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Biomedical subjects

V Pietrini

Publications and source records attributed to V Pietrini.

30 records · Page 2Linked to original sources
PubMed1983

Megalencephaly with formation of Rosenthal fibers in symmetric subependymal gliomatous proliferations: clinicopathologic report.

Clinical and neuropathologic observations are reported concerning a girl with retarded psychomotor development accompanied with a steady increase in the volume of the cranium and brain, tetraparesis, and a serious epileptic syndrome resulting in death at the age of 13 years. The neuropathologic study revealed a marked megalencephaly and the presence of two bilateral and symmetric areas of atypical glial proliferation, corresponding with the optostriatal groove, with endoventricular protrusion: in this region the histologic findings gave evidence of a tapetum of Rosenthal fibers. Discussion is focused on the observed neuropathologic aspects in relation to the various diagnostic hypotheses: Alexander's disease, primary megalencephaly associated with astrocytomas or reactive gliosis, or megalencephaly associated with subependymal hamartomas.

Brain↗
PubMed1982

Anderson-Fabry's disease: neuropathological and neurochemical investigation.

A clinical, neuropathological and neurochemical study of a case of Anderson-Fabry's disease is described. The clinical course mainly consisted of repeated ictus with major involvement of the CNS. The neuropathological examination is dominated by severe alterations in the cerebral vessels due to glycolipid deposits on the walls, with reduction or occlusion of the lumen. This is correlated with secondary ischaemic foci scattered throughout the cortex as well as through the white matter. In addition, the cells of the cerebral cortex, thalamus, basal ganglia, amygdala, cerebellar and olivary nuclei show a marked accumulation of lipofuscin. Biochemical examination reveals a threefold increase in galactolipids due to the specific alpha-galactosidase deficiency. Cholesterol is reduced secondarily to ischaemic myelin damage. Glycosaminoglycans uronic acid is increased in cytosol and membrane-bound fractions which could be related to reactive gliosis. Glycoprotein sugars show a decrease in N-acetyl-neuraminic acid and fucose as well as an increase in hexosamines and hexoses in membrane-bound fraction, while in cytosol fraction all sugars are increased. This suggests that the alpha-galactosidase deficiency can alter not only the glycolipid but also the glycoprotein metabolism, resulting in a higher presence of hexosamines and hexoses-rich glycoproteins.

Amygdala↗
PubMed1981

Progressive supranuclear palsy in the course of subclavian steal syndrome.

A 70-year-old man manifested during four years a progressive clinical picture consisting in palsy of gaze, axial rigidity, disorders of standing and gait, dysarthria, dysphagia. Neuroradiological investigations demonstrated proximal thrombosis of the left subclavian artery with subclavian steal. At necropsy, degenerative changes in several areas of the basal ganglia and brain stem, with presence of globose neurofibrillary tangles, were found, consistently with the pathologic pattern of the Progressive Supranuclear Palsy (PSP). The association of PSP and subclavian steal syndrome has not been previously reported, to our knowledge. We hypothesize that chronic ischemia, due to subclavian steal syndrome, in the vertebral basilar system and its watershed versus carotid system may have favoured the appearance, in these same areas, of the changes of the PSP.

Aged↗
PubMed1981

On the pathology of meningiomas. A study of 412 cases.

Some pathological features of tumors arising primarily in the meninges are discussed on the basis of 412 surgically treated meningiomas during the period running from 1964 to 1978. Cases of meningeal sarcomas were not considered. Except for a few cases, pathology and clinical course have been generally in agreement. The incidence of histological types like meningotheliomatous, fibroblastic, angioblastic or atypical meningiomas has been verified in relation to sex, age and intracranial or spinal conventional locations. The problem of recurrencies has been studied either comparing the histological features with the first observation or comparing histopathological types with location and details of surgical procedure. Additional criteria like uni or multifocal origin of the tumors and histological features of the peri-lesional tissues, often harbouring small meningiomatous nests, have been taken into account.

Brain Neoplasms↗
PubMed1981

"Thalamic" dementia in herpes encephalitis: clinico-pathological report.

Herpes zoster (HZ) primary affections of the CNS are rare and, in most of the reported patients, are representing variously extended forms of ascending myelitis. Our examination concerns a man who at the age of 37 developed apathy after a feverish episode with iridocyclitis. Six months later an ophthalmic HZ was diagnosed and thenceforth the patient showed a dementia with Korsakow's syndrome, apathy and a right hemipalsy, and diplopia appeared; the later symptoms remitted after steroid therapy. Post-mortem examination revealed a slowly progressive encephalitis with symmetrical impairment of the anterior ventral, medial, and centrum medianum of the thalamus. The HZ origin of the lesions and the relation between their site and the peculiar form of dementia, to be ascribed to the "thalamic" ones, are discussed. A vasculitis process can be hypothesized considering both the symmetrical localisation and the microscopical aspects of the lesions.

