortex and cerebellum. To ascertain the specificity of this observation, we examined 27 additional brains from the pediatric autopsy service, including 1 from another patient with Down's syndrome. To our surprise, similar argyrophilic deposits were found in 16 of these cases. The deposits were equally well stained by three different silver stains and had the same size, shape, and distribution in gray matter as the diffuse amyloid plaques commonly seen in adults. However these structures appeared to be amyloid negative. There were no obvious differences in the primary diagnoses amongst the group of patients with argyrophilic deposits and the group without them, and the origin, permanence, and functional significance of these plaque-like deposits are still unknown. Nonetheless, their recognition is important since they may represent subtle brain injury and since similar structures in adults might easily be misinterpreted as true diffuse amyloid plaques.

Adult↗
PubMed1995

Stroke due to recurrent ipsilateral carotid artery dissection in a young adult.

BACKGROUND: Extracranial carotid artery dissection is a well-recognized cause of ischemic stroke. Recurrent carotid artery dissections are infrequent. Recurrent ipsilateral dissection has only rarely been documented and has not been pathologically verified. CASE DESCRIPTION: A 33-year-old woman presented with a left parieto-occipital ischemic stroke. Angiography demonstrated a pseudoaneurysm of the extracranial left internal carotid artery. There was no angiographic evidence of an underlying vasculopathy. The pseudoaneurysm was resected, and microscopic examination revealed features most consistent with fibromuscular dysplasia with areas of both chronic and recent dissection. CONCLUSIONS: This case suggests that the frequency of fibromuscular dysplasia as a cause for "idiopathic" spontaneous carotid artery dissection may be higher than previously recognized and that recurrent embolization may occur in the setting of chronic dissection due to redissection of the previously involved vessel.

Adult↗
PubMed1994

Creutzfeldt-Jakob disease in a pregnant woman with an implanted dura mater graft.

A 28-year-old woman with prior neurosurgery involving the placement of a cadaveric dural graft developed a rapidly progressive neurodegenerative disorder with prominent cerebellar dysfunction that was proven at autopsy to be Creutzfeldt-Jakob disease. She represents the second American to develop Creutzfeldt-Jakob disease in association with a dural graft. The unusual features of the case include the patient's initial clinical presentation with cerebellar ataxia in the absence of dementia, the widespread presence of kuru-type amyloid plaques on a histological examination of the brain, the development of clinical symptoms during pregnancy, and the subsequent delivery of a child who remains healthy at the age of 3 years.

Adult↗
PubMed1994

A comparison of commercially available probes for in situ hybridization to human papillomavirus DNA.

Several commercially prepared DNA probes to human papillomavirus are available for nonisotopic in situ hybridization. Our laboratory conducted a comparison of eight commercially available DNA probes for diagnostic purposes. Six type-specific and two wide-spectrum probes from two vendors (Enzo and Digene Diagnostics) were used on routinely processed, formalin-fixed, paraffin-embedded tissue sections. An additional wide-spectrum probe from Dako was also investigated, but testing was discontinued early because performance was unsatisfactory. An automated apparatus was used for this comparison study, and the protocols for the kits were adapted accordingly. Twenty-four cases were studied; these included four cases of normal cervical tissue, six cases of condyloma, six cases of mild (low-grade) dysplasia with condyloma, five cases of moderate to severe (high-grade) dysplasia, and three cases of carcinoma. All of the low-grade dysplasias and the condyloma hybridized with at least one wide-spectrum and one type-specific probe. By contrast, only half of the high-grade dysplasias and carcinomas hybridized to any probe. The results of hybridizing with the type-specific probes were equivocal. Four cases reacted with two different type-specific probes. We conclude that nonisotopic in situ hybridization is a reproducible technique for detection of human papillomavirus, and recommend this method as an adjunct for routine histology in equivocal cases of condyloma. The technique adapts to a mechanized system, increasing its utility in the clinical setting. In our laboratory, the Enzo wide-spectrum probe was easier to interpret, generating a stronger signal with less nonspecific binding of chromogen. The sensitivities of the probes tested were not significantly different.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Utility of postmortem magnetic resonance imaging in clinical neuropathology.

Over 200 brains were examined by postmortem magnetic resonance imaging to determine the utility of this imaging procedure as an adjunct to the standard postmortem examination of the brain and spinal cord. One unembalmed cadaver was also studied using a conventional 1.5-tesla (T) field-strength unit, and three formalin-fixed sections of the hippocampus were imaged using a high field-strength (7.0-T) prototype imaging system. The postmortem magnetic resonance images proved to be an invaluable aid that complemented the standard pathologic examination of the brain and spinal cord. The compelling advantages of this postmortem radiographic procedure included the three-dimensional aspects of the images; the ability to detect mineral (ie, iron) deposits; small focal lesions such as hemorrhages or infarcts; and the ability to evaluate the extent of cerebral edema. For the same reasons, as well as its archival potential for documenting the topographic distribution of pathologic processes, this technique has great promise for forensic cases. High field-strength (7.0-T) imaging brought the resolution of magnetic resonance to the microscopic level and reaffirmed the potential value of magnetic resonance imaging for diagnostic and investigative studies in which both the histologic and fine radiologic features of lesions are of interest.

