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Biomedical subjects

F F Cruz-Sanchez

Publications and source records attributed to F F Cruz-Sanchez.

At least 19 recordsLinked to original sources
PubMed2004

Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease.

BACKGROUND: Recently described neuronal intermediate filament inclusion disease (NIFID) shows considerable clinical heterogeneity. OBJECTIVE: To assess the spectrum of the clinical and neuropathological features in 10 NIFID cases. METHODS: Retrospective chart and comprehensive neuropathological review of these NIFID cases was conducted. RESULTS: The mean age at onset was 40.8 (range 23 to 56) years, mean disease duration was 4.5 (range 2.7 to 13) years, and mean age at death was 45.3 (range 28 to 61) years. The most common presenting symptoms were behavioral and personality changes in 7 of 10 cases and, less often, memory loss, cognitive impairment, language deficits, and motor weakness. Extrapyramidal features were present in 8 of 10 patients. Language impairment, perseveration, executive dysfunction, hyperreflexia, and primitive reflexes were frequent signs, whereas a minority had buccofacial apraxia, supranuclear ophthalmoplegia, upper motor neuron disease (MND), and limb dystonia. Frontotemporal and caudate atrophy were common. Histologic changes were extensive in many cortical areas, deep gray matter, cerebellum, and spinal cord. The hallmark lesions of NIFID were unique neuronal IF inclusions detected most robustly by antibodies to neurofilament triplet proteins and alpha-internexin. CONCLUSION: NIFID is a neuropathologically distinct, clinically heterogeneous variant of frontotemporal dementia (FTD) that may include parkinsonism or MND. Neuronal IF inclusions are the neuropathological signatures of NIFID that distinguish it from all other FTD variants including FTD with MND and FTD tauopathies.

Adult↗
PubMed2003

Mild hypercholesterolemia is an early risk factor for the development of Alzheimer amyloid pathology.

BACKGROUND: Epidemiologic and experimental data suggest that cholesterol may play a role in the pathogenesis of AD. Modulation of cholesterolemia in transgenic animal models of AD strongly alters amyloid pathology. OBJECTIVE: To determine whether a relationship exists between amyloid deposition and total cholesterolemia (TC) in the human brain. METHODS: The authors reviewed autopsy cases of patients older than 40 years and correlated cholesterolemia and presence or absence of amyloid deposition (amyloid positive vs amyloid negative subjects) and cholesterolemia and amyloid load. Amyloid load in human brains was measured by immunohistochemistry and image analysis. To remove the effect of apoE isoforms on cholesterol levels, cases were genotyped and duplicate analyses were performed on apoE3/3 subjects. RESULTS: Cholesterolemia correlates with presence of amyloid deposition in the youngest subjects (40 to 55 years) with early amyloid deposition (diffuse type of senile plaques) (p = 0.000 for all apoE isoforms; p = 0.009 for apoE3/3 subjects). In this group, increases in cholesterolemia from 181 to 200 almost tripled the odds for developing amyloid, independent of apoE isoform. A logistic regression model showed consistent results (McFadden rho2 = 0.445). The difference in mean TC between subjects with and without amyloid disappeared as the age of the sample increased (>55 years: p = 0.491), possibly reflecting the effect of cardiovascular deaths among other possibilities. TC and amyloid load were not linearly correlated, indicating that there are additional factors involved in amyloid accumulation. CONCLUSIONS: Serum hypercholesterolemia may be an early risk factor for the development of AD amyloid pathology.

Adult↗
PubMed1993

Brain banks and non nervous tissues.

Nervous system diseases may not be confined to neural tissue, but also affect other organs. These organs could be involved indirectly or could be simultaneously affected by the same pathological process. A brain bank (BB) should also guarantee the storage of specific organs primarily or secondary affected other than nervous system. Tissues from patients with primary nervous system diseases without or with unknown systemic involvement should also be stored. Samples stored will be identified and registered in a BB database for an accurate distribution and utilization of the material. To guarantee the best quality of the material stored, several techniques for the collection and preservation (cryopreservation, chemical fixation and microwave irradiation) and tissue management are described.

Brain↗
PubMed1992

Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer's changes. Antigenic determinants that distinguish cortical and subcortical neurofibrillary tangles.

