| 1. |
- Einarsdottir, Margret Jona, et al.
(författare)
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Intermittent high-dose glucocorticoid treatment does not cause adrenal insufficiency in patients with diffuse large B-cell lymphoma - a prospective study.
- 2023
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Ingår i: Acta haematologica. - 1421-9662.
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Tidskriftsartikel (refereegranskat)abstract
- Glucocorticoid (GC) treatment suppresses the hypothalamic-pituitary-adrenal axis and can cause GC-induced adrenal insufficiency. In this study we investigated the incidence of GC-induced adrenal insufficiency in patients receiving intermittent short-term high-dose oral GC treatment for newly diagnosed diffuse large B-cell lymphoma. Cosyntropin stimulation test was used to assess adrenal function at study entry (baseline), at 2 months (before the 5th cycle), and 6 months from baseline (3 months after the last cycle). Ten patients were included (40% women). Mean age was 61 years. The mean (range) plasma morning cortisol was 407 (320-530) nmol/L at baseline, 373 (260-610) nmol/L at 2 months, and 372 (230-520) nmol/L 6 months from baseline. All patients had normal response to cosyntropin stimulation at baseline as well as 2 and 6 months from baseline. Thus, none of the patients developed biochemically verified adrenal insufficiency. Therefore, short-term high-dose GC therapy, a commonly used adjuvant treatment in patients with malignant hematological diseases, does not seem to down-regulate the hypothalamic-pituitary-adrenal axis.
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| 2. |
- Hahne, Oscar, et al.
(författare)
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Epilepsy surgery in patients with hypothalamic hamartomas-Population-based two-year and long-term outcomes
- 2023
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Ingår i: European Journal of Paediatric Neurology. - 1090-3798. ; 46, s. 24-29
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Hypothalamic hamartomas are benign lesions associated with drug resistant epilepsy. Surgical treatment has become an increasingly utilised approach with promising results. This study aims to evaluate seizure outcome and complications after surgery in a population-based series of patients with intractable epilepsy and hypothalamic hamartoma.Methods: All patients with hypothalamic hamartoma treated with epilepsy surgery in Sweden since 1995 with at least two years of follow-up were included. Preoperative, two-, five- and ten-year prospective longitudinal data were collected from The Swedish National Epilepsy Surgery Register. Data included seizure types and frequency, duration of epilepsy, clinical characteristics, neurological deficits, cognitive level and complications. In a subgroup from Gothenburg, we also analysed data not included in the register such as classification of hamartomas, surgical procedures and gelastic seizures.Results: Eighteen patients were operated on during the period 1995-2020. The median age at epilepsy onset was 6 months and age at surgery 13 years. Four were seizure free and another four had >75% reduction in seizure frequency at the two-year follow-up. Two of the 13 patients with a long-term follow-up (five or ten years) were seizure-free and four had >75% reduction in seizure frequency. Three had an increased seizure frequency. No major complications were seen. Five had minor complications. In the Gothenburg subgroup all had open pterional disconnection or intraventricular endoscopic disconnection. Six of 12 were free from gelastic seizures at the two-year follow-up and six of eight at the long-term follow-up. Conclusion: This study supports surgical treatment of hypothalamic hamartomas as a safe method with a low risk of permanent complications. The seizure reduction seems to be persistent over time.
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| 3. |
- Kristjánsdóttir, Ragnhildur, et al.
(författare)
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Cerebrospinal fluid markers in children with cerebral white matter abnormalities.
- 2001
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Ingår i: Neuropediatrics. - : Georg Thieme Verlag KG. - 0174-304X .- 1439-1899. ; 32:4, s. 176-82
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Tidskriftsartikel (refereegranskat)abstract
- Disorders of the cerebral white matter in children constitute a heterogeneous group and the diagnostic work is often complicated. Clinical and radiological characteristics can provide diagnostic clues but there is a need for further diagnostic methods. This study focused on assessing neurochemical "markers" in the cerebrospinal fluid considered to reflect damage to white matter components such as myelin and glial cells as well as neurones with their axons and synapses. The aim was to evaluate whether they contributed to the elucidation of pathogenic processes and the direction of further diagnostic efforts. Seventeen of the 26 cases had increased levels of the glial cell marker ganglioside GD3, indicating gliosis, or of the CNS myelin marker sulfatide, indicating myelin disturbance. As signs of disturbed maturation or sustenance, the nerve cell markers GD1 b, GT1 b and total gangliosides were reduced, as was the synapse marker GD1a. Increased 5-HIAA indicated increased serotonergic turnover. Children with an increased level of the axonal marker Tau protein had a progressive disease whereas GD1a was reduced in the progressive group (n = 11). In contrast, GD3 and HVA were increased in the non-progressive group (n = 15). The chemical profiles were found to be useful, in combination with clinical and radiological findings, when investigating children with white matter abnormalities.
