[Glutamate--the most important transmitter in the brain].
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Biomedical subjects
Publications and source records attributed to Espen Dietrichs.
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BACKGROUND: One of several probable causation theories of Parkinson disease postulates that brain tissue cannot generate sufficient levels of various growth factors required to sustain the viability of dopamine-producing nerve cells in the presence of as yet unknown toxic factors. The study reported here evaluates the ability of externally applied growth factors to protect the dopamine fibres in the basal ganglia in a toxin-induced animal model of the disease. MATERIALS AND METHODS: All animals (rats) were subjected to selective destruction of the dopamine-producing cells in substantia nigra. The rats were divided into three groups. Two groups received intracerebral treatment with either glia-cell derived neurotrophic factor (GDNF) or a combination of brain-derived neurotrophic factor (BDNF) and GDNF. The third group acted as untreated controls and were given sterile saline. The growth factors were infused directly into the brain by an osmotic pump over a period of 28 days. Brain sections taken from all three groups were evaluated by immunocytochemistry. RESULTS: The two groups of rats that received growth factor infusion displayed a significant improvement in their motor behaviour compared to control animals. Immunocytochemistry studies demonstrated that the group receiving a combination of GDNF and BDNF had an increased number of surviving active fibres in the dopamine system striatum in comparison to the control and GDNF groups. In addition the infusion of growth factors resulted in a proliferation of subventricular cells in the basal ganglia. CONCLUSION: The improved motor function following growth factor treatment in this rat model might be due to a delayed retrograde degeneration of the nigrostriatal nerve fibers. Growth factor infusion also clearly stimulated endogenous stem cells and caused their migration towards the striatum. Our observations indicate that the infusion of growth factors into the brain have a symptomatic and neuroprotective effect in this model.
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Serious infection with the bacterium L. monocytogenes mainly manifests as sepsis and/or meningitis. A particular entity is Listeria brain stem encephalitis, which is characterized by progressive brain stem deficits. The condition is fatal unless early treated. The purpose of the present study was to assess the incidence of brain stem encephalitis in a population-based listeriosis material. Medical records from 212 of the 240 patients with serious listeriosis reported in Norway from 1977 to 2000, as well as autopsy material from 8 of these patients, were available. This material was searched for clinical and neuropathological evidence of brain stem infection. Findings indicating brain stem encephalitis were present in 19 of the 172 patients with adult listeriosis (11%) but none of the 40 pregnancy-related listeriosis cases. None of the 19 patients had been diagnosed with Listeria brain stem infection originally. We conclude that brain stem encephalitis is relatively common in this Norwegian listeriosis material.
Brain stem encephalitis is a particular manifestation of infection with the bacterium Listeria monocytogenes. Here, we present the neuropathological findings in 9 such cases. In the brain stem, the inflammatory infiltrates were located predominantly within nuclei and tracts of cranial nerves innervating the oropharynx. These findings support the hypothesis that the food-borne bacterium Listeria monocytogenes invades the brain stem along cranial nerves.
All patients seeking medical or surgical treatment for hemifacial spasm (HFS) in Oslo, Norway were identified in a service-based prevalence study. Only four hospital departments offered services for Oslo citizens with HFS. Fifty patients with HFS were treated. The total prevalence was 9.8 per 100,000. The prevalence increased with age to 39.7 among those older than 70 years. The use of antihypertensive drugs was significantly more common in HFS patients (36%) than in Oslo's general population.
BACKGROUND: Hereditary movement disorders include spinocerebellar disorders, a large and heterogeneous group of syndromes with ataxia or spasticity as the prominent symptom. In spite of the vast clinical and genetic heterogeneity, patterns of pathogenesis slowly emerge and help us understand these disorders. MATERIAL AND METHODS: This review is based on personal experience and recent literature. RESULTS: More than 20 types of hereditary spastic paraparesis have been reported. Dominant SPG4 and SPG3 with mutations in the spastin or the atlastin gene have been identified in many countries. The most prevalent type of recessive ataxia in Europe, Friedreich's ataxia, has become a model of integrated clinical-molecular-therapeutic research. More recessive ataxias (AOA1-2) have been described recently. More than 20 autosomal dominant ataxias have been reported, with 12 identified genes including the episodic ataxias, and 9 mapped. SCA7 appears to be the most frequent type in some Nordic countries. INTERPRETATION: A striking feature of many of these diseases is the involvement of very different genes for similar phenotypes. Conversely, very heterogeneous phenotypes are due to single-gene defects. Recently there has been considerable progress in the clinical description of movement disorders and the understanding of their genetic basis. Possible therapies are emerging.
BACKGROUND: Several dystonias, Huntington's disease, essential tremor and various rare conditions are among the hereditary movement disorders, disorders with considerable genetic and clinical heterogeneity. It may be hoped that better understanding of the genetics and pathogenetic mechanisms involved will improve management. MATERIAL AND METHODS: This review is based on personal experience and recent literature. RESULTS: At least 13 types of dystonias are genetically defined. DYT 1 seems to be the most important, and is easily tested. The Huntington gene is readily available for symptomatic as well as presymptomatic testing. Presymptomatic testing gives rise to ethical concerns and require thorough genetic counselling. Also essential tremor seems to show an autosomal dominant inheritance, but the responsible gene(s) has not been identified. INTERPRETATION: As for other movement disorders, involvement of different genes may give similar phenotypes and defects in a single gene give rise to various different phenotypes.
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All patients seeking medical treatment for primary focal and segmental dystonia in Oslo, Norway, were identified in a service-based prevalence study. A total of 129 patients with focal and segmental dystonia were treated. The total prevalence was 25.4 per 100,000, but the prevalence increased with age from 4.1 (age < 30) to 58.7 (age > 70). The prevalence in subjects of European descent was 28.3 per 100,000, much higher than among first-generation immigrants of Asian and African descent.
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Experimental and clinical studies have shown that the cerebellum participates in the regulation of various visceral responses, including micturition. It is not yet clear through which parts of the central nervous system such cerebellar influences are mediated. However, a series of investigations have shown that the cerebellum is directly or indirectly connected to various centres that appear to be involved in autonomic control. These include parts of the cerebral cortex, the hypothalamus, the periaquaductal grey, nuclei in and around the pontine micturition centre, the dorsal vagal nucleus and nucleus of the solitary tract, and the medullary reticular formation. This article examines some of the circuits that may be involved in cerebellar modulation of visceral reflexes, especially the micturition reflex.