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1.
J Neuropathol Exp Neurol ; 47(2): 158-65, 1988 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-2892894

RESUMEN

Scrapie-associated fibrils (SAF) are disease-specific markers for the unconventional agent-induced, transmissible spongiform encephalopathies. Polyclonal rabbit antiserum to SAF protein was reacted with brain sections from scrapie-infected mice, two familial cases of transmissible dementia, and three cases of Alzheimer's disease (AD). Specific immunostaining of cerebral amyloid plaques occurred in the scrapie-infected mice and in the two familial cases of transmissible dementia. No immunoreactivity was detected in senile plaques or neurofibrillary tangles in the three cases of AD. Our results suggest that SAF, the causative pathogenic agent, and extracellular deposits of amyloid in the brain are closely related. Immunohistochemical detection of SAF protein could serve as a useful diagnostic adjunct in the postmortem evaluation of difficult cases of dementia. The identification of SAF protein in the brains of two affected members of a family combining the clinical and pathological features of Creutzfeldt-Jakob disease (CJD) and the Gerstmann-Straüssler syndrome (GSS) substantiates earlier conclusions of a nosological relationship between the two. Our study provides further evidence of the similarity of SAF protein to prion protein (PrP 27-30).


Asunto(s)
Amiloide/inmunología , Demencia/genética , Proteínas del Tejido Nervioso/inmunología , Adulto , Síndrome de Creutzfeldt-Jakob/metabolismo , Síndrome de Creutzfeldt-Jakob/patología , Demencia/inmunología , Francia , Humanos , Sueros Inmunes/inmunología , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Proteína PrP 27-30 , Enfermedades por Virus Lento/metabolismo , Enfermedades por Virus Lento/patología
2.
Brain Pathol ; 5(4): 459-66, 1995 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-8974629

RESUMEN

Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed for the following disease entities: CJD--sporadic, iatrogenic (recognised risk) or familial (same disease in 1st degree relative): spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical grey matter; or encephalopathy with prion protein (PrP) immunoreactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types). Gerstmann-Sträussler-Scheinker disease (GSS) (in family with dominantly inherited progressive ataxia and/or dementia): encephalo(myelo)pathy with multicentric PrP plaques. Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the Fore population). Without PrP data, the crucial feature is the spongiform change accompanied by neuronal loss and gliosis. This spongiform change is characterised by diffuse or focally clustered small round or oval vacuoles in the neuropil of the deep cortical layers, cerebellar cortex or subcortical grey matter, which might become confluent. Spongiform change should not be confused with non-specific spongiosis. This includes status spongiosus ("spongiform state"), comprising irregular cavities in gliotic neuropil following extensive neuronal loss (including also lesions of "burnt-out" CJD), "spongy" changes in brain oedema and metabolic encephalopathies, and artefacts such as superficial cortical, perineuronal, or perivascular vacuolation; focal changes indistinguishable from spongiform change may occur in some cases of Alzheimer's and diffuse Lewy body diseases. Very rare cases might not be diagnosed by these criteria. Then confirmation must be sought by additional techniques such as PrP immunoblotting, preparations for electron microscopic examination of scrapie associated fibrils (SAF), molecular biologic studies, or experimental transmission.


Asunto(s)
Síndrome de Creutzfeldt-Jakob/patología , Enfermedades por Prión/patología , Enfermedad de Gerstmann-Straussler-Scheinker/patología , Humanos
3.
Brain Pathol ; 5(3): 319-22, 1995 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-8520732

RESUMEN

Despite many sensational and intimidating reports in the mass media, transmissible spongiform encephalopathies (prion disease) are not contagious in the usual sense. Successful transmission requires both specific material (an affected individual's tissue, from or adjacent to CNS) and specific modes (mainly penetrating contact with the recipient). Nevertheless, specific safety precautions are mandatory to avoid accidental transmission and to decontaminate any infectivity. Autopsy is essential for definite diagnosis of these disorders. Recommendations are given here for performance of the autopsy, for neuropathology service and appropriate decontamination; they are based on the current literature and on precautions taken in most laboratories with experience in handling tissue from transmissible spongiform encephalopathies. In particular, special care must be taken to avoid penetrating wounds, possible contamination should be kept to a minimum, and potential infectious material must be adequately decontaminated by specific means.


