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| 2. |
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| 3. |
- 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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| 4. |
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| 7. |
- Ansved, Tor, et al.
(författare)
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Enzyme-histochemical and morphological characteristics of fast- and slow-twitch skeletal muscle after brain infarction in the rat
- 1996
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Ingår i: Journal of the Neurological Sciences. - 1878-5883. ; 144:1-2, s. 14-20
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Tidskriftsartikel (refereegranskat)abstract
- The right middle cerebral artery was permanently occluded in 12-week-old male spontaneously hypertensive rats. After the surgery the rats were subjected to repeated behavioural tests during the observation period. Fourteen weeks after surgery the fast-twitch extensor digitorum longus (EDL) and the slow-twitch soleus muscle of both sides were removed and examined with regard to muscle fibre characteristics obtained by histochemical and morphometrical methods. Comparisons were made with age-matched controls. Limb placement and the ability to traverse a beam or a rotating pole were repeatedly tested 2-13 weeks after the operation. In spite of permanent sensorimotor deficits in limb placement and when traversing a rotating pole or beam, no increase in pathological changes was noted in either EDL or soleus. The number and proportion of fibre types remained unchanged in both muscles. There was no difference in muscle fibre size in either EDL or soleus. It is concluded that brain infarction in the rat, although causing marked impairment of contralateral motor function, does not have a major influence on the muscle-fibre morphology or fibre-type composition, irrespective of muscle type.
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| 13. |
- Boström, Inger, et al.
(författare)
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An ecological study of industry in a high-risk region of multiple sclerosis
- 2011
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Ingår i: Journal of the Neurological Sciences. - : Elsevier. - 0022-510X .- 1878-5883. ; 311:1-2, s. 50-57
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Tidskriftsartikel (refereegranskat)abstract
- The county of Varmland, Sweden, has shown a high frequency of multiple sclerosis in several investigations. It has been presented in three studies: a period prevalence study in 1925-1934, a mortality study during 1952-1992 and a prevalence investigation in 2002. The aim of this study was to investigate the pattern of industry in this high-risk area for multiple sclerosis. The three investigations were correlated with industry in 1913 and in the 1950s, all analyzed by the Kruskall-Wallis test. Select industries from wood-pulp, paper and iron/mechanical sectors were tested also in whole Sweden. The Spearman rank correlation was used for these data and forestry data in Varmland. In Varmland, industrial data from 1913 revealed that large sawmills were associated with the period prevalence in 1925-1934 and there was a possible correlation with the prevalence for 2002. Wood-pulp factories showed a possible association with the prevalence 1925-1934 and the mortality 1952-1992. Some industries in the 1950s were correlated with the prevalence 2002. Wood and paper industries in Sweden 1913 showed an association with the MS mortality 1952-1992. In summary, data on MS prevalence in Varmland and mortality both in Varmland and all Sweden from the past 100 years suggest an association with wood-related industries in 1913 and in the 1950s, whereas no consistent association was found for other industries.
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| 14. |
- Bostöm, I., et al.
(författare)
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Narcolepsy as a side effect of swine flu vaccination
- 2017
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 381:Supplement, s. 189-189
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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| 15. |
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| 16. |
- Bäckström, David, et al.
(författare)
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PITX3 genotype and risk of dementia in Parkinson's disease : A population-based study
- 2017
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Ingår i: Journal of the Neurological Sciences. - : ELSEVIER SCIENCE BV. - 0022-510X .- 1878-5883. ; 381, s. 278-284
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Tidskriftsartikel (refereegranskat)abstract
- Dementia is a devastating manifestation of Parkinson's disease (PD). This study investigates whether a common polymorphism in the PITX3 gene (rs2281983), which is of importance for the function of dopaminergic neurons, affects the risk of developing dementia in PD and whether it affects dopamine transporter (DAT) uptake. We PITX3 genotyped 133 patients with new-onset, idiopathic PD, participating in a population-based study in Sweden. Patients were followed prospectively during 6-11 years with extensive investigations, including neuropsychology and DAT-imaging with I-123 FP-CIT. The primary outcome was the incidence of PD dementia (PDD), diagnosed according to published criteria, studied by the Kaplan-Meier method and Cox proportional hazards. Performance in individual cognitive domains, the incidence of visual hallucinations, disease progression and striatal DAT uptake on imaging was also investigated. PD patients carrying the PITX3 C allele had an increased risk of developing PDD (hazard ratio: 2.87, 95% CI: 1.42-5.81, p = 0.003), compared to the PD patients homozygous for the T-allele. Furthermore, the PITX3 C allele carriers with PD had a poorer cognitive performance in the visuospatial domain (p < 0.001) and a higher incidence of visual hallucinations. A trend towards a lower striatal DAT uptake in the PITX3 C allele carriers was suggested, but could not be confirmed. Our results show that a common polymorphism in the PITX3 gene affects the risk of developing PDD and visuospatial dysfunction in idiopathic PD. If validated, these findings can provide new insights into the neurobiology and genetics of non-motor symptoms in PD.
