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R Beckmann

Publications and source records attributed to R Beckmann.

At least 91 records · Page 5Linked to original sources

Second and third visual areas of the cat: interindividual variability in retinotopic arrangement and cortical location.

1. The cortical location and the retinotopic arrangement of the second (V2) and third (V3) visual areas in the cat have been investigated with single and multiple unit recordings in anaesthetized and immobilized animals.2. V2 and V3 are arranged side by side anterior and medial to V1 and occupy the lateral gyrus and the postlateral sulcus. In addition, V2 spreads to postlateral parts of the lateral sulcus and, occasionally, to the posterior suprasylvian gyrus. The contralateral lower hemifield is represented on the lateral gyrus, the area centralis and the horizontal meridian are found in most animals in the anterior part of the postlateral sulcus, and the representation of the upper hemifield occupies the posterior part of the postlateral sulcus.3. The detailed retinotopic arrangement of the visual field maps shows two characteristic features. First, the retinotopy at the V2/V3 border differs between lower and upper hemifield. In the lower hemifield the periphery of the fields is represented, whereas in the upper hemifield the border between the representations is formed by a sector running along the horizontal meridian about 5-10 degrees in the upper hemifield. Thus the lower field arrangement resembles that of rodents, and the upper field arrangement is similar to that of primates. Secondly, the periphery of a part of the visual field is not continuously represented, but forms patches or islands (Donaldson & Whitteridge, 1977). The islands are bounded by visual field representations closer to the vertical meridian. The way the visual field is represented at the border between V2 and V3 introduces discontinuities into the visual field maps: adjacent parts of the visual field are not represented adjacently in these two prestriate areas.4. Cortical location and detailed retinotopic arrangement vary considerably from animal to animal, so that a representative map of V2 and V3 cannot be constructed. For example, the representation of the periphery of the horizontal meridian may be located either in the anterior portion of the postlateral sulcus or some mm more posteriorly, where the sulcus turns laterally. The representation of the area centralis in V3 is found either at the transition zone between lateral and postlateral sulcus, on the posterior suprasylvian gyrus, or in the posterior part of the postlateral sulcus.5. The entire hemifield is represented in V2 at least in some animals. In V3 the uppermost part of the vertical meridian seems not to be represented. In other animals only a restricted part of the contralateral visual field is represented in V2 or in V3. In these cases the receptive fields cover not more than 50 degrees out in the lower hemifield or on the horizontal meridian. In a few cases the periphery of the horizontal meridian and the upper hemifield are not at all represented in V3, or only in an incomplete manner.6. The magnification factors (Daniel & Whitteridge, 1961) become progressively smaller from V1 to V2 to V3. Hence cortical volume occupied decreases from V1 to V3. In V1 and in V2 the magnification is highest along the lower vertical meridian. In V2 the magnification along the horizontal meridian is the smallest, whereas in V1 the magnification decreases progressively from the lower vertical, to the horizontal and to the upper vertical meridian. The relationship between retinal ganglion cell densities and cortical magnification factors is discussed.

Animals↗

[Screening for elevated creatine kinase activities for the early diagnosis of Duchenne muscular dystrophy].

A screening test for the determination of creatine kinase in a dry spot of the whole blood is used for the early identification of boys with Duchenne muscular dystrophy and girls with carrier properties of this hereditary disease. In the absence of an effective medical therapy, such screening leads to genetic counselling of the affected families with the purpose of avoiding the birth of further cases of Duchenne muscular dystrophy in the same families. The first results of a voluntary screening program in Germany are discussed.

Creatine Kinase↗

[Expeimental studies in animals on a new lipid lowering compound: etiroxate hydrochloride (author's transl)].

D,L-alpha-Methyl-thyroxin-ethylester hydrochloride (etiroxate hydrochloride, CG 635, Skleronorm) has been proved to be highly effective in lowering serum lipids in rats. Even daily oral doses of 3.3 mumol etiroxate hydrochloride/kg (2.8 mg/kg) decrease serum cholesterol significantly in hypercholesterolemic rats. From a dose of 10 mg/kg upwards etiroxate hydrochloride also significantly reduces serum triglycerides. Etiroxate hydrochloride has much less effect on oxygen consumption, heart rate and heart weight of rats than have L-thyroxin and D-thyroxin, and its antigoitrogenic effect is also much slighter. As calculated from the ratio between relative effect on basal metabolism and relative effect on serum cholesterol, the relative therapeutic index of the compound is 10--35 in comparison to a relative therapeutic index of 1 for L-thyroxin and D-thyroxin.

Animals↗

[Cardiomyopathy in Duchenne muscular dystrophy. Part 2: serum enzymes, vector-cardiography, and echo-cardiography in 143 patients (author's transl)].