Asunto(s)
Síndrome de Creutzfeldt-Jakob/transmisión , Enfermedades por Prión/transmisión , Seguridad , Manejo de Especímenes , Autopsia , Síndrome de Creutzfeldt-Jakob/patología , Descontaminación , Humanos , Enfermedades por Prión/patología
4.
Arch Neurol ; 38(3): 186-90, 1981 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-6970563

RESUMEN

Among the hereditary affections of the nervous system associated with deafness, a rare condition called "progressive pontobulbar palsy with deafness" has been described. In this slowly progressive condition, hearing loss and vestibular are-flexia are almost always the first symptoms, occurring in late childhood or early adulthood. Only 18 cases-some sporadic, several familial-have been published without a full report of pathological findings. The clinical and pathological data of two new cases-one familial, one sporadic-are described here. There are differences from other forms of bulbar paralysis, lower motor neuron diseases, and some spinocerebellar hereditary affections. In view of the homochrony and homotypy in familial cases and the pathological findings, progressive pontobulbar palsy with deafness appears to be an abiotrophic process wih autosomal recessive inheritance.


Asunto(s)
Sordera/patología , Parálisis/patología , Adolescente , Encéfalo/patología , Tronco Encefálico/patología , Niño , Nervios Craneales/patología , Femenino , Humanos , Reflejo Anormal/patología , Vestíbulo del Laberinto
5.
Neurology ; 44(8): 1479-83, 1994 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-8058153

RESUMEN

OBJECTIVE: We tested adjuvant chemotherapy combining dibromodulcitol (DBD) and bischloroethylnitrosourea (BCNU) given postoperatively to adults with newly diagnosed supratentorial malignant gliomas. METHODS: We enrolled 269 patients, 255 of whom were eligible. After surgery, we treated all patients with radiation therapy, using a median dose of 60 Gy given in 30 fractions. After randomization, patients in the chemotherapy group also received (1) six weekly courses, administered during irradiation, of DBD 700 mg/m2 and (2) one to nine (median, four) courses, administered during the first year following radiation therapy, of DBD 1,000 mg/m2 on day 1 and BCNU 150 mg/m2 on day 2, with the course being repeated every 6 weeks. RESULTS: Patients treated with radiation therapy along with DBD plus BCNU (group 2) had significantly longer survival time (p = 0.044) and time to progression (p = 0.003) than did those treated with radiation therapy alone (group 1). The median survival time was 13.0 months for group 2 and 10.4 months for group 1; the median time to progression was 8.1 months for group 2 and 6.7 months for group 1. The percentage of patients alive at 18 and 24 months was 34% and 21% in group 2 compared with 21% and 12% in group 1. CONCLUSION: DBD plus BCNU is an effective adjuvant therapy for malignant glioma.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/mortalidad , Glioma/tratamiento farmacológico , Glioma/mortalidad , Adulto , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Carmustina/administración & dosificación , Quimioterapia Adyuvante , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mitolactol/administración & dosificación , Análisis de Supervivencia
6.
Neurology ; 33(12): 1613-5, 1983 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-6685833

RESUMEN

The case of an adolescent with idiopathic recurrent myoglobinuria is reported. The following features are emphasized: (1) persistence of limb weakness and elevated CK levels between the attacks, (2) a constant myopathic pattern in EMG, and (3) chronic morphologic abnormalities of myopathy in two muscle biopsies. All known causes of myoglobinuria were investigated and ruled out.


Asunto(s)
Mioglobinuria/patología , Rabdomiólisis/patología , Adolescente , Extremidades , Humanos , Masculino , Enfermedades Musculares/metabolismo , Enfermedades Musculares/patología , Mioglobinuria/metabolismo , Recurrencia
7.
Neurology ; 34(6): 730-5, 1984 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-6539433

RESUMEN

Clinical and pathologic findings in six autopsies and five biopsies of cerebral amyloid angiopathy associated with cerebromeningeal hemorrhages are presented. One patient had experienced a previous meningeal hemorrhage. Only two had chronic hypertension; the multiple fresh hematomas found in all the autopsied brains always spared the basal ganglia and brainstem, as did vascular lesions, which were mostly cortical and meningeal. Extensive lesions of Alzheimer's disease were found in the autopsied cases with dementia. The most significant feature for clinical diagnosis of hemorrhagic cerebral amyloid angiopathy is the presence of multiple hemorrhages in unusual locations in the absence of hypertension.


