| 1. |
- Danielsen, N, et al.
(författare)
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Nerve repair and axonal transport. Distribution of axonally transported proteins during maturation period in regenerating rabbit hypoglossal nerve
- 1986
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Ingår i: Journal of the Neurological Sciences. - 0022-510X. ; 73:3, s. 77-269
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Tidskriftsartikel (refereegranskat)abstract
- The distribution of fast migrating [3H]leucine-labelled proteins was studied in transected and repaired rabbit hypoglossal nerves. The nerves were repaired 90 days earlier with mesothelial chamber or epineurial suture technique. Fast migrating radiolabelled proteins were transported into the distal nerve segment and neurophysiological recordings from the tongue as well as the presence of myelinated axons in the distal nerve segment verified successful regeneration. The total amount of radioactivity was increased in repaired nerves as compared to contralateral nerves. In both groups there was a significant accumulation of radiolabelled proteins at the site of lesion. Nerves repaired with mesothelial chambers showed significantly more radioactivity in the distal nerve segment as compared to sutured nerves. The present study indicates long-standing effects on axonal transport system after both types of nerve repair. It is our opinion that axonal transport studies are a valuable complement when evaluating experimental nerve repair.
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| 2. |
- Ekström, Per A R, et al.
(författare)
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Impaired nerve regeneration in streptozotocin-diabetic rats. Effects of treatment with an aldose reductase inhibitor
- 1989
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Ingår i: Journal of the Neurological Sciences. - 0022-510X. ; 93:2-3, s. 231-237
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Forskningsöversikt (refereegranskat)abstract
- Rats with streptozotocin-induced diabetes have a decreased rate of sciatic nerve regeneration. We studied the effects on this defect of treatment with the aldose reductase inhibitor, ponalrestat (25 mg/kg per day via an endogastric tube). The nerves of diabetic rats were crush-injured at 5 weeks of diabetes and regeneration evaluated 7 days later with the pinch-reflex test. Ponalrestat treatment was started at day 3 after streptozotocin injection and was continued for the whole experimental period, i.e. until 6 weeks of diabetes. The treatment prevented effectively the accumulation of sorbitol and fructose in the nerves of diabetic rats, but was without effect on the sciatic nerve regeneration (controls 21.8 ± 1.2 mm/7 days (mean ± SEM, n = 6), untreated diabetics 15.8 ± 1.8 (n = 7), ponalrestat-treated diabetics 16.2 ± 1.0 (n = 10)). The results indicate that there is no connection between increased sorbitol pathway flux and impaired regeneration in streptozotocin diabetic rats.
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| 3. |
- Gustafson, Ingvar, et al.
(författare)
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Effects of ischemia on regional ligand binding to adrenoceptors in the rat brain
- 1992
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Ingår i: Journal of the Neurological Sciences. - 0022-510X. ; 113:2, s. 165-176
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Tidskriftsartikel (refereegranskat)abstract
- Changes in ligand binding to adrenoceptors ([3H]prazosin to α1-receptors, [3H]idazoxan to α2-receptors and [125I]cyanopindolol to β-receptors) following transient cerebral ischemia were investigated using autoradiographic methods. The binding was quantified in brain sections from control rats, rats subjected to 15 min of 2-vessel occlusion ischemia, and rats with recirculation times of 1 h, 1 week or 4 weeks after ischemia. No significant change in α1-receptor binding was observed during and immediately following ischemia, but a decrease was noted in the vulnerable hippocampal CA1 region following 1 week's survival. In the parietal cortex, the ligand binding to α1-receptors increased at 4 weeks. A reduced [3H]idazoxan binding was observed 1 h after ischemia in the temporal cortex and amygdala. No change in ligand binding to β-receptors was seen in the early phase postischemia, but a marked increase had occurred in the hippocampal CA1 region at 1 and 4 weeks after ischemia (+163% and +142%, respectively), presumably due to accumulation of macrophages expressing β-receptors. The early postischemic changes in receptor binding may represent downregulation of the adrenoceptors by processes activated during ischemia, while neuronal degeneration, compensatory mechanisms in surviving neurons and proliferation of non-neuronal cells may account for the subsequent changes.
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| 4. |
- Lexell, Jan, et al.
