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J F Llena

Publications and source records attributed to J F Llena.

At least 37 records · Page 2Linked to original sources
PubMed1992

Increased iron content in the putamen of patients with striatonigral degeneration.

We measured the total content of iron, copper, zinc and manganese in the putamen of four patients with striatonigral degeneration (SND) and age- and gender-matched normal controls. The iron content in the SND patients was five times greater than in the controls. Electron microscopic histochemistry revealed the iron reaction products in the pigments showing a triphasic pattern of coarse, electron-dense globules, fine granular and fibrillary materials, and lamellated structures. These findings suggest that increased iron deposition may be related to pigment formation in the putamen of SND.

Aged↗
PubMed1992

Ultrastructural identification of neurofibrillary tangles in the spinal cords in Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam.

The immunohistochemical and ultrastructural characteristics of spinal cord neurofibrillary tangles (NFTs) were examined in Guamanian amyotrophic lateral sclerosis and in parkinisonism-dementia complex on Guam. The spinal cord NFTs reacted with antibodies to tau protein (tau-2), ubiquitin and paired helical filaments (PHFs). Ultrastructurally, the components of the NFTs were seen as randomly arranged fibrils which were often associated with osmiophilic granules; small bundle-like arrangements were also occasionally observed. Individual NFT fibrils appeared as straight fibrils with a diameter of approximately 15 nm and constricted fibrils with a periodicity of approximately 80 nm. Ultrastructural microscopic examination of specimens stained by the modified Bielschowsky method and with the antibodies revealed silver particles and the products of the tau, ubiquitin and PHF immunoreactions on the NFT fibrils. This is the first demonstration of the fine structure of the spinal cord NFTs.

Adult↗
PubMed1992

Immunohistochemical, ultrastructural and immunoelectron microscopic studies of spinal cord neurofibrillary tangles in progressive supranuclear palsy.

Immunohistochemical, ultrastructural and immunoelectron microscopic studies of spinal cord neurofibrillary tangles (NFTs) in progressive supranuclear palsy (PSP) were performed. The spinal cord NFTs reacted with antibodies to tau protein (tau-2), ubiquitin and Alzheimer neurofibrillary tangles (ANTs, Ab 39). Ultrastructurally, the NFTs consisted of bundles of straight fibrils. In longitudinal sections, the individual NFT fibrils appeared as straight fibrils with a diameter of approximately 15 nm. In cross sections, circular structures approximately 15 nm in diameter were seen, and some had a central density. Electron microscopic examination of specimens stained with the antibodies and by the modified Bielschowsky method revealed the products of the tau, ubiquitin and ANTs immunoreactions and silver deposits on the NFT fibrils. This is the first demonstration of the ultrastructure of spinal cord NFTs in PSP.

Adult↗
PubMed1991

Primary central nervous system lymphoma in acquired immune deficiency syndrome. A clinical and pathologic study with results of treatment with radiation.

Primary central nervous system (CNS) lymphoma occurs frequently in patients with the acquired immune deficiency syndrome (AIDS). Seventeen patients with AIDS and biopsy-proven CNS lymphoma were treated with whole-brain radiation. At presentation, most patients were severely debilitated from previous AIDS-related illnesses. Patients generally had focal neurologic symptoms such as seizures and paralysis. Headaches and mental status changes, often noticed after hospital admission, seldom brought our patients to seek medical attention. Computed tomography (CT) scan showed low-density, contrast-enhancing, mass lesions with variable amounts of peritumor edema. Size, location, and pattern of contrast enhancement of the lesions varied. No specific pattern was seen that could be used to distinguish between CNS lymphoma, toxoplasmosis, or other CNS diseases that occur in patients with AIDS. Biopsy results showed angiocentric, high-grade, large cell tumors with frequent necrosis. Immunohistochemical analysis showed B-cell phenotype with small amounts of T-cells, presumably reactive. All patients received irradiation to the whole brain with parallel opposed fields. A variety of doses and treatment regimens were used. Mean survival was only 72 days. Survival was longer in patients with higher pretreatment Karnofsky scores. The correlation between dose and survival was not significant. At completion of therapy, most patients showed improvement in Karnofsky score and had partial improvement in neurologic symptoms. CNS lymphomas in patients with AIDS are responsive to radiation. Posttreatment CT scans showed regression of tumors. Autopsy examinations showed regression of tumors, but also showed concurrent CNS infections, AIDS encephalopathy, and radiation-induced changes within the normal CNS tissue. Opportunistic infections rather than cerebral herniation or uncontrolled lymphoma was the most common cause of death.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Argyrophilic ubiquitinated cytoplasmic inclusions of Leu-7-positive glial cells in olivopontocerebellar atrophy (multiple system atrophy).