Two cases with classical clinical manifestations of progressive supranuclear palsy (PSP) showed severe progressive dementia as an additional clinical feature. Neuropathological study demonstrated typical features of PSP in the brainstem. Additionally, histological criteria of Alzheimer's disease (AD) were observed. A topographic and immunohistological study (with neurofilament subunit and Tau and Ubiquitin antibodies) of the distribution of neurofibrillary tangles (NFTs) was performed in order to compare the characteristics of NFTs from cortex and brainstem. NFTs from cortex were positive with all antibodies used and were predominantly distributed in cortical layers III and V and affected medium size neurons. Brainstem NFTs were positive only for neurofilament subunits and Tau. Cortical and brainstem NFTs showed immunohistological differences. Cortical NFTs in our two cases had a similar distribution as in control AD cases. On the basis of our observations we believe (1) that cortical tangles in our PSP cases are related to Alzheimer's disease and (2) that the cortical NFTs of PSP and AD are morphologically and immunohistologically distinct. Mechanisms concerned with the production of cortical and brainstem NFTs in PSP and AD are discussed.

Aged↗
PubMed1992

Immunohistological study of grumose degeneration of the dentate nucleus in progressive supranuclear palsy.

The grumose degeneration observed in the dentate nuclei of 7 cases of progressive supranuclear palsy (PSP) was studied with a panel of antibodies which included 2 neurofilaments, Tau and ubiquitin. Dentate nucleus neurons were negative with all antibodies except ubiquitin which showed a slightly positive homogeneous pattern of staining. The amorphous material surrounding swollen or normal neurons was strongly positive for neurofilament and subunits and numerous torpedoes were observed in the granular layer of the cerebellar cortex. Our results confirm that grumose degeneration consists in degeneration of terminal axons of Purkinje cells in the dentate nucleus. The positivity of dentate nucleus neurons for ubiquitin may support the concept of synaptic dysfunction between Purkinje cells and dentate nucleus neurons.

Aged↗
PubMed1992

Ubiquitin in cerebral amyloid angiopathy.

Immunohistological findings in cerebral blood vessels of 4 cases with cerebral amyloid angiopathy (CAA) were compared with those of 4 Alzheimer's (AD) cases. A panel of antibodies against 2 neurofilament subunits (BF10 and RT97), a microtubule-associated protein (TAU) and ubiquitin were used. CAA cases showed a strong immunoreactivity for ubiquitin in blood vessel wall. Senile plaques (SPs) in CAA cases showed strong ubiquitin positivity but the central amyloid core was negative. AD brains showed immunoreactivity with all antibodies in SPs and neurofibrillary tangles (NFTs); blood vessels were consistently negative for ubiquitin. Control brains showed few SPs and NFTs; these were positive for ubiquitin, but blood vessels were negative. These results indicate that vascular amyloid deposition in CAA and AD may have different pathophysiological mechanisms.

Adenosine Triphosphate↗
PubMed1992

Ultraviolet irradiation induced brain oedema in rats. A microgravimetric study.

The cerebral cortex of 36 anaesthetized Wistar rats were exposed to ultraviolet irradiation (UV-I) for 6 min through a 2 x 2 mm left parietal craniotomy. Animals were killed at different times and brains were removed immediately after death. Three consecutive coronal sections were obtained and sampled for gravimetric study. The density of the samples was measured using a continuous gradient of organic solvents. Gravimetric results showed significant differences between brain samples. The irradiated left hemisphere was less dense than the right one, and maximum differences in density were found in the medial coronal section. Grey and white matter oedema in the non-irradiated hemisphere was compared with the irradiated hemisphere. Early and delayed onset of odema was observed in both hemispheres but it was more marked in the irradiated hemisphere. In conclusion, brain oedema induced by ultraviolet irradiation in various animals is also reproducible in rats with all the advantages involved in the use of these experimental animals. Microgravimetric study correlated with topographical analysis using this model may lead to an understanding of some of the dynamic aspects of cerebral oedema.

Animals↗
PubMed1991

Brain lesions following combined treatment with methotrexate and craniospinal irradiation.

Eight patients with meningeal seeding by carcinoma or lymphomas were treated with intravenous (i.v.) and/or intrathecal (i.th.) Methotrexate (MTX). Seven patients received additional craniospinal irradiation and in all seven a fatal encephalopathy developed. On the bases of clinical and morphological findings we identified an acute and a delayed form of encephalopathy and concluded that the concurrent administration of Methotrexate and of craniospinal irradiation increases considerably the risk of brain damage.

