Senile plaques, "aging" and Alzheimer's disease.
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Aged↗
Biomedical subjects
J F Foncin
Publications and source records attributed to J F Foncin.
Complete spontaneous thrombosis of a giant intracranial aneurysm.
In a 23-year-old woman CT demonstrated a patent giant intracranial aneurysm. MRI, CT and angiography one month later demonstrated complete spontaneous thrombosis of the aneurysm. The diagnosis was confirmed surgically and pathologically. This case demonstrates that a normal angiogram can be associated with a thrombosed giant aneurysm, and that this diagnosis should be considered in cases of angiographically-occult cerebral vascular malformations.
Adult↗
MR of isolated leptomeningeal glioma.
We report the MR study of a case of high-grade glioma that mimics leptomeningeal carcinomatosis. Superficial gliomas should thus be included in the differential diagnosis of isolated meningeal enhancement.
Adult↗
Do microglial cells phagocyte the beta/A4-amyloid senile plaque core of Alzheimer disease?
We performed EM, immuno-EM and light microscope immunohistochemistry studies on the topographic and functional relationships between microglial cells and amyloid senile plaque core in Alzheimer's disease. Microglial cells with cytologic characteristics of phagocytic function were associated to amyloid fibrils and to other neuropathological degenerative processes. On the periphery of the amyloid plaque core, microglial cells contain intracytoplasmic bundles of membrane bound fibrils. These fibrils, like plaque core fibrils, are immunodecorated. Immunostaining was observed neither in secretory organelles nor in hyalaplasm. Preamyloid deposits in superficial layers were not associated to microglial cells. These data lead us to conclude that the microglial cells participate to phagocytosis of beta/A4 amyloid and do not secrete this substance in Alzheimer's disease.
Adult↗
Paraganglioma of the cauda equina: magnetic resonance imaging.
We report the clinical and imaging features of a paraganglioma of the cauda equina. Raised CSF protein caused an unusual appearance of the spinal canal below the obstruction. No specific identifying features were encountered; paraganglioma is uncommon in this site and usually thought to be an ependymoma or a neuroma; histopathological examination is necessary for correct diagnosis.
Cauda Equina↗
Analysis of the c-FOS gene on chromosome 14 and the promoter of the amyloid precursor protein gene in familial Alzheimer's disease.
The c-FOS gene product, a putative transacting transcriptional regulator of the amyloid precursor protein (APP) gene, is a candidate locus for the familial Alzheimer's disease (FAD) mutation on chromosome 14 (FAD14). In light of this functional relationship, we investigated the nucleotide sequence and segregation of c-FOS and the nucleotide sequence of the 5' APP promoter. Single-stranded conformational polymorphisms (SSCPs) in the c-FOS gene revealed that c-FOS closely cosegregates with the FAD14 gene but does not show allelic association with FAD. A conservative third-position T-->C mutation was demonstrated in exon 2 (codon 84) of c-FOS, and a C-->G substitution was detected at -209 bp in the 5' promoter of APP. Neither were unique to FAD and are unlikely to be pathogenic or secondary modifiers of the FAD phenotype. We conclude that the c-FOS open reading frame is probably not the site of the FAD14 locus, but we cannot exclude the existence of modifier loci on chromosome 21.
Adult↗
Supratentorial low-grade astrocytomas in adults.
One hundred seventy-nine adult patients with supratentorial low-grade astrocytomas were treated during a 10-year period. Retrospectively, a series of nine factors were evaluated with simple and multivariate analysis to determine their importance in predicting length of survival. Four appear highly significant (P < 0.005): age, preoperative Karnofsky Performance Scale score, histological grade, and type of surgical removal. Eighty percent of patients with total removal were alive at 5 years compared with 50% with incomplete surgery and 45% with biopsy. For the group under study, the mean time for recurrence was 52 months. For patients surviving for more than 1 year, the chance of being recurrence free went from 75% at 3 years to 25% at 10 years. Among the entire population, the influence of radiotherapy was not obvious: 65% of patients were alive at 5 years without radiotherapy compared with 55% with radiotherapy. A significant difference appeared only for patients older than 40, with incomplete removal (P < 0.05); this difference did not apply to younger patients. The need for postoperative radiotherapy in all patients with subtotal removal, irrespective of their age, has not been demonstrated by this study.
Adult↗
A novel but non-pathogenic mutation in exon 4 of the human amyloid precursor protein (APP) gene.
Mutations in the beta-amyloid precursor protein (APP) gene have been associated with both familial Alzheimer disease (FAD) and with hereditary cerebral haemorrhage. The polymerase chain reaction was used to both amplify and sequence exon 4 of the APP gene from genomic DNA of subjects with FAD and normal control subjects. A novel, rare, conservative DNA sequence variant was discovered at nucleotide 459 of codon 153 (valine) in exon 4 of the APP gene in an affected member of a large FAD pedigree. Segregation studies indicate that this mutation is likely to be non-pathogenic, but must be recognized and discriminated from pathogenic mutations during sequencing studies of the APP gene in patients with FAD.
