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Biomedical subjects

E Reske-Nielsen

Publications and source records attributed to E Reske-Nielsen.

At least 19 recordsLinked to original sources

Stereological estimates of nuclear volume and other quantitative variables in supratentorial brain tumors. Practical technique and use in prognostic evaluation.

The use of morphometry and modern stereology in malignancy grading of brain tumors is only poorly investigated. The aim of this study was to present these quantitative methods. A retrospective feasibility study of 46 patients with supratentorial brain tumors was carried out to demonstrate the practical technique. The continuous variables were correlated with the subjective, qualitative WHO classification of brain tumors, and the prognostic value of the parameters was assessed. Well differentiated astrocytomas (n = 14) had smaller estimates of the volume-weighted mean nuclear volume and mean nuclear profile area, than those of anaplastic astrocytomas (n = 13) (2p = 3.1.10(-3) and 2p = 4.8.10(-3), respectively). No differences were seen between the latter type of tumor and glioblastomas (n = 19). The nuclear index was of the same magnitude in all three tumor types, whereas the mitotic index was significantly increased in glioblastomas (2p = 0.01). Three-dimensional, shape-independent estimates of macroscopical tumor volume were not different in anaplastic astrocytomas and glioblastomas (2p = 0.39). Histological type of tumor and mitotic index were of significant prognostic value (2p = 8.2.10(-6) and 2p approximately 0.05, respectively). Age above the median and short duration of symptoms were significantly associated with short survival (2p = 0.01). Further investigations of larger series of patients are needed to define the clinical usefulness of these objective, reproducible, and quantitative techniques in the prognostic evaluation of primary brain tumors.

Adolescent

Juvenile ceroid-lipofuscinosis and calcifications of the CNS.

The post-mortem brains and spinal cords of 20 juvenile ceroid-lipofuscinosis (JC-L) cases from 1973 to 1987 were investigated. Clinical course of the disease was characterized by impaired vision from the age of 5-8 years, progressive dementia, seizures, somatic retardation, and early death (16-29 years of age). Microscopy showed classic intracytoplasmic autofluorescent lipopigment in the nerve cells throughout the CNS and viscera. Immunoperoxidase staining for glial fibrillary acidic protein (GFAP) showed marked gliosis with enlarged reactive astrocytes mainly in the superficial layers of the cerebral cortex. Calcifications of the nervous system principally along the outer and inner brain surfaces were demonstrated by X-ray, macroscopic examination and microscopy. We suggest that the calcifications are secondary to a suspected generalized metabolic error.

Adolescent

Central nervous system calcifications following tap water rinsing in autopsy material from children. A pitfall.

CNS from 80 children collected consecutively at the Institute of Forensic Medicine, Aarhus, were investigated. Owing to peculiar calcifications of the first 41 CNS examined, we gradually came to suspect that some external factors had influenced the results. Different fixation methods used on the CNS, rinsed or not rinsed in tap water, led us to suggest that the relatively high calcium content in the tap water in our part of the country was the cause. The non-pathologic calcification could presumably mimic metabolic diseases.

Autopsy

The concentration of the Na,K-pump in skeletal and heart muscle in congestive heart failure.

Na,K-ATPase (or the Na,K-pump) is essential for excitability and contractility of muscle tissue. Previous studies have shown a decrease in the concentration of this pump in endomyocardial biopsies from patients with dilated cardiomyopathy. The effect of congestive heart failure on the concentration of Na,K-ATPase in skeletal muscle was assessed in 16 patients by measurement of binding of 3H-ouabain to biopsies of the vastus lateralis muscle. Ten patients had impaired left ventricular function with an ejection fraction of 0.32 +/- 0.03 and a concentration of the Na,K-pump of 229 +/- 15 pmol/g wet weight in the skeletal muscle, whereas 6 patients had an ejection fraction of 0.66 +/- 0.05 (P less than 0.001) and a concentration of 307 +/- 17 pmol/g wet weight (P less than 0.01). In endomyocardial biopsies, the concentration of Na,K-ATPase was 340 +/- 37 and 500 +/- 39 pmol/g wet weight (P less than 0.025) in patients with impaired and normal ventricular function, respectively. There was a significant correlation between the concentration of the Na,K-pump in the biopsies of the skeletal muscle and ejection fraction, as well as between its concentration in the endomyocardial and skeletal muscular biopsies (r = 0.56, P less than 0.025 and r = 0.72, P less than 0.005, respectively). The decrease in concentration of the pump in skeletal muscle may contribute to the limitation of exercise capacity in congestive heart failure.

