Sur les formes familiales précoces de la maladie d 'Alzheimer, pp. 275–301

Bogaert, Ludo van; Maere, Marcel; Smedt, Edmond de

doi:10.1159/000148593

Cited by 7 publications

(2 citation statements)

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“…23 An early description of the unusual combination of dominantly inherited Alzheimer-type presenile dementia with spastic paralysis was published in 1940. 28 An autopsied patient showed numerous neurofibrillary tangles as well as a multitude of various types of senile plaques not only in the cerebral but also in the cerebellar cortex. In addition, "drusenartige Entartung" of cerebral blood vessels as well as degeneration of the pyramidal tracts were observed.…”

Section: Unusual "Cotton Wool" Plaques In the Temporal Neocortex Ofmentioning

confidence: 99%

MICAL-1 Is a Negative Regulator of MST-NDR Kinase Signaling and Apoptosis

Zhou

Adolfs

Pijnappel

et al. 2011

Molecular and Cellular Biology

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Article abstract-Objective: To present the clinical, neuroimaging, and electrophysiologic characteristics of a variant AD phenotype. Background: The authors have identified a large Finnish kindred with presenile dementia and spastic paraparesis due to deletion of exon 9 of presenilin 1. Neuropathologic analysis showed unusual cortical "cotton wool" plaques, immunoreactive for the beta-amyloid peptide but lacking congophilic cores. Patients and Methods: Twenty-two affected individuals (16 men and 6 women) were identified in four successive generations. All surviving five patients were examined and subjected to molecular genetic analysis. In addition, the neurologic records of nine deceased patients were evaluated. Electrophysiologic investigations were available in eight cases. CT or MRI of the head had been performed on 11 patients and PET was performed on three patients. Result: The mean age at onset (ϮSD) was 50.9 Ϯ 5.2 years (range 40 to 61 years). Memory impairment was present in all patients. Memory impairment appeared simultaneously with or was preceded by walking difficulty due to spasticity of the lower extremities (10/14). Impaired fine coordination of hands (9/14) and dysarthria (6/14) in some patients suggested cerebellar involvement. EEG showed intermittent generalized delta-theta activity. Head MRI showed temporal and hippocampal atrophy; PET showed bilateral temporo-parietal hypometabolism. Conclusion: Spastic paraparesis or brisk stretch reflexes of lower extremities or clumsiness of hands combined with dementia suggests this variant of AD.

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Section: Unusual "Cotton Wool" Plaques In the Temporal Neocortex Ofmentioning

confidence: 99%

MICAL-1 Is a Negative Regulator of MST-NDR Kinase Signaling and Apoptosis

Zhou

Adolfs

Pijnappel

et al. 2011

Molecular and Cellular Biology

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“…Amyloidosis of the nervous system, especially of the brain, has hitherto been studied as an interesting rarity (Mignot & Marchand, 1911;Weimann, 1922;Schwartz, 1930;Fischer & Holfelder, 1930;Morgenstern, 1934;Saltykow, 1935;Gerstmann et aZ., 1936;Volland, 1938;van Bogaert et al, 1940;Worster-Drought, Greenfield & McMenemey, 1940;Lifers, 1947;Lowenberg-Scharenberg & Bassett, 1950;Krucke, 1950;Fisher & Preuss, 1951;Denny-Brown, 1951;Corsellis & Brierley, 1954;Ritama, 1954;Ritama & Bjbrkesten, 1954;Silva Horta, 1955;de Vries, 1959;Neumann, 1960;Silva Horta & TrincPo, 1963;Haberland, 1964). Now we have demonstrated that this disease, because of the frequency of its occurrence and clinical significance, occupies a foremost place in neuropathology, since no other morphologically defined cerebral disease appears to be so often associated with severe deficiencies.…”

Section: )mentioning

confidence: 99%

Senile Cerebral, Pancreatic Insular and Cardiac Amyloidosis*

Schwartz

1965

Trans NY Acad Sci

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We a r e accustomed to consider senile alterations of the human body as self-evident results of attrition, just as the deterioration of any structure exposed to incessant wear-and-tear. Atrophy of organs, their hardening (sclerosis), stiffening, o r rarefaction, and discoloration due to changes in their physical properties and in their chemical composition, seem to be no more noteworthy than the deterioration of any timeworn article in our daily experience.Since 1953, we have collected, investigated and classified material from approximately 1,000 postmortems on aged persons who died at the Warren State Hospital, Warren, Pa. We have encountered not only well-known and often reported senile phenomena, but also disorders unrecognized o r neglected until now, which have revealed new aspects of aging. We have very often Dbserved decrease in the excretory pancreatic tissue, a typical disease probably impairing the digestion of fatty and lipoidic as well as proteinic constituents of nutrition. We have designated it as fatty dysplasia of the pancreas because of its association with a compensatory proliferation of fatty connective tissue. Another remarkably frequent senile alteration is characterized by hemosiderinic deposits in the splenic capsule and in reticulo-endothelial elements of the spleen and liver; we refer to this change as senile mobilization of the iron mefabolism. A third group of findings in the aged implicates the internal secretory glands: we deal with benign tumoral, mostly focal proliferations in the cortex and medullary substance of adrenals, @f the thyroid gland, prostate and, sometimes, in the mammae of males which we consider to result from an enhanced activity of pituitary hormones after the climacteric decrease o r extinction of the gonadic function. We will report on all these observations in another publication, but intend to devote this present paper entirely to the most surprising finding we have made in the course of our systematic investigations on old age, namely the progressive infiltration of certain vital organs by amyloid. Haitinger and Geiser (1944), Vassar and associates (1959) as well as Hobbs and Morgan (1963),? demonstrated that amyloid deposits in spleen, liver and kidneys may be detected with particular reliability by employing fluorescence microscopy. On the other hand, after Bielschowsky a s early as 1911 had considered the possibility that senile plaques contain amyloid, on the basis of quite valid findings, Divry, since 1927 repeatedly pointed out that amyloid constitutes an essential component of both senile plaques and Alzheimer's fibrils. Although still opposed, doubted and neglected

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Die Verbindung der Pickschen Atrophie mit Hirnnervenkernver�nderungen und Pyramidenbahnsymptomatik

Poppe¹,

Tennstedt²

1964

Acta Neuropathol

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Sur les formes familiales précoces de la maladie d 'Alzheimer, pp. 275–301

Cited by 7 publications

References 0 publications

MICAL-1 Is a Negative Regulator of MST-NDR Kinase Signaling and Apoptosis

MICAL-1 Is a Negative Regulator of MST-NDR Kinase Signaling and Apoptosis

Senile Cerebral, Pancreatic Insular and Cardiac Amyloidosis*

Die Verbindung der Pickschen Atrophie mit Hirnnervenkernver�nderungen und Pyramidenbahnsymptomatik

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