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Biomedical subjects

M Swash

Publications and source records attributed to M Swash.

At least 19 recordsLinked to original sources
PubMed1992

Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia.

Amyotrophic lateral sclerosis (ALS) patients with dementia were found to have ubiquitin-immunoreactive (IR) inclusions in the dentate granule cells of the hippocampus. These inclusions were also present in some patients with minor cognitive changes but otherwise typical ALS. Ubiquitin-IR inclusions were also found in neurons of superficial layers of the frontal and temporal cortex and in the entorhinal cortex in patients with ALS and dementia. These ubiquitin-IR inclusions were non-argyrophilic, and were not labelled by antibodies which identify Alzheimer's neurofibrillary tangles and Pick bodies, nor were they typical of cortical Lewy bodies. Our findings indicate that ubiquitin-IR inclusions in small neurons of the hippocampus, entorhinal area and neocortex are a characteristic feature of degeneration of non-motor cortex in ALS, and are particularly associated with cognitive impairment and dementia of frontal lobe type.

Adult↗
PubMed1992

Fiber density in acute and chronic inflammatory demyelinating polyneuropathy.

The fiber density in the deltoid, extensor digitorum communis, and first dorsal interosseous muscles was measured using SFEMG in 11 patients with acute and chronic inflammatory demyelinating polyneuropathy. The fiber density was increased in 58% of the muscles studied. The deltoid muscle was the most abnormal of the 3 muscles studied. There was no correlation with the clinical syndrome, with conduction block or slowed motor conduction, or with the distribution of weakness. Changes were noted even in patients studied within 3 weeks of presentation, suggesting that reinnervation begins soon after the onset of the disease.

Adult↗
PubMed1992

Adverse effect of verapamil in myasthenia gravis.

Verapamil, a class IV anti-arrhythmic drug that blocks voltage-dependent calcium channels in cardiac and smooth muscle, also has effects on presynaptic and postsynaptic voltage-dependent calcium channels at the neuromuscular junction. In a postoperative patient with pre-existent myasthenia gravis, oral verapamil caused a marked exacerbation in myasthenic weakness.

Contraindications↗
PubMed1992

Cyclosporin A therapy in paraprotein-associated neuropathy.

A case of IgG paraprotein-associated demyelinating neuropathy complicated by respiratory failure, which was unresponsive to standard immunosuppressive drug therapy, is reported. Cyclosporin A therapy resulted in a marked clinical recovery with objective improvement in nerve conduction and vital capacity. The beneficial response suggests that cell-mediated immunity is an important pathogenetic mechanism, and that cyclosporin A may be useful in the treatment of other refractory cases of paraprotein-associated neuropathy.

Adult↗
PubMed1992

What do we really know about amyotrophic lateral sclerosis?

The cause of amyotrophic lateral sclerosis is unknown. In this review clinical and scientific data that are pertinent to understanding this disease are reviewed. There are currently several major controversies concerning the possible role of immunological factors, genetic factors, environmental toxins, and viral infection in pathogenesis. These concepts must be considered in relation to what is known about the disease in all its aspects, including epidemiological data, information on the classical and molecular pathology of the disease, and on associated involvement of other systems, e.g., the spinocerebellar pathways and frontal dementia. Only when all this information is assimilated can full understanding of the disease and, hopefully, a logical approach to treatment and prevention, be achieved.

Amyotrophic Lateral Sclerosis↗
PubMed1992

Criteria for diagnosis of familial amyotrophic lateral sclerosis. European FALS Collaborative Group.

Clinical criteria for the diagnosis of motor neuron disease, agreed at the inaugural meeting of the European Familial Amyotrophic Lateral Sclerosis Collaborative Group, are described. The criteria are derived from those developed for the study of sporadic amyotrophic lateral sclerosis, and allow the inclusion of certain recognized clinical sub-types of familial amyotrophic lateral sclerosis. They will require testing for consistency and sensitivity.

Amyotrophic Lateral Sclerosis↗
PubMed1992

Ultrastructure of pre-synaptic input to motor neurons in Onuf's nucleus: controls and motor neuron disease.

