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Biomedical subjects

E Sluga

Publications and source records attributed to E Sluga.

At least 37 records · Page 2Linked to original sources
PubMed1983

Chronic idiopathic polyneuritis.

Clinical assessment, CSF changes, electrophysiologic findings, and nerve biopsy investigations are reported for ten cases of chronic idiopathic polyneuritis. Seven cases developed a progressive, three a relapse course. Motor deficit syndromes predominated, often with proximal weakness. Sensory signs were less frequent and less intense, although exceptions were noted. Elevated CSF total protein and a disproportional increase in IgG developed, which increased with duration of the disease. Nerve conduction velocity was markedly reduced. Nerve biopsies showed cell-mediated demyelination, simultaneous de- and remyelination, considerable alteration of Schwann cells, and long-persisting glycolipid-rich myelin degradation products. Particular features of chronic polyneuritis are delineated. The demyelinating process seems to be "self-perpetuating" and not monophasic as the acute forms. Schwann cells or their surface antigens seem to be targets of the immune response. Chronic polyneuritis responds to treatment with corticosteroids. Long-term treatment in combination with immunosuppressants and plasmapheresis is often required.

Adolescent↗
PubMed1982

[WPW syndrome combined with AV block 2 in an adult with glycogenosis (Type II)].

A 31 year-old female with a five year history of muscle weakness, cardiac palpitations and elevation of activity of some serum enzymes of muscular origin, showed signs of the WPW syndrome on ECG, often in combination with grade 2 A-V block. Type II glycogenosis (Pompe's disease) was diagnosed on the basis of the results of physical examination, laboratory findings--especially subtotal deficiency of acid maltase (a-1,4 glucosidase) activity-and morphological aspects of light and electron microscopy of a quadriceps muscle biopsy specimen. To our knowledge the coincidence of such a rarely encountered arrhythmia with glycogenosis type II in an adult has never been reported so far.

Adult↗
PubMed1980

[Differential diagnosis of ocular myopathies].

While the ocular myopathies were hitherto classified in various syndromes solely on the basis of symptom complexes, biochemical and electron-microscopic studies are leading to new findings in the pathogenesis of these conditions, which are probably enzymopathies. At the same time, with the aid of electron microscopy, a classification of the myopathies is attempted on the basis of the morphological changes seen in the affected cells. However, the occurrence of giant mitochondria is no longer characteristic for myopathies. An acumulation of lipid vacuoles in the cells is currently designated "sudanophilic myopathy", and is frequently found in cases suffering from a carnitine deficiency. However, it is still difficult to demonstrate the enzyme defect responsible for the disease. -- Two cases of "sudanophilic myopathy" are reported. At first, a carnitine deficiency was assumed, but this could not be proved in subsequent follow-ups; the condition was therefore probably due to another type of enzyme deficiency in these two cases.

Adolescent↗
PubMed1980

[Fatal hyperthermic crisis after premedication. Human stress syndrome or trigger effect of promethacine (author's transl)].

A case report of a fatal hyperthermic crisis after premedication and before introduction of anaesthesia is given. Based on the typical clinical course, CK isoenzyme patterns and preliminary results of familial investigation the diagnosis of malignant hyperthermia could be established. According to literature reports, psychic stress and/or phenothiazine medication may be assumed as triggering factors. Until a potential causality between malignant hyperthermia and phenothiazine medication can definitely be ruled out, their use in suspicious cases of susceptibility to malignant hyperthermia should be avoided.

Adolescent↗
PubMed1979

Primary intracranial malignant lymphomas a fine structural cytochemical and CSF immunological study.

Cytochemical and electron microscopical studies in six cases of primary intracranial malignant lymphomas, four of which were diagnosed by biopsy, confirmed their ultrastructural identity with extraneural malignant lymphomas classified as immunoblastomas and immunocytomas. Demonstration of transformation of 'blast-like' cells (immunoblasts) toward rough E. R. developing cells in all these tumours argue for the B-dell origin of these types of cerebral lymphomas, previously referred to as reticulum cell sarcomas/microgliomas. CSF studies showed increased levels of B cells with surface IgG in two cases of immunoblastoma, and increased IgG levels with light chains in two cases each immunoblastoma and of pleomorphic immunocytoma, while one case of immunoblastoma showed a normal T cell to B cell ratio with increased monoclonal and surface, IgM suggesting some morphological and immunological heterogeneity of these lymphomas.

B-Lymphocytes↗
PubMed1977

[Myocardial involvement in a special form of Erb's muscular dystrophy (type Becker-Kiener) (author's transl)].

A 22-year-old man with cardiac symptoms had been treated for myocarditis for two years. But the clinical course and special tests, including isoenzyme measurements, electromyography, peripheral muscle and myocardial biopsy, established the diagnosis of X chromosome-linked muscular dystrophy (type Becker-Kiener) with an associated cardiomyopathy (raised left-ventricular end-diastolic pressure). Light- and electron-microscopic studies of the (right ventricular) myocardial biopsy revealed degenerative changes like those seen in peripheral muscle.

Adult↗
PubMed1977

[Comparative bioptic and electrophysiological investigations in polyneuropathies (author's transl)].

