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- Brinkmalm-Westman, Ann, 1966, et al.
(author)
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Proteomics/peptidomics tools to find CSF biomarkers for neurodegenerative diseases.
- 2009
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In: Frontiers in bioscience : a journal and virtual library. - : IMR Press. - 1093-4715. ; 14, s. 1793-806
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Research review (peer-reviewed)abstract
- Neurodegenerative diseases are characterized by premature neuronal loss in specific brain regions. During the past decades our knowledge on molecular mechanisms underlying neurodegeneration has increased immensely and resulted in promising drug candidates that might slow down or even stop the neuronal loss. These advances have put a strong focus on the development of diagnostic tools for early or pre-clinical detection of the disorders. In this review we discuss our experience in the field of neuroproteomics/peptidomics, with special focus on biomarker discovery studies that have been performed on CSF samples from well-defined patient and control populations.
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3. |
- Hallböök, Tove, et al.
(author)
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Beneficial effects on sleep of vagus nerve stimulation in children with therapy resistant epilepsy.
- 2005
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In: European Journal of Paediatric Neurology. - : Elsevier BV. - 1090-3798. ; 9:6, s. 399-407
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Journal article (peer-reviewed)abstract
- The study purpose was to evaluate sleep structure following Vagus Nerve Stimulation (VNS) in 15 children with therapy resistant epilepsy and to correlate possible alterations with changes in epileptiform activity and clinical effects. Fifteen children were examined with ambulatory polysomnographic recordings initially, and after 3 and 9 months of VNS-treatment. Sleep parameters, all-night delta power activity and movement times (MTs), used to account for arousals were estimated. Epileptiform activity was evaluated by spike detection. Seizure frequency was recorded in a diary. The severity of the seizures was scored with the National Hospital Seizure Severity Scale (NHS3). Quality of life (QOL) was assessed by a visual analogue scale. Behaviour problems were quantified by using the total score of the Child Behaviour Checklist (CBCL). VNS induces a significant increase in slow wave sleep (SWS) and a decrease in sleep latency and in stage 1 sleep. The number and density of MTs during total night sleep were significantly increased. There was also a significant increase in the number of MTs immediately related to the VNS stimulation periods. Of the 14 children with increased MTs, 10 had a reduction in epileptiform activity, and in clinical seizures, all had an improvement in NHS3, and 11 in QOL. Of the 10 children with increased SWS, eight also improved in QOL and eight in behaviour. Our findings indicate that VNS counteracts known adverse effects of epilepsy on sleep and increases slow wave sleep. This possibly contributes to the reported improvement in well-being. We also see an increase in MTs. This arousal effect seems to be of minor importance for QOL and could possibly be related to the antiepileptic mechanisms in VNS.
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- Hallböök, Tove, et al.
(author)
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Long term effects on epileptiform activity with vagus nerve stimulation in children.
- 2005
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In: Seizure. - : Elsevier BV. - 1532-2688 .- 1059-1311. ; 14:8, s. 527-533
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Journal article (peer-reviewed)abstract
- Purpose: We report tong-term effects of vagus nerve stimulation (VNS) on epileptiform activity in 15 children, and how these changes are related to activity stage and to clinical effects on seizure reduction, seizure severity (NHS3) and quality of life (QOL). Methods: Initially, and after 3 and 9 months of VNS-treatment, 15 children were investigated with 24 h ambulatory EEG monitoring for spike detection. The number of interictal epiteptiform discharges (IEDs) and the inter spike intervals (ISIs) were analysed during 2 h in the awake state, and 1 h of rapid eye movement (REM)-, spindle- and delta-steep, respectively. Total number and duration of electrographic seizure episodes were also analysed. Results: At 9 months the total number of IEDs was significantly reduced (p = 0.04). There was a tendency of reduction in all activity stages, and significantly so in delta-steep (p = 0.008). Total etectrographic seizure number was significantly reduced in the 24 h EEG at 3 and 9 months (p = 0.03, 0.05). There was a significant concordance in direction of changes in epileptiform activity and etectrographic seizures at 9 months (p = 0.04). Concordance in direction of changes was seen in 9 of 15 children between clinical seizures and IED (p > 0.3), in 10 of 15 children between QOL and IED (p = 0.3) and in 8 of 15 children between NHS3 and IED (p > 0.3). There was no direct correlation between the extent of improvement in these clinical data and the degree of spike reduction. Conclusion: This study shows that VNS reduces IEDs especially in REM and delta steep, as well as the number of electrographic seizures. It also shows a concordance between reduction in IEDs and etectrographic seizures.