(författare)
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Distribution of different fibre types in human skeletal muscles. A statistical and computational model for the study of fibre type grouping and early diagnosis of skeletal muscle fibre denervation and reinnervation
- 1983
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Ingår i: Journal of the Neurological Sciences. - 1878-5883 .- 0022-510X. ; 61:3, s. 301-314
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Tidskriftsartikel (refereegranskat)abstract
- To define fibre type grouping in terms of random and non-random arrangements of the two fibre types, type 1 (ST) and type 2 (FT), we adopted the measure of counting the number of "enclosed fibres". The statistical properties of the number of enclosed fibres, and the number and size of groups of enclosed fibres were studied in computer-simulated muscle cross-sections, using a model based upon hexagonal-shaped fibres. The effects on the results of differences in the sizes of the muscle fibres were considered. The applicability of the model, and the derived results and methods of analysis were tested on 10 samples from a cross-section of a whole human muscle. The results show that the model can be applied to various shapes and sizes of muscle samples and various sizes of muscle fibres. The number of enclosed fibres within a muscle sample is the best of the three measures of non-randomness considered. A test is also described for assessing whether or not the observed number of enclosed fibres is random at a given significance level.
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| 5. |
- Lindman, Rolf, et al.
(författare)
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Abnormal palatopharyngeal muscle morphology in sleep-disordered breathing
- 2002
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Ingår i: Journal of the Neurological Sciences. - : Elsevier. - 1878-5883 .- 0022-510X. ; 195:1, s. 11-23
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Tidskriftsartikel (refereegranskat)abstract
- The aim of the present study was to investigate whether histopathological changes can be detected in two soft palate muscles, the palatopharyngeus and the uvula, in 11 patients with long duration of sleep-disordered breathing (SDB). Muscle samples were collected froth patients undergoing uvulo-palatopharyngoplasty (UPPP). Reference samples from the corresponding areas were obtained at autopsy froth five previously healthy subjects. Muscle morphology, fibre type and myosin heavy chain (MyHC) compositions were analysed with enzyme-histochemical, immunohistochemical and biochemical techniques. The muscle samples from the patients, and especially those from the palatopharyngeus, showed several morphological abnormalities. The most striking findings were (i) increased amount of connective tissue, (ii) abnormal variability in fibre size, (iii) increased proportion of small-sized fibres, (iv) alterations in fibre type and MyHC compositions, (v) increased frequency of fibres containing developmental MyHC isoforms. Our findings point towards a pathological process of denervation and degeneration in the patient samples. Conclusively. the morphological abnormalities suggest a neuromuscular disorder of the soft palate in SDB patients. (C) 2002 Elsevier Science B.V. All rights reserved.
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| 6. |
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| 7. |
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| 8. |
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| 9. |
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| 10. |
- Dahle, Charlotte, et al.
(författare)
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T helper type 2 like cytokine responses to peptides from P0 and P2 myelin proteins during the recovery phase of Guillain-Barré syndrome
- 1997
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 153:1, s. 54-60
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Tidskriftsartikel (refereegranskat)abstract
- T-lymphocytes are probably involved in the pathogenesis of Guillain-Barré syndrome (GBS). T-helper-1 (Th1) cytokines activate macrophages and induce a delayed type hypersensitivity (DTH) inflammatory response, consistent with the morphology of the demyelination in GBS. Th2 cytokines encourage antibody production and downregulate Th1 responses. To study the Th1/Th2 cytokines in relation to the clinical course of GBS an ELISPOT method for determination of single cells secreting interferon-γ, IFN-γ (Th1) or interleukin-4, IL-4 (Th2) was used. We serially investigated antigen-induced cytokine secretion from circulating T-cells stimulated with human peptides from the P0 and P2 proteins in seven patients and compared to results from seven serially investigated healthy controls. Most patients (five of seven) showed IL-4 responses during the plateau- or recovery-phase as compared to controls. One patient with a prolonged disease course, on the other hand, had an IFN-γ dominated reactivity. We suggest that the IL-4 responses are beneficial in GBS, and may have a role in terminating the disease process in this self-limiting inflammatory disease.
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| 11. |
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| 12. |
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| 13. |
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| 14. |
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| 15. |
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| 16. |
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| 17. |
- Grundström, Eva, et al.