Asunto(s)
Amiloidosis/complicaciones , Encefalopatías/complicaciones , Hemorragia Cerebral/etiología , Anciano , Amiloidosis/patología , Encefalopatías/patología , Corteza Cerebral/irrigación sanguínea , Corteza Cerebral/patología , Hemorragia Cerebral/patología , Femenino , Humanos , Masculino , Meninges/irrigación sanguínea , Meninges/patología , Persona de Mediana Edad , Estudios Retrospectivos , Enfermedades Vasculares/complicaciones , Enfermedades Vasculares/patología
8.
J Nucl Med ; 38(9): 1459-62, 1997 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-9293808

RESUMEN

UNLABELLED: Gliomas are regionally heterogeneous tumors. The local relationship between histologic features and radiotracer uptake evaluated by PET should therefore influence analysis and interpretation of PET results on gliomas. This study explored this local relationship as a result of PET guidance of stereotactic biopsies. METHODS: Local histology was confronted to the regional uptake of 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) and 11C-methionine (11C-MET) in 14 patients with high-grade glioma diagnosed during a procedure of PET-guided stereotactic biopsies. We analyzed the uptake of both tracers in regions of interest centered on the stereotactic coordinates of 93 biopsy samples. RESULTS: A semiquantitative analysis revealed a significant regional correlation between 11C-MET and 18F-FDG uptakes. Uptake of both tracers was significantly higher on the site of tumor samples showing anaplastic changes than in the rest of the tumor. Presence of necrosis in anaplastic areas of the tumor significantly reduced the uptake of 11C-MET. CONCLUSION: PET with 11C-MET and 18F-FDG may help to evaluate, in vivo, the metabolic heterogeneity of human gliomas. Anaplasia is a factor of increased uptake of both tracers, but microscopic necrosis in anaplastic areas influences their uptake differently. This finding probably relates to the differences in tracer uptake by non-neoplastic components of necrotic tumors. These results underline the complementary role of 18F-FDG and 11C-MET for the study of brain tumors and favors their use for stereotactic PET guidance of diagnostic or therapeutic procedures.


Asunto(s)
Biopsia con Aguja , Neoplasias Encefálicas/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Radioisótopos de Carbono , Desoxiglucosa/análogos & derivados , Radioisótopos de Flúor , Glioma/diagnóstico por imagen , Metionina , Técnicas Estereotáxicas , Tomografía Computarizada de Emisión , Adulto , Anciano , Encéfalo/patología , Neoplasias Encefálicas/patología , Femenino , Fluorodesoxiglucosa F18 , Glioma/patología , Humanos , Masculino , Persona de Mediana Edad , Radiofármacos
9.
Am J Med Genet ; 56(3): 276-80, 1995 Apr 10.
Artículo en Inglés | MEDLINE | ID: mdl-7778589

RESUMEN

We have studied a girl with multiple congenital anomalies, growth and mental deficiency, characteristic facial anomalies, cataracts, cerebellar atrophy, and severe hypocholesterolemia. Death occurred at age 7 years. After excluding several syndromes, i.e., peroxisomal disorders, mevalonic acidaemia, and Marinesco-Sjögren syndrome, it is concluded that this girl had severe Smith-Lemli-Opitz Syndrome (SLOS) with exceptionally long survival. This diagnosis was confirmed through assay of 7-dehydrocholesterol in cultured fibroblasts.


Asunto(s)
Anomalías Múltiples/patología , Colesterol/metabolismo , Errores Innatos del Metabolismo Lipídico/patología , Anomalías Múltiples/sangre , Anomalías Múltiples/genética , HDL-Colesterol/sangre , LDL-Colesterol/sangre , Resultado Fatal , Femenino , Humanos , Recién Nacido , Errores Innatos del Metabolismo Lipídico/sangre , Errores Innatos del Metabolismo Lipídico/genética , Hígado/patología , Fenotipo , Síndrome
10.
Immunobiology ; 166(1): 62-71, 1984 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-6325328

RESUMEN

Injection of silica did not brake the resistance against MHV3 conferred to C57BL/6 mice by injection of C. parvum. However, silica itself had a marked protective effect against MHV3 infection that was maximal when injecting 1 mg 2 hrs before virus infection. The protective effect of silica was observed in a number of inbred mouse strains that differ in their relative resistance to MHV3 infection. No viral titers were observed in the spleen and liver of mice which had received MHV3 plus silica, whereas high titers were observed in the virus-infected controls. Injection of silica caused a marked decrease in the number of esterase-positive macrophages in the peritoneal wash-out population, that may be compatible with the possibility that the cause of the protection is the depletion of target cells for the viral infection. This latter effect, however, was short-lived and 24-48 hrs after injection of silica, high numbers of esterase-positive cells were again observed. This may explain why only little protection was observed when silica was administered 2 days before virus infection.