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| 4. |
- Kristjánsdóttir, Ragnhildur, et al.
(författare)
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Clinical characteristics of children with cerebral white matter abnormalities.
- 2000
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Ingår i: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. - : Elsevier BV. - 1090-3798. ; 4:1, s. 17-26
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Tidskriftsartikel (refereegranskat)abstract
- The rapidly expanding use of magnetic resonance imaging (MRI) in children with neurological impairments of unknown aetiology has revealed a large number of children with abnormalities of the cerebral white matter, some with leukodystrophy-like white matter abnormalities on MRI, but non-progressive in clinical presentation and course. The aim of this study was to investigate the clinical and neuroradiological characteristics of 26 children with white matter abnormalities of unknown origin and to find diagnostic clues or indicators of progressive versus nonprogressive disease. The typical child with white matter abnormalities was characterized by onset of symptoms within the first year of life, most often presenting as general developmental delay and hypotonia. Later-appearing signs were spasticity and ataxia and as a rule severe learning and motor disabilities. Serious ophthalmological signs were frequently seen. Perinatal adverse events were rare, infectious aetiologies not indicated but prenatal stigmata relatively common. The clinical course was progressive in 11 children and non-progressive in 15. Late onset presentation was associated with a progressive course whereas prenatal stigmata and asymmetrical white matter lesions only were found in children with a non-progressive disorder. The MRI showed three main patterns: a) a generalized increase of the T2 signal of the white matter in 12 children, b) a bilateral, symmetric but not generalized abnormality in nine and c) asymmetric, focal or multifocal pathology in five. Useful information as to clinical entities and course was obtained from the combined clinical and radiological assessment. A precise nosological diagnosis could be made in six cases. The study showed that white matter abnormalities in children constitute a heterogeneous group of rare and 'anonymous' conditions, motivating collaborative studies for further clarification of background and management.
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| 5. |
- Kristjánsdóttir, Ragnhildur
(författare)
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Disorders of the cerebral white matter in children
- 2001
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- The aims of this thesis were to describe the clinical spectrum in a large group of children characterised as having an abnormal magnetic resonance imaging (MRI) signal from cerebral white matter (WM) and to study the radiological, clinical and ophthalmological features in that subgroup where aetiology was unknown. We also wanted to look at biochemical markers in the cerebrospinal fluid (CSF) to elucidate pathological processes which were responsible for the WM abnormalities. A further aim was to identify useful diagnostic clues and to differentiate a progressive disease from non-progressive disorders.Methods: I. MRI and clinical data (medical records) from 100 children considered to have abnormal cerebral WM were assessed. II. Those 26 children with abnormal WM of unknown cause were studied in detail using clinical, radiological, ophthalmic and neurochemical methods. The ophthalmic examination included visual evoked potentials (VEP). The neurochemical investigations included analyses of CSF markers for myelin (sulfatide), gliosis (ganglioside GD3, glial fibrillary acid protein -GFAP), nerve cells (gangliosides), dendrites (ganglioside GD1b), axons (neurofilament - NFL, tau protein), synapses (gangliosides GM1 and GD1a) and neurotransmission (monoamines: 5-HIAA, HVA, HMPG) as well as seeking signs of infectious or inflammatory processes (protein quantitation -albumin, IgG, IgM- and oligoclonal bands). Results: I. Progressive diseases (for example leukodystrophies and other neurodegenerative disorders) were found in 45 % of the children, a relapsing-remitting course in 15 % (for example MS, ADEM and SLE) and non-progressive disorders (for example maldevelopment and sequel after insults) in 26%. In 14% the clinical course could not be categorised. In about 60 % of the children, the nature or cause of the disorder was unknown. II. The abnormal MRI signal could be classified as: generalised (involving all supratentorial WM) in 12 children; bilateral (symmetric but sparing parts of the WM) in 9 and asymmetric (focal or multifocal) in 5. Progressive disease was indicated by increasing MRI pathologic findings and infratentorial involvement, abnormal