Ultrastructural analyses of sphincteric motoneurons in Onuf's nucleus at S2 were undertaken in human spinal cord obtained 3-6 h post-mortem from three subjects with no neurological disease ('controls') and five in which death was due to motor neuron disease (MND). Neurons in specified locations within Onuf's nucleus of control subjects ranged between 17.8 and 71.7 microns diameter (mean 38.6 microns). Analyses of synaptology revealed five ultrastructural classes of presynaptic terminal synapsing with the neuronal surface membrane. When classified by size, vesicle morphology, and synaptic site structure these conformed to the S, F, T, M and C-terminals defining somatic motoneurons. No terminals characteristic of autonomic motoneurons were found. In MND subjects, neurons in Onuf's nucleus at S2 were preserved despite a paucity of neurons in medial and lateral motor nuclei and were of similar size range to those in control subjects. The morphological classes of pre-synaptic terminal found in controls, also characterized sphincteric motoneurons in MND subjects, including the C-type terminal. The presence of C-terminals indicates (i) that sphincteric motoneurons are somatic alpha-motoneurons, and (ii) that hypotheses explaining the survival of sphincteric motoneurons in MND on the basis of Onuf's nucleus being an extension of the pre-ganglionic parasympathetic nucleus, or having intrinsic autonomic properties are incorrect.

Adult↗
PubMed1992

Botulinum toxin in the treatment of chronic urinary retention in women.

Six women were identified as having difficulty in voiding or complete urinary retention due to abnormal myotonic-like electromyographic (EMG) activity in the striated muscle of the urethral sphincter. An attempt was made to improve voiding by injection of botulinum toxin into the striated sphincter muscle. Although 3 patients then developed transient stress incontinence, demonstrating that sufficient botulinum toxin had been given to cause sphincter weakness, no patient had significant symptomatic benefit.

Adult↗
PubMed1991

Expression of heat shock protein epitopes in tubular aggregates.

Tubular aggregates may be found in a variety of conditions and have been associated with a wide range of chemical and ischemic insults. We report clinical and histological features in a case of myopathy with tubular aggregates. The structure of these tubular aggregates was examined using antibodies to cytoskeletal proteins and heat shock proteins. Epitopes of the 72 kD heat shock protein were expressed in the areas of abnormality in this case and in a case of hypokalemic periodic paralysis with tubular aggregates. Heat shock proteins have a role in the modulation of the tertiary structure of proteins and may be involved in the pathogenesis of tubular aggregates and other microtubular abnormalities in muscle.

Adult↗
PubMed1991

Purkinje cell toxicity of beta-aminopropionitrile in the rat.

Compounds causing neurolathyrism are putative aetiological agents in neurodegenerative disorders including amyotrophic lateral sclerosis. beta-Aminopropionitrile (BAPN) is one such compound. We have administered this lathyrogenic agent at a dose of 1 g/kg by the intraperitoneal route in experiments in adult Sprague-Dawley rats during a period of 10 weeks. The rats developed marked kyphoscoliosis, ataxia with paralysis and muscle wasting of the hind limbs. Vacuolation and loss of Purkinje cells developed, but no anterior horn cell degeneration was noted. Immunohistochemical studies of phosphorylated neurofilaments and the 72 kDa heat shock protein were normal and no intraneuronal ubiquitinated inclusions were seen. High-dose intraperitoneal BAPN in the rat causes Purkinje cell changes, but no other central nervous system abnormalities.

Aminopropionitrile↗
PubMed1991

Hereditary internal anal sphincter myopathy causing proctalgia fugax and constipation. A newly identified condition.