Adult↗
PubMed1991

Characterization of the mononuclear cell infiltrate and HLA-Dr expression in 19 oligodendrogliomas.

We have studied frozen tissue from 19 oligodendrogliomas with a panel of antibodies to lymphocytes and their subsets, macrophages, natural killer cells, and HLA-Dr antigens. Macrophages were detected in moderate numbers in 60%-100% of tumors depending on the antibody used. T lymphocytes were fewer in number than macrophages and were present in 62% of cases. Most of the T lymphocytes were of the CD8 phenotype. CD4 lymphocytes were very few in number and present in only 18%. B cells and natural killer cells were absent from all cases. HLA-Dr antigens were expressed by macrophages in all cases but never on tumor cells. The implications of these findings are that macrophages and, to a lesser extent, CD8 lymphocytes are the predominant cells infiltrating oligodendrogliomas and that they may exert cellular immune functions.

Antibodies, Monoclonal↗
PubMed1991

Adult type of leukodystrophy. Krabbe's disease?

A 24-year-old man developed progressive dementia in seven years. The patient suffered a severe bronchopneumonia and eventually died few days later. Brain coronal sections showed a soft gray-brownish discoloration of white matter of centrum ovale but the subcortical arcuate fibers and the interne capsule were preserved. Microscopically, the white matter showed marked loss of myelin and oligodendrocytes, abundant hypertrophic astrocytes and numerous "globoid cells". The latter showed strong positivity in immunostains for a mouse monoclonal antigalactocerebroside antibody. The presence of these cells in the brain white matter might be the morphological basis to classify the present case as one of Krabbe's Leukodystrophy.

Adult↗
PubMed1991

Oligodendrogliomas: a clinical, histological, immunocytochemical and lectin-binding study.

We have studied 27 oligodendrogliomas with a panel of antibodies (vimentin, GFAP, S-100 protein, myelin basic protein, CAM 5.2) and of lectins (WGA, Con A, PNA, RCA, DBA, SBA) to different glycoproteins. There were 16 well-differentiated tumours, including one gliofibrillary and 11 anaplastic oligodendrogliomas, three of which were gliofibrillary. Four cases showed positivity for vimentin, three of which were anaplastic tumours. Fifteen cases were positive for S-100 protein (nine well-differentiated and six anaplastic tumours) and 13 contained GFAP-positive cells (three well-differentiated and 10 anaplastic tumours). WGA binding was positive in 75% of well-differentiated and 63% of anaplastic oligodendrogliomas, the corresponding figures were 50% and 45% for PNA, 37% and 81% for Con-A and 25% and 54% for RCA. On the basis of the results with lectin binding, we believe that there are changes in the spectrum of tumour cell-associated lectin-like proteins during malignant transformation. Our observations also suggest that the pattern of lectin expression can undergo substantial changes in the course of differentiation.

Adult↗
PubMed1989

Medulloblastoma. An immunohistological study of 50 cases.

Fifty paraffin-embedded medulloblastomas (31 in children and 19 in adults) were reacted with a panel of ten antibodies to glial, neuronal, mesodermal and epithelial antigens. The tumours were divided according to their histological features into three groups: classic, desmoplastic and highly vascular. Reactivity for glial fibrillary acidic protein was observed in 20 cases. Forty tumours reacted with PGP9.5 (neuronal marker) in clusters of poorly differentiated cells, cell cords and some scattered cells. Cells forming rosettes were mostly negative except for slight central reactivity. Eight of the 40 tumours contained neurofilaments. In scattered cells somatic reactivity for vimentin was found in 14 tumours. Ten cases showed positivity for S-100 with a nuclear and perinuclear pattern. No difference in reactivity in relation with age was observed. Desmoplastic medulloblastomas showed less reactivity for glial and neural markers. It was concluded that medulloblastoma shows degrees of differentiation as evidenced by the expression of various proteins. Differentiation occurs along two lines: glial and/or neuronal. Most tumours also contain a component of poorly differentiated cells which may differentiate into one of these two lines or act as primarily stem cells.

Adolescent↗
PubMed1989

The mononuclear cell infiltrate compared with survival in high-grade astrocytomas.