(författare)
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Increased expression of glial cell line-derived neurotrophic factor mRNA in muscle biopsies from patients with amyotrophic lateral sclerosis
- 1999
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 162:2, s. 169-173
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Tidskriftsartikel (refereegranskat)abstract
- The expression of glial cell line-derived neurotrophic factor (GDNF) mRNA and brain-derived neurotrophic factor (BDNF) mRNA were studied in muscle biopsies from five patients with amyotrophic lateral sclerosis (ALS), six patients with other neuromuscular diseases and eight healthy control persons. All five patients with ALS had higher GDNF mRNA expressions in their biopsies than the healthy control group (almost a three fold increase). Among the other patients only one, who had a rapidly progressing toxic polyneuropathy, showed a GDNF mRNA expression above those of the controls. The BDNF mRNA expressions in the biopsies from the ALS patients were in the same range as those from the healthy controls, although the mean value of the ALS patients was higher. The only biopsy that showed a markedly higher BDNF mRNA expression was taken from one patient with progressive muscular atrophy. These results suggest that increased GDNF mRNA expression in muscle is an unspecific response to ongoing denervation and that this response is maintained in ALS, at least temporarily. If increased GDNF mRNA in muscle proves to be a constant finding in ALS the rationale for the use of GDNF as a therapeutic agent in ALS must be questioned.
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| 18. |
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| 19. |
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| 20. |
- JOHANSSON, O
(författare)
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The innervation of the human epidermis
- 1995
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Ingår i: Journal of the neurological sciences. - : Elsevier BV. - 0022-510X. ; 130:2, s. 228-228
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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| 21. |
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| 22. |
- Lexell, Jan, et al.
(författare)
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Distribution of different fibre types in human skeletal muscles : A statistical and computational study of the fibre type arrangement in m. vastus lateralis of young, healthy males
- 1984
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 65:3, s. 353-365
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Tidskriftsartikel (refereegranskat)abstract
- To test whether the arrangement of fibre types in the human muscle (m. vastus lateralis) from clinically healthy and young males can be regarded as random, fascicles at different parts of the muscle and with different fibre type proportions were studied. The randomness of the arrangement of fibre types was assessed by the number of enclosed fibres in a fascicle and, on the basis of a model, tested by simulating muscle cross-sections using a microcomputer. The fibre type proportion was found to vary within a fascicle, so the original model for the test of randomness was modified to allow for different fibre type proportions on the border of the fascicle and internally. The effect of different sizes of the fibre types was also considered. The various aspects considered had only a marginal effect on the original model. For this muscle (m. vastus lateralis) the arrangement of fibre types was therefore considered random. Thus, a sample from this muscle, taken from individuals of the same sex and age group, can be tested for non-randomness, as an indication of a successive denervation and reinnervation process
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| 23. |
- Lexell, Jan, et al.
(författare)
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Distribution of different fibre types in human skeletal muscles : fibre type arrangement in m. vastus lateralis from three groups of healthy men between 15 and 83 years
- 1986
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 72:2-3, s. 211-222
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Tidskriftsartikel (refereegranskat)abstract
- The effects of age on the fibre type arrangement in the human muscle m. vastus lateralis were studied. There were 10, 6 and 8 healthy men in the three age-groups with means 24, 52 and 77 years, respectively. For each fascicle considered, the numbers of type 1 (ST) and type 2 (FT) fibres on the boundary and internally, and the numbers of enclosed fibres of either type, were counted. The randomness of the fibre type arrangement was considered in terms of the numbers of enclosed fibres and assessed by a Monte Carlo significance test. Fibre type grouping was shown to increase with increasing age. The proportion of type 2 fibres on the boundary of a fascicle was consistently greater than internally, but the difference was less pronounced in the old group. It is argued that the process of denervation and reinnervation of individual fibres has started before the age of 50, is a major factor in a progressive reduction of fibres with increasing age and is probably caused by a continuous loss of motor neurons in the spinal cord.
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| 24. |
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| 25. |
- Lexell, Jan, et al.
(författare)
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What is the cause of the ageing atrophy? : number, size and proportion of different fiber types studied in whole vastus lateralis muscle from 15- to 83-year-old men
- 1988
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 84:2-3, s. 275-294
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Tidskriftsartikel (refereegranskat)abstract
- In order to study the effects of increasing age on the human skeletal muscle, cross-sections (15 micron) of autopsied whole vastus lateralis muscle from 43 previously physically healthy men between 15 and 83 years of age were prepared and examined. The data obtained on muscle area, total number, size, proportion and distribution of type 1 (slow-twitch) and type 2 (fast-twitch) fibers were analysed using multivariate regression. The results show that the ageing atrophy of this muscle begins around 25 years of age and thereafter accelerates. This is caused mainly by a loss of fibers, with no predominant effect on any fiber type, and to a lesser extent by a reduction in fiber size, mostly of type 2 fibers. The results also suggest the occurrence of several other age-related adaptive mechanisms which could influence fiber sizes and fiber number, as well as enzyme histochemical fiber characteristics
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| 26. |
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| 27. |
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| 28. |
- Libelius, Rolf, et al.