A newly identified myopathy of the internal anal sphincter is described. In the affected family, at least one member from each of five generations had severe proctalgia fugax; onset was usually in the third to fifth decades of life. Three members of the family have been studied in detail. Each had severe pain intermittently during the day and hourly during the night. Constipation was an associated symptom, in particular difficulty with rectal evacuation. Clinically the internal anal sphincter was thickened and of decreased compliance. The maximum anal canal pressure was usually increased with marked ultraslow wave activity. Anal endosonography confirmed a grossly thickened internal anal sphincter. Two patients were treated by internal anal sphincter strip myectomy; one showed marked improvement and one was relieved of the constipation but had only slight improvement of the pain. The hypertrophied muscle in two of the patients showed unique myopathic changes, consisting of vacuolar changes with periodic acid-Schiff-positive polyglycosan bodies in the smooth muscle fibers and increased endomysial fibrosis. In vitro organ-bath studies showed insensitivity of the muscle to noradrenaline, isoprenaline, carbachol, dimethylpiperazinium, and electrical-field stimulation. Immunohistochemical studies for substance P, calcitonin gene-related peptide, galanin, neuropeptide Y, and vasoactive intestinal peptide showed staining in a similar distribution to that in control tissue. A specific autosomal-dominant inherited myopathy of the internal anal sphincter that causes anal pain and constipation has been identified and characterized.

Adult↗
PubMed1991

Why is the gracilis muscle relatively uninvolved in neuromuscular disorders?

CT scanning of thigh muscles has shown that the gracilis is often relatively resistant to degeneration in neuromuscular disorders. We have compared the histometric features of the gracilis muscle with the biceps femoris and vastus lateralis muscles in the right and left thighs of 12 subjects without neuromuscular disease. There was striking variation between individuals, but marked similarities between the two limbs in individuals. The gracilis and vastus lateralis muscles contained fewer Type 1 fibres than the biceps femoris. The Type 2 fibres were slightly larger in vastus lateralis than in the other two muscles. There is thus no evident morphological difference between the gracilis muscle and other thigh muscles that can be correlated with the different susceptibility of these muscles in neuromuscular diseases. However, these muscles are subject to differing physiological stresses in movement, and these may be important in determining the response to disease.

Female↗
PubMed1991

Digital subtraction angiography (DSA) of the extracranial cerebral vessels: a direct comparison between intravenous and intra-arterial DSA.

Fifty patients being investigated for ischaemic cerebrovascular disease underwent both intravenous digital subtraction angiography (IV-DSA) and intra-arterial arch digital subtraction angiography (arch-DSA) to enable a direct comparison to be made between the two techniques. The overall quality of the images obtained by arch-DSA was better than that obtained by IV-DSA, but there was no significant difference between the number and gradation of stenoses detected by the two techniques. No significant stenosis was missed by IV-DSA and the technique was found to be sufficiently accurate for it to be used as a screening procedure for carotid bifurcation stenoses suitable for carotid endarterectomy. The advantages and limitations of both techniques are discussed.

Adult↗
PubMed1991

Ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis. Morphology, distribution, and specificity.

Antibodies to ubiquitin have been used to search for evidence of abnormal protein degradation in amyotrophic lateral sclerosis--motor neuron disease (ALS). Anterior horn cell ubiquitin-immunoreactive (IR) inclusions were present in all of 31 ALS cases but in none of 23 neurologically normal and in only 1 of 22 neurologically abnormal controls. These inclusions, which were present in familial and sporadic ALS cases, and in cases with dementia, took the form of dense rounded or irregular ubiquitin-IR cytoplasmic inclusions (dense bodies), or loosely arranged bundles ('skeins') of filamentous-appearing material. The presence of ubiquitin-IR inclusions corresponded to the pattern of selective neuronal vulnerability in ALS, although inclusions in pyramidal neurons of the motor cortex were infrequent and were noted in only a minority of cases. Ubiquitin-IR inclusions were more prevalent than Bunina bodies. The latter were present in 67% of ALS cases but were seldom labelled by antibodies to ubiquitin. Intraneuronal inclusions resembling Lewy bodies were present in 23% of ALS cases and were often identified by antibodies to ubiquitin. We conclude that the presence of ubiquitin-IR inclusions in lower motor neurons represents a characteristic pathological feature of ALS in its various clinical forms. Ubiquitin-IR inclusions in ALS differ from ubiquitinated inclusions in other neuronal degenerations in that they are not readily identified by antibodies to cytoskeletal proteins. They may represent accumulations of altered or abnormal neuronal proteins resistant to degradation via the ubiquitin proteolytic pathway.

Adult↗