Biomedical subjects
E Sluga
Publications and source records attributed to E Sluga.
Tubulovesicular structures in Creutzfeldt-Jakob disease.
By electron microscopy tubulovesicular structures (TVS) have been consistently observed in brain tissue of transmissible spongiform encephalopathies such as natural and experimental scrapie, bovine spongiform encephalopathy and experimentally induced, but not naturally occurring, Creutzfeldt-Jakob disease (CJD). For the first time we report here the presence of TVS in human brains with CJD as detected by transmission electron microscopy. TVS were observed in all three CJD specimens (two biopsies, one autopsy), but they were rare and were found only in one or two location(s) per grid. TVS were seen in distended pre- and postsynaptic terminals and measured approximately 35 nm in diameter; they were smaller and of higher electron density than synaptic vesicles. Their occurrence in all types of transmissible spongiform encephalopathies irrespective of the affected host and the strain of the infectious agent suggests their biological significance.
Cholinergic markers in ALS spinal cord.
We analyzed binding sites for quinuclidinyl benzilate (QNB) and hemicholinium-3 (HC-3) by quantitative slice autoradiography and the activities of choline acetyltransferase (ChAT) and acetylcholinesterase (AChE) in spinal cord of 5-7 patients with amyotrophic lateral sclerosis (ALS). In the ventral horn, QNB binding sites were markedly reduced (38% of controls; P less than 0.001), whereas HC-3 binding sites were only moderately affected (76%, P less than 0.01). Losses in cholinergic marker enzymes were inconsistent. The loss of muscarinic binding sites in the ventral horn was the most reliable cholinergic disease marker in ALS.
Amyotrophic lateral sclerosis: changes of noradrenergic and serotonergic transmitter systems in the spinal cord.
Noradrenaline (NA), dopamine (DA), serotonin (5-HT) and 5-hydroxyindoleacetic acid (5-HIAA) were measured in discrete subdivisions of cervical, thoracic and lumbar spinal cord segments obtained at autopsy of 4 subjects with amyotrophic lateral sclerosis (ALS) and 7 control patients. NA concentrations in thoracic and lumbar spinal cord of ALS patients were 2- to 4-fold higher compared with values obtained in control patients. 5-HT levels were unchanged at the cervical and thoracic level and slightly above normal in lumbar spinal cord, while the concentration of 5-HIAA was lowered in cervical and thoracic, but within the control range, in lumbar spinal cord. As a result, the molar ratios of 5-HT/5-HIAA were increased at all spinal levels in ALS. No difference in spinal DA concentration was found between ALS and control patients. The changes in the noradrenergic and serotonergic transmitter systems reported here most probably reflect a decreased release of these transmitter substances in ALS spinal cord. Since lack of the facilitatory monoaminergic influence would necessitate an increase in the excitatory, potentially neurotoxic glutamatergic input onto the motoneurones, we hypothesize that this could contribute to the progressive loss of spinal motoneurones in amyotrophic lateral sclerosis.
Tubulovesicular structures in human and experimental Creutzfeldt-Jakob disease.
Tubulovesicular structures (TVS) have been consistently observed in brain tissue of animals with transmissible spongiform encephalopathies such as natural and experimental scrapie, bovine spongiform encephalopathy, and experimental Creutzfeldt-Jakob disease (CJD). In this communication we demonstrate for the first time the presence of TVS in natural CJD. TVS were detected in all 3 CJD specimens. However, they were rare and were found only in one or two locations per grid. They were seen in distended pre- and postsynaptic terminals and measured approximately 35 nm in diameter, and they were smaller and of higher electron density than synaptic vesicles. Their occurrence in all types of spongiform encephalopathies irrespective of the affected host and the strain of infectious agent emphasizes their biological significance.
Amyotrophic lateral sclerosis: glutamate dehydrogenase and transmitter amino acids in the spinal cord.