(författare)
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Uptake of horseradish peroxidase in denervated skeletal muscle occurs primarily at the endplate region
- 1984
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 66, s. 273-281
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Tidskriftsartikel (refereegranskat)abstract
- The spatial distribution of horseradish peroxidase (HRP) uptake has been studied by light- and electron microscopy in the denervated hemidiaphragm of the mouse. Segments with high HRP uptake were observed in a band centrally located in the denervated muscle. This distribution is similar to the well-known innervation pattern of the diaphragm. Ultrastructural studies demonstrated a high incidence of postsynaptic folds in close proximity of fibre areas with high intracellular content of HRP. 8–12 days after denervation a large number of fibres showed segments of high HRP uptake. 2–4 days after denervation very few such segments were observed. Biochemical studies also demonstrated an increase in HRP uptake after denervation occurring primarily in the endplate region. The activities of the lysosomal enzymes N-acetyl-β-d-glucosaminidase, acid phosphatase and cathepsin D all increased after denervation, most prominently in the endplate region.It is suggested that the observed segmental uptake of HRP and lysosomal activation reflects a process for rapid membrane turnover in denervated muscle.
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| 29. |
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| 30. |
- Mezei, M., et al.
(författare)
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Motor system abnormalities in heterozygous relatives of a D90A homozygous CuZn-SOD ALS patient of Finnish extraction.
- 1999
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 169:1-2, s. 49-55
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Tidskriftsartikel (refereegranskat)abstract
- Presently, 64 mutations in the gene encoding the enzyme CuZn-superoxide dismutase have been found in a small fraction of amyotrophic lateral sclerosis patients worldwide. All but one of these mutations show autosomal dominant inheritance. In Scandinavia, the D90A mutation is inherited as an autosomal recessive trait and patients have an easily recognizable characteristic phenotype with little variation among patients, even amongst different families. Importantly, all D90A heterozygous relatives of Scandinavian D90A homozygous patients have been reported as clinically unaffected. We have investigated a Canadian family of Finnish extraction in which the D90A homozygous proband developed ALS with the characteristic phenotype. Remarkably, two D90A heterozygous relatives show slight symptoms and signs of motor system involvement, suggesting that the final phenotype of an individual with a CuZn-superoxide dismutase mutation is shaped by the combination of the particular CuZn-SOD mutation, other polymorphic modifying genes elsewhere in the genome, stochastics and possible environmental factors.
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| 31. |
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| 32. |
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| 33. |
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| 34. |
- Tågerud, Sven, et al.
(författare)
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An ultrastructural study of the segmental uptake of horseradish peroxidase in the endplate region of denervated skeletal muscle fibres
- 1986
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 75:2, s. 141-157
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Tidskriftsartikel (refereegranskat)abstract
- The segmental uptake of horseradish peroxidase (HRP) in the endplate region of denervated skeletal muscle fibres has been studied ultrastructurally using a method for selecting single muscle fibres with high segmental peroxidase staining from denervated mouse tibialis anterior muscle.Segments containing large peroxidase positive phagosomes could already be seen 10–15 min after i.v. injection of HRP. Such segments were still present 24 h after HRP injection. The localization of phagosomes, deep in the fibres rather than immediately under the sarcolemma, suggests that the uptake occurs from t-tubuli. Vivid proliferation of t-tubuli, consisting of vesiculation, enlargement and encircling of cytoplasmic components, was also observed. The HRP accumulates in phagosomes of varying size and shape. Similar membrane-limited bodies without or with very weak peroxidase staining were also observed. The peroxidase-positive phagosomes participate in autophagic processes as suggested by their content of undegraded cellular material. Golgi profiles, which occurred deep in the muscle fibres, and enlarged components of the sarcoplasmic reticulum were frequently encountered in the segments. Myofibrillar degeneration occurs in the segments and progresses with time after denervation.The described segments may be related to the increased membrane turnover in denervated muscle fibres and/or they may be related to processes aimed at establishing new synaptic contacts.
