RESUMO
UNLABELLED: We report on two Turkish children who presented with progressive airway obstruction. Open lung biopsy revealed follicular bronchiolitis. The children were treated with systemic steroids and various topical medications. Whereas the respiratory situation of patient 1 required immunosuppressive therapy, the condition of patient 2 stabilised without systemic medication. CONCLUSION: Diagnosis of follicular bronchiolitis should be considered when children present with recurrent respiratory tract infections, progressive dyspnoea, and chronic bronchial obstruction. Children in whom follicular bronchiolitis is suspected should undergo open lung biopsy for confirmation of diagnosis.
Assuntos
Bronquite/patologia , Biópsia , Bronquite/tratamento farmacológico , Bronquite/imunologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , TurquiaRESUMO
The distribution of type VII collagen was examined in the normal human nervous system, in brain tumour biopsies and in glioma cell lines by immunohistochemistry and western blotting. In normal tissue, positivity was observed beneath choroid plexus epithelial cells and around pineal gland and pituitary gland cell nests, while other brain regions and peripheral nerves were negative. Expression was preserved in most related tumours (choroid plexus papilloma, pineoblastoma, pituitary adenoma). Scattered abnormal vessels showed neo-expression of type VII collagen in about half of the astrocytic and ependymal tumours. Glioma cells in situ were consistently negative for type VII collagen, whereas the glioblastoma cell lines were positive. Our results suggest that anchoring fibrils or at least epitopes of their major structural component are present in normal and pathological cerebral structures, indicating a unique distribution of type VII collagen in the nervous system.
Assuntos
Química Encefálica , Neoplasias Encefálicas/química , Colágeno/análise , Adulto , Idoso , Anticorpos Monoclonais/imunologia , Western Blotting , Humanos , Técnicas Imunoenzimáticas , Nervo Sural/químicaRESUMO
In patients with intravascular lymphomatosis (IL) a broad spectrum of neuro-psychiatric disorders including dementia, focal neurological signs and seizures has been reported. Clinical diagnosis is difficult since neuroimaging findings are nonspecific. The clinical histories, biopsy and autopsy findings of three patients with IL, one with brain biopsy, are described. Two of them presented with rapidly progressive, fluctuating dementia. The third patient suffered from seizures followed by aphasia. Histology revealed large-cell lymphoma of the B cell type. The prognosis of IL, in general, is poor.
Assuntos
Neoplasias Encefálicas/patologia , Encéfalo/patologia , Linfoma de Células B/patologia , Linfoma Difuso de Grandes Células B/patologia , Idoso , Idoso de 80 Anos ou mais , Encéfalo/irrigação sanguínea , Neoplasias Encefálicas/irrigação sanguínea , Neoplasias Encefálicas/complicações , Demência/etiologia , Feminino , Humanos , Linfoma de Células B/complicações , Linfoma Difuso de Grandes Células B/complicações , Masculino , Pessoa de Meia-IdadeRESUMO
A 45-year-old male developed a transverse lesion of the spinal cord two years after resection of a pulmonary adenocarcinoma. Laminectomy revealed a spindle cell tumor, immunohistochemically considered as leiomyosarcoma. Autopsy disclosed a spindle cell variant of an adenocarcinoma of the lung. The present report demonstrates that spindle cell formations can be found not only in squamous cell carcinomas but also in adenocarcinomas of the lung, and that metastases may be composed of this spindle cell component only.
Assuntos
Adenocarcinoma/patologia , Carcinoma/secundário , Neoplasias Pulmonares/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias da Medula Espinal/secundário , Carcinoma/química , Proteínas do Citoesqueleto/análise , Humanos , Leiomiossarcoma/patologia , Masculino , Pessoa de Meia-Idade , Neoplasias da Medula Espinal/químicaRESUMO
Based on a consecutive autopsy series of 184 patients with AIDS, a critical review of the pathology of the central nervous system (CNS) is given. The lesions can be divided into three groups: 1. opportunistic/non-opportunistic infections, 2. changes due to the human immunodeficiency virus (HIV), and 3. neoplasms. The frequency and morphology of CNS lesions in our cohort are compared with those in other series. Marked lesions of the CNS were found in 111 patients (60%), while mild/nonspecific changes were seen in 52 cases (28%). Toxoplasmosis (23%) was the most frequent CNS infection, followed by cytomegalovirus (17%), and papovirus (5%). HIV giant cell encephalitis, HIV leukoencephalopathy, vacuolar myelopathy and leukoencephalopathy were observed in 11%. Primary CNS lymphomas were present in 6%, while secondary involvement of the CNS in systemic lymphomas was seen in only two cases (1%). Lesions due to CNS infections in patients with AIDS often show atypical patterns, and frequently, there are multiple infections with simultaneous involvement of the CNS by lesions of different etiology. The present study confirms the frequent involvement of the CNS in AIDS, although there are differences in the incidence and pattern of lesions related to geographic and/or demographic factors.
Assuntos
Complexo AIDS Demência/patologia , Infecções Oportunistas Relacionadas com a AIDS/patologia , Neoplasias Encefálicas/patologia , Soropositividade para HIV/patologia , Áustria , Encéfalo/patologia , Humanos , Linfoma de Células B/patologiaRESUMO
In serum and cerebrospinal fluid of 6 out of 15 patients with small cell lung cancer circulating antineuronal antibodies could be detected by indirect immunofluorescence. None of the patients showed signs of a neurological paraneoplastic syndrome, usually associated with the presence of these antibodies. On sections of frontal brain, cerebellum, dorsal root ganglia and peripheral nerve, obtained at autopsy, a direct immunofluorescence test was performed. Only in antibody-positive individuals were membrane-bound immunoglobulin deposits detected on neurons of dorsal root ganglia and Purkinje cells. The present study showed that circulating antineuronal antibodies reach the central and peripheral nervous system in vivo. These findings support the theory that these antibodies might be directly involved in the pathogenesis of neurological paraneoplastic syndromes.
