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Biomedical subjects

F Gullotta

Publications and source records attributed to F Gullotta.

At least 127 records · Page 7Linked to original sources

Cytopathology of an unusual case of centronuclear myopathy. Light- and electron-microscopic investigations.

The results of enzyme-histochemical and electron-microscopic investigations of a patient with centronuclear myopathy combined with targets, cores and prevalence of type-I fibers are presented. The patient had suffered from perinatal hypoxic brain damage, causing enlargement of the ventricular system, slight frontal atrophy and right-sided hemiparesis. Morphologic investigation of muscle fibers demonstrated dynamic activity of an autophagic system represented by a distinct increase of Golgi components adhering to the nuclear surface. By analysing the lytic events ad their sequences, a postnatal secondary migration of subsarcolemmal nuclei to the central regions of muscle fibers can be postulated.

Adenosine Triphosphatases↗

[Diphenylhydantoin, epilepsy, cerebellar atrophy--histological and electron microscope examinations (author's transl)].

Neuropathological investigations of CNS of two epileptic patients (a man and a woman), who had been treated over years with diphenylhydantoin, disclosed in both cases severe cerebellar atrophy, with almost complete loss of Purkinje cells. Hypoxic tissue alterations were found neither in cerebellum nor in cerebrum. Ultrastructural examination of formalin fixed tissue revealed in cytoplasm of degenerating Purkinje cells multilamellar structures similar to those reported by other authors, presumably consisting of proliferated cisternae of endoplasmic reticulum, degenerating mitochondria and "multilamellar cytoplasmic bodies". However, these findings are very probably not specific; they were detected also in Purkinje cells of a non-epileptic patient of the same age-group, suddenly deceased by car accident. Reversible cerebellar dysfunction is a common manifestation of acute phenytoin toxicity. However, further factors can influence the action of DPH, leading to chronic intoxication and irreversible cerebellar damage. A review of these factors is given. The periodic monitoring of serum DPH-concentration is emphasized.

Adult↗

Clinical and morphological investigations on ependymomas and their tissue cultures.

A morphological investigation was carried out on 56 ependymomas cultivated in vitro as short-term cultures in roller tubes. The tumours had been histologically classified as cellular and fibrillary ependymomas, subependymomas, myxopapillary and malignant ependymomas (Table 1). A very good growth was detected in 32 cases, most of them being cellular and malignant ependymomas (Table 2). The prevailing growth pattern was epithelial in type, i.e. proliferating cells forming a carpet. In some cases, in the first stages of growth elongated bipolar cells did appear, but they evolved later as flattened epithelial elements. In four cases, a mixed proliferation of piloid astrocytes and ependymal cells was seen; these tumours were regarded as mixed gliomas. In 46 cases an exact evaluation of the history was possible. Although no correlation could be found between histology and survival time (Table 4), the longest survival was observed in spinal tumours (Table 3). Tumours in children had a slightly worse prognosis in comparison with adults (Table 5). A radical removal of the tumour was generally followed by a longer survival time (Table 6), although the operative procedure employed did not seem to influence the development of recurrences (Table 7).

Adult↗

Giant cell gliomas of the temporal lobe.

Giant cell tumours of the brain are commonly a) monstrocellular sarcomas, b) giant cell glioblastomas or gliosarcomas, or c) "giant cell gliomas", i.e. gliomas characterized by many atypical, giant-sized cells with monstrous nuclei. This last group is commonly found in the temporal region and should not be mistaken for a) and b), because, in spite of their "malignant" appearance, these tumours can present benign biological behaviour. Three such cases are described. In two cases the patients received postoperatively neither radio- nor chemotherapy: seven and three years respectively after operation no signs of recurrences were present. A third patient died within two years after operation. However, his tumour had histological signs of malignancy. The importance of exact histological diagnosis is stressed. This prevents misinterpretations in estimating the efficacy of postoperative therapy.

Adult↗

Immunoelectrophoresis in the diagnosis of neuroectodermal and mesodermal intracranial tumours, especially those of the posterior fossa.

