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Biomedical subjects

A P Anzil

Publications and source records attributed to A P Anzil.

At least 19 recordsLinked to original sources

Microencephaly in children congenitally infected with human immunodeficiency virus--a gross-anatomical morphometric study.

A quantitative technique involving serial sectioning and semiautomatic morphometric analysis was used to assess the severity of the reduction in size of the major brain structures in cerebral hemispheres of children congenitally infected with HIV-1. Cerebral hemispheres from 12 children (18-48 months of age) who died of AIDS were sectioned into 5-mm-thick serial slabs and photographed. The cross-sectional areas of grossly recognizable brain structures were digitized, and the volumes were calculated according to Cavalieri's principle. The results were compared with those of an identically processed group of control brains from non-AIDS children. Analysis of the brain weight showed that there was a significant reduction in supratentorial and infratentorial weight in the AIDS group. The results of the morphometric study revealed that the loss in brain mass was associated with a statistically significant reduction in the total volume of both hemispheres, the entire cortex, white matter, and basal ganglia. Detailed analysis of individual brain structures also showed a significant reduction in volume of all cortical regions and most of the subcortical gray matter (e.g., caudate nucleus, putamen, globus pallidus, claustrum, and thalamus). It appears that in the microencephaly observed as a frequent sequel in pediatric AIDS, the loss of brain tissue is global and includes an almost proportional loss of cortex, subcortical gray matter and white matter.

Acquired Immunodeficiency Syndrome

Eccrine syringofibroadenoma surrounding a squamous cell carcinoma: a case report.

A 91-year-old man presented with a 9.0 x 7.0 cm exophytic mass on the dorsum of the right foot, surrounded by a scaling hyperkeratotic plaque-like lesion that had been present for many years. He had similar long-standing hyperkeratotic plaque-like lesions on both legs. Histopathologic examination of the exophytic mass revealed a well-differentiated squamous cell carcinoma surrounded by an eccrine syringofibroadenoma (ESFA). Histochemistry, immunohistochemistry and electron microscopy support this diagnosis. To our knowledge, this is the only reported case of ESFA being intimately associated with a malignant neoplasm.

Adenoma, Sweat Gland

Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases.

This report summarizes neuropathological, clinical, and general autopsy findings in 105 individuals with nonneoplastic syringomyelia. On the basis of detailed histological findings, three types of cavities were distinguished: 1) dilations of the central canal that communicated directly with the fourth ventricle (47 cases); 2) noncommunicating (isolated) dilations of the central canal that arose below a syrinx-free segment of spinal cord (23 cases); and 3) extracanalicular syrinxes that originated in the spinal cord parenchyma and did not communicate with the central canal (35 cases). The incidence of communicating syrinxes in this study reflects an autopsy bias of morbid conditions such as severe birth defects. Communicating central canal syrinxes were found in association with hydrocephalus. The cavities were lined wholly or partially by ependyma and their overall length was influenced by age-related stenosis of the central canal. Non-communicating central canal syrinxes arose at a variable distance below the fourth ventricle and were associated with disorders that presumably affect cerebrospinal fluid dynamics in the spinal subarachnoid space, such as the Chiari I malformation, basilar impression, and arachnoiditis. These cavities were usually defined rostrally and caudally by stenosis of the central canal and were much more likely than communicating syrinxes to dissect paracentrally into the parenchymal tissues. The paracentral dissections of the central canal syrinxes occurred preferentially into the posterolateral quadrant of the spinal cord. Extracanalicular (parenchymal) syrinxes were found typically in the watershed area of the spinal cord and were associated with conditions that injure spinal cord tissue (for example, trauma, infarction, and hemorrhage). A distinguishing feature of this type of cavitation was its frequent association with myelomalacia. Extracanalicular syrinxes and the paracentral dissections of central canal syrinxes were lined by glial or fibroglial tissue, ruptured frequently into the spinal subarachnoid space, and were characterized by the presence of central chromatolysis, neuronophagia, and Wallerian degeneration. Some lesions extended rostrally into the medulla or pons (syringobulbia). Although clinical information was incomplete, simple dilations of the central canal tended to produce nonspecific neurological findings such as spastic paraparesis, whereas deficits associated with extracanalicular syrinxes and the paracentral dissections of central canal syrinxes included segmental signs that were referable to affected nuclei and tracts. It is concluded that syringomyelia has several distinct cavitary patterns with different mechanisms of pathogenesis that probably determine the clinical features of the condition.

Adolescent

Stenosis of central canal of spinal cord in man: incidence and pathological findings in 232 autopsy cases.