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- Hallböök, Tove, et al.
(author)
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Vagus nerve stimulation in 15 children with therapy resistant epilepsy; its impact on cognition, quality of life, behaviour and mood.
- 2005
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In: Seizure. - : Elsevier BV. - 1532-2688 .- 1059-1311. ; 14:Feb 19, s. 504-513
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Journal article (peer-reviewed)abstract
- PURPOSE: Vagus nerve stimulation (VNS) is a neurophysiologic treatment for patients with refractory epilepsy. There is growing evidence of additional quality of life (QOL) benefits of VNS. We report the effects of VNS on seizure frequency and severity and how these changes are related to cognitive abilities, QOL, behaviour and mood in 15 children with medically refractory and for surgery not eligible epilepsy. METHODS: Initially, and after 3 and 9 months of VNS-treatment, 15 children were investigated with Bayley Scales of Infant Development (BSID), Wechsler Preschool and Primary Scale of Intelligence (WPPSI-R), Wechlser Intelligence Scales for Children (WISC-III) depending on the child's level of functioning, a Visual Analogue Scale for validating QOL, Child Behaviour Checklist (CBCL) for quantifying behaviour problems, Dodrill Mood Analogue Scale and Birleson Depression Self-Rating Scale, and the National Hospital Seizure Severity Scale (NHS3). A diary of seizure frequency was collected. RESULTS: Six of 15 children showed a 50% or more reduction in seizure frequency; one of these became seizure-free. Two children had a 25-50% seizure reduction. Two children showed increased seizure frequency. In 13 of 15 children there was an improvement in NHS3. The parents reported shorter duration of seizure and recovery phase. There were no changes in cognitive functioning. Twelve children showed an improvement in QOL. Eleven of these also improved in seizure severity and mood and five also in depressive parameters. CONCLUSION: This study has shown a good anti-seizure effect of VNS, an improvement in seizure severity and in QOL and a tendency to improvement over time regarding behaviour, mood and depressive parameters. The improvement in seizure severity, QOL, behaviour, mood and depressive parameters was not related to the anti-seizure effect.
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- Hellström-Westas, Lena, et al.
(author)
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Amplitude-integrated encephalography in pyridoxine-dependent seizures and pyridoxine-responsive seizures.
- 2002
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In: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 91:8, s. 977-980
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Journal article (peer-reviewed)abstract
- Pyridoxine-dependent seizures are rare in newborn infants, although recent data suggest that the prevalence probably is underestimated. In all newborn infants with recurrent epileptic seizures the general recommendation is to administer pyridoxine and simultaneously record an electroencephalogram (EEG). Conclusion: One infant with pyridoxine-responsive seizures and another with pyridoxine-dependent seizures had different electroclinical responses on amplitude-integrated EEG monitoring (aEEG) when pyridoxine was administered.
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- Hellström-Westas, Lena, et al.
(author)
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Low risk of seizure recurrence after early withdrawal of antiepileptic treatment in the neonatal period
- 1995
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In: Archives of disease in childhood. Fetal and neonatal edition. - 1359-2998. ; 72:2, s. 97-101
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Journal article (peer-reviewed)abstract
- The risk of seizure recurrence within the first year of life was evaluated in infants with neonatal seizures diagnosed with a combination of clinical signs, amplitude-integrated electroencephalogram (EEG) monitoring, and standard EEG. Fifty eight of 283 (4.5%) neonates in tertiary level neonatal intensive care had seizures. The mortality in the infants with neonatal seizures was 36.2%. In 31 surviving infants antiepileptic treatment was discontinued after one to 65 days (median 4.5 days). Three infants received no antiepileptic treatment, two continued with prophylactic antiepileptic treatment. Seizure recurrence was present in only three cases (8.3%)--one infant receiving prophylaxis, one treated for 65 days, and in one infant treated for six days. Owing to the small number of infants with seizure recurrence, no clinical features could be specifically related to an increased risk of subsequent seizures. When administering antiepileptic treatment, one aim was to abolish both clinical and electrographical seizures. Another goal was to minimise the duration of treatment and to keep the treatment as short as possible. It is suggested that treating neonatal seizures in this way may not only reduce the risk of subsequent seizure recurrence, but may also minimise unnecessary non-specific prophylactic treatment for epilepsy.