Amyloid beta-Protein Precursor↗
Genetic evidence for a novel familial Alzheimer's disease locus on chromosome 14.
Familial Alzheimer's disease (FAD) has been shown to be genetically heterogeneous, with a very small proportion of early onset pedigrees being associated with mutations in the amyloid precursor protein (APP) gene on chromosome 21, and some late onset pedigrees showing associations with markers on chromosome 19. We now provide evidence for a major early onset FAD locus on the long arm of chromosome 14 near the markers D14S43 and D14S53 (multipoint lod score z = 23.4) and suggest that the inheritance of FAD may be more complex than had initially been suspected.
Aged↗
Relationship between non-fibrillary amyloid precursors and cell processes in the cortical neuropil of Alzheimer patients.
We examined the ultrastructural localization of amyloid beta-protein in 8 Alzheimer neocortical biopsies. Intense immunoreactivity was located extracellularly on amyloid fibrils and amorphous material. Amorphous labelled material was also found in cell processes. No ultrastructural cell marker, such as glial fibrils, glycogen, tubules, paired helical filaments (PFHs) or synaptic vesicles could be seen in these processes that could allow their identification as glial processes, neurites or presynaptic terminals, respectively; occasional membrane stacks were observed. These findings suggest that preamyloid deposits are related to cell processes and, by elimination, that postsynaptic terminals may be involved in abnormal metabolism of the amyloid fibril precursors.
Aged↗
Genotypic differences in hemangiopericytic meningioma.
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Alleles↗
[The transition zone of the central nervous system-peripheral nervous system of the adult rat; ultrastructural and immunocytochemical studies: a new function of the astroglia?].
At the transition between central nervous system (CNS) and peripheral nervous system (PNS), the CNS compartment forms cone-shaped incursions into the peripheral part of the dorsal root. The ultrastructural study of the CNS-PNS transitional zone shows that this region is particularly rich in astrocyte processes. In an attempt to investigate the possible role of the CNS-PNS interface astrocytes in myelin formation, a photonic microscopy immunocytochemical study has been done with anti-GFAP and anti-MBP sera. The CNS glial expansion shows an important GFAP immunoreactivity with intimate association between astrocyte processes and myelinated axons. This may indicate that the transitional myelin originates from astrocytes. The same region is also MBP-positive. Two explanations are considered: some astrocytes form transitional myelin sheathes and express MBP epitopes, or oligodendrocytes, with cell bodies distant from the CNS-PNS interface, send myelinating cytoplasmic expansions which are not shown by the techniques we used.
Animals↗
Genetic linkage studies suggest that Alzheimer's disease is not a single homogeneous disorder.
Alzheimer's disease, a fatal neurodegenerative disorder of unknown aetiology, is usually considered to be a single disorder because of the general uniformity of the disease phenotype. Two recent genetic linkage studies revealed co-segregation of familial Alzheimer disease with the D21S1/S11 and D21S16 loci on chromosome 21. But two other studies, one of predominantly multiplex kindreds with a late age-of-onset, the other of a cadre of kindreds with a unique Volga German ethnic origin, found absence of linkage at least to D21S1/S11. So far it has not been possible to discern whether these conflicting reports reflect aetiological heterogeneity, differences in methods of pedigree selection, effects of confounding variables in the analysis (for example, diagnostic errors, assortative matings), or true non-replication. To resolve this issue, we have now examined the inheritance of five polymorphic DNA markers from the proximal long arm of chromosome 21 in a large unselected series of pedigrees with familial Alzheimer's disease. Our data suggest that Alzheimer's disease is not a single entity, but rather results from genetic defects on chromosome 21 and from other genetic or nongenetic factors.
Alzheimer Disease↗
Intracranial meningiomas in elderly patients. Postoperative morbidity and mortality. Factors predictive of outcome.
We studied retrospectively a series of 96 patients (36 men, 60 women), older than 65 years of age (mean age: 70 +/- 4 years, range 65-82), operated upon for an intracranial meningioma from October 1978 to December 1988. Fifty-two patients (54%) were under 70, 32 between 71 and 75 and 12 over 75 (46%). The tumours were diagnosed for all the patients by CT scan. Thirty-four (35%) were located over the convexity, 24 (25%) in the falx/parasagittal region, 38 (40%) in the base, tentorium and posterior fossa. Neurological and physical conditions were assessed preoperatively and at the closing date in June 1989. Operative mortality was 16% (15/96). Patients were divided into two groups: poor outcome, defined by the death or a post-operative Karnofsky index less than or equal to 70 (n = 36), and good outcome defined by a Karnofsky index of 80 or more (n = 60). The two groups did not differ regarding age, sex ratio, tumour size and peritumoural oedema. The only predictors of poor outcome were poor preoperative general health condition (stage III of the American Society of Anesthesiology classification), (p less than 0.01), poor preoperative neurological condition (Karnofsky's index) (p less than 0.001), and location of the tumour on the base or in posterior fossa (p = 0.02).
