1/4. serum positive botulism with neuropathic features.A 32-year-old man presented with multiple cranial neuropathies and his serum was positive for botulism type B. However, serial electrodiagnostic studies were consistent with a primarily neuropathic process, such as Fisher syndrome, rather than a neuromuscular junction disorder. Electrodiagnostic study findings in patients with presumed neuromuscular junction disorders may mimic findings suggestive of a neuropathic process, or the bioassay for botulism may be falsely positive in patients with Fisher Syndrome.- - - - - - - - - - ranking = 1keywords = neuromuscular junction, junction (Clic here for more details about this article) |
2/4. Childhood miller fisher syndrome: case report and review of the literature.Three years ago Ray Buncic and I were having a break from the lectures at an ophthalmology meeting. Ray told me of his earnest desire to provide Canadian ophthalmologists with a series of updates in pediatric ophthalmology. After a few days we were both back to the "busi-ness" of medicine, with little time to devote to such a project. Then, along came the COS Council on Continuing Professional Development (CPD) and the MaintCert program--a bold initiative to draw talent of Canadian ophthalmology into a program of continuing medical education (CME). One initiative was to use the Canadian Journal of ophthalmology as a CME tool. This article by Eedy Mezer and Ray Buncic is highlighted as a Section 3 learning activity (self-assessment). I am delighted to have seen Ray bring forward his idea in this manner. The Canadian Journal of ophthalmology has initiated this project in conjunction with the Council on CPD. There are a number of questions that relate to this article that can be answered on the COS Web site (http://www.eyesite.ca). Participants will be provided with an aggregate score and a certificate that can be printed to allow them to record this activity for Section 3 credits with the Royal College of physicians and Surgeons of canada.- - - - - - - - - - ranking = 7.8610620431279E-5keywords = junction (Clic here for more details about this article) |
3/4. Electrophysiological evidence by single fibre electromyography of neuromuscular transmission impairment in a case of miller fisher syndrome.miller fisher syndrome is an autoimmune neuropathy characterised by ataxia, areflexia and ophthalmoplegia, with minimal if any limb weakness, and in the majority of cases by high titres of IgG anti-GQ1b ganglioside antibodies. in vitro electrophysiological experiments have demonstrated that these antibodies induce a transmission blockade at neuromuscular junction either pre- or post-synaptically. We report the case of a 63-year-old man with MFS that shows blood serum negative for anti-GQ1b but presents an impairment of neuromuscular transmission detected by single fibre electromyography. To the best of our knowledge, this represents the first case in the literature using jitter technique and suggests that other antibodies may be involved in the function of motor end plates by bindings to the synaptic membranes.- - - - - - - - - - ranking = 0.5keywords = neuromuscular junction, junction (Clic here for more details about this article) |
4/4. Single-fiber electromyography shows terminal axon dysfunction in miller fisher syndrome: a case report.We studied a patient with ophthalmoparesis and pupillary areflexia 2 weeks after a viral syndrome. miller fisher syndrome was suspected but GQ1b antibodies were not detected. To define neuromuscular involvement we performed electrodiagnostic studies. Single-fiber electromyography (SFEMG) in the extensor digitorum communis (EDC) showed abnormal jitter and axonal blocking, suggesting terminal axon dysfunction. Subsequent GQ1b antibody titers were elevated to borderline levels. Clinical symptoms gradually resolved. SFEMG may help characterize neuropathies associated with antibodies to neuronal ganglioside and identify involvement of the terminal axon and neuromuscular junction.- - - - - - - - - - ranking = 0.5keywords = neuromuscular junction, junction (Clic here for more details about this article) |