Adult

Distribution of dietary mercury in a dog. Quantitation and localization of total mercury in organs and central nervous system.

An Alsatian dog which had been fed fish contaminated with methyl mercury for 7 years was examined after its death at the age of 12, 4 years after the exposure to methyl mercury had ceased. Two dogs of the same age and breed served as controls. In the exposed dog, mercury was found in all of the organs examined; the highest concentrations were found in the kidneys, and the lowest in the gastrointestinal tract and skeletal muscles. In the central nervous system (CNS) the mercury was fairly uniformly distributed, with 93% in the inorganic state, whereas the skeletal muscles contained approximately 30% inorganic mercury. This demonstrates time-dependent demethylation and suggests a variation in the rate from one type of tissue to another. At the time of death, the mercury level in the dog was still falling. In the control dogs, detectable amounts (0.01 mg kg-1) of mercury were only found in the kidney and liver. The distribution of mercury was determined by a histochemical method (autometallography) for locating mercury in tissue sections. Sections from autometallography of the central nervous system showed large deposits of mercury in all areas of the cerebral hemispheres, the brainstem and the spinal cord, including nerve cells, astrocytes, microglial cells and vessel walls. The granular layer of the cerebellar hemispheres was especially loaded, while only a few granules were present in the Purkinje cells. In the leptomeninges the vessels and the macrophages were heavily encrusted. High amounts of histochemically demonstrable mercury were observed in the liver, thyroid gland and kidney. In the control dogs, all the organs examined were practically devoid of deposits.

Animals

Deficiency of reticular fibers in cerebral arteries. On the etiology of saccular aneurysms in childhood.

A 5-year-old, previous healthy, girl developed a large hematoma of the right cerebral hemisphere and died. An aneurysm was strongly suggested. As previously found in adult aneurysm patients, histological and morphometric investigations showed an abnormality of the reticular fibers of the cerebral arteries. We suggest that arterial deficiency of reticular fibers is of importance in formation and rupture of saccular aneurysm, also in childhood.

Age Factors

X-linked Duchenne muscular dystrophy. Motor functions and prognosis.

69 patients with x-linked Duchenne Muscular Dystrophy (DMD) were included in a retrospective investigation from 1975-1986. A mean profile of the decline of the motor functions was made by using the median age at which the patients were unable to perform specific motor functions by request. It was found that 81.2% of the personal profiles followed the pattern shown in the mean profile of the disease and that DMD is progressing in a uniform way, but with a different progression rate in every DMD patient. Good correlation between two motor functions made it possible to predict a personal prognosis of the decline of the motor functions in the DMD patient. No correlation between late walkers (later than 18 months) and rapid progression was found.

Chromosomes

Calcification of the central nervous system in a new hereditary neurological syndrome.

A case of a new hereditary neurological condition with extensive calcifications of the central nervous system is described. The calcium deposits were especially localized to the leptomeninges, the first layer of the cerebral and cerebellar cortex, and along the ventricular wall. The neuropathological findings were in accordance with the clinic. The case was familial and the pedigree suggested an X-linked recessive inheritance.

Adult

Lafora's disease and brain calcifications.

The brain from a 22-year-old man with progressive myoclonal epilepsy (Lafora's disease) was examined. Besides widespread inclusion bodies in the nerve cells calcifications were seen on the inner and outer surface of the brain. No gliosis was present but the astrocytes were enlarged. They were unrelated to the inclusion bodies, as demonstrated in combined stainings with glial fibrillary acidic protein (GFAP)-PAS or GFAP-Bodian. A transport of an abnormal metabolic product is suggested.

Adult

Lethal autosomal recessive arthrogryposis multiplex congenita with whistling face and calcifications of the nervous system.