Assuntos
Autoanticorpos/análise , Carcinoma de Células Pequenas/imunologia , Neoplasias Pulmonares/imunologia , Neurônios/imunologia , Química Encefálica , Carcinoma de Células Pequenas/sangue , Carcinoma de Células Pequenas/líquido cefalorraquidiano , Cerebelo/química , Imunofluorescência , Gânglios Espinais/química , Humanos , Neoplasias Pulmonares/sangue , Neoplasias Pulmonares/líquido cefalorraquidiano , Nervos Periféricos/química , Células de Purkinje/químicaAssuntos
Autoanticorpos/análise , Biomarcadores Tumorais/análise , Carcinoma de Células Pequenas/imunologia , Carcinoma de Células Pequenas/patologia , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/patologia , Neurônios/imunologia , Síndromes Paraneoplásicas/imunologia , Síndromes Paraneoplásicas/patologia , Adulto , Idoso , Carcinoma de Células Pequenas/mortalidade , Feminino , Humanos , Pulmão/patologia , Neoplasias Pulmonares/mortalidade , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Síndromes Paraneoplásicas/mortalidade , Taxa de SobrevidaRESUMO
The detection of antineuronal antibodies against cytoplasm and nuclei of Purkinje cells is considered to be an essential factor in the diagnosis of neurologic paraneoplastic syndromes. Published data rely on different immunological methods and relatively small numbers of patients. The clinical relevance of this phenomenon is not clear from the literature. A positive correlation between antibody detection and the appearance of neurologic paraneoplastic syndromes has been reported, but this finding has been questioned in critical reviews.
Assuntos
Autoanticorpos/análise , Autoantígenos/imunologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/imunologia , Proteínas de Bactérias/imunologia , Proteínas de Ligação a DNA/imunologia , Proteínas de Neoplasias/imunologia , Proteínas do Tecido Nervoso , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/imunologia , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/imunologia , Imunofluorescência , Humanos , Técnicas ImunoenzimáticasRESUMO
The expression of type VIII collagen was examined in the normal and diseased human brain. Focal immunoreactivity was seen in histologically abnormal vessels of all four angiomas and 40 of 52 brain tumours (gliomas, meningiomas and schwannomas). An extended staining pattern, as well as a punctate distribution, was frequently observed in affected vessels. Staining was not apparent in nine normal brains and in 15 pathologic brains showing various cerebrovascular abnormalities, including Alzheimer's, Leigh's and Wernicke's diseases. Immunoblotting of glioblastomas revealed two bands at 56 kD and 67 kD which were also present at low levels in normal frontal cortex. The extracellular distribution of type VIII collagen was different from that of the other collagen types which have been described in brain and resembles patterns of expression described for certain tissues during mammalian embryogenesis (Kapoor et al., 1988). Our results provide additional evidence for the participation of type VIII collagen in some types of angiogenesis.
Assuntos
Química Encefálica , Encefalopatias/metabolismo , Neoplasias Encefálicas/química , Colágeno/análise , Adulto , Idoso , Colágeno/imunologia , Humanos , Pessoa de Meia-IdadeRESUMO
An 87-year-old male presented with rapidly progressive dementia, disorder of consciousness and myoclonus, suggesting Creutzfeldt-Jakob disease, but with nonspecific EEG and negative CT findings. Autopsy revealed a malignant angiotropic lymphoma with accumulation of tumor cells within small vessels of the brain and visceral organs. Immunohistochemistry disclosed a large cell lymphoma of B cell type.
Assuntos
Neoplasias Encefálicas/patologia , Demência/diagnóstico , Hemangioendotelioma/patologia , Idoso , Idoso de 80 Anos ou mais , Cadáver , Síndrome de Creutzfeldt-Jakob/diagnóstico , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Linfoma de Células B/patologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Vísceras/irrigação sanguíneaRESUMO
Acute polyradiculitis ("acute inflammatory demyelinating polyradiculitis--AIDP" or the Landry-Guillain-Barré syndrome--GBS) is an acute inflammatory disease of the peripheral nervous system. Despite extremely severe courses and complications, the prognosis is favourable for the majority of patients. The typical clinical course is featured by non-characteristic sensory symptoms following an infection, with ensuing ascending motor signs and symptoms which, in 80% of the patients, reach a maximum within two weeks. The legs are usually involved before the arms. About 50% of the patients show involvement of cranial nerves. In the acute phase, respiratory insufficiency and autonomous dysfunctions may occur. For diagnosis, predominantly clinical criteria are used according to the criteria summarized by Asbury.
Assuntos
Polirradiculoneuropatia/diagnóstico , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Doenças dos Nervos Cranianos/fisiopatologia , Diagnóstico Diferencial , Humanos , Dor/fisiopatologia , Paralisia/fisiopatologia , Polirradiculoneuropatia/fisiopatologia , Prognóstico , SensaçãoRESUMO
The appearance of circulating antineuronal antibodies (CANA) in patients with malignant tumors has been correlated with the occurrence of paraneoplastic neurological syndromes. However, the effect of CANA on the peripheral nervous system is poorly understood. The reactivity of sera from CANA-positive and -negative patients were investigated on cryostat sections of peripheral nerves and skeletal muscle, and on nerve tease-fiber preparations. Only CANA-positive sera showed staining of Schwann cell nuclei on cryostat sections, whereas nerve tease-fiber preparations and sections of skeletal muscle remained negative. Positive direct immunofluorescence of small cell lung cancer (SCLS) cells was confined to CANA-positive patients only. These findings suggest the existence of a common antigen between SCLC and Schwann cells.