We described cytoplasmic inclusions in glial cells in 18 patients with olivopontocerebellar atrophy (OPCA) (multiple system atrophy, MSA). These glial inclusions showed intense argyrophilia with modified Bielschowsky's and Bodian's silver impregnation techniques, and were observed in the pons, cerebellar white matter, midbrain, medulla oblongata and basal ganglia, as well as cerebral white matter and spinal cord. None of the 54 control cases had glial argyrophilic inclusions. Immunohistochemically, these inclusions were intensely labeled by anti-ubiquitin antibody. Some of them reacted with an antibody to Rosenthal fiber (RF) protein. The cytoplasm of ubiquitinated inclusion-bearing glial cells was immunostained by anti-Leu-7 antibody, but not by anti-GFAP antibody. Ultrastructurally, the glial inclusions were composed primarily of approximately 24- to 40-nm fibrils, which were coated with osmiophilic granular material along their length in longitudinal section. These fibrils appeared as annuli in cross section. Often, a central granule approximately 5 nm in diameter was seen in the lucent lumen of a cross-sectioned fibril. The granule-coated fibrils were not seen in the glial filament-containing astrocytes. Electron microscopic examination of silver-impregnated specimens revealed that the granule-coated fibrils had strong affinity for silver. Immunoelectron microscopy using the indirect immunoperoxidase techniques with antibodies to ubiquitin and RF protein revealed that the electron-dense reaction products respective to both were located on constituents of glial inclusions. Our observation that Leu-7-positive glial cells, mainly oligodendroglial cells, had argyrophilic ubiquitinated inclusions may be of significance for the evaluation of the pathology of OPCA(MSA).

Adult↗
PubMed1991

Immunohistochemical study of granular cell tumors of the neurohypophysis.

Six granular cell tumors (GCT) of the neurohypophysis were studied by immunohistochemical techniques. They were all labeled by peanut lectin (Arachis hypogaea) and three showed reactivity for S-100 protein. Unlike extracranial GCT, neuron specific enolase (NSE), myelin basic protein (MBP) and vimentin were not detected in the tumor cells. Glial fibrillary acidic protein (GFAP), keratin and desmin were also not observed. On the other hand, some showed reactivity for alpha-1-antitrypsin (AAT), alpha-1-antichymotrypsin (AAC) and cathepsin B. These results suggest that neurohypophysial GCT have some features different from extracranial GCT and that they may not be derived from Schwann cells.

Adult↗
PubMed1991

Human immunodeficiency virus-1 infection of the nervous system: an autopsy study of 268 adult, pediatric, and fetal brains.