Autopsy↗
PubMed1993

Increased amyloid beta-peptide deposition in cerebral cortex as a consequence of apolipoprotein E genotype in late-onset Alzheimer disease.

Amyloid beta-peptide (A beta) deposition in senile plaques and cerebral vessels is a neuropathological feature of Alzheimer disease (AD). We examined the possibility that commonly observed variability in A beta deposition in late-onset AD might be related to apolipoprotein E genotype (APOE gene; the two most common alleles are 3 and 4), since APOE4 is a susceptibility gene for late-onset AD and apolipoprotein E interacts strongly with A beta in vitro. In an autopsy series of brains of late-onset AD patients, we found a strong association of APOE4 allele with increased vascular and plaque A beta deposits. Late-onset AD patients with one or two APOE4 alleles have a distinct neuropathological phenotype compared with patients homozygous for APOE3.

Aged↗
PubMed1992

Evaluation of cerebral biopsies for the diagnosis of dementia.

To identify those patients most likely to benefit from a cerebral biopsy to diagnose dementia, we reviewed a series of 14 unselected biopsies performed during a 9-year period (1980 through 1989) at Duke University Medical Center, Durham, NC. Pathognomonic features allowed a definitive diagnosis in seven specimens. Nondiagnostic abnormalities but not diagnostic neuropathologic changes were seen in five additional specimens, and two specimens were normal. Creutzfeldt-Jakob disease was the most frequent diagnosis. One patient each was diagnosed as having Alzheimer's disease, diffuse Lewy body disease, adult-onset Niemann-Pick disease, and anaplastic astrocytoma. We conclude that a substantial proportion of patients presenting clinically with atypical dementia are likely to receive a definitive diagnosis from a cerebral biopsy. However, in those with coexisting hemiparesis, chorea, athetosis, or lower motor neuron signs, cerebral biopsies are less likely to be diagnostic.

Adult↗
PubMed1992

Macrophage markers in diagnostic neuropathology.

It can be difficult to differentiate the cells of infiltrating gliomas from macrophages associated with demyelinating lesions or cerebral infarcts, especially in tissue with freezing artifact or tissue that is technically suboptimal. We therefore sought to identify a reliable immunohistochemical marker for central nervous system macrophages that could be used on routinely processed tissue. The reactions of commercially available markers [Ham-56, Mac 387, CD68, CR3/43, DR alpha, lysozyme, CD16, and Ricinus communis agglutinin-1 (RCA-1)] were studied in normal cerebellum, subacute infarcts, viral encephalitis with microglial nodules, radiation necrosis, Alzheimer's disease, medulloblastoma, T-cell lymphoma, oligodendroglioma, gliomatosis cerebri, and microgliomatosis cerebri. We also performed a more extensive investigation of Ham-56 with largely surgical material. We concluded that (a) RCA-1 is a sensitive marker for microglia, either resting or responding to injury, but staining must be interpreted carefully because endothelial cells and reactive astrocytes frequently stain as well. This marker was useful in highlighting focal inflammation in HIV encephalitis. (b) CD68 is a specific marker for resting microglia. A case of microgliomatosis cerebri was intensely stained. (c) Ham-56 is a sensitive, specific (with the exception of endothelial cells), and reliable marker of central nervous system macrophages in routinely processed autopsy and surgical material. It is especially useful in distinguishing the macrophages in demyelinating disease and cerebral infarcts from neoplastic glial cells.

Adolescent↗
PubMed1991

Progressive multifocal leukoencephalopathy. Diagnosis by in situ hybridization with a biotinylated JC virus DNA probe using an automated Histomatic Code-On slide stainer.

The accurate surgical pathological diagnosis of progressive multifocal leukoencephalopathy (PML) depends on the demonstration of pathognomonic histological features in cerebral biopsy tissue. The diagnosis may be difficult, however, if only small tissue fragments are submitted from the center of a demyelinating lesion. Previous studies by other authors have established that in situ hybridization with a biotinylated JC virus DNA probe can be a valuable diagnostic adjunct because it identifies the virally infected cells with great specificity and does not depend on the larger specimen, which may be necessary for a firm histological diagnosis. To confirm and extend these findings, we have used a commercially available biotinylated JC virus DNA probe to demonstrate the presence of viral DNA in formalin-fixed, paraffin-embedded tissues from four open biopsies, four needle biopsies, and two autopsies of patients with PML. With the goal of making this procedure applicable to the general surgical pathology laboratory, this method was adapted to the Histomatic Code-On slide stainer. The Histomatic is a programmable, robotic instrument with walk-away capability for hybridization histochemistry. Operation of this instrument requires the same expertise as execution of immunocytochemistry. With the advent of commercially available JC virus DNA probes and an automated system for hybridization histochemistry, this technology for diagnosis of PML may enter the routine diagnostic surgical pathology laboratory.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Immunological aspects of Alzheimer disease: a review.