The central canal of the spinal cord is generally regarded as a vestigial structure that is obliterated after birth in 70% to 80% of the general population. This report describes the first detailed histological study of the human central canal in 232 subjects ranging in age from 6 weeks' gestation to 92 years. Whole spinal cords were harvested at autopsy and sectioned serially from the conus medullaris to the upper medulla. Histological findings and morphometric analysis of the cross-sectional luminal area were used to grade stenosis at seven levels of the canal. Varying grades of stenosis were present at one or more levels in none (0%) of 60 fetuses, one (3%) of 34 infants, three (18%) of 17 children, 21 (88%) of 24 adolescents and young adults, 67 (96%) of 70 middle-aged adults, and all 27 adults aged 65 years or older (100%). The stenotic process was most pronounced in the thoracic segments of the canal and involved more levels with higher grades of stenosis in older individuals. Histological findings consisted of disorganization of the ependymal epithelium, formation of ependymal rosettes or microcanals, proliferation of subependymal gliovascular buds, and intracanalicular gliosis. These features are consistent with a pathological lesion involving ependymal injury and scarring and are less compatible with an involutional or degenerative process. Stenosis of the central canal probably influences the anatomical features of syringomyelia and may account for variations in cavity formation such as the prevalence of holocord syrinxes in children, the formation of focal and paracentral syrinxes in adults, and the rare incidence of syrinx formation in many older individuals with acquired lesions known to produce syringomyelia.

Adolescent

Spinal cord pathology.

The subject of spinal cord pathology can be addressed in several ways. This article tackles spinal cord pathology by examining the topic according to purely nosologic criteria. Topics discussed include malformations, traumatic injuries, vascular and circulatory diseases, tumors, infections and inflammatory diseases, demyelinating diseases, toxic-metabolic and nutritional diseases, degenerative diseases, and miscellaneous other disorders.

Diagnosis, Differential

Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma.

BACKGROUND: Patients who survive retinoblastoma (RB) are at risk for having second nonocular tumors, usually osteosarcomas, which often are fatal. Such patients almost always have bilateral RB. METHODS: This article reports a woman who, at the age of 1 year had been cured of a unilateral RB by radiation therapy and enucleation. Eighteen years later, she had a sinonasal small cell tumor that rapidly recurred and proved fatal 2 months after surgical debulking. The tumor was studied by immunohistochemistry and electron microscopic (EM) examination. RESULTS: It showed diffuse neuron-specific enolase staining, focal weak staining for chromogranin, synaptophysin, and Leu-7 monoclonal antibodies in paraffin-embedded, B5-fixed tissue (Great Lakes Diagnostics, Troy, MI). EM study showed an undifferentiated primitive neuroectodermal tumor with many polyribosomes, simple cell junctions, few microtubules, and rare dense core granules. CONCLUSIONS: The combined immunohistochemical, ultrastructural, and clinical features of the tumor were interpreted as a sinonasal primitive neuroectodermal tumor with early neuronal differentiation. The tumor was pathologically indistinguishable from poorly differentiated olfactory neuroblastoma (ONB) and Ewing sarcoma.

Chromogranins

Active muscle length reduction progressively damages soleus in hindlimb-suspended rabbits.

This study describes the morphologic changes in rabbit soleus muscle following hindlimb suspension (HS) for 1 to 4 weeks (group A); or following HS with hindfeet passively dorsiflexed, by means of an elastic band, for 1 to 2 weeks (group B). In the latter, elastic band use allowed phasic contractions of foot extensor muscles against resistance and prevented 35% chronic soleus shortening, which occurred in group A animals. In group A, the soleus revealed progressive muscle atrophy and myofibrillar damage. Myofibrils underwent dissolution, muscle regeneration was ineffective, and adipose tissue developed from about 2-week suspension onward. Conversely, passive dorsiflexion of unloaded hindfeet was essential in maintaining mass and structural muscle integrity in the soleus of group B. It is hereby demonstrated that HS-induced soleus damage in the rabbit is progressive, and can be prevented, avoiding long-term shortening of soleus and its phasic unloaded contractions. Soleus sensitivity to unloading conditions, such as HS, tenotomy, and hypogravity, may depend on the particular physiology of this tonic antigravity muscle, engaged mainly in developing long-lasting isometric contractions in a stretched length.

Animals

Myofibrillar disruption in the rabbit soleus muscle after one-week hindlimb suspension.

