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Biomedical subjects

D Gambarelli

Publications and source records attributed to D Gambarelli.

At least 91 records · Page 5Linked to original sources

Apomorphine-induced circling behaviour in hamsters following unilateral injection of scrapie gent in the striatum.

Twenty golden hamsters received a microinjection of scrapie agent into the left striatum. At different times after inoculation animals were injected intraperitoneally with apomorphine, a direct dopamine receptor agonist. Two types of effects developed simultaneously, starting at about 80 days after infection. First, apomorphine induced a rotational behaviour which showed a progressive destruction of the striatal neurones at the site of injection. This suggest a local spread of scrapie agent by cell to cell transfer in the striatum. Secondly, the clinical signs of scrapie developed, indicating a more widespread distribution of agent throughout the brain.

Animals↗

Germinomas of the brain. Light and electron microscopic study. A report of seven cases.

The authors report seven cases of intracerebral germinomas studied on autopsy and/or biopsy material. Electron microscopy was done in five cases. Tumors were located on the middle line of the brain (pineal area, suprasellar region, third ventricle). Light microscopic features were stereotyped with PAS + round tumor cells and inflammatory mononuclear cells. By electron microscopy, fenestrated nucleoli, glycogen storage and annulate lamellae were always found, as well as a striking macrophagic activity of histiocytes. This immune reaction is supposed to contribute to the relatively favorable prognosis of these original brain tumors.

Adolescent↗

Anatomical study of experimental kuru in the Rhesus monkey.

The authors report the morphology and topography of CNS lesions in experimental kuru (third passage) in the rhesus monkey. The main lesions encountered are spongiosis of the neuropile with neuronal vacuolization, moderate astrocytic gliosis, and slight neuronal depopulation. In all cases, lesions predominated in the grey matter of the cerebral hemispheres (cortex, neostriatum). Lesions were minimal in cerebellum and brain stem. The authors compare their results with these observed species of Primates (chimpanzee, spider monkey, marmoset, squirrel monkey).

Animals↗

Melanocytic involvement in giant axonal neuropathy.

The skin of a patient with giant axonal neuropathy was studied by light and electron microscopy. Enlarged melanocytes protruded into the papillary dermis. An increased number of microfilaments (100 A), which accumulated in rounded or irregular clusters was the main pathological feature. Melanosomes and melanisation were normal. A similar increase of microfilaments was seen in axons and Schwann cells of peripheral nerves, fibroblasts and endothelial cells. This suggests that this metabolic, probably inborn and genetic disorder, does not only affect the nervous system but also the whole microfilament system (cytoskeleton).

Axons↗

[Primary brain lymphomas. Anatomoclinical, ultrastructural and immunocytochemical study about 23 cases (author's transl)].

Twenty-three cases of primary brain lymphomas were morphologically studied. Electron microscopy was done in twelve cases and immunoperoxidase reactions for light chains of immunoglobulins in ten cases. The patients were fourteen males and nine females. Except two children, the mean age was 50 years. Clinical features and pre-operative investigations lacked any specificity. Post-operative radiotherapy could be performed in thirteen patients, eleven of them being still alive with a follow-up varying from 2,5 months to 4,5 years. All the other patients persued fulminating course (max: 4,5 months). Tumors were preferentially located in frontal and temporal lobes. On gross examination, well limited tumors were more frequent than ill-defined or purely infiltrating tumors. Microscopically, all the lymphomas were of the diffuse type. Perivascular clusters of lymphoma cells, meningeal and parenchymal infiltrates and sub-ependymal sprouts were found in the peritumoral areas. The lymphomas were cytologically characterized according to the modified Kiel classification (Lennert et al, 1975) using light and electron microscopic data. So were identified thirteen lymphoblastic lymphomas (with one convoluted-cell type), one centroblastic lymphoma, eight immunoblastic lymphomas (two with plasmacytic differentiation). Light chains of human immunoglobulins were immunocytologically demonstrated in six of twelve tumors tested, all the positive cases corresponding to immunoblastic lymphomas. A unique case of primary meningeal Hodgkin's disease was found. These results were compared with the rare previous reports associating these different morphological investigations.

Adolescent↗

The retina in Lafora disease: light and electron microscopy.

Lafora bodies are described in the retina of a 16 year old female who died five years after the onset of a typical familial progressive myoclonus epilepsy which was diagnosed as Lafora disease by brain biopsy and by autopsy findings. The patient was the offspring of consanguinous parents who had three affected siblings out of nine. The fine structure and distribution of Lafora bodies, which represent a specific non-lysosomal cell storage disorder, is reported for the first time in the human retina. The nature of the abnormal material in the Lafora bodies, which are identical to those present in the brain, heart and liver tissues in the same patient and in her brother, is discussed according to their iodide spectrum. In this respect, Lafora disease might be related to the inborn errors of carbohydrate metabolism and its relationship with Type IV Glycogenosis (Anderson's disease) must be verified by further investigations.

Adolescent↗

Macrophagic activity in intracerebral germinoma: ultrastructural study of a case.