Asunto(s)
Hepatitis Viral Animal/prevención & control , Dióxido de Silicio/uso terapéutico , Adyuvantes Inmunológicos , Animales , Macrófagos/inmunología , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Endogámicos , Virus de la Hepatitis Murina , Propionibacterium acnes/inmunología
11.
Antiviral Res ; 3(5-6): 325-33, 1983 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-6200068

RESUMEN

We have studied the susceptibility of SM/J mice to intraperitoneal (i.p.) infection with herpes simplex virus type 1 (HSV) and have searched for correlations of susceptibility with the activation of Natural Killer (NK) cells and with local induction of interferon. SM/J were exceedingly susceptible to virus infection as they could be killed by less than 10 plaque forming units (PFU). The NK cell system of these mice, as measured by the activity of spleen cells against YAC-1 lymphoma cells, was hyperreactive, which is in agreement with previous findings of others. The peritoneal exudate cells (PEC) of uninfected mice had no activity. However, 18 h after i.p. injection of HSV NK cell activity was detected in the PEC population, which was at least as high as that in C57BL/6 mice that are resistant to HSV infection. Thus it appears as if the NK cell system does not play a major role in antiviral resistance in our experimental system. In contrast, from our previous work it would rather appear that the magnitude of the early local interferon response is important for resistance. The current data obtained in SM/J mice are in accordance with this, in that these highly susceptible mice are deficient in their early interferon response. Homozygous beige mice were found to be as resistant to infection with HSV as C57BL/6 mice. While the NK cell activity in their PEC population after injection of HSV was low, the titers of locally induced interferon were as high as those in the controls.


Asunto(s)
Herpes Simple/inmunología , Interferones/biosíntesis , Células Asesinas Naturales/inmunología , Animales , Herpes Simple/metabolismo , Inmunidad Innata , Macrófagos/microbiología , Masculino , Ratones , Ratones Endogámicos
12.
Invest Radiol ; 15(6 Suppl): S254-9, 1980.
Artículo en Inglés | MEDLINE | ID: mdl-6894139

RESUMEN

Novel nonionic contrast media were tested by injection into the subarachnoid space of the anterior fossae of guinea pigs, followed by EEG recording and histologic examination. Iopamidol was found to be more epileptogenic than metrizamide and P-297.


Asunto(s)
Sistema Nervioso Central/efectos de los fármacos , Medios de Contraste/toxicidad , Convulsiones/inducido químicamente , Animales , Sistema Nervioso Central/diagnóstico por imagen , Sistema Nervioso Central/fisiología , Medios de Contraste/administración & dosificación , Electroencefalografía , Cobayas , Inyecciones Espinales , Neurorradiografía/métodos , Solubilidad
13.
Radiat Res ; 147(5): 621-30, 1997 May.
Artículo en Inglés | MEDLINE | ID: mdl-9146708

RESUMEN

Hemodynamic parameters such as total cerebral blood volume (total CBV), cerebral parenchymal blood volume (CBV), cerebral blood flow (CBF) and cerebral blood velocity index were measured in rats 6, 12 and 18 months after single exposures of brain to 5, 10, 15 and 20 Gy X rays for total CBV, CBF and blood velocity index, and only 20 Gy for CBV. Total CBV and blood velocity index were determined by a noninvasive blood dilution method using [99mTc]pertechnetate and CBF by [131I]iodoantipyrine brain extraction. The CBV was obtained from both parenchymal plasma and erythrocyte volumes measured in isolated brain by 125I-labeled serum albumin and 51Cr-labeled erythrocytes, respectively. Neither the dose nor the time after irradiation influenced total CBV. Nevertheless, CBV decreased slightly while CBF decreased strongly at 12 and 18 months after 20 Gy. In contrast, the blood velocity index increased progressively at 12 and 18 months after 15 Gy and at all times after 20 Gy. According to the coexistence in irradiated brains of a remodeling with microvascular occlusions and dilated abnormal vessels, this lowered CBF can be explained by the smaller number of open capillaries and a "steal phenomenon" through low-resistance channels developed in the parenchymal and extraparenchymal vasculatures. Such a "steal phenomenon" is also supported by the response of the blood velocity index, which appears to be the earliest sensitive index for the detection of hemodynamic changes with respect to time (6 months) and dose of radiation (15 Gy).