Frozen samples from 92 malignant astrocytomas were stained with a panel of monoclonal antibodies directed against macrophages and lymphocytes. A follow-up to death was available on 68 cases which form the basis of this study. Large numbers of macrophages were found in all cases; T lymphocytes, mostly of the CD8 phenotype were also seen in moderate numbers in 70% of cases. CD4-positive cells were present in small numbers in 32% and B cells were seen in only 8% of cases. Analysis of the survival showed no demonstrable correlation between the numbers of macrophages or CD4 lymphocytes and survival. The survival curves for parenchymal CD8 infiltration diverged after 9 months suggesting increased survival for those patients without such an infiltration but the difference failed to reach statistical significance (P = 0.37). No correlation between lymphocytic cuffing and survival was seen after studying all paraffin-embedded material. We conclude that there is no significant statistical correlation between survival and the various types of mononuclear cell infiltrating malignant astrocytomas.

Antibodies, Monoclonal↗
PubMed1989

Pelizaeus-Merzbacher disease with thiamine deficiency or Leigh disease with extensive involvement of white matter? Case report.

We report on a case of a 30-month-old child who presented with a clinical syndrome compatible with leucodystrophy and in whom neuropathological features of both Pelizaeus-Merzbacher disease and subacute necrotizing encephalopathy were shown. The significance of the neuropathological findings is discussed in the light of a possible coexistence of both diseases which has not previously been reported.

Brain↗
PubMed1989

Choroid plexus papillomas: an immunohistological study of 16 cases.

Eleven benign and five malignant choroid plexus papillomas in children and adults were studied immunohistologically with a panel of antibodies against glial fibrillary acidic protein, S-100 protein, vimentin, desmin, epithelial membrane antigen and two different cytokeratins (LP34 and CAM 5.2). Glial fibrillary acidic protein was focally present in epithelial tumour cells, in cells within solid areas and in clusters of cells within the stroma. S-100 protein was diffusely present in tumour cells with focal accentuation. Vimentin was present in all cases, the epithelial tumour cells demonstrating strong and diffuse positivity with perinuclear accentuation; malignant tumours, however, showed stronger positivity than benign ones. Desmin was negative in all tumours. Epithelial membrane antigen and cytokeratin (LP34) were demonstrated in four of five malignant tumours but were absent in the benign ones; CAM 5.2 reacted with four of five malignant tumours and also reacted with eight of the 11 benign ones. The significance of these findings is discussed in respect of the ontogeny of these tumours.

Adolescent↗
PubMed1988

Histologic characterization of 41 ependymomas with the help of a personal computer.

Fourty-one ependymomas were histologically analyzed in relation to patient age and sex and tumor location. A discriminant analysis model using Bayes' formula and a personal computer were employed. Ependymomas situated in the posterior fossa had a higher incidence in children. Ependymoblastomas were all situated above the tentorium and occurred only in young children. We identified three tumor groups on the basis of common histologic characteristics: benign ependymomas, anaplastic ependymomas, and ependymoblastomas. The main features useful for the grouping were the degree of differentiation and anaplasia. Ependymomas from the cauda equina showed histologic characteristics that allowed them to be differentiated from other benign ependymomas. In each group the particular histologic characteristics, age, and location were significant in terms of diagnosis and prognosis. This method of analysis may help to more precisely define ependymomas and may provide pathologists and clinicians with a quantifiable diagnostic tool that may be of help in establishing the appropriate treatment.

Adolescent↗
PubMed1988

Ependymoblastoma: a histological, immunohistological and ultrastructural study of five cases.

Five ependymoblastomas were studied by means of routine histological techniques, immunohistology and electron microscopy. The tumours were characterized histologically by medium sized, poorly differentiated cells with round or oval nuclei frequently in mitosis and by ependymoblastic rosettes. Reactions for cytokeratin and neurofilament were negative and tubular material positive for glial fibrillary acidic protein was scanty. All five tumors demonstrated positivity for vimentin and S-100 protein. Electron microscopy showed poorly differentiated cells with high nucleo-cytoplasmic ratio and scanty cytoplasmic organelles. Sparse rosettes were present and the cells were united by junctional complexes. Frequent rudimentary or incomplete cilia, a few basal bodies and a few short intercellular glial-like filaments were seen. Features differentiating ependymomas and anaplastic ependymomas from ependymoblastomas are discussed and the need for a definite category separating ependymoblastomas from the former tumours is emphasized.

Adolescent↗