Immunoelectrophoresis of extracts of 200 intracranial tumours against rabbit anti-glioblastoma serum gave positive results (= precipitation) in all cases of tumours of neuroectodermal origin such as glioblastoma, astrocytoma, oligodendroglioma, ependymoma, neurinoma, and spongioblastoma. No immunoelectrophoretic precipitation was seen for any of the tumours of mesenchymal origin, for instance meningioma and metastases of cancer. On the basis of these findings, immunoelectrophoresis is considered to be a reliable method for differentiation between tumour tissue of neuroectodermal and non-neuroectodermal origin. Among the 41 posterior fossa tumours some unusual observations were made. Cerebellar angioblastoma (Lindau tumour) showed an atypically located precipitation line, which for the present is interpreted as an immunological reaction to vascular wall tissue. Furthermore, among the group of so-called medulloblastomas, two subgroups were distinguished on the basis of three parameters. The first of these subgroups comprises tumours whose immunoelectrophoretic pattern resembles that of gliomas, which are histologically characterized by neuroectodermal structures and which occur in younger children (5--10 years). The tumours of the second subgroup, which do not show this neuroectodermal immunoelectrophoretic pattern, have a sarcomatous character histologically, and occur in patients aged between 10 and 50 years. The view that medulloblastoma comprises a number of different types of tumour seems to be confirmed by this finding.

Animals↗

Krabbe's disease with unusual clinical and morphological features.

A progressive encephalopathy appeared in two sibs aged 7 and 5 months. The children died at 23 respectively 29 months of age. Autopsy of the second child disclosed a severe demyelination involving the whole brain. Only few globoid cells were identified. Ultrastructural and biochemical investigations confirmed the diagnosis of Krabbe's disease.

Astrocytes↗

[Carcinomas and malignant papillomas of choroid plexus (author's transl)].

Carcinomas and malignant papillomas of choroid plexus occur more frequently in children and are mostly localized in lateral ventricles. Their clinical picture corresponds to that of plexus papillomas. The histological diagnosis is based upon unmistakeable criteria of malignancy in a tumour deriving from choroid plexus. Five such cases have been studied; in three of them the increase of malignancy was proved by the investigation of recurrences. One case was investigated electronmicroscopically.

Adult↗

Schwartz-Jampel syndrome in two daughters of first cousins.

The clinical and pathological features of two sisters born from consanguineous parents and affected by the rare Schwartz-Jampel syndrome are reported. The parental consanguinity of these two patients and the findings of electromyographic changes in the mother strongly support an autosomal recessive pattern of inheritance. No response of growth hormone secretion to arginine and insulin stimulation tests was found.

Abnormalities, Multiple↗

[Glycogen storage disease (Pompe's disease) presenting as myopathy in the adult (author's transl)].

In a 46-year-old woman with progressive atypical limb-girdle dystrophy for eight years the electromyogram revealed a myopathic pattern with signs of denervation activity. Enzymehistochemical and biochemical investigations of muscle and liver biopsies indicated type II glycogen storage disease (Pompe's disease). This type, with its prolonged course and the almost exclusive clinical involvement of proximal muscles mimics a "degenerative" neuromuscular disease, particularly muscle dystrophy, in the adult. Caused by acid maltase deficiency it is not a rare disease.

Age Factors↗

[Contribution to the pathogenesis of intrasellar and intraventricular cysts (author's transl)].

The histological structure of Rathke's cyst having an epithelial mono- or multiple layer wall with glands and mucous secreting cells is indicative of their origin from the stomodaeum. These cysts are morphologically and genetically simialr to the so-called cysts of the foramen of Monro (colloid cysts of the third ventricle). According to Stochdorph, the origin of the latter cysts is from ectopic cerebral inclusions of the upper respiratory tract.

Cerebral Ventricle Neoplasms↗

The protracted form of juvenile neuronal ceroid-lipofuscinosis.

Clinical and ultrastructural findings consisting of curvilinear and fingerprint residual bodies, in a protracted juvenile form of NCL are reported from a woman who died at the age of 35 years. Homochrony and homotypy of her brother's illness emphasize intrafamilial similarities within subgroups of lysosomal disorders.

Adult↗