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| 17. |
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| 18. |
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| 19. |
- Chroni, Elisabeth, et al.
(författare)
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Neurophysiological characteristics of MuSK antibody positive Myasthenia Gravis mice : Focal denervation and hypersensitivity to acetylcholinesterase inhibitors
- 2012
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 316:1-2, s. 150-157
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Tidskriftsartikel (refereegranskat)abstract
- Myasthenia Gravis (MG) patients with antibodies against the muscle specific tyrosine kinase (MuSK+) typically present with focal fatigue and atrophy of the facial and bulbar muscles, along with unbeneficial reactions upon administration of acetylcholinesterase inhibitors (AChEIs). This study addresses the neurophysiological characteristics in facial versus limb muscles, before and after intraperitoneal injection of AChEIs, in mice immunized with MuSK. We performed in-vivo neurophysiological examinations in the masseter and gastrocnemius muscles of mice with MuSK+experimental autoimmune MG (EAMG) and in healthy control mice before and after administration of AChEIs. Abnormal spontaneous activity (fibrillations) was observed in the masseter muscle of MuSK+mice. Furthermore, 94% of MuSK-immunized mice displayed so called extra discharges (EDs) upon administration of a therapeutic AChEI dose, in contrast to 22% of the control mice, indicating neuromuscular hyperactivity. These findings support functional denervation in the masseter muscle and neuromuscular hypersensitivity already at a standard dose of AChEIs in MuSK+EAMG.
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| 20. |
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| 21. |
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| 22. |
- Chruzander, Charlotte, et al.
(författare)
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Longitudinal changes in sickness absence and disability pension, and associations between disability pension and disease-specific and contextual factors and functioning, in people with multiple sclerosis.
- 2016
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 367, s. 319-325
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Even though it is well known that disability due to MS is highly associated with employment status, the long-term longitudinal perspective on sickness absence and disability pension over the MS trajectory is lacking. In addition, further knowledge of risk factors for future disability pension is needed.OBJECTIVES: To explore long-term longitudinal changes in the prevalence of sickness absence and disability pension in people with MS (PwMS), as well as to explore associations between disease-specific factors, contextual factors and functioning, and the outcome of future full-time disability pension.METHODS: A prospective, population-based survival cohort study, with a nine year follow-up, including 114 PwMS was conducted by combining face-to-face collected data and register-based data.RESULTS: The prevalence of full-time disability pension increased from 20% to 50%, however 24% of the PwMS had no disability pension at all at end of follow-up. Sex, age, disease severity and impaired manual dexterity were associated with future full-time disability pension.CONCLUSIONS: The large increase in prevalence of PwMS on full-time disability pension during the MS trajectory, calls for the development and implementation of evidence-based interventions, aiming at keeping PwMS in the work force. Modifiable factors, such as manual dexterity should be targeted in such interventions.
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| 23. |
- 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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| 24. |
- Damangir, Soheil, et al.
(författare)
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Multispectral MRI segmentation of age related white matter changes using a cascade of support vector machines
- 2012
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 322:1-2, s. 211-216
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Tidskriftsartikel (refereegranskat)abstract
- White matter changes (WMC) are the focus of intensive research and have been linked to cognitive impairment and depression in the elderly. Cumbersome manual outlining procedures make research on WMC labor intensive and prone to subjective bias. We present a fast, fully automated method for WMC segmentation using a cascade of reduced support vector machines (SVMs) with active learning. Data of 102 subjects was used in this study. Two MRI sequences (T1-weighted and FLAIR) and masks of manually outlined WMC from each subject were used for the image analysis. The segmentation framework comprises pre-processing, classification (training and core segmentation) and post-processing. After pre-processing, the model was trained on two subjects and tested on the remaining 100 subjects. The effectiveness and robustness of the classification was assessed using the receiver operating curve technique. The cascade of SVMs segmentation framework outputted accurate results with high sensitivity (90%) and specificity (99.5%) values, with the manually outlined WMC as reference. An algorithm for the segmentation of WMC is proposed. This is a completely competitive and fast automatic segmentation framework, capable of using different input sequences, without changes or restrictions of the image analysis algorithm.
