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Other Neurologic Diseases in Pregnancy
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Loralei L. Thornburg, Meredith L. Birsner
Typically medications that are managing symptoms well should be continued in pregnancy, with discussion of risk of these medications to the fetus. Uncontrolled MG is a significant risk to maternal respiratory status and health, which will also affect the pregnancy, and the risk/benefits must be carefully considered. Pyridostigmine is considered safe in pregnancy at doses <600 mg/day, while IV choline esterase inhibitors may produce uterine contractions and are typically avoided [60].
Neurologic disorders in pregnancy
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Robert Burger, Terry Rolan, David Lardizabal, Upinder Dhand, Aarti Sarwal, Pradeep Sahota
Myasthenia gravis (MG) occurs predominantly in women of reproductive years with peak incidence in third decade (41). Management of MG in a pregnant woman is a unique challenge needing many therapeutic decisions at different stages. The course of MG during pregnancy is unpredictable with exacerbation in about 40%, remission in 30%, and no change in the remaining (24,42,43). The risk of worsening is the greatest during first trimester and early puerperium, and in women with recent diagnosis of MG (31). Women with MG who are considering pregnancy should be instructed on optimal disease control, minimizing oral immunosuppressants, and risks to mother and fetus. Elective thymectomy if being considered should be done before pregnancy as the benefit of thymectomy is delayed (24,41). Treatment with acetylcholinesterase inhibitors (pyridostigmine, edrophonium) and prednisone is safe during pregnancy. Immunosuppressive agents such as azathioprine and mycophenolate mofetil are contraindicated (41,43). Aggressive treatment is needed in patients with bulbar or respiratory involvement. Plasmapheresis or intravenous immunoglobulin therapy can be used for control of severe symptoms and in myasthenic crisis (41). Maintenance IVIG therapy can help to avoid the use of immunosuppressive agents. There is higher risk of maternal mortality in myasthenic women especially within 1year of diagnosis (42). It is advisable for MG women to delay childbirth for 2years after the onset of illness.
Neurology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
Clinical features: weakness that is often fatiguable – may be purely ocular (ptosis, ophthalmoplegia) or more generalised (proximal limbs +/- weakness in face or neck, dysarthria, dysphagia, dyspnoea). Investigations: diagnosis is made with EMG (decrement on repetitive stimulation) and/or positive acetylcholine receptor (AChR) antibodies. AChR antibody-negative cases may be MuSK antibody +ve. Tensilon tests are now performed less often. Mediastinal imaging (CT or MRI) for thymus enlargement/thymoma. Management: symptomatic treatment is with pyridostigmine. Immunosuppression (steroids initially then normally azathioprine). Other treatments: IVIg or plasma exchange if severe/relapse; thymectomy. Avoidance of drugs that exacerbate symptoms.
Effects of rhythmic auditory stimulation on walking during the 6-minute walk test in patients with generalised Myasthenia Gravis
Published in European Journal of Physiotherapy, 2022
Linda Kahr Andersen, Nanna Witting, John Vissing
Patients with gMG, 18-80years of age, were invited to participate in a 2-hour visit for testing. The patients were recruited from our neuromuscular clinic during their routine follow-up visit with their neurologist. The diagnosis of gMG was based on a typical clinical history and symptom improvement with acetylcholinesterase inhibitors coupled with either positive acetylcholine receptor antibodies and/or significant decrement/increased jitter on electromyography. Patients with a medical condition that could interfere with the interpretation of walking capability, e.g. heart failure or rheumatoid arthritis, were excluded. The included patients were treated by pyridostigmine, immunosuppressive drugs or a combination of both. For patients taking pyridostigmine, the tests were performed ½–2h after intake of the drug.
Electrophysiologic evaluation of myasthenia gravis and its mimics: real-world experience with single-fiber electromyography
Published in Hospital Practice, 2022
Anthony Khoo, Hnin Hay Mar, Maria Victoria Borghi, Santiago Catania
The outcome of SFEMG was recorded, as were details of muscles tested, total pairs obtained, number of abnormal pairs, degree of jitter and presence of blocking. Results of RNS were noted including which muscles were examined and the timing of any significant decrement. Repetitive nerve stimulation of the median and ulnar nerve at the wrist were considered distal sites, whereas RNS of facial and spinal accessory nerves were considered proximal sites. At each site, RNS involved a train of 10 pulses at 3 Hz, and was performed before and at one-minute intervals after one minute of muscle contraction. Standard operating procedure at our center mandates skin temperature recordings >32 degree Celsius although this was not systematically recorded in most studies. Repetitive nerve stimulation was considered abnormal if there was a decrement of >10% in comparing the baseline compound motor action potential (CMAP) amplitude or area with the 4th or 5th CMAP recorded. For every patient, we recorded whether pyridostigmine had been taken and the timing of the last dose. Where performed, findings of routine nerve conduction studies and electromyography of proximal muscles were also recorded.
Longstanding and Refractory Anti-Muscle Specific Tyrosine Kinase Antibody-Associated Myasthenia Gravis (Anti-MuSK-MG) in a Child Successfully Treated with Rituximab
Published in Journal of Binocular Vision and Ocular Motility, 2019
Steven Weger, Juan Pablo Appendino, Ian H. Clark
Myasthenia gravis was suspected. A 20-min ice-test improved eyelid opening dramatically, but only for 10–15 s after which the upper lids fell rapidly. Neurology was consulted; they prescribed pyridostigmine 5.2 mg/kg/day in four divided doses. Her ptosis further deteriorated prior to starting this medication. At this time, her right and left upper marginal reflex distances (RMRD1 and LMRD1) were 0 mm and 1 mm, respectively. Right and left lower marginal reflex distance (RMRD2 and LMRD2) were 3 mm and 4 mm, respectively. Levator function was 5 mm on the right and 7 mm on the left. She developed bulbar symptoms including difficulty and fatigability with her chewing and swallowing, and coughing when drinking fluids. Her ocular motility also deteriorated in all directions of gaze in both eyes: left adduction was best preserved at −1.5, whilst left gaze and infraduction of both eyes were −3.5. Vertical elevation was −3 bilaterally. Her esotropia was unchanged at this time.