Relevant muscle- and species-specific differences may be found in the reaction of muscles to hindlimb suspension. This problem has been studied in 5 rabbits following a one-week hindlimb suspension, and in 5 ground-based controls. The soleus and the tibialis were prepared for light and transmission electron microscopy. In suspension the animals occasionally extended and flexed the hindlimbs, but, when standing still, their hindfeet were plantar-flexed to an angle of 180 degrees. In this position the length of the soleus was determined to be 35% less than in controls, whereas that of the tibialis was 30% more. Histologically, the tibialis fibers usually exhibited a preserved sarcomeric pattern, whereas soleus fibers displayed a regular sequence of areas of shortened sarcomeres, alternating with areas of myofibrillar disruption. These findings demonstrated that hindlimb suspension induces a focal breakdown of the soleus myofibrils, probably dependent on the reduced longitudinal tension of the suspended soleus and its phasic contractions against no load. It is conceivable that similar factors could also be responsible for soleus muscle atrophy induced by hypogravity as well as by other clinical conditions during which a stressful plantar flexion of the feet occurs against no load.

Animals

Morphological, immunocytochemical and growth characteristics of three human glioblastomas established in vitro.

The human glioblastoma-derived cell lines 86HG-39, 87HG-28 and 87HG-31, used for the production of monoclonal antibodies (mAbs) against glioma-associated antigens (GAA), were characterized in terms of morphology, growth behaviour, chromosomes and antigen expression. In the primary tumours, differential expression of glial fibrillary acidic protein, S100 protein, Leu-7 and GAA as defined by mAbs MUC 2-39, MUC 2-63 and MUC 8-22 was demonstrated. Receptors for epidermal growth factor (EGFr) and nerve growth factor (NGFr) were found in many cells in short-term cultures, but the transferrin receptor (Tr) was found in only a few cells of 87HG-28. In permanent cell lines, differentiation antigens and EGFr decreased and Tr increased markedly. NGFr and GAA remained stable. Transplantation tumours of 86HG-39 were partly positive for Tr and GAA. Chromosomal analysis revealed that the 86HG-39 and 87HG-28 cell lines had a hypodiploid or diploid stem line with lines in the hypotetraploid to tetraploid region for 50 in vitro passages. The 87HG-31 cell line had chromosomal patterns in the hypotriploid to triploid region. A gain of chromosomes was seen in the groups C7, C8, C10, D14, F19, F20, G21, G22. The variability of antigens in these tumours and especially during long-term cultivation probably reveals an ability to influence the growth of malignant glioma cells via the respective effector molecules.

Antibodies, Monoclonal

Amebic meningoencephalitis in a patient with AIDS caused by a newly recognized opportunistic pathogen. Leptomyxid ameba.

A fatal case of meningoencephalitis due to a leptomyxid ameba in a patient with the acquired immunodeficiency syndrome is presented. This opportunistic organism has not been previously recognized as a human pathogen. A 36-year-old male intravenous drug abuser died after an 18-day hospital course heralded by fever and headache and followed by nuchal rigidity and hemiparesis. Computed tomography of the head showed multiple hypodense lesions. Neuropathologic examination showed that in addition to human immunodeficiency virus encephalomyelitis, there was multifocal meningoencephalitis with trophozoites and cysts morphologically indistinguishable from those of Acanthamoeba. These organisms were also found in the kidneys and adrenal glands. By immunofluorescence, the parasites showed antigenic identity with a free-living leptomyxid ameba and failed to react with any of a spectrum of antiacanthamoeba antisera. This emphasizes the importance of immunofluorescence identification of morphologically indistinguishable ameba species.

Acquired Immunodeficiency Syndrome

Medullomyoblastoma in an adult.

Medullomyoblastoma is a rare histologic variant of medulloblastoma. Of the 20 cases reported in the literature, 19 were in children ages 2.5 to 10.5 years and one was in a 26-year-old woman. In the reported adult case the myogenic component of the tumor was leiomyosarcomatous. The authors report a case of medullomyoblastoma with a rhabdomyosarcomatous component in a 40-year-old man with light microscopic, immunohistochemical, and ultrastructural findings. The histogenetic theories regarding this tumor include that it is a teratoma, or that the myogenic component arises from the perivascular or leptomeningeal ectomesenchyme, or pluripotential neuroectodermal cells, or endothelial cells. The authors' findings do not elucidate the histogenesis but argue against an endothelial origin of the rhabdomyoblastic component.

Adult

Muscle fibre loss and reinnervation after long-term denervation.

