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Biomedical subjects

D Troost

Publications and source records attributed to D Troost.

At least 109 records · Page 6Linked to original sources
PubMed1990

Polar spongioblastoma: an immunohistochemical and electron microscopical study.

A case is reported of a 9-year-old boy with a cerebral polar spongioblastoma. This neoplasm, first described by Russell and Cairns in 1947, is morphologically a distinct entity characterized by bipolar tumor cells with palisading nuclei. In the case under study immunoreactivity for neuron-specific enolase was found and ultrastructural features of developing neuronal elements were present. A neuro-endocrine nature was suggested by de Chadarévian et al. (1984) in a morphologically similar case. These findings are in contrast with the long-held view that the polar spongioblastoma is cytogenetically related to the embryonal radial glial cells.

Astrocytoma↗
PubMed1990

Immunohistochemical characterization of the inflammatory infiltrate in amyotrophic lateral sclerosis.

In order to test the hypothesis that the immune system plays a role in the pathogenesis of amyotrophic lateral sclerosis (ALS), the cellular composition of the spinal cord inflammatory infiltrate was analysed in eight cases of sporadic ALS by a panel of monoclonal antibodies. The majority of the many diffusely scattered lymphocytes seen in the anterior and lateral corticospinal tracts and anterior horns belonged to the suppressor/cytotoxicity T-cell subset and were admixed with variable numbers of macrophages. Helper-inducer T-cells were rare and B-cells were conspicuously absent. Compared to controls, ALS specimens exhibited an increase in major histocompatibility complex (MHC) products or human leucocyte antigens (HLA) in the corticospinal tracts and anterior horns. HLA-ABC antigens were expressed in the honeycomb pattern of the glial matrix of the spinal cord, and HLA-DR antigens were strongly expressed by large dendritic cells. In addition, macrophages and endothelial cells were labelled by HLA-DR. These findings suggest that an autoimmune process or infectious agent may play a role in ALS.

Adult↗
PubMed1990

Therapy for cytomegalovirus polyradiculomyelitis in patients with AIDS: treatment with ganciclovir.

Six AIDS patients with progressive cytomegalovirus (CMV) polyradiculomyelitis were treated with ganciclovir in an open study. The diagnosis was based on the presence of a distinct clinical syndrome with progressive flaccid paraparesis, preserved proprioception and urinary retention with specific cerebrospinal fluid (CSF) findings. Ganciclovir therapy, 5-10 mg/kg per day, instituted 3-6.5 weeks after onset of symptoms, was ineffective in four patients with severe paraparesis. One patient developed CMV polyradiculomyelitis while receiving ganciclovir and further deteriorated during foscarnet therapy. One patient however, showing minor paresis of one leg, improved after institution of ganciclovir therapy 1 week after onset of symptoms. It is concluded that a presumptive diagnosis of CMV polyradiculomyelitis can be made on the basis of distinct clinical findings and CSF pleocytosis with predominance of polymorphonuclear leukocytes in patients with AIDS. Ganciclovir therapy does not appear to be beneficial for patients with advanced paresis in the doses used. Further investigations are needed in order to determine if early intervention with ganciclovir, when paresis is mild, or higher doses in advanced paresis, might be of some benefit.

Acquired Immunodeficiency Syndrome↗
PubMed1990

Predominance of polymorphonuclear leukocytes in cerebrospinal fluid of AIDS patients with cytomegalovirus polyradiculomyelitis.

Cytomegalovirus (CMV) polyradiculomyelitis was diagnosed in 4 of 241 consecutive neurologically assessed human immunodeficiency virus type (HIV-1) seropositive patients. CMV-related neurologic disease was suspected on clinical grounds and was subsequently confirmed by CMV culture from cerebrospinal fluid (CSF) and/or CMV in situ hybridization on specific specimens. All four patients showed CSF pleocytosis with predominance of polymorphonuclear leukocytes (PMNs). Retrospective analysis of the results of CSF examination, performed in 143 of 241 patients with neurologic symptoms, showed pleocytosis in 58 of 143 patients. Predominance of PMNs was found in seven patients, including the four with CMV polyradiculomyelitis. It is concluded that in HIV-1 seropositive patients with a clinical diagnosis of polyradiculomyelitis, a predominance of PMNs in CSF could be an indication that the condition is CMV related. This should lead to early diagnosis and institution of specific antiviral therapy.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Multifocal rhabdomyoma of the neck. Report of a case studied by fine-needle aspiration, light and electron microscopy, histochemistry, and immunohistochemistry.