The central nervous system (CNS) of 221 adults and 31 infants or children with the acquired immunodeficiency syndrome (AIDS) was examined with immunocytochemistry for infectious agents and for human immunodeficiency virus-1 (HIV-1) antigen (gp41). Since the major risk factor in this population was intravenous drug abuse, there were more female and pediatric patients than in other neuropathology autopsy series. Although children had a different spectrum of pathologic changes, including less frequent opportunistic infections, women did not differ from men in terms of types or incidence of opportunistic infections, vascular disease, neoplasia, and subacute AIDS encephalitis (SAE). Subacute AIDS encephalitis was detected in 26% of adult and 48% of pediatric brains. Immunocytochemical analysis of 100 adult and 20 pediatric brains revealed gp41 immunoreactivity in 78% and 40%, respectively. Virtually all adult brains with SAE had gp41 immunoreactivity in macrophages and microglia. Even brains with no significant pathology had frequent gp41 immunoreactivity, especially in the basal ganglia. In pediatric brains, including cases with SAE, gp41 immunoreactivity was less abundant, suggesting the possibility of latent infection or viral clearance. Spinal cords with vacuolar myelopathy or corticospinal tract degeneration had only rare gp41-positive cells. Brains from 16 aborted fetuses from HIV-1-seropositive women were all negative for gp41 immunoreactivity, but 12 brains were positive for HIV-1 by the polymerase chain reaction. These results may indicate that HIV-1 infection in fetal brains is below the limits of detection of immunocytochemistry. The differences noted between adults and children suggest that adults more often have productive CNS HIV-1 infection.

AIDS Dementia Complex↗
PubMed1990

Ubiquitin immunoreactivity in kuru plaques in Creutzfeldt-Jakob disease.

Cerebellar kuru plaques in 2 cases of Creutzfeldt-Jakob disease were studied immunohistochemically. Similar to cerebellar senile plaques in Alzheimer's disease, many kuru plaques contained ubiquitin-positive, tau-negative small granular elements, presumably representing dystrophic neurites. Our results suggest that similar mechanisms are involved in neuritic changes in cerebellar plaques in Creutzfeldt-Jakob and Alzheimer's diseases despite differences of amyloid proteins in the plaques.

Aged↗
PubMed1990

Stroke in pediatric acquired immunodeficiency syndrome.

In a 4 1/2-year period, 4 of 68 children in a longitudinal study of neurological complications of human immunodeficiency virus (HIV) infection had clinical and/or neuroradiological evidence of stroke, yielding a clinical incidence of stroke in this population of 1.3% per year. During this period, 32 subjects died, and permission for autopsy was granted in 18 of the patients, including 3 of 4 who had clinical evidence of stroke. The prevalence of cerebrovascular pathological features in our consecutive autopsy series was higher than the clinical incidence. At autopsy cerebrovascular disease was documented in 6 (24%) of 25 children with HIV infection, including all 3 children who had clinical evidence of stroke. Four patients had intracerebral hemorrhages, 6 patients had nonhemorrhagic infarcts, and 3 had both. Hemorrhage was catastrophic in 1 child and clinically silent in 3 children, all of whom had immune thrombocytopenia. One child had an arteriopathy that affected meningocerebral arteries. In another child, the arteries of the circle of Willis were aneurysmally dilated. Two children had coexisting cardiomyopathy and subacute necrotizing encephalomyelopathy with vascular proliferation. These results suggest that stroke should be considered when children with HIV infection develop focal neurological signs.

Acquired Immunodeficiency Syndrome↗
PubMed1990

Modified Bielschowsky stain and immunohistochemical studies on striatal plaques in Alzheimer's disease.

The nature of senile plaques (SP) in the striatum in 14 cases of Alzheimer's disease (AD) was investigated with the modified Bielschowsky stain and immunohistochemistry using antibodies to a beta amyloid synthetic peptide, ubiquitin, tau protein, and paired helical filaments (PHF). Striatal SP, composed of beta amyloid deposits with or without neuritic elements, were demonstrated in all AD cases examined. Compact and perivascular amyloid deposits were concentrated in the ventral striatum, including the nucleus accumbens. Many diffuse amyloid deposits in the ventral striatum contained ubiquitin-positive granular elements, presumably representing dystrophic neurites, whereas most of those in the dorsal striatum did not have such elements. On the other hand, most compact amyloid deposits in both ventral and dorsal striatum had ubiquitin immunoreactivity. Dystrophic neurites with tau or PHF immunoreactivity were detected particularly around compact amyloid deposits. Our results indicate that the ventral striatum, which is closely affiliated with the limbic system, is frequently affected by amyloid deposits with dystrophic neurites, and suggest that the ventral striatum is particularly vulnerable to AD. Furthermore, our results suggest that amyloid deposits, especially compact deposits, may induce dystrophic neurites.