Adult↗
PubMed1980

[Fabry's disease and familial lymphedema. Description of 2 cases].

Two cases of Fabry's disease (FD) with lymphedema of the lower limbs are reported. On the basis of lymphographic investigations showing lymphatic aplasia, the hypothesis of an inborn error in the development of the lymphatic system of the lower limbs--Familial Lymphedema--controlled by a gene associated with FD gene on the same chromosome, is suggested.

Adult↗
PubMed1979

Orthochromatic leukodystrophy with pigmented glial cells. An adult case with clinical-anatomical study.

The case history is reported of a woman who died at the age of 36, at the conclusion of 11 years progressive neurological and psychiatric symptomatology. The anatomical and histological examination demonstrated an orthochromatic leukodystrophy with pigmented glial cells. Attention is drawn to the difficulty of finding these cells, which serve to differentiate between the unusual and the "simple" form of the disease. In the reported patient the pigmented cells were found around the vessels and only in specific cerebral locations. It is emphasized that the form is extremely rare (this is the tenth case so far reported). The significance of whether the iron content should be considered as an incidental or necessary finding is discussed. Systematic research for pigmented casts of this kind is taken to be important for all brains presenting a diffuse sclerosis after a protracted clinical course, mainly in adult patients.

Adult↗
PubMed1979

Fabry's disease with familial lymphedema of the lower limbs. Case report and family study.

The case of a 49-year-old man with Fabry's disease (FD), confirmed by histopathological findings of kidney and skin biopsies and enzymatic studies, is reported. Clinical symptoms mainly consisted in severe neurological involvement, and in conspicuous lymphedema of the lower limbs. Two decreased brothers of the patient were also affected with symptons strongly suggesting FD, as well as the lymphedema of the lower limbs. On the basis of these data, the association of FD with familial lymphedema of the lower limbs is discussed: a lipid accumulation in the lymphatic as well as the blood vessel wall is proposed as a possible explanation; the hypothesis of an inborn error in the development of the lymphatic system, controlled by a gene closedly associated with the FD gene on the same chromosome can also be advanced.

Cerebroside-Sulfatase↗
PubMed1978

[Intracranial and spinal dermo-epidermoid tumours. Anatomoclinical study (author's transl)].

The authors review the previous report of intracranial or spinal dermo-epidermoid tumours, in concern with both nosographical and biological problems. They report their clinical and anatomical findings on a patient who presented the same tumours within the skull and in the vertebral canal. The authors underscore the rarity of such finding, the length of the course. albeit a few symptoms and no positive neuroradiological findings had been remarked, and the quite atypical eventual troubles.

Brain↗
PubMedDate not supplied

Adult metachromatic leucodystrophy: clinicopathological report of two familial cases with slow course.

Two cases of adult metachromatic leucodystrophy in one family are reported. The main clinical features in both were predominantly psychiatric with alcoholism and an extremely long duration of the illness. Neuropathological examination revealed a similar distribution of the lesions in both, and scanty metachromatic accumulation in the CNS and not at all elsewhere. The great variability of the lengths of the courses is stressed. The duration in no way seems to be linked to the age of onset, except in the typical infantile form. The authors argue that different lengths of history correlate with distinct neuropathological findings, and may possibly be related to qualitative differences in the involved enzyme disturbance. Therefore, the authors suggest that the classification based on the age of onset be enlarged with a further one distinguishing between 'rapid' and 'slow' course types.

Aged↗
PubMedDate not supplied

Panencephalopathic type of Creutzfeldt-Jakob disease with neuropathologic features similar to Pick's disease.

The panencephalopathic type of Creutzfeldt-Jakob disease is characterized by a serious degeneration of the white matter in addition to the other pathological features of the classic Creutzfeldt-Jakob disease. The clinical and neuropathological findings of a new case are described in a woman aged 62, who died after a year of illness. The brain appeared seriously affected by atrophy and white matter degeneration. Microscopically, it showed a marked cortical spongiosis, with gemistocytic astrogliosis and degeneration of the white matter of both hemispheres. Although a serious loss of nerve cells was evident, some residual neurons with a ballooned aspect were found in the fronto-temporal cortex. Other neurons presented argyrophilic inclusions similar to Pick bodies. By means of immunohistochemical techniques and monoclonal antineurofilaments antibodies some neurons with swollen cytoplasm and enlargement of the first tract of the neurites were detected in the basal layers of the frontal and temporal cortex. These abnormal features were due to the accumulation of phosphorylated 200 Kd neurofilaments. The relations between Creutzfeldt-Jakob and Pick's diseases are analyzed on the basis of the neuropathological findings. There is evidence from the immunohistochemical data of an interference in the axonal transport of neurofilaments.

Axonal Transport↗