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- Nordgren, Ann, et al.
(author)
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Identification of numerical and structural chromosome aberrations in 15 high hyperdiploid childhood acute lymphoblastic leukemias using spectral karyotyping
- 2001
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In: European Journal of Haematology. - : Wiley. - 1600-0609 .- 0902-4441. ; 66:5, s. 297-304
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Journal article (peer-reviewed)abstract
- Spectral karyotyping (SKY) on metaphase spreads from 15 high hyperdiploid (>51 chromosomes) childhood acute lymphoblastic leukemias (ALL), which typically display a poor chromosome morphology, was performed in order to investigate the pattern of numerical abnormalities, reveal the chromosomal origin of marker chromosomes, and identify translocations and other interchromosomal rearrangements not detected by G-banding analysis. In all cases the numerical changes could be fully characterized, and a non-random pattern of chromosomal gain was identified, with chromosomes X, 21, 14, 17, 6, 18, 4, and 10 being most frequently gained. The numerical changes had been partly misinterpreted in 12 of the 15 ALL patients using G-banding, and the present study hence emphasizes the importance of SKY in identifying such anomalies, some of which, i.e. +4 and +10, have been suggested to be prognostically important. The chromosomal origin of all marker chromosomes and of seven structural rearrangements, one of which was the prognostically important Philadelphia chromosome, could be identified. Five rearrangements [der(1)t(1;14)(q32;q21), der(2)t(2;8)(q36;?), der(3)t(2;3)(q21;?), der(8)t(8;14)(?;?), and t(9;21)(q12;q22)] have previously not been reported in ALL, emphasizing the value of SKY in identifying novel chromosomal rearrangements.
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11. |
- Ors, Marianne, et al.
(author)
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Auditory event-related brain potentials in children with specific language impairment
- 2002
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In: European Journal of Paediatric Neurology. - : Elsevier BV. - 1090-3798. ; 6:1, s. 47-62
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Journal article (peer-reviewed)abstract
- Event-related brain potentials evoked by tone and speech stimuli were recorded in ten children with specific language impairment and a control group. The patient group showed prolonged P3 latencies in both tasks compared with the controls and smaller P3 amplitude in the speech task. There were no group differences concerning the N1-P2 components. The findings indicate that the children with language impairment showed signs of deficient late-stage auditory perceptual processing whereas the earlier sensory stages as indexed by the N1 were no different from the controls. The results are further discussed within the framework of context updating in working memory.
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12. |
- Ors, Marianne, et al.
(author)
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Auditory event-related brain potentials in parents of children with specific language impairment
- 2002
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In: European Journal of Paediatric Neurology. - : Elsevier BV. - 1090-3798. ; 6:5, s. 60-249
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Journal article (peer-reviewed)abstract
- Auditory event-related brain potentials evoked in response to tone stimuli and to speech stimuli were recorded in a group of parents of children with specific language-impairment and a group of parents of normally speaking children. The parents of the language-impaired children showed longer P3 latencies than the parental control group in the speech task requiring a phonological discrimination, but did not differ from the controls in the linguistically non-demanding tone discrimination task. The longer P3 latency was associated with a positive parental history of language delay. There were no group differences concerning the N1 component in any of the tasks. The findings indicate that parents of children with specific language impairment show signs of deficient late-stage perceptual higher order linguistic processing, whereas the earlier central sensory detection stage of the phonological information is no different from the controls. Our observations are particularly interesting with regard to a study of the children of these two parental groups, where the language-impaired children showed longer P3 latencies than controls in both a tone task and a speech task, whereas there were no differences between the children concerning the N1 component. We propose that deficient late-stage auditory higher order perceptual processing as indexed by the longer P3 latency to speech stimuli observed both in children with specific language-impairment and in their parents may represent a constitutional trait, contributing to the language acquisition difficulties in these children.
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13. |
- Ors, Marianne, et al.
(author)
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SPECT findings in children with specific language impairment.
