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Biomedical subjects

V Pietrini

Publications and source records attributed to V Pietrini.

6 recordsLinked to original sources
PubMed1992

Creutzfeldt-Jakob disease presenting as Wernicke-Korsakoff syndrome.

A 47-year-old man began to suffer from progressive truncal ataxia and mental alterations typical of Wernicke-Korsakoff syndrome. He showed confusional state, hallucinations, delirium of jealousy and a serious impairment of recent memory. The symptomatology lasted 13 months, but only in the last weeks was it complicated by myoclonias. Triphasic pseudoperiodic sharp-waves characterized the EEG-recordings only in the final stage. Macroscopic examination of the brain showed marked atrophy of the mammillary bodies and superior vermis. However, the histological features were consistent with Creutzfeldt-Jakob disease (CJD) with focal accentuation of the changes in the latter structures. This case supports the hypothesis that CJD-changes begin focally in the CNS and, subsequently, spread along neuronal pathways, probably via central axons. Only in the final stage does the pathological process involve most parts of the gray matter. A focal accentuation of the CJD process in the cerebello-mammillo-thalamic system caused in this case a Wernicke-Korsakoff-like syndrome.

Alcohol Amnestic Disorder↗
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↗