Three children from a sibship of four had congenital contractures, scarce facial expressions, central nervous system dysfunction, and early death. Extensive deposits of calcium compounds were found at postmortem examination of the nervous system and of skeletal muscle. The disorder in these sibs is presumably inherited as an autosomal recessive trait. The metabolic basis for the calcium deposition has yet to be discovered.

Arthrogryposis

Sudden death from cerebral cysticercosis.

A case of sudden death from cysticercosis cerebri is described. A 38-year-old Ecuadorian woman suffered from headache for a few weeks which was interpreted as migraine. Later she developed extremely severe headache with seizures and died. A forensic autopsy showed normal organs. Neuropathologic examination revealed cysticercosis racemosus with reactive chronic ependymitis and meningitis and secondary hydrocephalus.

Adult

A simple mechanical model using a piston to produce localized cerebral contusions in pigs.

A simple mechanical model using a piston to produce localized cerebral contusions in pigs, is presented. The precision and reproducibility of the method are described by the biomechanical and pathological results. There are only pathological changes with haemorrhage and laceration close to the place of entry of the piston. The changes in the physiological parameters also indicate that the damage is focal. In this model, when kept intact, the dura mater offers considerable protection as no pathological changes in the brain are observed even when the energy at the time of the contusion is increased to twice the values which, when the dura is open, cause considerable damage.

Animals

Postmortem Menkes diagnosis from carrier testing of female relatives.

A boy who died at 6 months of age was noted to have sparse, stubby and light hair, pili torti were observed microscopically, and his skin was dry and redundant. As a suspicion of Menkes disease was first raised after his death, serum copper and ceruloplasmin in serum were not measured. Unfortunately, no fibroblasts were available - only fixed and paraffin-embedded samples of brain, spleen and liver. The copper contents of the brain and the liver were lower than in an age-matched control. Fibroblast cultures from the mother, the maternal grandmother, and a maternal aunt of the index patient were analysed for 64Cu-uptake. All these females showed the uptake values expected for Menkes carriers, thus supporting the clinical suspicion of Menkes disease in the index patient. From the above-mentioned results it was highly likely that the index patient had suffered from Menkes disease. Adequate genetic counseling could thus be offered to the family, and in the next pregnancy a first trimester prenatal diagnosis was performed.

Brain Chemistry

Familial centronuclear myopathy: a clinical and pathological study.

A congenital myotubular myopathy in a family is presented. An elderly woman, her daughter and her granddaughter showed the characteristic clinical and histological pattern of the disease. It is still a matter of debate whether the disease is of myopathic or neuropathic origin. The similarity of the muscle fibre with a myotube of the fetus might point towards an arrest of the maturation after 20th week of gestation perhaps caused by a lack of a trophic factor from the motor nerve.

Adult

Histological and morphometric observations on the reticular fibers in the arterial beds of patients with ruptured intracranial saccular aneurysms.

Histological and morphometric studies on the collagenous, reticular, and elastic fibers of the tunica media were performed on the middle cerebral and brachial arteries of patients with ruptured intracranial saccular aneurysms and controls. There was no difference between aneurysm patients and controls in the amount of collagenous and elastic fibers. The amount of reticular fibers was, however, reduced about 35% in aneurysm patients within both the middle cerebral artery (P less than 0.01) and the brachial artery (P less than 0.02). The reticular fibers were irregularly distributed and often appeared shorter and coarser than normally. The observations point to a generalized abnormality of the arterial bed in some patients with rupture of saccular aneurysms.

Adult

Astrocytes in the prenatal central nervous system. From 5th to 28th week of gestation. An immunohistochemical study on paraffin-embedded material.

The CNS from 30 normal fetuses aged 5-28 weeks were studied in GFAP stained paraffin-embedded material. The technique of preparation, autopsy and fixation is described in details. GFAP reacting glial cells developed first in the spinal cord at 7 weeks, and appeared in all regions of CNS during fetal life in a systematic way but with a temporal variation. The supporting and guiding properties of the fibrillary astrocytes are stressed.

Astrocytes