Measurements were taken of the activity of glutamate dehydrogenase (GDH) and the levels of transmitter amino acids in anatomically dissected regions of cervical and lumbar spinal cord in eight patients dying with amyotrophic lateral sclerosis (ALS) and in 11 neurologically normal controls. GDH activity was considerably increased in lateral and ventral white matter and in the dorsal horn of the ALS cervical spinal cord, but normal in the ventral horn and the dorsal columns. Similar, although less pronounced, GDH changes were found in the lumbar enlargement. The mean concentrations of aspartate and glutamate were reduced in all regions of ALS spinal cord investigated. Taurine concentrations were significantly increased in several subdivisions of cervical spinal cord, but normal in lumbar regions. Glycine levels were significantly reduced in lumbar ventral and dorsal horns. There was no striking change in spinal cord GABA levels in our ALS patients. It is suggested that the reduced levels of glutamate and aspartate as well as the elevated GDH activity in the spinal cord of ALS patients may reflect an overactivity of the neurons releasing these potentially excitotoxic amino acids and thus may be causally related to the spinal neuro-degenerative changes characteristic of ALS.
Serum plasmalogens in ischemic cerebrovascular disease.
Plasmalogens, a subclass of glycerophospholipids are ubiquitous constituents of cellular membranes and serum lipoproteins. Comparing concentrations of plasmalogens in sera from patients suffering from ischemic cerebrovascular disease with serum levels in a normal population significantly lower values were found for patient sera.
Fatal encephalitis in a patient with chronic graft-versus-host disease.
A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. Splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
Diffuse cerebrospinal gliomatosis presenting as motor neuron disease for two years.
A patient with symptoms and signs of motor neuron disease for 2 years finally developed sensory disturbances and increased intracranial pressure. MRI and CT showed enlargement of the right side of the cerebellum, the brainstem and parts of the cerebral hemisphere with focal hyperperfusion demonstrated by SPECT. Necropsy revealed a diffuse cerebrospinal gliomatosis with loss of spinal motor neurons in tumour infiltration of the anterior horns. This type of spinal cord involvement is considered responsible for the unusual clinical presentation of the neoplasm.
[Flow measurements in extracranial carotid arteries by means of duplex sonography. Results in normal subjects].
Carotid arteries were examined by Duplex sonography in 116 healthy volunteers of between 16 and 78 years of age. We measured the internal carotid artery and common carotid artery peak systolic velocities and their ratio, the common carotid artery time-averaged velocity, the maximal diameter of the common carotid artery, and the common carotid artery volume flow (ml/min). Flow velocities and volume flow showed an age-dependent decrease.
Neuropathy associated with acrodermatitis chronica atrophicans. Clinical and morphological features.
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[Ichthyosiform scaling in alpha-1,4-glucosidase deficiency].
In two patients suffering from infantile and juvenile types of alpha-1,4 glucosidase deficiency (Pompe's disease, glycogen storage disease types 2a and 2b) with typical lysosomal glycogen storage, widespread dry "ichthyosiform" scaling skin was observed. The clinical and microscopical findings resembled those of ichthyosis vulgaris. Even in the cytoplasm of keratinocytes vacuolar glycogen accumulation was demonstrated, suggesting a correlation between this pathological storage process and the symptom of "scaling". Therefore, ichthyosiform scaling conditions should be investigated not only for disorders of lipid metabolism but also for a possible disturbance of the carbohydrate-digesting enzymes.
[Malignant hyperthermia in Austria. II. A comparison of the results of diagnostic test procedures].
