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1.
Neurology ; 29(1): 100-6, 1979 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-154627

RESUMO

An acute reversible myopathy characterized by extensive lysis of myosin occurred in a patient who suffered from severe shock, hypoxia, and acidosis. This new clinical pathologic entity illustrates an unusual structural change in muscle associated with these catastrophic clinical circumstances.


Assuntos
Doenças Musculares/patologia , Miosinas/metabolismo , Doença Aguda , Adenosina Trifosfatases/metabolismo , Adulto , Feminino , Humanos , Músculos/ultraestrutura , Doenças Musculares/metabolismo , Miofibrilas/ultraestrutura
2.
Neurology ; 41(1): 135-40, 1991 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-1824643

RESUMO

We studied muscles from 3 patients with centronuclear myopathy (CNM) by immunocytochemistry using myosin heavy chain (MHC)-specific monoclonal antibodies to determine whether subtypes of CNM express prenatal MHC and to assess if there is an arrest in development of these muscles. Muscle from a woman with childhood-onset CNM did not express prenatal MHC, yet this prenatal MHC was strongly expressed in the muscle fibers of 2 brothers with X-linked CNM. This finding represents the 1st immunocytochemical evidence of the expression of a prenatal myosin isoform in nonregenerating postnatal human muscle and suggests that the X-linked form of CNM differs from the other types because of a true arrest in maturation of the muscle.


Assuntos
Doenças Musculares/patologia , Miosinas/química , Adulto , Anticorpos Monoclonais , Biópsia , Feminino , Genes Recessivos , Ligação Genética , Histocitoquímica , Humanos , Imuno-Histoquímica , Lactente , Recém-Nascido , Masculino , Músculos/patologia , Doenças Musculares/classificação , Doenças Musculares/enzimologia , Doenças Musculares/genética , Cromossomo X
3.
J Histochem Cytochem ; 37(10): 1539-43, 1989 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-2476479

RESUMO

We report the characterization of monoclonal antibody (MAb) ALD 180, prepared against the myosin of slow avian muscle, for studies of human muscle development and disease. With the use of radioimmunoassays, Western immunoblots of native and denatured myosins, and epifluorescent indirect immunocytochemistry, we show that ALD 180 is specific for an epitope in human prenatal skeletal muscle myosin heavy chain (MHC), which is expressed in diminishing abundance in fetal fibers from at least 19-22 weeks' gestation to term and also in regenerating muscle fibers seen in diseased muscles from both children and adults. ALD 180 recognizes an epitope apparently different from those reacting with anti-prenatal human myosin MAb previously described, and therefore affords a complementary reagent for use in future studies of human myosin isoform expression and regulation.


Assuntos
Anticorpos Monoclonais/imunologia , Músculos/embriologia , Miosinas/imunologia , Laranja de Acridina , Trifosfato de Adenosina/análise , Trifosfato de Adenosina/metabolismo , Adulto , Criança , Epitopos , Imunofluorescência , Humanos , Músculos/análise , Distrofias Musculares/metabolismo , Miosinas/análise , Miosite/metabolismo , Especificidade da Espécie
4.
J Neurol Sci ; 95(2): 183-92, 1990 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-2324769

RESUMO

It has been suggested, on the basis of mostly morphological and some biochemical evidence, that defective innervation of muscle of patients with Werdnig-Hoffmann (WH) disease results in maturational arrest of the fibers at a stage comparable to 20-week gestational muscle. Therefore, with the use of recently developed and characterized, myosin-isoform-specific monoclonal antibodies (McAbs), an immunocytochemical study of muscle of 6 children with Werdnig-Hoffmann disease was done to determine if the pattern of expression of myosin heavy chain isoforms (MHC) in these fibers was similar to that of 20-week gestation muscle. This work showed that the MHC isoform expression in the muscle of the children with WH did not mimic that seen in 20-week gestation muscle since only a few fibers (less than 1-11%) in each specimen expressed prenatal MHC as detected by reactivity to McAb, ALD 180 (specific for a prenatal MHC) whereas virtually all of the fibers from 20-week gestation muscle were strongly reactive with ALD 180. The majority of the fibers expressed either adult fast MHC or adult slow MHC similar to that seen in normal muscle, although some co-expressed multiple MHC isoforms. Our results indicate that the difference of adult MHC isoforms in the muscle fibers of WH patients either proceeds in the absence of innervation or that denervation of muscle fibers is subsequent to the neural input required to initiate myosin isoform transitions to the adult isoforms.