A 32-year-old Caucasian man presented with a tumor in the right side of the neck. Preoperative fine-needle aspiration showed large multinucleated cells with abundant granular cytoplasm that were consonant with the features of a granular cell tumor. At surgery, two separate tumors were found. Histologically, the tumor proved to be an adult rhabdomyoma, the 11th such multifocal case reported. The striated muscle origin of this benign tumor was confirmed by immunohistochemical and ultrastructural studies. The tumor cells were desmin-, myoglobin-, and actin-positive. They showed variable numbers of thick and thin filaments, as well as hypertrophic Z-band material. Histochemical studies showed the presence of basophilic muscle cells, vesicular nuclei, ragged red fibers, and diffuse acid phosphatase positivity. These features, together with the absence of actual muscle cell proliferation and the assumption that the mass of the tumor could be explained by the enormous swelling of the muscle cells, lead us to conclude that an adult rhabodomyoma is merely the result of a process of disorderly degeneration and regeneration rather than a real neoplasm or a hamartomatous lesion.

Acid Phosphatase↗
PubMed1988

Oligodendroglioma. A comparison of two grading systems.

In order to compare the grading system for oligodendrogliomas described by M.T. Smith (1983) with the conventional grading system according to Kernohan (1938), specimens from 72 patients were graded according to both systems, and survival times of the patients were compared. Survival rates decline in older patients. No interaction between the age of the patient and the degree of the tumor was found. No influence of localization of the tumor on survival was found. Similar to the system of Kernohan, the grading system of Smith distinguishes between only three groups of patients with significantly different survival times. In Smith's Grade A and Kernohan's Grade 1 the longest survivals are found; while in Smith's Grade D and Kernohan's Grade 4 the shortest survivals are found. Smith's Grades B and C as well as Kernohan's Grades 2 and 3 were intermediate with respect to the survival times of the patients and did not significantly differ from each other. With the independently significant features (cell density, pleomorphism, and necrosis) evaluated according to simple on-off scoring, and with the reduction from four grades to three, the grading system according to Smith would provide a simple and good, concise grading system for oligodendrogliomas of the brain.

Age Factors↗
PubMed1988

Enterogenous cyst of the brainstem--a case report.

A four-year-old child with an enterogenous cyst located lateral to the brainstem is reported. The endodermal origin of the cyst was histologically and immunohistochemically established. The unusual localization of the cyst as well as its destructive biological behavior as a result of inflammation is discussed.

Brain Diseases↗
PubMed1987

Gliomatosis cerebri, report of a clinically diagnosed and histologically confirmed case.

Pathological criteria for diffuse gliomatosis are clearly demarcated but its clinical diagnosis has always been hampered by the imaging techniques used. Although with the advent of CT scanning clinical possibilities were improved, isodense brain lesions are difficult to detect and in these cases the diagnosis gliomatosis cerebri can only be suspected from the clinical signs and the absence of radiological signs. We have studied a young patient with a peculiar clinical history, in whom comparison of the CT-scan and NMR scan yielded remarkable results. Our clinical diagnosis of (diffuse) gliomatosis was confirmed by biopsy.

Adolescent↗
PubMed1986

Maturation of a primitive neuroectodermal brain tumor? A case study with some remarks on the classification and nomenclature of 'primitive' CNS tumors.

A brain tumor in a 4-year-old child is described. The neoplasm was partly cystic and showed an a-typical multi-differentiated aspect. Microscopically the neoplasm had a clear-cut 'malignant' morphology. This tumor represents possibly a partly maturated primitive neuroectodermal brain tumor. The term PNET is briefly discussed in relation to the clinical implications.

Brain Neoplasms↗
PubMed1984

Cerebral calcifications and cerebellar hypoplasia in two children: clinical, radiologic and neuropathological studies--a separate neurodevelopmental entity.