To diagnose a possible latent or manifested cardiomyopathy, 143 male patients between 2 and 21 years of age with confirmed Duchenne muscular dystrophy were examined for serum enzymes, by electrocardiography, vector-cardiography, and echo-cardiography. The results contain information on 1. the quantitative cellular myocardial degeneration process, 2. the disturbed cellular depolarization and nerve-conduction processes in the area of the right and left ventricular myocard, and 3. the disturbed left ventricular function which, in the initial state, is only documented by a discrete decrease of contractility caused by a manifested decreased ejection output. The pathological contraction and relaxation process of the heart muscle cell and its dependency on calcium ion transport as pathogenic background is discussed.

Adolescent↗

Creatine kinase in human erythrocytes: a newly detected genetic anomaly.

In a family of Italian origin, we found four members with a considerable activity of creatine kinase inside their erythrocytes. All other clinical and hematological findings were normal. The enzyme anomaly seems to be inherited in the autosomal mode. The creatine kinase CK) activity in freshly drawn blood was about 12 U/g Hb. The activity was higher in young red cells than in older ones. Studies with specific antibodies against human CK isoenzymes revealed the CK activity in the probands' red cells to be due to about 90% to the BB-isoenzyme normally found in brain and nerve tissue. The presence of CK in the erythrocytes does not seem to have any consequences for the energy metabolism of the cells. Creatine concentration was slightly elevated, but creatine phosphate could not be detected.

Adult↗

[Liver disease in homozygous alpha1-antitrypsin deficiency (author's transl)].

Among twelve patients with homozygous alpha1-antitrypsin deficiency (Pi-type Z), five cases of infantile liver disease were diagnosed. The course of the disease was extremely variable; only one patient died of liver cirrhosis at the age of fourteen. In four cases the clinical, biochemical and histological (2 cases) findings became normal over a follow-up period of one to fifteen years. The results of these observations demonstrate that in alpha1-antitrypsin deficiency even when associated with proven liver disease the prognosis need not be unfavorable.

Adolescent↗

Freeze-fracturing studies of mitochondrial myopathy. A correlated clinical, biochemical and morphological investigation.

Freeze-fracture studies of pathologically changed mitochondria in situ from muscle biopsies of a 9.5-year-old girl with a mitochondrial myopathy were correlated with clinical, biochemical and histochemical investigations. In the ultrathin sections giant mitochondria with densely packed cristae membranes - often reoriented to concentric circles - and, in addition, paracrystalline mitochondrial inclusions were found. The freeze fracture faces of such transformed mitochondria and preparations of their inner and outer membranes provided a morphological insight in the macromolecular structure of the mitochondrial membrane under such pathological conditions. The results lead to the hypothesis that part of the transformed mitochondria stay active functionally for an extended period by maintaining the delimitation from the cytoplasm and by preserving the macromolecular membrane architecture. This hypothesis could explain the slow progression of the myogenic symptoms.

Child↗

Creatine kinase isoenzyme patterns in Duchenne muscular dystrophy.

Serum creatine kinase isoenzymes were studied in 41 patients suffering from Duchenne type muscular dystrophy and 20 mothers of patients (carriers) by cellulose acetate electrophoresis. Both the MM and MB types were found in all cases of Duchenne type dystrophy patients, and in carriers with highly elevated total creatine kinase activity BB was not observed above the detection limits of the methods used. However, a so-called atypical CK--BB band has been demonstrated.

Creatine Kinase↗

[Duchenne-type muscular dystrophy: problems, early diagnosis, early treatment (author's transl)].

The aetiology and pathogenesis of the Duchenne-type muscular dystrophy (pseudohypertrophic muscular dystrophy) are still largely unknown. The possibilities of treating the disease are rather limited. Treatment is the more successful, the earlier diagnosis was possible, and the earlier treatment is initiated. The CK-Screening Test is an important aid for early diagnosis. The CK-Screening Test is also valuable for genetic consultation and advice, because it helps to identify women who are conductors or carriers of the disease. Current hypotheses on aetiology and pathogenesis are mentioned. Progress made in the fields of biochemistry, including enzyme histochemistry, and electron microscopy, raise hopes of finding more efficient therapeutic possibilities in the future. The many interests of patients with muscular diseases are being looked after by the European Alliance of Muscular Dystrophy Associations (EAMDA). Thirteen European associations are members of this organisation, including the German association "Bekämpfung der Muskelkrankheiten e.V." The number of sponsoring members of the EAMDA is at present about 300,000. 3 international congresses have already been held on the problems of muscular diseases. The fourth congress is scheduled to take place in Montreal in 1978.

Cell- and Tissue-Based Therapy↗

Inclusion body myositis. A "slow-virus" infection of skeletal musculature?

We report the case of a 56-years old patient with clinical symptoms of an unresolved neuromuscular disease. The light microscopic studies of a muscle biopsy from the m. triceps shows the picture of a diffuse muscular atrophy. By electron microscopy, myelin-like degeneration zones with tubular-filamentous inclusions can be shown in the cytoplasma of the atrophic muscle cells. These filamentous structures correspond morphologically to the nucleocapside of paramyxoviruses. These results lead, even without the proof of inflammatory cells, to the diagnosis of an "inclusion body" myositis also taking into account the clinical and electrophysiological findings.

Biopsy↗