Comparative bioptic and electrophysiological investigations were performed in 30 cases of polyneuropathy of varying aetiology. Axonal and demyelinating neuropathies were distinguishable concordantly on the basis of both investigations. Maximum motor nerve conduction velocity (NCV) and compound potential (CP) were regularly examined in several nerves of each patient. Low (NCV) values (critical limit 25 m/sec) and long-standing high CP values were characteristic of demyelinating processes, whereas axonal neuropathies showed a slight to moderate decrease in NCV with early decrease in CP, often rapidly followed by loss of nerve excitability. Nonclassifiable stages and diseases are discussed. In biopsies, ultrastructural examination in particular, not only allowed the differentiation of primary axonal and primary demyelinating lesions and the respective neuropathic syndromes, but permitted moreover, further classification of these heterogenous groups of neuropathies. According to the sequence of lesions of the nerve fibre classes on the one hand, and to the nature of the primary lesion of the nerve fibres on the other hand, the following types of neuropathies were distinguished: in axonal neuropathies of the myelinated fibres, dystrophic and disintegrative forms were differentiated; axonal neuropathies of unmyelinated fibres correlated with sensory neuropathies. Demyelinating neuropathies were represented by hypertrophic, leucodystrophic and paraproteinaemic forms. The correlation of these types of neuropathies to the different clinical and aetiological forms of polyneuropathies is diagrammatically shown. The connection between the electrophysiological data and the basic nerve fibre alterations of the different neuropathies is discussed.

Demyelinating Diseases↗
PubMed1977

[Ocular myopathy with giant mitochondriae (author's transl)].

A case of progressive external ophthalmoplegia and a slight coincident affection of the scapulohumeral muscle group is recorded. Thorough examination including EMG and biopsy from ocular and skeletal muscle tissue confirmed the clinical diagnosis of myopathy. By means of electron microscopy the affection could be identified as a type of myopathy characterized by giant mitochondriae. Causative factors were not detectable. The function of the thyroid and the carnitine level in the muscle tissue and blood serum were normal.

Biopsy↗
PubMed1976

Spastic paraplegia associated with Addison's disease: adult variant of adreno-leukodystrophy.

Clinical and pathological features of an adult variant of adreno-leukodystrophy (ALD) are presented. A male with clinical and laboratory signs of Addison's disease (AD) developed at age 22 a slowly progressing paraplegia with slight sensory deficits in both legs and bladder and sphincter dysfunctions; he died at age 24 in an AD crisis. Autopsy revealed hyperplasia of lymphatic tissues, lymphocytic infiltrates in various organs including the CNS and adrenocortical atrophy with prominence of large ballooned, sometimes bizarre and occassionally striated cortical cells. CNS lesions consisted in incomplete demyelination of long tracts of brain stem and spinal cord with accentuation in the pyramical tracts; in these areas, perivascular cuffs of "epitheloid" histiocytic cells contained a strongly PAS-positive non-sudanophilic material. Electron microscopy demonstrated massive stroge of leaflet structures in perivascular histiocytes identical to the lamellar profiles previously described as specific for ALD. Some leaflets were found in close contact with compact lamellar arrays and with an electron-dense fingerprint material within astrocytes. In our case, the spastic paraplegia-AD syndrome which has been described previously in several clinical observations could be neuropathologically classified as an adult variant of ALD. Several differences to "classical" ALD occurring in young boys are stressed: the predominance of the endocrine disorder probably accounting for some of the perivascular lymphocyte infiltrates within the CNS; the absence of both clinical and pathological signs of diffuse cerebral involvement and the peculiar topistic pattern of CNS lesions and the very slow evolution of neurological signs paralleled by the absence of active sudanophilic demyelinating lesions. The possible mechanism of demyelination and the nature of the suggested metabolic defect in ALD are discussed. The ultrastructurally prominent leaflet structures may originate from myelin remnants, thus relating ALD to pathological storage of a myelin degradation product.

Addison Disease↗
PubMed1975

[Slow-virus "encephalitis" following measles in a child with cytostatically-treated leukaemia (author's transl)].

A child with acute infantile lymphoblastic leukaemia in remission after cytostatic therapy contracted apparently uncomplicated measles. 8 weeks later, an acute neurological syndrome developed, which led to death within 2 weeks. At autopsy, a peculiar type of inclusion body "encephalitis" almost without signs of inflammatory infiltration was found. Electron microscopic examination showed changes of paramyxovirus infection, which were attributable to slow measles virus disease of the CNS. There was evidence of disturbed nucleocapside synthesis, which is considered to be one of the basic defects in the development of slow-virus processes and their altered viral maturation. Possible pathogenetic factors are discussed in the light of our case.

Cell Nucleus↗
PubMed1975

SSPE-like inclusion body disorder in treated childhood leukemia.

Clinico-pathological report on a boy with cytostatically treated leukemia, dying with cerebral symptoms after passing clinical measles 10 weeks before death. At autopsy, numerous nuclear inclusion bodies in glial and nerve cells were found. By electron microscopy, nuclear inclusions appeared as loosely arranged smooth tubules, corresponding to paramyxovirus nucleocapsids. Frequently, cytoplasmic changes appeared too, consisting of incomplete tubular structures and an abundant dense "fuzzy" material. No regular tubuli of the coated granular type were present, as in common measles virus infection, nor any mature viral structures or differentiation of the surface membrane. The lack of maturation in cytoplasm together with a predominance of nuclear changes suggested a slow type of measles virus infection, while the particular cytoplasmic changes suggested a defect in synthesis of granular nucleocapsids, possibly a basic factor for the slow type of the viral infection. Possible pathogenetic factors are discussed.

Brain↗