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Biomedical subjects

G Macchi

Publications and source records attributed to G Macchi.

At least 73 records · Page 4Linked to original sources

[Alzheimer's presenile dementia transmitted in an extended kindred].

Forty-three patients affected with Alzheimer's disease were identified in a kindred of Italian origin, emigrated in part to the U.S.A. and France. Thirteen were known by history, 21 by medical record, and 9 by personal examination, of whom 5 were confirmed histopathologically. The clinical picture was fairly uniform: the first symptom was memory loss beginning around age 40. Psychotic-like symptoms often followed, with rapid evolution into profound dementia, and death around age 50. Akinesia was prominent at a late stage, often with myoclonus. Grand mal seizures sometimes occurred, with occasional interictal spike and wave discharge; repetitive paroxystic periodic discharges were never recorded. A genealogical study, as far as possible free from line bias, has been conducted mainly by analysis of municipal records. 1 435 subjects in 10 generations, linked to affected subjects through ascent/descent or marriage, were listed in a computer file; the corresponding genealogical tree or selected part thereof are generated by computer. Application of Bayesian techniques to demographic data makes possible an estimation of disease probability in subjects for which no clinical data were available: such an estimate was confirmed by the later discovery of a living patient in descent of a subject with 0.7 estimated disease probability. No patient was found in descent from an inbred union known as such. Patients are the only transmitters. The sex ratio is not significantly different from 1. There is no detectable maternal effect. The segregation ratio, as calculated from extensively known sibships, lies in the range 0.65 to 0.89; the lower value itself is significatively higher than the 0.5 value expected in an autosomal dominant monogenic Mendelian transmission. An environment factor is ruled out by the diversity of locations and circumstances in kindred members. Such a kindred may represent an useful model for fundamental studies in Alzheimer's disease and senile dementia of the Alzheimer type.

Adolescent↗

The thalamo-caudate versus thalamo-cortical projections as studied in the cat with fluorescent retrograde double labeling.

The distribution of thalamic cells projecting to the head of the caudate and their interrelations with thalamo-cortical cells were studied in the cat with different combinations of fluorescent tracers. Injections in the head of the caudate were combined with the injections in the pericruciate, proreal, suprasylvian, anterior cingulate, occipital and ectosylvian cortices. The following results were obtained: (i) Injections in the head of the caudate resulted in retrograde labeling of thalamic cells medially and laterally to the anteromedial (AM) nucleus, and in the medioventral part of the ventral anterior (VA) nucleus. Further, labeled cells were distributed throughout the anterior intralaminar central medial (CeM), paracentral (Pc) and central lateral (CL) nuclei, and the posterior intralaminar center median-parafascicular complex (CM-Pf). Labeled cells were mainly grouped in the mediodorsal parts of the anterior intralaminar nuclei; they were also found in the more dorsal part of the mediodorsal (MD) nucleus, ventral to the thalamic paraventricular (Pv) nucleus and to the habenular complex. (ii) Thalamo-cortical and thalamo-caudate cells overlapped in the medial part of the VA; in the anterior intralaminar nuclei they were either intermingled or were distributed in separate clusters or longitudinal bands. The two cell populations also overlapped in the posterior intralaminar complex. The greatest overlap occurred with the thalamic cell population projecting to the pericruciate cortex. (iii) Thalamic cells bifurcating to the head of the caudate and to the pericruciate cortex were found lateral to the AM, within the VA, and throughout the anterior intralaminar nuclei, especially in the CeM and in the posterior part of the CL; a few branched cells were also found in the CM. Thalamic cells bifurcating to caudate and anterior suprasylvian cortex were also found in the VA. Very few cells (scattered in the anterior thalamus lateral to the AM, as well as in the CeM, Pc and CL) were found to bifurcate to the head of the caudate and the other cortical fields here examined.

Animals↗

On the white matter lesions of the Creutzfeldt-Jakob disease. Can a new subentity be recognized in man?

One case of CJD with severe involvement of the white matter is discussed. The patient was admitted after a 3-month clinical course with rapidly increasing mental deterioration, coma vigil-like state, myoclonic twitching of the limbs and of the facial muscles. The EEG showed the typical features of CJD. The first CT scan, performed 3 months after onset, revealed only a mild cortical and subcortical atrophy of the brain. The second CT scan, 12 months later, showed a considerable cortical and subcortical atrophy of the brain. The patient died 18 months after onset. Neuropathological examination showed a severe degeneration in the gray matter, with spongiosis, loss of neurones and hypertrophic glial reaction. The white matter was also involved with severe spongiosis, demyelination and hypertrophic glial proliferation. The case is discussed in relation to the data in the literature. It is argued that cases of CJD with severe involvement of the white matter should be classified as a new neuropathological subentity of CJD.