Asunto(s)
Encéfalo/irrigación sanguínea , Flujo Sanguíneo Regional/efectos de la radiación , Animales , Velocidad del Flujo Sanguíneo/efectos de la radiación , Volumen Sanguíneo/efectos de la radiación , Gasto Cardíaco/efectos de la radiación , Femenino , Ratas , Ratas Sprague-Dawley , Factores de Tiempo , Distribución Tisular , Rayos X
14.
J Clin Pathol ; 25(8): 705-7, 1972 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-5076805

RESUMEN

An investigation on human peripheral blood lymphocyte chromosomes in chronic active hepatitis was carried out. A higher percentage of chromatid and chromosome lesions was recorded in all patients studied as compared with control groups-normal individuals, healthy subjects who had suffered from acute viral hepatitis, patients with alcoholic liver disease, and patients with mechanical jaundice due to cancer. The possible origin of these abnormalities is discussed.


Asunto(s)
Aberraciones Cromosómicas/complicaciones , Hepatitis/genética , Adulto , Anciano , Consumo de Bebidas Alcohólicas , Aneuploidia , Cromátides , Trastornos de los Cromosomas , Enfermedad Crónica , Femenino , Hepatitis A/genética , Humanos , Ictericia/genética , Cirrosis Hepática/genética , Neoplasias Hepáticas/genética , Linfocitos/citología , Masculino , Persona de Mediana Edad , Poliploidía
15.
J Neurol ; 233(5): 267-70, 1986 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-3772406

RESUMEN

Previously undescribed morphological changes are reported in two cases of recent, non-septic cerebral embolism. These consisted of an arterial rupture involving the whole thickness of the wall. A direct tear by a sharp embolus seems unlikely but, as proposed by Villaret et al. in 1937, the arterial spasm may have occurred at the time of cerebral embolism. The embolic occlusion would prevent narrowing of the vascular lumen, so that focal spasm would rupture the arterial wall even at the level of a healthy and relatively thick media.


Asunto(s)
Embolia y Trombosis Intracraneal/complicaciones , Ataque Isquémico Transitorio/complicaciones , Arterias/patología , Encéfalo/irrigación sanguínea , Femenino , Humanos , Embolia y Trombosis Intracraneal/patología , Ataque Isquémico Transitorio/patología , Persona de Mediana Edad , Rotura Espontánea
16.
J Neurol ; 235(6): 352-4, 1988 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-2845007

RESUMEN

Sensory subacute neuropathies associated with sicca syndrome without any systemic involvement have been reported rarely. A sixth case is described with what appears to be the first report of muscle and nerve biopsy findings. The histological studies revealed axonal degeneration without vasculitis in the sural nerve, and a slight denervation process and a discrete myositis in the gastrocnemius muscle, reflecting a subtle systemic disorder. The clinical course of a long-standing subacute sensory neuropathy, the biopsy-documented axonal degeneration, and the neurophysiological findings suggest involvement of the dorsal root ganglia.


Asunto(s)
Neuronas Aferentes/ultraestructura , Enfermedades del Sistema Nervioso Periférico/etiología , Síndrome de Sjögren/complicaciones , Potenciales Evocados Somatosensoriales , Femenino , Humanos , Microscopía Electrónica , Persona de Mediana Edad , Degeneración Nerviosa , Conducción Nerviosa , Neuronas Aferentes/fisiopatología , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Síndrome de Sjögren/patología , Síndrome de Sjögren/fisiopatología
17.
J Neurol ; 233(1): 41-3, 1986 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-3512781

RESUMEN

A 30-year-old, previously healthy, non-addicted man presented with a chronic spinal meningitis complicated by arachnoiditis and spinal cord compression. Biopsy showed a chronic granulomatous leptomeningitis, in which some cells contained branching septate organisms that were immunostained with an antiserum to Aspergillus fumigatus. Precipitins to A. fumigatus were detected in cerebrospinal fluid (CSF), but not in blood, and aspergillus infection was apparently restricted to the leptomeninges. Clinically successful treatment led to the disappearance of CSF precipitins and oligoclonal bands.