Aged↗
PubMed1990

Epithelial cysts in the central nervous system, characteristic expression of cytokeratins in an immunohistochemical study.

Nineteen epithelial cysts in the central nervous system including six colloid cysts of the third ventricle, seven Rathke's cleft cysts in the sella, two enterogenous cysts in the posterior fossa, two epithelial cysts in the spinal canal and two neuroectodermal cysts in the cerebrum were examined immunohistochemically for expression of intermediate filament proteins-simple type, stratified type and skin type cytokeratins and GFAP. Colloid cysts of the third ventricle, Rathke's cleft cysts in the sella and epithelial cysts in the spinal canal expressed complex type cytokeratins while enterogenous cysts and neuro-ectodermal cysts showed only simple type cytokeratins. In addition, Rathke's cleft cysts expressed GFAP in occasional lining cells. The characteristic composition and distribution of cytokeratins in various kinds of epithelial cysts in the central nervous system are demonstrated and discussed with regard to their origins.

Adult↗
PubMed1990

Modified Bielschowsky and immunocytochemical studies on cerebellar plaques in Alzheimer's disease.

Senile plaques (SP) in the cerebellum of 23 cases of Alzheimer's disease (AD), three with widespread amyloid angiopathy, were studied with a modified Bielschowsky stain and immunocytochemical methods using antibodies to a beta-amyloid synthetic peptide (beta ASP), phosphorylated neurofilament proteins, ubiquitin, tau protein, and glial fibrillary acidic protein (GFAP). The four subtypes of SP (diffuse plaques, compact plaques, perivascular plaques, and subpial fibrillar deposits) that were observed with the modified Bielschowsky stain were also stained with antibodies to beta ASP. Many cerebellar SP contained ubiquitin-positive granular elements resembling dystrophic neurites. In contrast to neuritic elements in cerebral SP in AD, ubiquitin-positive elements in cerebellar SP were not labeled with antibodies to phosphorylated neurofilament or tau proteins. Various degrees of glial reaction were observed in all subtypes of SP except diffuse plaques. The absence of phosphorylated neurofilament and tau epitopes in neuritic elements in cerebellar SP is not surprising since paired helical filaments have not been seen in the cerebellum. Nevertheless, our results suggest that cerebellar SP are frequently associated with dystrophic neurites.

Aged↗
PubMed1990

[Epithelial cyst in the central nervous system--characteristic expression of cytokeratin].

Twenty one cases of epithelial cysts in the central nervous system including six colloid cysts of the third ventricle, eight Rathke's cleft cysts in the sella, two enterogenous cysts in the posterior fossa, two epithelial cysts in the spinal canal and three neuroectodermal cysts in the cerebrum were examined immunohistochemically for expression of intermediate filamentous proteins-simple type, stratified type and skin type cytokeratins and GFAP. Colloid cysts of the third ventricle. Rathke's cleft cysts in the sella and epithelial cysts in the spinal canal expressed complexed type cytokeratins while enterogenous cysts and neuroectodermal cysts showed only simple type cytokeratins. In addition, several Rathke's cleft cysts demonstrated skin type differentiation and expressed GFAP in occasional lining cells. The characteristic composition and distribution of cytokeratins in various kinds of epithelial cysts in the central nervous system are demonstrated and discussed with regards to their origins.

Adult↗
PubMed1989

Distribution of epithelial membrane antigen in normal and neoplastic human ependyma.