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| 25. |
- Eckerström, Carl, et al.
(författare)
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Small baseline volume of left hippocampus is associated with subsequent conversion of MCI into dementia. The Göteborg MCI study.
- 2008
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 272:1-2, s. 48-59
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Tidskriftsartikel (refereegranskat)abstract
- Background: Earlier studies have reported that hippocampal atrophy can to some extent predict which patients with mild cognitive impairment (MCI) will subsequently convert to dementia, and that converters have an enhanced rate of hippocampal volume loss. Objective: To further validate the hypothesis that hippocampal atrophy predicts conversion from MCI to dementia, to relate baseline hippocampal volume to different forms of dementia, and to investigate the role of hippocampal side differences and rate of volume loss over time. Patients: The subjects (N = 68) include patients with MCI at baseline and progression to dementia at the two-year follow-up (N = 21), stable MCI patients (N = 21), and controls (N = 26). Among the progressing patients, 13 were diagnosed as having AD. Methods: The Göteborg MCI study is a clinically based longitudinal study with biannual clinical assessments. Hippocampal volumetry was performed manually on the MRI investigations at baseline and at the two-year follow-up. Results: Hippocampal volumetry could predict conversion to dementia in both the AD and the non-AD subgroup of converters. Left hippocampal volume in particular discriminated between converting and stable MCI. Cut off points for individual discrimination were shown to be potentially useful. The converting MCI group had a significantly higher rate of hippocampal volume loss as compared to the stable MCI group. Conclusions: In MCI patients, hippocampal volumetry at baseline gives prognostic information about possible development of AD and non-AD dementia. Contrary to earlier studies, we found that left hippocampal volume has the best predictive power. Reliable predictions appear to be possible in many individual cases.
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| 26. |
- Edvardsson, Bengt, et al.
(författare)
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Polyneuritis cranialis presenting with anti-GQ1b IgG antibody.
- 2009
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 1878-5883 .- 0022-510X. ; 281, s. 125-126
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Tidskriftsartikel (refereegranskat)abstract
- A 52-year-old man developed diplopia, a nasal voice, dysphagia, hoarseness and slight bilateral facial palsies. There was no ataxia, areflexia, limb weakness or sensory involvement. Serum anti-GQ1b IgG antibody was present. Treatment with intravenous immunoglobulin started, and the patient responded with a rapid resolution of symptoms. The diagnosis is consistent with polyneuritis cranialis which is considered to be a Guillain-Barre syndrome variant, a forme fruste, but very rare. The diagnosis can be difficult and a thorough investigation is required. Electrophysiological examination, laboratory evaluations, imaging and cerebrospinal fluid examination are often required in the investigations. Cranial neuropathy can be the presentation of many disorders. Determination of anti-ganglioside antibodies as anti-GQ1b is valuable to the diagnosis, and shows the association with the Guillain-Barre syndrome.
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| 27. |
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| 28. |
- Ekegren, Titti, et al.
(författare)
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Maintained regulation of polyamines in spinal cord from patients with amyotrophic lateral sclerosis
- 2004
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Ingår i: Journal of the Neurological Sciences. - : Elsevier. - 0022-510X .- 1878-5883. ; 222:1-2, s. 49-53
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Tidskriftsartikel (refereegranskat)abstract
- Levels of the polyamines putrescine, spermidine, and spermine were investigated in postmortem spinal cord from seven patients with amyotrophic lateral sclerosis (ALS) and seven control subjects. The method consisted of precolumn derivatization of the polyamines, followed by high-performance liquid chromatography (HPLC) analysis and fluorescence detection. The stability of the polyamines was examined in rat spinal cord during the interval of 0–36 h postmortem. The levels of putrescine, spermidine, and spermine increased by 32%, 15%, and 2%, respectively. Polyamine levels did not differ significantly between the ALS group and the control group, suggesting a maintained regulation of polyamines in the end stage of the disease. However, an effect of gender on the levels of spermidine and spermine was observed. Levels of spermidine and spermine in the ventral horn region of female ALS patients were significantly higher in comparison with the same region of the male ALS group (p< 0.05). The female ALS group also presented significantly higher levels of spermidine in comparison with female controls (p< 0.05).
