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Biomedical subjects

S Pelc

Publications and source records attributed to S Pelc.

At least 19 recordsLinked to original sources

S-100 protein expression in satellite and Schwann cells in neuroblastoma. An immunohistochemical and ultrastructural study.

Immunohistochemical evidence has recently been provided that in the normal adrenal medulla as well as in autonomic ganglia, satellite cells and Schwann cells react with S-100 protein antiserum. In the light of these data, we investigated primary peripheral neuroblastoma and ganglioneuroblastoma to determine firstly whether both cell populations actually exist in the malignancies, using the definite criteria of electron microscopy for their identification, and secondly whether they express S-100 protein using on immunohistochemical technique and light microscopy. The results indicate that in both neuroblastoma variants, satellite and Schwann cells are present and specifically express the S-100 antigen.

Adrenal Gland Neoplasms

Immunohistochemical distribution of glial fibrillary acidic protein, neurofilament polypeptides and neuronal specific enolase in the human cerebellum.

The human normal cerebellar cortex has been investigated using paraffin-embedded sections and a panel of antibodies against GFAP, NSE, NF 70 Kd and 200 Kd triplet proteins. The study has shown that: antiserum to GFAP labelled all and only astroglial elements; antibodies to NSE, NF 70 Kd and 200 Kd stained the perikaryon of all neurons, with two noticeable exceptions: Purkinje cells, which remained unresponsive to all 3 reagents, and basket cells which only became labelled with the NSE antiserum; the staining of dendrites and axons was exquisitely selective with both the NF and the NSE antibodies, each of which displayed unique binding patterns. These immunohistochemical features, first to be described in man, are compared with those so far reported in rodents.

Astrocytes

Peroneal motor nerve conduction velocity in diabetic children and adolescents. Relationships to metabolic control, HLA-DR antigens, retinopathy, and EEG.

To investigate incipient diabetic neuropathy, peroneal motor nerve conduction velocity (PMNCV) was measured in 61 diabetic children and adolescents whose type 1 diabetes became clinically apparent before the age of 14 years. PMNCV in diabetic patients (48.3 +/- 5.6 m/s) was significantly lower than in controls (56.5 +/- 5.5 m/s), 23 diabetics (36%) having a value more than 2 SD below the mean for normals. There was a highly significant negative correlation between PMNCV and HbA1 levels concomitant with PMNCV measurement or mean annual HbA1 concentrations preceding PMNCV. The relationship between PMNCV and the clinical score of diabetic control since the onset of the disease was also significant. Age, duration of diabetes and HLR-DR antigens were unrelated to PMNCV. EEG abnormalities and retinopathy, whose pathogenesis is different, were not necessarily associated with subclinical neuropathy. Being easy and sensitive, PMNCV determination provides the paediatric diabetologist and the patient himself with an important motivation to improve diabetic control.

Adolescent

The Mohr syndrome: are there two variants?

Two female siblings with the oral-facial-digital type II syndrome, or Mohr syndrome, presented an associated brain malformation: the Dandy-Walker syndrome. Up till now, patients with the Mohr syndrome have been considered to be free of mental deficits and/or brain defects. After reviewing the literature and considering the current criteria for OFD type II syndrome, other possible cases of Mohr syndrome associated with brain abnormality are discussed.

Abnormalities, Multiple

Burkitt lymphoma. EBV nucleoid-like structures and lack of capsids in lymphoblasts.

Burkitt lymphoma biopsies and the CSF malignancy have been investigated ultrastructurally. The tumor cells were found to contain vermicellar bodies formed by rod-like structures similar to those suggested to represent HSV nucleoids in HSV encephalities. But, unlike the HSV disease and BL in culture, no nucleocapsids nor empty capsids were detected. It is suggested that the rods in the Burkitt lymphoblasts may well represent EBV nucleoids and that the absence of capsids may be another characteristic of the EBV in BL in situ.

Antigens, Viral

Burkitt's lymphoma--a correlated light and electron microscopic study on the malignancy in the CSF.

In a sporadic case of primary maxillar Burkitt's lymphoma and secondary spinal epidural dissemination, the massive malignancy in the CSF was investigated by light and electron microscopy. At both levels the CSF tumor cells were strikingly similar, with the undifferentiated lymphoblasts that characterize Burkitt's lymphoma in tissue. However, the starry-sky appearance in the CSF was produced more often by degenerating malignant cells than by histiocytes as classically described in tissue with Burkitt's lymphoma.

Burkitt Lymphoma

Immunoblastic sarcoma of the B-cell type in the CSF. Light- and electronmicroscopic study of the malignant cells and unusual macrophages.