VEP, spasticity and positive heredity or by increased levels of NFL, tau protein and very high GFAP in the CSF. Increased GD3 and HVA were found in non-progressive disorders.Conclusions: There is great variability and heterogeneity of cause and manifestations for WM abnormalities in children. The disorders are characterised by signs and symptoms presenting early in life, most often in the form of psychomotor delay and hypotonia. Signs appearing later are spasticity, ataxia and severe motor and learning disabilities. Ophthalmic abnormalities predominate, most often in the form of visual impairment, optic nerve pathology and aberrant VEP. The neurochemical characteristics are signs of increased turnover of myelin, gliosis, neuroaxonal degeneration and disturbed nerve cell maturation and sustenance as well as impaired function of dopaminergic and serotonergic transmitter systems. This study found a combination of radiological, clinical, ophthalmological and neurochemical methods to be the most useful for diagnostic and prognostic purposes.
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| 6. |
- Kristjánsdóttir, Ragnhildur, et al.
(författare)
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Disorders of the cerebral white matter in children. The spectrum of lesions.
- 1996
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Ingår i: Neuropediatrics. - : Georg Thieme Verlag KG. - 0174-304X .- 1439-1899. ; 27:6, s. 295-8
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Tidskriftsartikel (refereegranskat)abstract
- The use of magnetic resonance imaging (MRI) has resulted in the detection of an increasing number of children with an apparently leukodystrophic white matter. Laboratory tests and the clinical presentation, however, often do not correspond to any known entity and the course is sometimes not progressively deteriorating. Such children with white-matter changes and no known diagnosis were the subject of this Swedish multicentre study, in which MRI findings and clinical data from 100 children considered to have white-matter abnormalities were assessed during the period 1992-1995. At re-evaluation of MR images by an established "white-matter group" of neuroradiologists, paediatric neurologists, neurologists and neurochemists, the MRI signal of the white matter was considered normal in eleven children and eleven had mainly a grey matter affection. Of the remaining 78 children with white matter abnormalities, a diagnosis was found in 32, but in 46 children no diagnosis could be established. A progressive downhill course characterised 17, probably representing hitherto undefined types of leukodystrophies. Five children had a relapsing-remitting course, and in 11 it was difficult to establish whether the course was progressive or stationary. The disease was non-progressive in 13. This group of non-leukodystrophic white-matter changes obviously represents maldevelopments of myelin formation, thus dys- or hypomyelination rather than demyelination.
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| 7. |
- Kristjánsdóttir, Ragnhildur, et al.
(författare)
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Glial fibrillary acidic protein and neurofilament in children with cerebral white matter abnormalities.
- 2001
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Ingår i: Neuropediatrics. - : Georg Thieme Verlag KG. - 0174-304X .- 1439-1899. ; 32:6, s. 307-12
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Tidskriftsartikel (refereegranskat)abstract
- Glial fibrillary acidic protein (GFAP) is the major structural protein of the intermediate filaments found in glial cells. Increased levels in the cerebrospinal fluid (CSF) have been found to indicate gliosis. Neurofilament (NFL) is a structural element of neurons, mainly found in large myelinated axons. Its presence in the CSF has been suggested to reflect destruction of axons. The aim of this study was to see if GFAP and NFL in the CSF of children with neurological disabilities and an abnormal signal on magnetic resonance imaging (MRI) of the cerebral white matter could be used to clarify the underlying neuropathology. The potential of GFAP and NFL to differentiate a progressive disease from a stationary disorder was investigated, as was the correlation with disability and clinical findings. CSF from 26 children, eleven with progressive and 15 with non-progressive disorders, was analysed. GFAP was increased in all, interpreted to reflect gliosis. NFL was elevated in seven and considered to indicate ongoing neuroaxonal damage as all but one patient were found to have a progressive disease. GFAP did not differentiate between progressive and non-progressive disorders, although low levels were found in stationary and high levels in progressive disorders. The severity of the disability correlated with the NFL levels, but not with the concentration of GFAP.