During the last 4 years different diagnostic procedures for the detection of malignant hyperthermia (MH) susceptibility have been used at the authors' clinical unit; this study was designed to compare the results of these tests. PATIENTS AND METHODS. Since March 1983, 158 patients have been referred for the following reasons: group A: probands (n = 17) who had had symptoms of MH during anesthesia; group B: patients of probands (n = 48) if the latter were not tested because of age (n = 24) or death (n = 2); group C: relatives from MH families (n = 86); group D: patients (n = 5) who developed fever during stress and/or physical activity (n = 3), had myotonia (n = 1), or developed rhabdomyolysis during intensive care (n = 1); group E: controls (n = 2). Two static halothane and two static caffeine tests according to the European protocol were performed in all patients (n = 158). Histological examinations of skeletal muscle (fixed in glutaraldehyde, stained with hematoxylin-eosin, Gieson, and toluidine blue) were done in the first 100 patients; all specimens were scored by the same investigator (E.S.). Score 0: normal; 1: increased number of sarcolemma cores; 2: 1+cores forming groups; 3: 1+2+fiber degeneration; 4: specific changes-myopathies. Plasma levels of creatine kinase (CK) were determined in the first 50 patients. Complete neurological examinations, including electromyography (EMG), were done in ten patients who had increased CK levels as well as histological scores of 3 or 4 (Table 1).(ABSTRACT TRUNCATED AT 250 WORDS)
Neurological manifestations of Borrelia burgdorferi-infections: the enlarging clinical spectrum.
We report on the clinical symptoms of 50 patients with serologically proven B. burgdorferi infection in stage 2 Lyme disease. Besides the typical pattern of the GBB-syndrom myelitis, encephalitis, cranial nerve neuritis others than Bell's palsy, painful neuritis without CSF-pleocytosis and meningitis without other neurological findings were observed.
High-dose penicillin therapy in meningopolyneuritis Garin-Bujadoux-Bannwarth. Clinical and cerebrospinal fluid data.
Clinical data of 19 patients with meningopolyneuritis Garin-Bujadoux-Bannwarth (MPN-GBB), treated with 2 X 10 million units intravenous sodium penicillin for 10 days, were evaluated at the beginning of therapy, 3 weeks thereafter, and 6 months after onset of the neurological disease. Cerebrospinal fluid (CSF) was analysed in 14 patients at the onset of therapy and 3 1/2 weeks thereafter. At the same interval antibodies against B. burgdorferi were measured by enzyme-linked immunosorbent assay (ELISA) in the CSF and sera of 12 patients. Clinical data and all CSF results, with exception of specific antibody titers, were compared with those of patients who had suffered from MPN-GBB between 1979 and 1983, and who had not received antibiotic or corticosteroid therapy. Comparing the clinical data of all treated patients with those of all non-treated controls, no significant difference could be observed. A significant improvement could however be detected in those patients who had their treatment begun 5 weeks within onset of the neurological disease. Changes in CSF 3 1/2 weeks after onset of treatment showed slight differences when compared with controls.
The rigid spine syndrome--a myopathy of uncertain nosological position.
Four patients meeting the clinical criteria of the rigid spine syndrome are presented; they are one girl with a positive family history and three boys. Clinical and histological findings are discussed in relation to the 14 cases of rigid spine syndrome reported in the literature. The delineations of the syndrome from other benign myopathies with early contractures are discussed suggesting that the rigid spine syndrome probably does not represent a single nosological entity.
[Apolipoproteins A and B in cerebrovascular diseases].
The present study has proved unequivocally the value of determining apolipoproteins A and B and the APO-L A/B quotient for establishing the risk for cerebrovascular disease. The determination of apolipoproteins should be preferred to the determination of HDL, LDL and VLDL, which has been practised so far. While only 30% of a group of 88 patients suffering from cerebrovascular disease had serum cholesterol levels of more than 250 mg/dl and approx. 57% showed HDL values below 40 mg/dl, a reduced APO-L A/B relation (less than 1.75) was demonstrated in 74%. A reduced APO-L A/B quotient was also observed in more than 70% of 62 CVD patients whose cholesterol level was within the range of normal. Determination of apolipoproteins may, therefore, be regarded as further progress in risk factor diagnostic of arteriosclerotic disease.
[Clinical aspects of polyneuropathies].
Polyneuropathic diseases can exhibit sensory, motor and autonomous symptoms. Subjective disorders and objective manifestations of polyneuropathies are characterized, the main clinical syndromes are described. Furthermore, different types of course and distribution can be distinguished. So far 162 etiological factors are known which correspond to 5 main groups of causes. Their relation to the clinical syndromes is discussed. For exact diagnosis in the individual case electrophysiological and bioptical investigations have to complete the spectrum of findings.