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| 29. |
- Elf, Kristin, et al.
(författare)
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Vitamin D deficiency in patients with primary immune-mediated peripheral neuropathies
- 2014
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 345:1-2, s. 184-188
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: T cells are important in the immunopathology of immune-mediated peripheral neuropathies (PNP) and activated vitamin D regulates the immune response through increasing the amount of regulatory T cells. An association between vitamin D deficiency and polyneuropathy has been stipulated; hence we assessed whether patients with primary immune-mediated PNP have low vitamin D [25(OH)D] levels.METHODS: Plasma levels of 25(OH)D were analyzed in 26 patients with primary immune-mediated PNP, 50 healthy matched blood donors and 24 patients with motor neuron disease (MND). INCAT score was assessed in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. ALSFRS-R score was applied to MND patients and the modified Rankin (mRankin) scale compared disability among patient groups.RESULTS: Mean 25(OH)D value in PNP patients was 40±16nmol/l, compared to 69±21nmol/l in healthy blood donors (p<0.001). MND patients had a higher mean 25(OH)D than PNP patients (59±26nmol/L; p=0.006) and comparable levels to healthy blood donors (p=0.15). Mean 25(OH)D value was not higher in PNP patients with pre-existing vitamin D3 supplementation of 800IU/day (N=6; 35±18nmol/L) than in unsupplemented PNP patients (42±16nmol). INCAT score ranged from 0 to 10 (mean 3.5) and ALSFRS-R ranged from 11 to 44 (mean 31). mRankin score was more severe in MND patients (mean 3.5) compared to PNP patients (mean 2.1).CONCLUSIONS: All patients with primary immune-mediated PNP were diagnosed with vitamin D deficiency and they had significantly lower 25(OH)D values than healthy control persons and MND patients. We suggest monitoring of vitamin D status in patients with autoimmune PNP, since immune cells are responsive to the ameliorative effects of vitamin D.
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| 30. |
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| 31. |
- Filioglo, A, et al.
(författare)
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Outcomes after reperfusion therapies in patients with ACA stroke: A multicenter cohort study from the EVATRISP collaboration.
- 2022
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Ingår i: Journal of the neurological sciences. - : Elsevier BV. - 1878-5883 .- 0022-510X. ; 432
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Tidskriftsartikel (refereegranskat)abstract
- Patients with stroke secondary to occlusions of the anterior cerebral artery (ACA) often have poor outcomes. The optimal acute therapeutic intervention for these patients remains unknown.Patients with isolated ACA-stroke were identified from 10 centers participating in the EndoVascular treatment And ThRombolysis in Ischemic Stroke Patients (EVATRISP) prospective registry. Patients treated with endovascular thrombectomy (EVT) were compared to those treated with intravenous thrombolysis (IVT). Odds ratios with 95% confidence intervals (OR; 95%CI) were calculated using multivariate regression analysis.Included were 92 patients with ACA-stroke. Of the 92 ACA patients, 55 (60%) were treated with IVT only and 37 (40%) with EVT (±bridging IVT). ACA patients treated with EVT had more often wake-up stroke (24% vs. 6%, p=0.044) and proximal ACA occlusions (43% vs. 24%, p=0.047) and tended to have higher stroke severity on admission [NIHSS: 10.0 vs 7.0, p=0.054). However, odds for favorable outcome, mortality or symptomatic intracranial hemorrhage did not differ significantly between both groups. Exploration of the effect of clot location inside the ACA showed that in patients with A1 or A2/A3 ACA occlusions the chances of favorable outcome were not influenced by treatment allocation to IVT or EVT.Treatment with either IVT or EVT could be safe with similar effect in patients with ACA-strokes and these effects may be independent of clot location within the occluded ACA.
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| 32. |
- Forsberg, Anette, 1965-, et al.