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| 8. |
- Kristjánsdóttir, Ragnhildur, et al.
(författare)
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Ophthalmological abnormalities in children with cerebral white matter disorders.
- 2002
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Ingår i: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. - : Elsevier BV. - 1090-3798. ; 6:1, s. 25-33
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Tidskriftsartikel (refereegranskat)abstract
- The use of magnetic resonance imaging (MRI) in children with severe neurological impairment has defined a subgroup with increased T2-signals from cerebral white matter. The causes of white matter abnormalities are for the most part unknown, despite extensive investigation. Their clinical correlates and characteristics have still to be systematically analysed and described. We have compared clinical, ophthalmological and electro-ophthalmological findings in such children to delineate neurological and MRI patterns and have sought to correlate with the progression of disease. Clinical and electro-ophthalmological investigations were performed in 26 children with cerebral white matter abnormalities of unknown aetiology; 25 of the 26 children showed abnormalities, 23 clinical and 18 electro-ophthalmological. Optic nerve abnormalities, severe visual impairment and strabismus were the most common. Electro-ophthalmological abnormalities were increased latencies and abnormal waveform of the visual evoked potentials (VEP). Children with progressive disease all had abnormal VEP, whereas none of the ten children with a normal VEP deteriorated. We conclude that children with cerebral white matter abnormalities almost invariably had ophthalmological and often VEP abnormalities. Normal VEP was correlated with non-progressive disorder, as was hypoplasia or malformation of the papilla, whereas abnormal VEP were associated with progressive disease.
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| 9. |
- Sofou, Kalliopi, et al.
(författare)
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Management of Prolonged Seizures and Status Epilepticus in Childhood: A Systematic Review.
- 2009
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Ingår i: J Child Neurology. - : SAGE Publications. - 1708-8283 .- 0883-0738. ; 24:3, s. 918-26
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Forskningsöversikt (refereegranskat)abstract
- Pediatric prolonged seizures and status epilepticus are medical emergencies necessitating immediate life-support and seizure-control measures. A systematic review of published data on the management of prolonged seizures and status epilepticus showed that buccal midazolam was significantly more effective than rectal diazepam, reaching a seizure- control rate of 70% and recurrence rate of 8%. Intranasal lorazepam was as effective as intramuscular paraldehyde in a cost-restrained setting. In refractory status epilepticus, both intravenous midazolam and valproate were equally effective to intravenous diazepam, with valproate exhibiting significantly faster seizure cessation and safer profile than diazepam, even in infancy. In conclusion, buccal midazolam is efficacious and safe thanks to its convenient route of administration, which may serve as first-line in the treatment of prolonged seizures. Intranasal lorazepam is an effective, easy-to-use, and safe drug for prolonged seizures. Intravenous valproate exhibits favorable efficacy and safety profile as third-line in status epilepticus, refractory to diazepam and phenytoin.
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| 10. |
- Viggedal, Gerd, 1950, et al.
(författare)
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Cognitive development from two to ten years after pediatric epilepsy surgery.
- 2012
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Ingår i: Epilepsy & behavior : E&B. - : Elsevier BV. - 1525-5069. ; 25:1, s. 2-8
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Tidskriftsartikel (refereegranskat)abstract
- The development of cognitive functions and the sustainability of seizure control between two and ten years after epilepsy surgery were prospectively investigated in 17 children and adolescents. Intelligence quotient remained stable. Learning capacity improved. Verbal memory improved in half of the subjects and declined in half, whereas figurative memory declined in most patients. Working memory improved as did attention regarding sustained attention and impulse control. In contrast, reaction times were longer, and the auditory attention span was shorter. Executive functions were not affected. Six subjects (35%) were seizure free at the 10-year follow-up, and a seizure reduction of more than 75% had been achieved in 13 (76%). Seizure control improved in five and seizures recurred in two subjects between the two- and the 10-year follow-up.
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