Asunto(s)
Aracnoiditis/patología , Aspergilosis/patología , Meningitis/patología , Adulto , Aracnoides/patología , Aspergillus fumigatus/ultraestructura , Biopsia , Proteínas del Líquido Cefalorraquídeo/metabolismo , Humanos , Masculino , Meninges/patología , Compresión de la Médula Espinal/patología , Pulgar/lesiones , Heridas Penetrantes/complicaciones
18.
AJNR Am J Neuroradiol ; 13(1): 295-7, 1992.
Artículo en Inglés | MEDLINE | ID: mdl-1595463

RESUMEN

Two cases of tuberous sclerosis are presented. Extensive superficial occipital calcifications were found as classically described in Sturge-Weber syndrome. Other radiologic signs of tuberous sclerosis, such as subependymal calcifications in both patients and surgically proved giant cell astrocytoma in one patient, were present. At pathologic examination, the calcifications appeared to be located in extensive cortical tubers.


Asunto(s)
Encefalopatías/diagnóstico por imagen , Calcinosis/diagnóstico por imagen , Síndrome de Sturge-Weber/diagnóstico por imagen , Esclerosis Tuberosa/congénito , Diagnóstico Diferencial , Femenino , Humanos , Recién Nacido , Masculino , Tomografía Computarizada por Rayos X , Esclerosis Tuberosa/diagnóstico por imagen , Esclerosis Tuberosa/patología
19.
J Neurosurg ; 83(5): 919-22, 1995 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-7472566

RESUMEN

Nerve rhabdomyomas are exceedingly rare benign tumors of the peripheral nerves consisting of well-differentiated striated muscle fibers admixed with parental nerve fibers. Only one case of intracranial nerve rhabdomyoma has been described, which affected the trigeminal nerve. This report presents the detailed neuropathological description of a nerve rhabdomyoma arising in the schwannian portion of the facial nerve root in a 41-year-old Caucasian man. The nerve fibers were arranged chaotically as in a traumatic neuroma. Because of the intimate intermingling of this slow-growing tumor with the parental nerve fibers, complete excision should be avoided.


Asunto(s)
Neoplasias Encefálicas/cirugía , Neoplasias de los Nervios Craneales/cirugía , Nervio Facial , Rabdomioma/cirugía , Adulto , Neoplasias Encefálicas/patología , Neoplasias de los Nervios Craneales/patología , Humanos , Masculino , Rabdomioma/patología
20.
J Neurosurg ; 88(1): 129-32, 1998 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-9420085

RESUMEN

Angiolipomas (ALs) are hamartomas composed of abnormally differentiated vessels and mature adipose tissue. Although they are most commonly found in peripheral tissues, ALs sometimes grow in the spinal epidural space. Intracranial ALs (ICALs) are rare: only seven cases have been reported in the literature. The authors describe the case of a 70-year-old woman who presented with ocular symptoms from a clinically and radiologically progressing parasellar ICAL. The radiological as well as the microsurgical findings are illustrated and compared with the seven previously published cases. The most frequent location of ALs is the skull base, especially the parasellar region. Other ICALs were diagnosed as components of cerebral arteriovenous malformations and were not symptomatic by themselves. Neuroradiological studies of ICALs usually demonstrate the characteristics of both adipose and vascular tissues. However, a review of the literature shows that the diagnosis had not been suspected preoperatively in any of the cases. Operative descriptions emphasize that most neurosurgeons were caught off guard by the profuse bleeding and the unusual relationship of this unexpected lesion to the cavernous sinus, so that removal was rarely complete. The authors conclude that preoperative diagnosis of ICALs is achievable based on magnetic resonance analysis, which should help optimize the microsurgical management of these lesions.


Asunto(s)
Angiolipoma/diagnóstico , Angiolipoma/cirugía , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirugía , Anciano , Angiolipoma/complicaciones , Angiolipoma/patología , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/patología , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Microcirugia , Tomografía Computarizada por Rayos X
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