Assuntos
Regulação da Expressão Gênica , Atrofia Muscular Espinal/metabolismo , Miosinas/genética , Atrofias Musculares Espinais da Infância/metabolismo , Anticorpos Monoclonais , Criança , Pré-Escolar , Humanos , Imuno-Histoquímica , Lactente , Miosinas/metabolismo
5.
J Neurol Sci ; 80(1): 1-12, 1987 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-2440999

RESUMO

An immunocytochemical study was done on the skeletal muscles of human fetuses (19-36 weeks gestation), infants and adults using a new monoclonal antibody (McAb) ALD-47. The antibody was generated against slow myosin of chicken and is specific for myosin heavy chain (MHC). In human infants and adults the type I muscle fibres are strongly reactive with this McAb and the type II fibres uniformly non-reactive. In the fetuses from 19-20 weeks gestation (in whom the fibre types are not distinguishable by the histochemical myosin ATPase test) a proportion of muscle fibres react specifically with ALD-47. Other muscle fibres at this stage react positively with a fast specific MHC McAb HM-1.2 or are negative to both ALD-47 and HM-1.2 antibodies. These McAbs, thus, identify three distinct fibre populations in the early fetal muscle which by histochemical staining appears homogeneous. The percentage of ALD-47 positive fibres increases in fetuses at later gestational periods; at all stages these fibres lack reactivity with the HM-1.2 antibody. Because of its selective fibre type reactivity in differentiating muscles, the McAb ALD-47 in conjunction with HM-1.2 should be useful in immunoaffinity fractionation and biochemical studies of myosin isoforms in developing human muscles.


Assuntos
Adenosina Trifosfatases/metabolismo , Músculos/metabolismo , Miosinas/metabolismo , Fragmentos de Peptídeos/metabolismo , Anticorpos Monoclonais , Epitopos , Imunofluorescência , Idade Gestacional , Histocitoquímica , Humanos , Músculos/embriologia
6.
J Neurol Sci ; 63(1): 75-84, 1984 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-6321669

RESUMO

A well documented case of I-cell disease is presented. Light- and electron-microscopic studies of muscle revealed marked accumulation of characteristic I-cell inclusions in satellite cells and only scattered autophagic vacuoles in muscle fibers. Correlation with previous tissue culture studies indicated an amelioration of structural abnormalities with differentiation from satellite cell to mature muscle fiber. Histochemically, the muscle demonstrated paucity of type I fibers without evidence of denervation thus suggesting a developmental disturbance in motor unit organization. Selective type I fiber dysfunction and reduced satellite cell regenerative capacity may be related factors in the neuromuscular disability of patients with I-cell disease.


Assuntos
Mucolipidoses/patologia , Músculos/patologia , Pré-Escolar , Feminino , Fibroblastos/ultraestrutura , Histocitoquímica , Humanos , Corpos de Inclusão/ultraestrutura , Microscopia Eletrônica , Mucolipidoses/metabolismo , Músculos/metabolismo , Músculos/ultraestrutura
7.
Drug Alcohol Depend ; 1(4): 247-54, 1976 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-797562

RESUMO

The brains of ten narcotic addicts who had died from an overdose of methadone, and the brains of ten rats, given methadone for one month, were examined by the immunofluorescent technic. Positive neuronal fluorescence was seen primarily in the limbic systems of both species as well as in closely associated areas. In the human, Purkinje cell fluorescence was seen in the cerebellum, whereas only stellate cell staining was observed in rat cerebellum. Neurons of the hippocampal denate gyrus often fluoresced in man while only pyramidal cell staining was seen in this region of rat brain. The method should prove to be of value in the detection and tracing of narcotic drugs and may be helpful in investigations of drug tolerance and dependence.


Assuntos
Encéfalo/metabolismo , Metadona/metabolismo , Animais , Encéfalo/citologia , Imunofluorescência , Humanos , Masculino , Neurônios/metabolismo , Ratos , Transtornos Relacionados ao Uso de Substâncias/metabolismo , Transtornos Relacionados ao Uso de Substâncias/patologia
8.
J Neurosurg ; 43(4): 483-5, 1975 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-1159487

RESUMO

The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete.


Assuntos
Anemia Falciforme/complicações , Sistema Hematopoético/patologia , Compressão da Medula Espinal/etiologia , Adulto , Anemia Falciforme/patologia , Humanos , Masculino , Paraplegia/etiologia
9.
J Neurosurg ; 49(3): 445-9, 1978 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-682008

RESUMO

A child treated for a desmoplastic medulloblastoma of the left cerebellar hemisphere at the age of 10 months developed a malignant astrocytoma in the same site 11 years later. Theories of origin of the second tumor, particularly in relation to concepts of the genesis of medulloblastoma in general, are discussed.