Two siblings with cerebral calcifications are described, clinically characterized by the early onset of general regression and epileptic seizures, followed by cerebral blindness and spastic tetraplegia. No remarkable biochemical abnormalities were found. Death ensued before the age of seven years. Radiology of the skull in the second case showed widespread calcifications, situated periventricularly, in the basal nuclei and in the cerebellum. In both cases postmortem examination revealed widespread calcifications, corpus callosum hypoplasia and hydrocephalus "a vacuo". Moreover retrocerebellar leptomeningeal "cysts" and hypoplasia of the cerebellum were found. A diffuse lack of axons appeared to be the main cause of the poor staining of the white matter. The diagnostic criteria are neurological regression, progressive calcification, hydrocephalus and hypoplasia of the corpus callosum and cerebellum. Five similar cases were found in the literature. Our cases and those from the literature probably belong to a separate entity, originally published by Laubenthal et al (1940).

Brain Diseases↗
PubMed1982

Menkes' kinky hair disease. II. A clinicopathological report of three cases.

The neuropathologic abnormalities in three new cases of Menkes' kinky hair disease are described. Principally the three cases were the same. Hypoplasia of the cerebellum, with a basal arachnoïdal cyst, was present in all three cases. The cysts were not described before in Menkes' disease. There was nerve cell loss and gliosis in the cerebral cortex, cerebellum and thalamus. The reduction of myelinated axons was widespread and the disease does not belong to the leukodystrophies. Cortical lamination disturbances were present indicating that the disease develops as early as the sixth fetal month. Abnormal arborization of Purkinje cells with swelling of dendrites was present and thought not to be identical with the Purkinje cell abnormalities seen in amaurotic idiocy. The difference in severity of the copper deficiency in 2 patients is compared with the situation in "brindled" and "blotchy" mutant mice.

Brain↗
PubMedDate not supplied

Alternative splicing of glutamate transporter EAAT2 RNA in neocortex and hippocampus of temporal lobe epilepsy patients.

RATIONALE: Altered expression of glutamate transporter EAAT2 protein has been reported in the hippocampus of patients with temporal lobe epilepsy (TLE). Two alternative EAAT2 mRNA splice forms, one resulting from a partial retention of intron 7 (I7R), the other from a deletion of exon 9 (E9S), were previously implicated in the loss of EAAT2 protein in patients with amyotrophic lateral sclerosis. METHODS: By RT-PCR we studied the occurrence of I7R and E9S in neocortical and hippocampal specimens from TLE patients and non-neurological controls. RESULTS: Both splice forms were found in all neocortical specimens from TLE patients (100% I7R, 100% E9S). This was significantly more than in controls (67% I7R, 60% E9S; P < 0.05). We also detected I7R and E9S in all seven motor cortex post-mortem samples from patients with amyotrophic lateral sclerosis. Within the TLE patient group, both splice variants appeared significantly more in non-sclerotic (100%), than in sclerotic hippocampi (69%, P < 0.05). CONCLUSION: These data indicate that the epileptic brain, especially that of TLE patients without hippocampal sclerosis, is highly prone to alternative EAAT2 mRNA splicing. Our data confirm that the presence of alternative EAAT2 splice forms is not disease specific.

Adult↗
PubMedDate not supplied

Delusional misidentification and subsequent dementia: a clinical and neuropathological study.

An 81-year-old man presented with psychotic episodes involving the delusion that his wife had been replaced by a closely resembling double (the so-called Capgras delusion). A comprehensive neuropsychological investigation revealed no signs of dementia at that time, but in the subsequent course the patient became demented and died 5 years later. Neuropathological and neurochemical examination displayed many diffuse plaques in the cortex that escaped detection by Congo-red but were clearly demonstrated by antibodies raised to beta-amyloid protein. Neurofibrillary tangles were absent in the cortex. Antiubiquitin immunohistochemistry revealed some cortical and subcortical Lewy bodies. The hippocampus and the majority of the nigral cells were intact. However, in the frontal and parietal white-matter many small lacunar infarcts were found due to arteriosclerotic and hypertensive vessel wall changes. Though the etiology of psychotic symptoms in the context of dementia is complex, this study shows the possible significance of white-matter lesions in the pathogenesis of delusions and subsequent dementia.

Aged↗