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Biomedical subjects

P Cras

Publications and source records attributed to P Cras.

101 records · Page 6Linked to original sources
PubMed1985

Familial erythrophagocytic lymphohistiocytosis. A neuropathologic study.

The neuropathologic study of a 7-month-old female patient affected by familial erythrophagocytic lymphohistiocytosis (FEL) reveals three main features: (1) a lymphohistiocytic leptomeningitis with erythrophagocytosis; (2) perivascular lymphohistiocytic cuffs in the cerebral and cerebellar white matter and, to a lesser extent, in the thalamus, the reticular formation of the brain stem and the griseum pontis; (3) perifocal gliosis and demyelination, especially in the cerebellar white matter. The lesions remind of the perivenous post-infectious encephalomyelitides. Perifocal demyelination has been reported in FEL very seldom. Although its pathogenesis is not known, immune mechanisms could play a role by analogy with perivenous encephalomyelitis.

Arachnoid↗
PubMedDate not supplied

The role of cytokines, astrocytes, microglia and apoptosis in Creutzfeldt-Jakob disease.

In order to investigate inflammation and apoptosis in Creutzfeldt-Jakob disease (CJD) patients, we analyzed astrocytes, microglia and apoptotic neurons in brain and IL-1beta in cerebrospinal fluid (CSF). Our results showed increased numbers of astrocytes in CJD and increased numbers of microglia and apoptotic neurons both in CJD and Alzheimer's disease (AD) as compared to controls. All these markers correlated (P < 0.001) with the severity of the neuropathological lesions. An increased IL-1beta concentration was found in AD and CJD CSF that correlated with the number of microglia and which did not change in the disease course of CJD.In conclusion, apoptotic neurons in CJD correlates to the neuropathological lesions and are probably related to the presence of inflammatory cells and cytokines which are present during the whole CJD disease process.

Aged↗
PubMedDate not supplied

Failure of familial Alzheimer's disease to segregate with the A4-amyloid gene in several European families.

The gene coding for the amyloid protein, a component of neuritic plaques found in brain tissue from patients with Alzheimer's disease, has been localized to chromosome 21, and neighbouring polymorphic DNA markers segregate with Alzheimer's disease in several large families. These data, and the association of Alzheimer's disease with Down's syndrome, suggest that overproduction of the amyloid protein, or production of an abnormal variant of the protein, may be the underlying pathological change causing Alzheimer's disease. We have identified a restriction fragment length polymorphism of the A4-amyloid gene, and find recombinants in two Alzheimer's disease families between Alzheimer's disease and the A4-amyloid locus. This demonstrates that the gene for plaque core A4-amyloid cannot be the locus of a defect causing Alzheimer's disease in these families. These data indicate that alterations in the plaque core amyloid gene cannot explain the molecular pathology for all cases of Alzheimer's disease.

Adult↗
PubMedDate not supplied

Recent developments in Alzheimer's disease.

Alzheimer's disease is the most frequent form of dementia and it is estimated that its prevalence will quadruple by the year 2050. In the past decade, a number of important new developments have provided insight in the pathogenesis, improved diagnosis and allowed therapy of dementia. Several new mutations in the amyloid protein precursor gene, presenilin-1 and -2 genes and the influence of the apolipoprotein E gene isotypes on the disease phenotype have been described. The role of secretases in the generation of amyloid in senile plaques has been determined and this may provide important new therapeutic approaches in the future. The role of vascular lesions in the development of dementia and relationship with the Lewy body variant of Alzheimer's disease have been refined. Acetylcholine is deficient in Alzheimer's disease and can be supplemented in part by treatment with acetylcholinesterase inhibitors. Recently, surprising results of vaccination with amyloid in a transgenic mouse model have opened a completely new perspective in the prevention and treatment of Alzheimer's disease.

Aged↗
PubMedDate not supplied

Malignant pigmented spinal nerve root schwannoma metastasizing in the brain and viscera.