Assuntos
Astrocitoma/patologia , Neoplasias Cerebelares/patologia , Meduloblastoma/patologia , Neoplasias Primárias Múltiplas/patologia , Criança , Humanos , Masculino , Estadiamento de Neoplasias , Fatores de Tempo
10.
J Child Neurol ; 1(2): 149-57, 1986 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-2439564

RESUMO

Nine cases of marginal glioneuronal heterotopias over the cerebral cortex were reviewed from the morphological point of view. There were developmental disabilities in all cases except one (case 8), who was stillborn. All subjects died before 1 year of age except one (case 5). The common features of small glioneuronal heterotopias and abundant heterotopic glioneuronal proliferation are described. The correlation of glioneuronal heterotopias with polymicrogyria and other cortical malformations, as well as their appearance over a normal cortex, are described. The glioneuronal heterotopias are considered to be a separate type of malformation that could arise during the second half of intrauterine life. A breach of the neuropial border seems to be the most acceptable pathomechanism for our presented cases. Their morphological features indicate that damage to this barrier leads to involvement of glioneuronal heterotopias in fusion of opposite cortical convolutions.


Assuntos
Neoplasias Encefálicas/patologia , Córtex Cerebral/patologia , Coristoma/patologia , Deficiências do Desenvolvimento/patologia , Neuroglia , Humanos , Lactente , Recém-Nascido
11.
Brain Dev ; 6(1): 60-4, 1984.
Artigo em Inglês | MEDLINE | ID: mdl-6203423

RESUMO

This case report is a neuropathological study of a ten-month-old infant with unilateral megalencephaly . In this anomaly neuronal migration defect and disturbances of cortical organization resulting in micropolygyria were the most striking neuropathological feature.


Assuntos
Córtex Cerebral/anormalidades , Deficiências do Desenvolvimento/patologia , Espasmos Infantis/patologia , Astrócitos/ultraestrutura , Calcinose/patologia , Córtex Cerebral/patologia , Feminino , Humanos , Lactente , Bainha de Mielina/ultraestrutura , Neurônios/ultraestrutura
12.
Brain Dev ; 5(3): 302-10, 1983.
Artigo em Inglês | MEDLINE | ID: mdl-6614389

RESUMO

The present study is a review of four new cases of lissencephaly and two others previously reported. This study demonstrates that lissencephaly is a gross feature of the brain occurring in two different groups of cortical malformations. The first group, the classic agyria syndrome extensively analyzed by Jellinger and Rett [8] includes two types of abnormal cortical organization. They may be found in familial syndromes and also can appear sporadically. The second group includes smooth brains with the internal features of polymicrogyria and a more severely disorganized cortex. This type appears in familial lissencephaly in the cerebro-oculo-muscular syndrome, belonging to the same group as Fukuyama congenital-cerebro-muscular dystrophy. The other incidences of this type of cortical malformation require further investigation. The clinico-pathological differential diagnosis of two types of lissencephaly are also discussed.


Assuntos
Encéfalo/anormalidades , Anormalidades Múltiplas/patologia , Córtex Cerebelar/patologia , Córtex Cerebral/patologia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Deficiência Intelectual/patologia , Masculino , Músculos/patologia , Distrofias Musculares/patologia , Neurônios/ultraestrutura
13.
Clin Neuropathol ; 8(2): 85-91, 1989.
Artigo em Inglês | MEDLINE | ID: mdl-2721045

RESUMO

The Galloway syndrome is a rare autosomal recessive disease consisting of congenital microencephaly associated with congenital nephrotic syndrome, and in some cases with hiatus hernia [Galloway and Mowatt, 1968]. The case presented is that of a microencephalic infant with the nephrotic syndrome who died at 11 3/4 months after a course characterized by convulsions, developmental delay, hypotonia and hyperreflexia. Brain weight was 270 g. The frontal, parietal, and rostral temporal cortex was pachygyric. Microscopically there was lack of cortical stratification, immature cortical neurons, improper orientation of cortical neurons (seen in the Golgi stained sections), and glioneuronal ectopias in the leptomeninges. There was hypomyelination in the brain stem and spinal cord, and no myelin in the hemispheres. There was also complete absence of the internal granular layer of the cerebellum. The dentate gyrus within the hippocampal formation was absent and the inferior olivary nuclei were hypoplastic. The mechanism of neuronal migration abnormalities and the significance of associated nephrosis is discussed.