- 2005
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In: Cortex. - 1973-8102. ; 41:3, s. 316-326
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Journal article (peer-reviewed)abstract
- Findings from 99mTc-HMPAO SPECT measurements at rest in a group of 19 school-age children with specific language impairment (SLI) were compared to a group of 12 children with attention-deficit hyperactivity disorder (ADHD). The regional cerebral blood flow (CBF) distributions were different in the two groups. Children with SLI showed significantly lower CBF values in the right parietal region and in the subcortical region compared to the ADHD group. In addition, the SLI group had symmetric CBF distributions in the left and right temporal regions, whereas the ADHD group showed the usual asymmetry with left-sided hemispheric predominance in the temporal regions. The findings give further evidence for anomalous neurodevelopment with deviant hemispheric lateralization as an important factor in the aetiology of SLI. They also point to the role of subcortical structures in language impairment in childhood. Earlier focus on cortical structures in SLI research needs to be widened to include subcortical regions as well.
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- Portelius, Erik, 1977, et al.
(author)
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Exploring Alzheimer molecular pathology in Down's syndrome cerebrospinal fluid.
- 2014
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In: Neuro-degenerative diseases. - : S. Karger AG. - 1660-2862 .- 1660-2854. ; 14:2, s. 98-106
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Journal article (peer-reviewed)abstract
- Individuals with Down's syndrome (DS) develop early Alzheimer's disease (AD) with β-amyloid (Aβ) plaque pathology. The extra amyloid precursor protein (APP) gene copy in DS is believed to result in a 50% increase in Aβ production, but it is unclear how this relates to the development of other AD hallmarks, including axonal degeneration and microglia cell activation, and to other neurological problems in DS, including disturbed sleep regulation.
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16. |
- Schain, Frida, et al.
(author)
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Real-world clinical characterization, healthcare resource utilization and productivity loss in chronic graft versus host patients exposed to extracorporeal photopheresis in Sweden
- 2023
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In: Transfusion and apheresis science. - : Elsevier BV. - 1473-0502 .- 1878-1683. ; 62:3
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Journal article (peer-reviewed)abstract
- Background:Extracorporeal photopheresis (ECP) is frequently used to treat moderate-severe chronic graft versus host disease (cGVHD), however limited data exists describing ECP treatment effects on healthcare and societal costs. We aimed to characterize clinical and health economic outcomes and productivity loss in cGVHD patients exposed to ECP.Methods:We identified 2708 patients aged ≥ 18 years with a record of allogeneic hematopoietic stem cell transplantation (HSCT) in the Swedish Patient Register between 2006 and 2020. Patients exposed to ECP from 3-months post HSCT (index) were included (n= 183). Data was linked to the Prescribed Drug Register, the Cause of Death Register, and the Longitudinal Integrated Database for Health Insurance and Labor Market Studies (LISA).Results:The median patient age at index was 51 years (IQR1–3; 38–61). In the 3-month period before ECP initiation compared to 9–12 months post-ECP, the cumulative three-month dose per patient decreased prednisolone/prednisone (1,381 mg vs. 658 mg, p < 0.001) and cyclosporin (12,242 mg vs. 3,501 mg, p < 0.001). Infection incidence also decreased over the same period (79.2% vs 59.1%, p < 0.001). Time spent in healthcare decreased from 68.9% to 22.1% from the first and fifth follow-up year respectively, and corresponding annual healthcare cost reduced from €27,719 to €1,981. Among patients < 66 years of age, sickness-related workplace absence decreased from 73.2% to 31.9% between the first and fifth follow-up year, with median annual productivity loss decreasing from €20,358 to €7,211 per patient.Conclusions:ECP was associated with reduced use of corticosteroids, immunosuppressive agents, and fewer infections. Furthermore, cost and healthcare utilization decreased over time.
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17. |
- Sundgren, P C, et al.
(author)
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Adrenoleukodystrophy--unusual CT and MR findings in two siblings. Case reports
- 1998
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In: Acta Radiologica. - 1600-0455. ; 39:1, s. 77-80
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Journal article (peer-reviewed)abstract
- PURPOSE: To describe the unusual appearance of adrenoleukodystrophy (ALD) in MR imaging. MATERIAL AND METHODS: An analysis was made of MR findings, histopathology and clinical course in the cases of two brothers with ALD. RESULTS: The older brother presented with frontal contrast-enhanced lesions, including a cyst. These were initially misinterpreted as a tumour at both radiology and pathology. The correct diagnosis was made after the discovery of very-long-chain fatty acids in the blood. The younger brother presented initially with involvement of various segments of the corticospinal tract. CONCLUSION: It is important that the radiologist be aware of the variants of ALD in MR imaging so as to avoid diagnostic mistakes.
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