In a patient with systemic and nervous system IBS, the CSF cells were investigated by light and electron microscopy. An IBS of the B-cell type was identified in the CSF which proved, at both morphological levels, to be identical to that recorded in IBS in lymphoid tissue with regard to the malignant cell types and the absence of surface specialization. In contrast, unusual macrophages were observed in the CSF instead of the reticular elements associated with IBS in lymph nodes which present desmosomes. They demonstrated both phagocytosis and emperipolesis and also displayed autodesmosomes. The CSF phagocytes are discussed in relationship with the phagocytes described in the brain with malignant lymphoma.

B-Lymphocytes

CSF cells in tuberculous meningitis. Humoral and cellular immune response.

In 10 patients with tuberculous meningitis both T and B lymphocyte activation was observed in the CSF from clinical onset of disease. During the course of the illness, the humoral response remained most marked, disappearing by 2 months, while the cellular immune response continued for about another 3 months. This contrasts with acute viral meningitis, in which both responses cease within 1-2 weeks of disease.

Adolescent

Tubular arrays in cerebrospinal fluid cells: their location within smooth endoplasmic reticulum cisternae.

Unique ultrastructural formations termed tubular arrays have been reported in a variety of renal diseases, viral infections, malignant tumors, and other circumstances. This report describes identical structures in monocytes in the cerebrospinal fluid in a case of pneumococcal meningitis. The occurrence, previously unreported, of tubular arrays in this infection and this kind of cell supports the growing view that tubular arrays probably do not represent viral components, but rather a general reactive phenomenon of the cell. In addition, ultrastructural findings are presented suggesting that tubular arrays appear to be located in the cisternae of the endoplasmic reticulum with major involvement of its smooth compartment.

Cerebrospinal Fluid

CSF cytology of acute viral meningitis and meningoencephalitis.

The CSF of 12 patients with acute meningitis and meningoencephalitis of viral nature (proven in 10 and probably in 2) was investigated by light microscopy. In early CSF, 1--31% reactive lymphocytes and 1--6% plasma cells were found consistently. In addition, various lymphoid cell features of marked immune activation were disclosed in association: cell polymorphism, normal-appearing mitosis and binucleation, abnormal nuclear lobulation and even partition, as well as Russell bodies, morular appearance and clasmatosis in plasma cells. The CSF findings are discussed with respect to those described in tuberculous meningitis and multiple sclerosis and distinguishing features are suggested to occur in early CSF of viral meningitis.

Acute Disease

Fundamental aspects of the developing brain: synapse formation.

The phenomena presiding over synaptogenesis in the developing brain, notably death of neurons and the nerve growth factor are described as well as the neurobiological and ultrastructural features of synapse maturation. Synapse and connection specificity in the brain versus plasticity are given consideration. Infantile neuroaxonal dystrophy is suggested as a possible example of defective synaptogenesis.

Brain

EEG abnormalities in diabetic children: influence of hypoglycemia and vascular complications.

The effect of diabetic control upon EEG has seldom been studied. In the present investigation, a significant positive correlation between EEG abnormalities and degree of diabetic control was found, but no definite increase was noted in relation to the duration of diabetes. Eighty per cent of our patients having more than 5 severe hypoglycemic attacks showed evidence of abnormal EEG, suggesting that hypoglycemic coma or convulsions are closely related to EEG abnormalities (minor hypoglycemic episodes had no effect on the EEG). With the sensitive technique of fluorescein angiography, we demonstrated a clear correlation between incipient retinal angiopathy and EEG abnormalities. The factors that most positively relate to pathologic electrocerebral (EEG) activity in diabetic children are frequent and severe hypoglycemic attacks, comas and/or convulsions, and vascular changes in the retina.

Adolescent

Farber's disease as a ceramidosis: clinical, radiological and biochemical aspects.

A case of Farber's disease associated with athyreosis is reported in a Belgian infant born from consanguineous parents. A detailed clinical observation made from the early onset of symptoms until death of the patient at age of 22 months, together with radiological, morphological and biochemical data confirmed the diagnosis of Farber's disease and its specific storage process. Cultured fibroblast studies disclosed an abnormal catabolism of ceramides, presumably related to the deficiency in lysosomal ceramidase. Family history confirms that the disease is inherited as an autosomal recessive trait.

Ceramides

Temporal lobe oligodendroglioma developing from infancy into adulthood.

Clinical, radiological, surgical, and histological features are reported for a patient with a temporal lobe oligodendroglioma that had developed in infancy and was diagnosed and partially resected at age 10. The patient is presently 24 years old and still well. To the best of our knowledge this is the first report of an oligodendroglioma with probable onset at 5 months of life and satisfactory course over 24 years.

Adolescent

Subdural empyema.

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Brain Abscess

[Happy-puppet syndrome].

Six cases of this syndrome previously reported in Great Britain and in Canada are reviewed in relation to a personal case, a 17-month-old boy. The syndrome's main features are microbrachycephaly and peculiar face, mental deficiency with euphoria and laughter, ataxia and epilepsy consisting in infantile spasms and grand-mal attacks. Though its precise nature and cause still remain unknown, the syndrome seems neither familial nor progressive.

Electroencephalography