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| 35. |
- Tågerud, Sven, et al.
(författare)
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Biochemical and ultrastructural effects of chloroquine on horseradish peroxidase uptake and lysosomal enzyme activities in innervated and denervated mouse skeletal muscle
- 1986
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 75:2, s. 159-171
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Tidskriftsartikel (refereegranskat)abstract
- The effects of chloroquine treatment on horseradish peroxidase (HRP) uptake and lysosomal enzyme activities in innervated and denervated mouse skeletal muscle have been studied using biochemical, histochemical and ultrastructural techniques.Chloroquine treatment caused a large (59–101%) increase in the activity of cathepsin D in both innervated and denervated muscle. The activity of N-acetyl-β-d-glucosaminidase also increased slightly in denervated muscle. No effect was observed on acid phosphatase activity. The in vivo uptake of HRP in innervated and denervated muscle was unaffected by chloroquine treatment. The results show that the activities of certain lysosomal enzymes may increase in skeletal muscle without an increase in endocytic activity. This is discussed in comparison to what is seen in denervated and dystrophic muscle.Histochemical and ultrastructural studies showed the HRP uptake to occur segmentally in denervated muscle fibres from untreated as well as chloroquine-treated animals. Ultrastructurally the peroxidase-positive phagosomes occurring in these segments were found to contain increased levels of undegraded material after chloroquine treatment suggesting that these phagosomes are of a lysosomal nature and also participate in autophagic processes.
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| 36. |
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| 45. |
- Agerskov, Simon, et al.
(författare)
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The phenotype of idiopathic normal pressure hydrocephalus-a single center study of 429 patients
- 2018
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X. ; 391, s. 54-60
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Tidskriftsartikel (refereegranskat)abstract
- Introduction Idiopathic Normal Pressure Hydrocephlaus (iNPH) is, despite a vastly improved knowledge of the disorder since its first description still underdiagnosed and undertreated. Because of this, there is a need for further large studies describing the typical symptomatology and reversibility of symptoms in iNPH, which was the aim of this study. Methods In all, 429 patients (mean age 71years) were included. Detailed pre- and postoperative examinations of symptoms and signs were analyzed. A composite outcome measure was constructed. Results Sixty-eight % improved after surgery. Preoperatively, 72% exhibited symptoms from three or four of the assessed domains (gait, balance, neuropsychology and continence) while 41% had symptoms from all four domains. Ninety % had gait disturbances, of which 75% had broad-based gait, 65% shuffling gait and 30% freezing of gait. These disturbances coexisted in most patients preoperatively, but were more likely to appear as isolated findings after surgery. Impaired balance was seen in 53% and retropulsion in 46%. MMSE <25 was seen in 53% and impaired continence in 86%. Improvements were seen in all symptom domains postoperatively. Conclusions The iNPH phenotype is characterized by a disturbance in at least 3/4 symptom domains in most patients, with improvements in all domains after shunt surgery. Most patients present with a broad-based and shuffling gait as well as paratonia. Present symptoms in all domains and a shuffling gait at the time of diagnosis seem to predict a favorable postoperative outcome, whereas symptom severity does not.
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| 46. |
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| 47. |
- Ali, Zafar, et al.
(författare)
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Novel SACS mutations associated with intellectual disability, epilepsy and widespread supratentorial abnormalities
- 2016
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 371, s. 105-111
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Tidskriftsartikel (refereegranskat)abstract
- We describe eight subjects from two consanguineous families segregating with autosomal recessive childhood onset spastic ataxia, peripheral neuropathy and intellectual disability. The degree of intellectual disability varied from mild to severe and all four affected individuals in one family developed aggressive behavior and epilepsy. Using exome sequencing, we identified two novel truncating mutations (c.2656C>T (p.Gln886*)) and (c.4756_4760delAATCA (p.Asn1586Tyrfs*3)) in the SACS gene responsible for autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). MRI revealed typical cerebellar and pontine changes associated with ARSACS as well as multiple supratentorial changes in both families as likely contributing factors to the cognitive symptoms. Intellectual disability and behavioral abnormalities have been reported in some cases of ARSACS but are not a part of the characteristic triad of symptoms that includes cerebellar ataxia, spasticity and peripheral neuropathy. Our combined findings bring further knowledge to the phenotypic spectrum, neurodegenerative changes and genetic variability associated with the SACS gene of clinical and diagnostic importance.
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