Cutaneous pectoris muscles of frog (Rana temporaria) were investigated 19.5-40 months after denervation. On whole mounts a heavy reduction in size and number of muscle fibres is noticed; in two muscles studied with semithin and ultrathin sections the number of remaining muscle fibres is 149 and around 120, while one of the contralateral muscles contains 250 and control muscles of equal sized frogs between 220 and 320 (n = 18) fibres. By electron microscopy muscle fibres undergoing degeneration or phagocytosis can be seen (3 of 20 muscle fibres present in a single ultrathin cross-section). On the other hand several profiles contained within one common basal lamina sheath are present in 14 of 20 fibres, indicating satellite cell proliferation. In one preparation 40 months after denervation not a single muscle fibre or axon is present, suggesting that eventually, without nerve supply, muscle fibres entirely disappear. Upon spontaneous reinnervation or implantation of the hypoglossal nerve 16 months after denervation, synapses are formed with the remaining muscle fibres. When studied 3.5-24 months after nerve implantation muscles innervated by few axons only (less than 10, 10-20 axons) contain a low number of muscle fibres (mean 44 +/- 41 SD, n = 6), while all muscles with a larger number of axons have more than 150 muscle fibres (n = 6). This indicates that unless large numbers of axons regenerate and/or when reinnervation is delayed muscle fibre loss continues to occur. The presence in one muscle of motor axons but only six muscle fibres 24 months after nerve implantation indicates that muscle fibre loss cannot be reversed, or recovery is extremely slow. This observation is interpreted as evidence for the exhaustibility of the satellite cell pool.

Action Potentials

Myopathy with tubular aggregates in a patient adrenalectomized for Cushing's syndrome.

We report a patient with attacks of muscle weakness and mild myopathy with tubular aggregates, following bilateral adrenalectomy for adrenal Cushing's syndrome and replacement therapy with cortisone acetate and 9 alpha-fluorohydrocortisone. The replacement of 9 alpha-fluorohydrocortisone therapy by desoxycorticosterone acetate therapy led to the cessation of the attacks.

Adrenalectomy

Persistence of nerve sprouting with features of synapse remodelling in soleus muscles of adult mice.

An increase in the number of nerve branches of the unmyelinated axon terminals with increasing age was observed in normal adult mouse motor endplates. In addition, ultrastructural investigation revealed signs of nerve retraction. A combined light and electron microscopic investigation was performed on zinc-iodine-osmium stained endplates in soleus muscles. The number of branch points in a synapse, endplate length and muscle fiber diameter were evaluated in "young adult" (3 months) and adult (6 and 11 months) mice. For all 3 parameters, 3-month-old animals had the lowest values. Eleven-month-old animals had more branch points and larger endplate lengths than 6-month-old animals while there was no significant difference in fiber diameters. Branch point numbers and endplate length were correlated in each muscle while fiber diameters did not correlate with any of the other parameters. The ultrastructure of 15 thin nerve branches--likely candidates for new branches--was investigated in serial section and in 14 of them synaptic contacts were found. Near such contacts, empty gutters, possibly abandoned former synaptic sites, were present in several cases. It is concluded that there is continual nerve sprouting in synapses of adult mice and that sprouts form synaptic contacts. The possible signs of nerve retraction observed indicate that, as in the frog, synaptic contacts in mouse muscles undergo some continual remodeling.

Aging

Light and electron microscopic identification of nerve terminal sprouting and retraction in normal adult frog muscle.

A combined light and electron microscopic study was performed on neuromuscular junctions of normal adult frogs. In a previous investigation signs of new synapse formation, as well as abandoned former synaptic sites, have been observed in normal muscles (Wernig, Pécot-Dechavassine & Stöver, 1980a, b). Here we performed a detailed light and electron microscopic correlation to investigate those parts of junctions which, after staining for cholinesterase (ChE) and presynaptic axon terminals, were suspected either to be newly formed or sites abandoned by the presynaptic nerve and the Schwann cell. Thin presynaptic nerve branches, enclosed by Schwann cell sheaths along most of their length, formed synaptic contacts with the muscle fibre only at small circumscribed areas. In these regions post-synaptic secondary folds (invariably present at mature synapses) were either missing or were less well developed. At these small contacts, binding sites for fluorescein-labelled alpha-bungarotoxin were usually present. At other sites the ChE reaction product was present but an axon could not be detected in silver-stained preparations. Electron microscopic observation revealed post-synaptic secondary folds filled with ChE reaction product while the presynaptic axon and Schwann cell were missing. The sites with ChE remnants can thus be regarded as abandoned former synaptic contacts. No binding of fluorescein-labelled alpha-bungarotoxin could be detected at such sites. These findings confirm earlier suggestions that synaptic contacts in frog muscle are normally undergoing continual remodelling. The lack of binding sites for fluorescein-labelled alpha-bungarotoxin at abandoned synaptic sites suggests that a neural or Schwann cell factor is important for the maintainance of synaptic acetylcholine receptors.

Animals

Presence and absence of virus particles in hybridomas secreting monoclonal antibodies against gliomas.

Hybridoma clones were produced by fusion of splenocytes from glioma-immunized hosts and the X63-Ag8.653 mouse myeloma line and Y3-Ag.1.2.3. rat myeloma line. Oncornavirus particles were found in all clones descending from the mouse myeloma line. No virus particles could be found in either the spleens of immunized Balb/c mice and Fischer rats or in the rat myeloma line and the hybridomas derived from it.

Animals