Atrophy↗

Claustroneocortical projections studied in the cat by means of multiple retrograde fluorescent tracing.

The topographical interrelations of claustroneocortical cells and their degree of divergent collateralization were investigated in cat by means of retrograde fluorescent double labeling. The tracers Fast Blue (FB) and Nuclear Yellow (NY) were injected in several combinations in two different cortical fields. FB-labeled and NY-labeled cells were found in all cases in the insular portion of the ipsilateral claustrum (CII). The distributions of labeled cells confirmed the organization reported in previous studies. In some cases the two labeled cell populations were segregated in different parts of the CII; in other cases there was more or less overlap in the two distributions. The degree of overlap was not simply related to the topographic proximity of the injected cortical territories. In all cases the vast majority of cells were single labeled. In one case both FB and NY were injected in the same cortical area in order to control that claustral axons can retrogradely transport two tracers back to the same parent cell bodies. In this control case CII cells were double labeled. All together these data indicate that widely divergent claustroneocortical projection take origin mainly from separate cells.

Animals↗

The organization of the claustroneocortical projections in the cat studied by means of the HRP retrograde axonal transport.

The projections from the claustrum to the cerebral cortex were examined in the cat by means of horseradish peroxidase (HRP) injections in various different areas of the lateral and medial surfaces of the neocortex. In all cases retrogradely labeled cells were observed in the ipsilateral claustrum insulare (ClI). In most of the cases some labeled cells were also observed in the contralateral ClI. The number of labeled cells was higher following large HRP injections in some cortical areas, such as the motor and visual cortex, than following large HRP injections in other areas, such as the second somatic sensory cortex. The patterns of distribution of labeled cells in the ClI following the different HRP injections evidenced a prevalent anteroposterior and dorsoventral topographical arrangement. However, some degree of overlapping was evident in the distribution of cells retrogradely labeled from injections in different cortical fields. The results of the present study indicate that: a) in all likelihood the claustral projections are distributed upon the entire neocortex; b) they are not uniformly distributed upon the neocortex from the quantitative point of view; c) the arrangement of the claustrocortical projections is predominantly but not strictly topographical. The widespread distribution of the claustral inputs suggests that they must be integrated in different models of cortical activity.

Animals↗

The cortical projections of the thalamic intralaminar nuclei, as studied in cat and rat with the multiple fluorescent retrograde tracing technique.

Two retrograde fluorescent tracers were injected in two different areas of the cerebral cortex in rats and in cats. In all the experiments many single labeled cells and only some double labeled ones were seen in the thalamic intralaminar nuclei. The present results suggest that the diffusely distributed intralaminar-cortical projections mainly consist of axons of separate cells, and only to a minor extent of axon collaterals of the same cells.

Animals↗

Jakob-Creutzfeldt disease: analysis of EEG and evoked potentials under basal conditions and neuroactive drugs.

EEG and evoked potential (EP) recordings of a female (aged 70) affected with Jakob-Creutzfeldt disease (JCD) are reported. A comparison of neurophysiological tests under basal conditions and pharmacological stimulation with methylphenidate, diazepam and piracetam was performed. Diazepam and methylphenidate produced a flattening of triphasic complexes and changes in background activity; piracetam did not seem to influence the abnormal brain bioelectrical environment. The authors conclude that EEG and EP abnormalities in JCD can be ascribed to two separate and interacting sources: (1) exaggerated massive excitatory input coming from a deep thalamic pace-maker throughout the diffuse projecting system, and (2) lack of inhibitory intracortical mechanisms.

Aged↗

Brain stem neurons projecting to neocortex: a HRP study in the cat.

The cortical projections of the brain stem were investigated in detail in the cat by means of the horseradish peroxidase (HRP) retrograde axonal transport. Most of the cells providing ascending fibers to the neocortex were located in the pons (locus coerulus and related structures, central gray substance, dorsal tegmental nucleus, raphe nuclei, reticular nuclei); labeled neurons were also identified in the mesencephalon, mainly in the periaqueductal gray and in the nucleus linearis rostralis. These projections, and particularly the pontine fibers, were diffusely distributed throughout the cerebral cortex.

Animals↗