Aged↗
[Loss of dendritic spines in Alzheimer's disease].
Neurones from five patients with Alzheimer's disease were studied semi-quantitatively on Golgi impregnated samples of cerebral cortex. They showed a dramatic reduction in dendritic spine number of pyramidal and non-pyramidal cells of all neocortical layers, as compared with control samples matched for age. Dendritic spine loss could be an initial phenomenon in Alzheimer's disease pathology.
Aged↗
[Ependymoma of the intradural filum terminale in adults. 20 cases].
Twenty cases of ependymomas of the intradural filum terminale in adults have been reviewed. Their pathology was quite uniform, of a myxopapillary type, similar to the low grade ependymoma described by Kernohan, which represent about 23% of the tumours of cauda equina. Mean age of the patients was 35.7 years. Mean time between the first symptom and the diagnosis was 46 months. Clinical symptoms were often non specific, with low back pain and radiculalgias. At the time of operation, clinical signs were essentially motor deficits usually moderate (11 cases), sphincter disturbances (10 cases), and sensory loss (9 cases). In 3 patients with rapid worsening, an intratumoral haemorrhage was found. In 2 other cases, intracranial hypertension was the main symptom: in the first, it was related to hydrocephalus probably caused by spinal subarachnoid haemorrhage; in the second, there was no ventricular dilatation. In this series, neuroradiological examinations had consisted mainly in myelographies. C.T. scan has been performed in 3 patients; in only one case it has allowed to visualize a presacral extension. One patient had preoperative M.R.I.: the association of an expansive lesion with upper cyst in conus medullaris and presence of blood in the sacral area permitted the diagnosis of ependymoma of the filum terminalis. The average size of the tumours was 8 cm. Total removal has been possible in 15 cases (and in 2 of the 5 giant tumours), subtotal removal in 2 cases, and partial removal in 3 cases. In 4 patients where existed an intraspinal cord extension above the conus, it has been resected completely, except for one case with recurrence. Patients with a total removal had a good functional recovery (13/15). No recurrence has been observed in this group. In conclusion, with M.R.I., one may hope an earlier diagnosis, condition of radical surgery. So, radiotherapy which is not without risk, could be avoided.
Adolescent↗
[Intradural extramedullary neuro-enteric cysts without associated malformation. 3 cases with magnetic resonance imaging].
We report 3 cases of extramedullary neurenteric cyst without associated dysraphic lesions. One of the cases had an ultrastructural study. Magnetic resonance imaging provided a preoperative diagnosis. The embryogenesis of neurenteric cysts, their main clinical aspects and their surgical treatment are reviewed.
Adult↗
[The prognosis of medulloblastoma in adults].
We studied retrospectively the series of 24 patients (17 men, 7 women), median age 25.5 years (range: 16-57), operated upon for a cerebellar medulloblastoma from March 1979 to June 1988. The tumors were diagnosed for all the patients by C.T. scan and by M.R. imaging for the six last patients. Seven tumors (29.2%) were located in the fourth ventricle, 2 in the vermis (8.3%), 9 in the cerebellar hemisphere (37.5%), 5 in the vermis and cerebellar hemisphere (20.8%). One patient had a diffuse infiltration of the cerebellum. All patients have been operated on (complete removal: 15 patients (62.5%), subtotal removal: 8 patients (33.3%), biopsy: 1 patient). Operative mortality was 8.3% (2/24). One patient died at two months from septicemia. The twenty-one surviving patients received radiotherapy. Twelve patients received both radiotherapy and chemotherapy. Six patients (25%) died during the follow up at 17, 22, 24, 60, 84 and 85 months. One patient is lost to follow up at 45 months and 13 patients are living at the closing date (June 1989). For the total group (n = 24) the probability of survival at 5 years was 64.8% (C.I. 95%: 42.8%-86.8%). Among the 21 patients who received radiotherapy alone or radiotherapy and chemotherapy the probability of survival at 5 years was 74.4% (C.I. 95%: 52.2%-96.7%). We studied the following predictors of poor outcome: age, sex, prognosis subgroups as defined by Chang et Coll., extension of tumor removal, adjunction of chemotherapy, histology. None of these factors was statistically related to the survival duration.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