(författare)
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Impairment in Guillain-Barré syndrome during the first 2 years after onset : A prospective study
- 2004
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 227:1, s. 131-138
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Tidskriftsartikel (refereegranskat)abstract
- ObjectivesTo provide a comprehensive description of impairment in patients with Guillain–Barré syndrome (GBS) in Sweden during the first 2 years after disease onset.MethodsIn this prospective multi-centre study, 42 patients, mean age 52 years, were evaluated at 2 weeks, 2 months, 6 months, 1 year and 2 years. Evaluations made use of validated, reliable measures of muscle strength, grip strength, finger dexterity, balance, facial-muscle function, respiratory function, gait, motor performance and sensory examination, and included patients' owns assessments of pain, fatigue and paraesthesia.ResultsMechanical ventilation was required in 21% of patients. At 2 weeks, 1 year and 2 years after GBS onset: 100%, 62% and 55% of patients had submaximal overall muscle strength; 98%, 38% and 31% subnormal grip strength; and 38%, 14% and 12% affected facial-muscle function. At the same time points, 62%, 10% and 7% of patients were unable to walk 10 m independently; and affected sensation was detected in 93%, 55% and 52%.ConclusionsRecovery occurred mainly during the first year after onset. At 2 years, motor impairment and sensory impairment were each still detectable in more than 50% of patients. We conclude that residual impairment is significant, somatically widespread and, likely, persistent.
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| 33. |
- Forsberg, Anette, 1965-, et al.
(författare)
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Residual disability 10 years after falling ill in Guillain-Barré syndrome : a prospective follow-up study
- 2012
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Ingår i: Journal of the Neurological Sciences. - Amsterdam, Netherlands : Elsevier. - 0022-510X .- 1878-5883. ; 317:1-2, s. 74-79
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To describe residual disability 10years after onset of Guillain-Barré syndrome (GBS) and longitudinal changes from 2weeks after onset until 10years afterwards. The Erasmus GBS Outcome score (EGOS) was applied for predicting prognosis at 2 and 10years.Methods: Twenty-nine patients, mean age at onset 49years, were followed prospectively from 2weeks to 10years after GBS onset. Measures included; GBS disability score, EGOS, Barthel Index, Frenchay Activity Index, Sickness Impact Profile (SIP), Overall Neuropathy Limitations Scale (ONLS), Walk-12, and Fatigue Severity Scale.Results: At 10years, the facial paralysis found in 5 participants at 2years was still present, 11 participants (38%) experienced paresthesia, 6 (21%) had limitations in their arms, and 15 (52%) had limitations in walking. Decreased health-related quality of life on comparison to the general population was seen in the physical dimension of SIP at 10years. The median EGOS at 2weeks was 4.5, which correlated highly only with the Barthel Index at 2years and the ONLS arm scale at 10years.Conclusion: The residual disabilities at 1-2years comprised mainly of reduced walking ability, and are still persistent 10years after GBS onset. For some individuals, facial paralysis caused major disability. The EGOS only partly predicted residual disability at 2 and 10years after onset.
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| 34. |
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| 35. |
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| 36. |
- Ghali, MGZ, et al.
(författare)
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Galenic Pial Arteriovenous Fistulas in Adults
- 2020
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Ingår i: Journal of the neurological sciences. - : Elsevier BV. - 1878-5883 .- 0022-510X. ; 416, s. 117014-
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Tidskriftsartikel (refereegranskat)
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| 37. |
- 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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| 38. |
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| 39. |
- Gustafson, Deborah, 1966
(författare)
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Adiposity hormones and dementia.
- 2010
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Ingår i: Journal of the neurological sciences. - : Elsevier BV. - 1878-5883 .- 0022-510X. ; 299:1-2, s. 30-344
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Tidskriftsartikel (refereegranskat)abstract
- Adipose tissue is an endocrine and paracrine organ that contributes to both metabolic and vascular homeostasis. Overweight and obesity due to excess adipose tissue, are cornerstones of vascular risk and increase risk for late-onset dementia. Vascular risk does not exist in isolation, and is accompanied by alterations in hormonal metabolism and metabolic syndromes. Thus, while vascular risk is highlighted as a primary mechanism for elevated dementia occurrence due to obesity, hormonal risk states may also precede or result from underlying dementia-related neuropathologies and direct neuronal toxicity. This is exemplified during the prodromal phase of dementia, as vascular and metabolic parameters decline in relation to dementia development, and potentially in a way that is different from 'normal' aging. In this review will be presented a review of the epidemiology of adiposity and dementia; adipose tissue biology; and two major hormones produced by adipose tissue, leptin and adiponectin, that interact directly with the brain. In addition, a synthesis related to other lines of supporting evidence for the role of adipose hormones in dementia will be provided. Understanding the role of adipose tissue in health of the brain is pivotal to a deeper understanding of dementia processes.
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| 40. |
- Haghighi, Sara, et al.
