Rev Neurol France
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Urea cycle disorders (UCD) usually present after 24 h to 48 h of life with failure to thrive, lethargy and coma leading to death, but milder forms may occur from infancy to adulthood. ⋯ Potentially fatal acute hyperammonemia may occur in male or female patients at any age. Ammonia should be measured promptly in case of acute neurological and psychiatric symptoms or coma.
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Our university hospital serves a population of 300 000 inhabitants. Stroke is the leading cause of admission in our department of neurology. In June 2003, when the Emergency Department (ED) was closed in our institution, was created an acute stroke network (ASN), comprising 2 beds of direct admission and thrombolysis in the intensive care unit, and 4 beds dedicated to stroke care in the department of neurology, in which standardized stroke care protocols were implemented. ⋯ Creation of an ASN was associated with a significant decrease of admission and imaging delays, due to a strong collaboration with EMS, and with a better outcome for hemorrhagic stroke patients. Treatment with intravenous thrombolysis in the first 3 hours could be used widely and was efficient and safe. However, the creation of dedicated stroke units for all stroke patients remains necessary to improve quality of care and outcome.
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Dysimmune neuropathies, in common with other neuropathies, comprise an axonal impairment that it is primary or secondary to a demyelinating process. We consider here axonal impairment in the course of certain dysimmune neuropathies, such as the Guillain Barré syndrome, chronic inflammatory demyelinating polyradiculoneuritis and multiple conduction block neuropathy. We mention the fact that it is not always easy to evidence the axonal impairment, its severity and its potential for regeneration. ⋯ It is generally recognized that it is only useful if applied for a period of weeks, although this is currently a matter of debate. Other therapeutic options have been discussed and proposed, although to date there is a lack of proven efficiency: such treatments include neuroprotective agents and drugs which block sodium/potassium ion channels. It is increasingly difficult to propose new treatments with validated efficiency, due to the small number of patients presenting dysimmune neuropathies of the type discussed here that are both typical and suitable for inclusion in medium to long term studies.
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T cell apoptosis has been studied in animal models for human autoimmune disorders of the nervous system and in other tissues devoid of specialized immune-defense mechanisms. Our data suggest that the central nervous system has a high potential to eliminate T cell inflammation, whereas this mechanism is less effective in the peripheral nervous system, and even more in muscle and skin. In-vitro experiments indicate different scenarios how specific cellular and humoral elements in the nervous system may synergize and sensitize T cells for apoptosis in-vivo. ⋯ This is further substantiated since neutralization of TNF-alpha in MS patients increased cellular inflammation and relapses. Therapeutically several conventional and novel approaches like glucocorticosteroids and high-dose antigen therapy induce T cell apoptosis in-situ. We also discuss regulatory, proapoptotic mechanisms such as the Fas/FasL system and counterregulatory mechanisms that have been utilized to limit tissue damage.
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Case Reports
[Transient high-intensity T2 signal in the splenium of the corpus callosum in a non-epileptic patient].
Transient splenial lesions of the corpus callosum have been mainly reported in epileptic patients. We report the case of a non-epileptic woman with bipolar affective disorder treated by oxcarbazepine which was withdrawn because of a mild hyponatremia (128 mmol/l). A confusional state followed withdrawal and the electroencephalogram was free of spike or sharp waves. ⋯ Different mechanisms of this brain MRI abnormality are discussed including the sudden withdraw of oxcarbazepine. Prognosis of transient splenial lesions of the corpus callosum is good. Clinicians should search for recent metabolic disorders and therapeutic modifications.