Assuntos
Encéfalo/anormalidades , Hérnia Hiatal/congênito , Hérnias Diafragmáticas Congênitas , Síndrome Nefrótica/congênito , Encéfalo/patologia , Sobrevivência Celular , Cerebelo/patologia , Córtex Cerebral/patologia , Feminino , Humanos , Lactente , Microcefalia/genética , Bainha de Mielina/patologia , Síndrome
14.
Arch Pathol Lab Med ; 115(1): 21-5, 1991 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-1987909

RESUMO

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.


Assuntos
Síndrome da Imunodeficiência Adquirida/parasitologia , Amebíase/parasitologia , Amoeba/isolamento & purificação , Meningoencefalite/parasitologia , Infecções Oportunistas/parasitologia , Síndrome da Imunodeficiência Adquirida/complicações , Síndrome da Imunodeficiência Adquirida/diagnóstico por imagem , Adulto , Amebíase/complicações , Amebíase/diagnóstico por imagem , Animais , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Imunofluorescência , Humanos , Meningoencefalite/complicações , Meningoencefalite/diagnóstico por imagem , Infecções Oportunistas/complicações , Infecções Oportunistas/diagnóstico por imagem , Tomografia Computadorizada por Raios X
15.
Surg Neurol ; 8(4): 273-6, 1977 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-898004

RESUMO

A case of diencephalic-midbrain hemorrhage presenting with excessive thirst, coma, and death is discussed. Some diencephalic dysfunction syndromes are reviewed, as well as their physiological basis. Thirst and drinking behavior can provide a valuable localizing symptom and sign in clinical-pathological correlations.


Assuntos
Hemorragia Cerebral/complicações , Hipotálamo , Mesencéfalo , Sede , Adulto , Hemorragia Cerebral/patologia , Feminino , Humanos
16.
Can J Ophthalmol ; 18(5): 248-50, 1983 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-6313171

RESUMO

This paper describes a man with previously undetected prostatic carcinoma who presented with mild symptoms due to an orbital metastasis. The case is unusual in that the patient was relatively asymptomatic and had 6/7 vision. Carcinoma of the prostate metastatic to the orbit is rare; this is only the 22nd case in the literature.


Assuntos
Adenocarcinoma Mucinoso/secundário , Neoplasias Orbitárias/secundário , Neoplasias da Próstata/patologia , Adenocarcinoma Mucinoso/radioterapia , Idoso , Biópsia , Humanos , Masculino , Neoplasias Orbitárias/radioterapia , Ultrassonografia
17.
Can J Ophthalmol ; 17(3): 116-20, 1982 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-6749266

RESUMO

The suitability and effectiveness of hydrophilic soft contact lenses and donor corneal buttons for interlamellar refractive keratoplasty were evaluated in a study with rabbits. In each experimental eye a trephined button of Bausch & Lomb Soflens (polymacon), Cooper Permalens (perfilcon A) or rabbit cornea 6.5 mm in diameter was inserted in a corneal pocket created with a lamellar dissector. Among the inserts available for long-term follow-up all five of the Soflens inserts showed "melting" (perforation and sloughing) by 3 weeks, whereas six of the seven Permalens inserts and all four of the donor corneal inserts were clear at 3 to 6 months. Myopia was induced and the corneal curvature steepened with all but the high-minus Soflens inserts. Thus, interlamellar refractive keratoplasty with soft contact lenses of high water content or lathe-cut donor corneal buttons has potential for the correction of aphakia in rabbits and may well have similar potential in humans.


Assuntos
Lentes de Contato Hidrofílicas/normas , Transplante de Córnea , Animais , Córnea/patologia , Córnea/cirurgia , Modelos Biológicos , Miopia/complicações , Complicações Pós-Operatórias , Coelhos , Refração Ocular
18.
J N J Dent Assoc ; 65(4): 29-33, 1994.
Artigo em Inglês | MEDLINE | ID: mdl-9520699

RESUMO

Temporization of teeth prepared for porcelain laminate veneers is sometimes necessary to preserve occlusal relationships, prevent sensitivity or maintain esthetics. The literature describes several techniques which satisfy different requirements for temporization. A modified technique is presented that satisfies at once: occlusion, sensitivity and esthetic needs. Clinical time spent with the patient is minimized by fabricating a matrix on a diagnostic cast prior to the preparation/ impression appointment.


Assuntos
Porcelana Dentária , Restauração Dentária Temporária/métodos , Facetas Dentárias , Colagem Dentária/métodos , Técnica de Fundição Odontológica , Técnica de Moldagem Odontológica , Oclusão Dentária , Estética Dentária , Humanos , Placas Oclusais , Fatores de Tempo
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