(författare)
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Increased CSF sulfatide levels and serum glycosphingolipid antibody levels in healthy siblings of multiple sclerosis patients
- 2013
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 326:1-2, s. 35-39
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Tidskriftsartikel (refereegranskat)abstract
- A proportion of healthy siblings of multiple sclerosis (MS) patients have an oligodonal immunological reaction in their cerebrospinal fluid (CSF) termed the "MS oligoclonal trait". The CSF levels of the major myelin glycosphingolipid sulfatide and serum antibodies against the glycosphingolipids sulfatide and galactosylceramide were recently reported to be increased in MS patients. We studied the levels of these substances in pairs of 46 patients and their 46 healthy siblings and 50 unrelated healthy blood donors (HBD). The sulfatide concentration in CSF was assayed by thin layer chromatography and immunostaining, and the concentration of galactosylceramide by densitometry after thin layer chromatography. Anti-glycosphingolipid antibody levels were assayed by ELISA. In the healthy siblings, the CSF sulfatide concentrations were markedly increased (p<0.001, age adjusted p = 0.025), and the serum IgM anti-GalCer antibodies were increased in healthy siblings compared with HBD (p = 0.02). The increased sulfatide or antibody levels did not co-segregate with the "MS oligoclonal trait" or the HLA-DR15 phenotype. In conclusion, a proportion of healthy siblings of MS patients have increased CSF sulfatide and anti-glycosphingolipid antibody levels, which may, analogous to the "MS oligoclonal trait", constitute an "MS glycosphingolipid endophenotype". Endophenotypes could potentially simplify the genetics of complex disorders
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| 41. |
- Hjæresen, Simone, et al.
(författare)
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High temperature requirement A1 and macrophage migration inhibitory factor in the cerebrospinal fluid; a potential marker of conversion from relapsing-remitting to secondary progressive multiple sclerosis
- 2024
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 457
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Tidskriftsartikel (refereegranskat)abstract
- Background: Predictive and prognostic biomarkers for multiple sclerosis (MS) remain a significant gap in MS diagnosis and treatment monitoring. Currently, there are no timely markers to diagnose the transition to secondary progressive MS (SPMS). Objective: This study aims to evaluate the discriminatory potential of the High temperature requirement serine protease (HTRA1)/Macrophage migration inhibitory factor (MIF) cerebrospinal fluid (CSF) ratio in distinguishing relapsing-remitting (RRMS) patients from SPMS patients. Methods: The MIF and HTRA1 CSF levels were determined using ELISA in healthy controls (n = 23), RRMS patients before (n = 22) and after 1 year of dimethyl fumarate treatment (n = 11), as well as in SPMS patients before (n = 11) and after 2 years of mitoxantrone treatment (n = 7). The ability of the HTRA1/MIF ratio to discriminate the different groups was determined using receiver operating curve (ROC) analyses. Results: The ratio was significantly increased in treatment naïve RRMS patients while decreased again in SPMS patients at baseline. Systemic administrated disease modifying treatment (DMT) only significantly affected the ratio in RRMS patients. ROC analysis demonstrated that the ratio could discriminate treatment naïve RRMS patients from SPMS patients with 91% sensitivity and 100% specificity. Conclusion: The HTRA1/MIF ratio is a strong candidate as a MS biomarker for SPMS conversion.
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| 44. |
- Jakobsson Larsson, Birgitta, et al.
(författare)
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Coping with amyotrophic lateral sclerosis; from diagnosis and during disease progression
- 2016
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 361, s. 235-242
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Tidskriftsartikel (refereegranskat)abstract
- To evaluate coping strategies among patients with Amyotrophic lateral sclerosis starting with diagnosis and during the disease progression, as well as investigate changes and correlations between coping strategies, emotional well-being and physical function. A total of 36 patients participated in the study. The patients filled out the Hospital Anxiety and Depression Scale and the Motor Neuron Disease Coping Scale. Physical function was measured using the revised ALS functional rating scale. Data were collected regularly from diagnosis and over a two years period. As a way to cope with the disease patients relied on both problem focused and emotional focused strategies. The use of coping strategies remained stable. Both physical disabilities and emotional well-being was related to some coping strategies, with some variation during the disease progression. Moreover, some coping strategies were related to symptoms of anxiety and depression. Irrespective of whether the coping strategies affect the emotional well-being or vice versa, the results show the importance of early and continuous evaluation of coping and emotional well-being to ease the emotional distress and provide support to the patient so that he/she can cope with the disease during the disease progression.