Clinical and neuropathological similarities between Alzheimer disease and the transmissible spongiform encephalopathies have sparked intensive investigation of the role of nearly every arm of the immune system in the etiology and pathogenesis of Alzheimer disease. These endeavors have yielded intriguing results but no clear-cut answers. Studies of humoral and cellular immune function in patients with dementia of the Alzheimer type (DAT), immunohistochemical examinations of Alzheimer brain tissue and HLA typing of both sporadic and familial DAT are reviewed. Sporadic DAT appears to be associated with the complement allotype C4B2 in the major histocompatibility complex, which is linked very closely to HLA. Mechanisms for the role of complement in the pathogenesis of DAT are postulated.

Aging↗
PubMed1987

Immunization of normal human splenocytes in vitro to produce human monoclonal antibodies to cellular antigens.

We have obtained human monoclonal antibodies to polymorphic cell surface determinants by immunizing normal human splenecytomy in vitro to allogeneic cells. Splenocytes from young patients undergoing splenectomy secondary to traumatic injury are separated into T and B lymphocyte populations. The T lymphocytes are irradiated with 1500 rad to selectively inactivate T suppressors. Responder T and B cells are then recombined at a 1:1 ratio. Maximal IgM and IgG production is obtained when pokeweed mitogen and irradiated stimulator cells are added to the cultures. Stimulator cell specific antibody levels peak at day 7 of in vitro immunization, and fall thereafter. Fusion of the immunized splenocytes to a human fusion partner as early as day 4 results in hybridomas secreting antibodies to cellular antigens. Transformation by EBV, expansion and fusion of the immunized cell line also yield hybridomas secreting stimulator cell specific antibody.

Antibodies, Monoclonal↗
PubMed1986

A human-human hybridoma secreting anti-HLA class II antibody.

In this report, we describe the production and characterization of the first human-human hybridoma secreting antibody to HLA Class II determinants. The hybridoma (GMEC101), which has been stable in tissue culture for greater than 20 mo, secretes 10 to 50 micrograms/ml of IgM-kappa antibody. This antibody binds to a wide range of human cell lines, but not to the HLA-A,B,C, and DR-negative K562 cell line. Functionally, GMEC101 strongly inhibits a unidirectional mixed lymphocyte reaction (MLR) at the level of the stimulator cell. Neither the cellular ELISA binding nor the MLR inhibition is lost after a triple platelet absorption (which removes Class I but not Class II activity). Because the binding and MLR blocking show no correlation with the known DR or DQ specificities, we suggest that GMEC101 may be detecting a novel HLA Class II determinant.

Antibody Specificity↗
PubMed1986

Cytomegaloviral epididymitis in a patient with the acquired immune deficiency syndrome.

We report on a patient with cytomegaloviral epididymitis and the acquired immune deficiency syndrome. The diagnosis was suggested by epididymitis that was refractory to antibiotics, and by isolation of cytomegalovirus from the urine and semen. The definitive diagnosis was made with immunohistochemical staining for cytomegaloviral antigens in the epididymal ductal cells. Because of the ineffectiveness of antimicrobial agents in this disorder, epididymectomy is the current treatment of choice.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Production of a human monoclonal antibody to HLA by human-human hybridoma technology. A preliminary report.

An Epstein-Barr-virus-transformed lymphoblastoid cell line (ECEBV) was derived from a multiply transfused renal dialysis patient. ECEBV was shown to secrete specific antibody in a cellular enzyme-linked immunosorbent assay (CELISA) and was hybridized with the mutagenized human fusion partner G M1500 resistant to 6-thioguanine and ouabain. Hybridomas surviving hypoxanthine-aminopterin-thymidine (HAT) and ouabain selection were cloned by limiting dilution. The hybridomas continue to secrete antibody which reacts with some human cells but not with others after 14 months in culture. None reacts with K562 (no HLA-A, -B, -C or -DR) or with Daudi (no HLA-A, -B, or -C). This is a preliminary report of the production of a human monoclonal antibody to HLA. Application of this technique could result in the large-scale production of human monoclonal antibodies for HLA typing, the production of anti-idiotype antibodies for use in transplant patients to prevent acute rejection, and for the study of the structure and function of HLA in man.

Antibodies, Monoclonal↗