Rev Neurol France
-
The purpose of the treatment of autoimmune myasthenia gravis is to directly improve neuromuscular transmission, and also to reduce the production or presence of the nicotinic acetylcholine receptor (achR). Acetylcholinesterase inhibitors are the first line treatment with the rapid onset of effect, for all types of myasthenia gravis (ocular, generalized myasthenia gravis, seronegative or seropositive patients). Plasmapheresis or intravenous immunoglobulin (IVIg) is the treatment for exacerbations. ⋯ Corticosteroids are considered a major treatment of myasthenia gravis but the doses and periods of time are still being debated. The combination of corticosteroids and immunosuppressive agents are recommended early to spare corticosteroids. The treatment of myasthenia gravis should be modulated regularly (minimal doses for example).
-
Spontaneous intracranial hypotension (SIH) is an uncommon cause of secondary headache due to a cerebrospinal fluid (CSF) hypotension. Lumbar epidural blood-patch (LEBP) is the most effective treatment and can be repeated in case of relapse. There is no standard therapeutic strategy for patients free of dural tears who fail to respond to several consecutive blood-patches. We report two cases of SIH successfully treated by an epidural saline infusion after two consecutive LEBP. ⋯ It is common procedure to search for a dural tear when patients fail to respond to several consecutive LEPB. Surgical repair is however exceptional. An epidural saline infusion might be an efficient therapeutic alternative despite the small number of cases reported in the literature.
-
The application of de Seze et al. criteria (2007) to patients presenting a first severe acute demyelinating event helps to distinguish acute disseminated encephalomyelitis (ADEM) from other CNS inflammatory diseases, with 83% sensitivity and 95% specificity. We applied these criteria to 14 patients who presented a first severe acute demyelinating event and whose later clinical course enabled clear identification of the neurological diagnosis. ⋯ Applying the new criteria, we did not find the same sensitivity, specificity, and positive and negative predictive values as in the original article. The lack of specificity arose from the misclassification of MS patients with a tumor-like presentation (two out of five false negatives). One of the explanations is that the clinical criteria used can be part of atypical forms of MS, in particular in its tumor-like presentation. De Seze et al. criteria can be an invaluable help for the clinician in the diagnosis of a first severe demyelinating event. Considering our results, these criteria should not be applied for patients with a tumor-like form of MS. A prospective study in a larger cohort is needed to confirm or invalidate these preliminary results.