A 46-year-old woman suffered a meningococcal meningitis followed by a rapidly progressive lumbosacral pluriradicular syndrome. Myelography showed multiple nodules on the lumbar radices. A biopsy showed tissue with numerous Verocay-like bodies, spindle shaped and lymphocytoid cells which was diagnosed as schwannoma. There was a small group of polygonal cells with somewhat irregular and hyperchromatic nuclei. Postoperatively, she developed intracranial hypertension and died. CT scan and MRI revealed multiple occipital lesions consistent with metastases. At autopsy the cauda equina showed multiple nodular lesions with morphology comparable to the biopsy. However, pigment producing cells were also present. There were metastases with distinct morphological features in the brain, myocardium, thyroid gland and pancreas. Some consisted of pigmented, large, pleomorphic cells, others of non-pigmented, spindle-shaped and less pleomorphic cells. In this case, the diagnosis of metastasizing pigmented schwannoma is the most plausible hypothesis.

Brain Neoplasms↗
PubMedDate not supplied

Primary thalamic malignant fibrous histiocytoma of the dominant hemisphere causing severe neuropsychological symptoms.

A 45-year-old man with a malignant fibrous histiocytoma (MFH) of the left thalamus is reported. This tumor only rarely originates within the brain. Primarily intracerebral MFH usually has a superficial localization and rarely involves the deep cerebral structures. Our patient presented with complex neuropsychological symptoms, including aphasia, hemispatial neglect and acalculia. These findings are further supporting evidence for the role of the thalamus, particularly of its posterior parts, in language, speech and other neuropsychological functions.

Anomia↗
PubMedDate not supplied

Primary intracerebral lymphoma in systemic lupus erythematosus treated with immunosuppressives.

A 38-year-old woman suffering from systemic lupus erythematosus was treated with cyclophosphamide and prednisone. Two months later she developed mononeuritis multiplex. After one year, she showed a personality change with aggressiveness and paranoid delusions. Grand mal epileptic fits began to occur. An unenhanced CT-scan showed extensive and bilateral frontal hypodense lesions originally ascribed to vasculitis-caused infarcts. On autopsy, a primary intracerebral lymphoma with extensive necroses was demonstrated. The relationship between SLE, immunosuppressive treatment and primary intracerebral lymphoma is discussed.

Adult↗
PubMedDate not supplied

Eosinophilia-myalgia syndrome: a clinicopathological study of four patients.

Eosinophilia-myalgia syndrome is an idiopathic disorder characterized by myalgia, especially of the extremities and peripheral eosinophilia. It is found in some patients as a result of tryptophan ingestion. We examined four patients showing clinical features and muscle biopsy changes consistent with eosinophilia-myalgia syndrome. The results of conventional histology were compared with immunohistochemical studies using monoclonal antibodies to human T lymphocytes, macrophages, HLA-DR antigen. Our findings confirm the potential importance of lymphocytes and macrophages in this syndrome. In particular, new observations are presented concerning the immunoreactivity of HLA-DR antigen.

Adult↗
PubMedDate not supplied

Cerebral complications of murine monoclonal CD3 antibody (OKT3): CT and MR findings.

Treatment of acute renal allograft rejection with mouse monoclonal antibody (OKT3) is associated with systemic and neurologic side effects. We describe cerebral abnormalities in a 13-year-old boy with steroid-resistant renal allograft rejection. After treatment with OKT3, an acute neurologic syndrome developed, including seizures, lethargy, and decreased mental function. CT and MR imaging revealed confluent cerebral lesions at the corticomedullary junction. Contrast-enhanced MR images showed patchy enhancement, indicating blood-brain barrier dysfunction. The diagnosis of OKT3-induced encephalopathy with cerebral edema and capillary leak syndrome was made. Although CT and MR findings are nonspecific, neuroradiologists should be aware of this condition in transplant patients treated with OKT3.

Adolescent↗