This report presents the ultrastructural study of a germinoma of the third ventricle occurring in a 13 year old boy. The electron microscopic data showed similarities linking this tumor to gonadal and mediastinal germinomas and emphasized the exceptional glycogenic storage in tumor and stroma cells. Another morphological analogy was the intense macrophagic activity that led to tumor cell phagocytosis. The authors discuss the meaning of such an immune response, which is usually not observed in cerebral tumors.

Adolescent↗

Experimental kuru in the rhesus monkey. A clinical study.

Serial neurologic examinations of six rhesus monkeys inoculated with a strain of kuru revealed complex clinical signs falling into two distinct syndromes. Experimental kuru in the rhesus monkey is different from the disease in man and to a lesser extent in the chimpanzee. Two factors are proposed to account for these observations: modification of the strain, and specific host characteristics.

Animals↗

Experimental kuru in the rhesus monkey: a study of EEG modifications in the waking state and during sleep.

EEG patterns recorded in the waking state and during sleep were studied in 6 rhesus monkeys inoculated with a strain of Kuru previously passaged in rhesus monkey (ENAGE strain, rhesus L6 56). The onset of the disease was confirmed by the appearance of various clinical signs in 4 monkeys 15 months after inoculation. At the 16th month, the first EEG modifications appeared during sleep, which became lighter. The waking EEG was abnormal during the mature phase of the disease; it was characterized by slow anomalies and scattered spikes. The sleep EEG still presented 3 stages of Slow Wave Sleep which, however, were totally unlike the physiological stages. REM sleep rapidly disappeared, as did the cyclic organization pattern. Irritative phenomena became very significant and, in particular, very frequent 'tonic seizures' were observed. Experimental Kuru thus appears, in the rhesus monkey, as an epileptogenic encephalopathy, which is differentiated from both the human disease and the experimental disease in the chimpanzee.

Animals↗

Appearance of EEG changes reminiscent of a secondary generalized epilepsy in a rhesus monkey inoculated with a strain of kuru.

In a group of rhesus monkeys (Macaca mulatta) inoculated intracerebrally and intravenously with a strain (Enage strain rhesus L6 56) of kuru already passaged in rhesus monkeys, 1 monkey presented the typical EEG pattern of epileptogenic encephalopathy reminiscent of the Lennox-Gastaut syndrome. This observation provides no direct evidence for the viral origin of epilepsies of this type. It does, however, show that it is possible to induce an epileptogenic encephalopathy by an unconventional infectious agent.

Animals↗

Intracranial supratentorial cysts in children excluding tumor and parasitic cysts.

The intracranial, liquid-containing cysts in children (excluding tumor and parasitic cysts) are relatively frequent in neurosurgical practice. They raise several problems about their nosology, etiology, clinical and radiological diagnosis, and treatment which are analyzed in a series of 36 cases of supratentorial cysts. The most frequent clinical feature is increased head circumference (22 cases) followed by epileptic fits (18 cases), as well as mental and motor retardation (19 cases). Fundi were found abnormal in only one case out of three. Electroencephalogram was abnormal in almost every case, showing either spikes, spikes and waves or localized slow waves, or an asymmetric depression of the electrical activity. Radiological investigation is essential for diagnosis. Plain radiographs of the skull may show an asymmetry (11 cases). Carotid angiogram and pneumoencephalography give the diagnosis of the lesion without accuracy as to the histology. Computerized axial tomography shows the position of the liquid cavity within the head. The surgical approach (simple shunting [6], direct approach [19], or both [7]) should be carefully considered according to anatomical variety and age of the child. An anatomical classification is proposed, based on the radiological, surgical and pathological findings. Three types of cysts are defined: external cysts (cortical or extracortical) which may or may not be communicating with the ventrioles or the subarachnoid space; internal cysts which again may or may not be communicating; and the corticoventricular cysts. The ultrastructural study represents further progress in the attempt to define the exact anatomical type.

Brain Diseases↗

[Craniostenoses].

Explore the source record for details and available documents.

Age Factors↗

Giant axonal neuropathy. Involvement of peripheral nerve, myenteric plexus and extra-neuronal area.

A case of giant axonal neuropathy in a 8 years old child is reported by light and electron microscopy. Clinically, this case is strikingly similar to the rare previous reports and characterized by a distal neuropathy, CNS symptoms and tightly curled hair. Giant axons were found in the sural nerve but had been absent at the onset of the illness. An increase in the number of neurofilaments was found in the axons and neurons of the myenteric plexus. The number of microfilaments was also increased in various types of cells namely Schwann and endothelial cells and fibroblasts: This suggests that the metabolic disorder, probably inborn and genetic, does not only affect the nervous system.

Axons↗

Granular cell myoblastoma.

Electron microscopic observations in three cases of granular cell myoblastoma revealed that this tumor is made up of two types of cells. 1) Granular cells grouped into nests, surrounded by a basement membrane, and displaying characteristic inclusions. Numerous processes were observed close to these cells, showing filamentous and microtubular components lying parallel to their axes. 2) Satellite fibroblasts which included angulated bodies. The relationship between these two types of cells is discussed.

Basement Membrane↗