The ependyma and choroid plexus of 23 normal brains and 20 ependymal tumors were examined immunohistochemically for expression of epithelial membrane antigen (EMA) using a specific monoclonal antibody. The ependyma of normal brains showed three patterns of immunoreactivity: membrane immunoreactivity confined to the luminal surface; irregular punctate intracytoplasmic immunoreactivity in the subependymal layer; and spherical and ring-like intracytoplasmic immunoreactivity in the subependymal layer. Of 13 differentiated ependymomas 11 reflected the immunoreactive patterns of normal ependyma. The anaplastic ependymomas and ependymoblastomas had no immunoreactivity. Our results indicate that EMA has a highly selective distribution in the ependyma, and is a marker for differentiated ependymoma.

Adult↗
PubMed1989

Pathology of the dentate nucleus in progressive supranuclear palsy: a histological, immunohistochemical and ultrastructural study.

Dentate nucleus pathology was studied histologically and immunohistochemically in four cases and ultrastructurally in three cases of progressive supranuclear palsy (PSP). In addition to neurofibrillary changes, there were ill-defined clumps of eosinophilic granular structures, named grumose degeneration (GD). GD was observed in three of the four cases; it was not seen in a case exhibiting severe Purkinje cell loss. In areas with prominent GD, neuronal loss was also marked and the remaining neurons were atrophic. GD, which was once believed to represent degenerated cell bodies of dentate neurons, could be histologically distinguished from perikarya of dentate neurons. Argentophilic rings and knobs were only a part of GD and the rest of GD was only faintly or not argentophilic. Immunostain for phosphorylated neurofilament proteins positively stained round structures of various sizes in GD, but a large part of GD did not react with the antibody. As described in other disorders, electron microscopy revealed that the GD consisted of clusters of numerous axon terminals and preterminal axons. Many appeared swollen to a varying extent and contained mitochondria, synaptic vesicles, neurofilaments, lamellar bodies, multivesicular structures, vacuoles or combinations of these organelles. A few were markedly swollen with accumulation of neurofilaments and other organelles, corresponding to the round structures which stained positively for phosphorylated neurofilament proteins. A considerable number of axon terminals with no apparent abnormal accumulations of organelles were found in areas of GD.

Aged↗
PubMed1989

Interface between the meningioma and the brain.

Though meningiomas are usually well demarcated from the brain, their interfaces with the brain take various forms. Based on 27 nonoperated supratentorial meningiomas from autopsies, the interfaces were classified into four types, that is, smooth, lobular, finger-like expansion, and invasive. Relationships between the types of interfaces and the so-called capsule of the tumor and subarachnoid vessels are discussed. Additionally, three surgical cases which showed more or less the invasive form are presented with neuroradiological findings.

Adult↗
PubMed1989

[Asymptomatic and symptomatic Rathke's cleft cysts. Histological study of 45 cases].

Pituitary cysts are fairly often found incidentally in anatomical studies, but symptomatic cysts are relatively uncommon. In this study, the histological characteristics of 42 asymptomatic and three symptomatic Rathke's cleft cysts were examined and compared. The 42 cysts found incidentally at autopsy were classified as small (less than 1 mm in diameter), medium (1 to 5 mm), or large (over 5 mm). There were 23 small (55%), 13 medium (31%), and six large (14%) cysts. The mean ages of the three patient groups were 66, 60, and 53 years, respectively. Flattened epithelial cells were the most common cells in small cysts, while cuboidal to columnar epithelial cells were more prevalent in medium and large cysts. The larger cysts tended to contain cilia. The three symptomatic cysts were histologically confirmed to be Rathke's cleft cysts by their characteristic ciliated columnar epithelial lining. No essential histological differences were observed between asymptomatic and symptomatic cysts, and the mechanism of change from asymptomatic to symptomatic Rathke's cleft cyst was not clarified. Small nests of epithelium at the junction of the pars tuberalis and pituitary stalk are considered to be remnants of Rathke's pouch and are thought to give rise to craniopharyngioma. In their most typical forms, Rathke's cleft cysts and craniopharyngiomas are histologically distinguishable, craniopharyngiomas being composed of stratified squamous epithelium and simple Rathke's cleft cysts of a layer of cuboidal or columnar epithelium on a basement membrane.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