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| 45. |
- Jakobsson Larsson, Birgitta, 1965-, et al.
(författare)
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Symptoms of anxiety and depression in patients with amyotrophic lateral sclerosis and their relatives during the disease trajectory
- 2023
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Ingår i: Journal of the Neurological Sciences. - : Elsevier. - 0022-510X .- 1878-5883. ; 455
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTS: The aim of this study was to describe the presence of anxiety and depression among patients with Amyotrophic Lateral Sclerosis (ALS) and their relatives from diagnosis and during the disease progression. An additional aim was to explore if the patient's physical function correlated with the patients' or relatives' anxiety and depression.METHODS: A prospective and longitudinal study, including 33 patients with ALS and their relatives who filled out the Hospital Anxiety and Depression Scale (HADS) at the time of diagnosis and over a period of two years. The patient's physical function was measured with the revised Amyotrophic Lateral Sclerosis Functional and Rating Scale (ALS FRS-R).RESULTS: The results showed that many patients (45%) and relatives (58%) had symptoms of anxiety and that 13% of the patients and 29% of the relatives had symptoms of depression soon after the patient had been diagnosed with ALS. The prevalence of anxiety decreased over time in the group of patients but remained stable in the group of relatives. Relatives had more symptoms of anxiety compared to patients. There was a correlation between the patient's physical function and HADS in the group of relatives; however, no correlation was found in the group of patients.CONCLUSION: The results showed that many patients and relatives suffered from symptoms of anxiety quite soon after their diagnosis, and that many relatives had symptoms of anxiety during the disease trajectory. This highlights the need to continuously measure patients' anxiety/depression level but also to pay attention to symptoms among relatives.
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| 48. |
- Johansson, Anders, et al.
(författare)
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Evidence for astrocytosis in ALS demonstrated by 11C(L)-deprenyl-D2 PET
- 2007
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Ingår i: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 255:1-2, s. 17-22
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Tidskriftsartikel (refereegranskat)abstract
- Objective: To use deuterium-substituted [11C](l)-deprenyl PET to depict astrocytosis in vivo in patients with amyotrophic lateral sclerosis (ALS). Background: In human brain, the enzyme MAO-B is primarily located in astrocytes. l-deprenyl binds to MAO-B and autoradiography with 3H-l-deprenyl has been used to map astrocytosis in vitro. Motor neuron loss in ALS is accompanied by astrocytosis and astrocytes may play an active role in the neurodegenerative process. Deuterium-substituted [11C](l)-deprenyl PET provides an opportunity to localize astrocytosis in vivo in the brain of patients with ALS. Methods: Deuterium-substituted [11C](l)-deprenyl PET was performed in seven patients with ALS and seven healthy control subjects. Results: Increased uptake rate of [11C](l)-deprenyl was demonstrated in ALS in pons and white matter. Conclusion: This study provides evidence that astrocytosis may be detected in vivo in ALS by the use of deuterium-substituted [11C](l)-deprenyl PET though further studies are needed to determine whether deuterium-substituted [11C](l)-deprenyl binding tracks disease progression and reflects astrocytosis.
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| 49. |
- Johansson, A, et al.
(författare)
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Hypercortisolism after stroke--partly cytokine-mediated?
- 1997
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Ingår i: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 147:1, s. 43-7
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Tidskriftsartikel (refereegranskat)abstract
- Increased activity of the hypothalamic-pituitary-adrenal (HPA) axis is common early after stroke. Hypercortisolism is a prominent manifestation. Normally the secretion of cortisol is regulated by adrenocorticotrophic hormone (ACTH), but recently an ACTH/cortisol dissociation after stroke was reported. Cytokines may influence the HPA axis, and plasma IL-6 levels are elevated following stroke. We investigated correlations between cortisol, ACTH, and cytokines, and between blood pressure and blood hormone levels early after stroke in seven stroke patients. All had neurological symptoms secondary to brain infarctions. Blood samples for analysis of cortisol, ACTH, IL-6, TNF alpha, norepinephrine, and epinephrine were collected four times daily, and 24-h blood pressure was measured. Plasma IL-6, but not ACTH, correlated significantly to serum cortisol. Catecholamine levels correlated with cytokine and cortisol levels. This study suggests that several routes for HPA-axis dysregulation is present early after stroke. Cytokine release may play an important role in this situation.
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