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Practice paper
Published in Andrew Schofield, Paul Schofield, The Complete SAQ Study Guide, 2019
Andrew Schofield, Paul Schofield
Myasthenia gravis is an autoimmune condition due to antibodies targeted against the nicotinic acetylcholine receptors on the post-synaptic neuromuscular junction. It is associated with thymomas and a number of autoimmune conditions. The clinical presentation of myasthenia gravis is of muscular weakness which is characterised by fatigability (i.e. worsens on exercise). The extraocular muscles are often the first muscle group affected, followed by bulbar, facial and proximal muscle groups. The Tensilon test is an investigation used to aid in diagnosis. This involves administering a short-acting acetylcholinesterase inhibitor (edrophonium) to the patient and watching for an improvement in the muscle weakness. However, due to the risk of increased vagal tone producing bradycardia, the test should only be performed where resuscitation facilities and atropine are to hand. Thymectomy is indicated in patients with a thymoma but may also benefit patients without one, so is often considered in younger patients and when acetylcholinesterase inhibitors are ineffective.
Head and neck
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Neurogenic Oculomotor nerve lesion.Horner’s syndrome (effects reduced with 10% phenylephrine hydrochloride).Myasthenia gravis (worse later in day). Tensilon test is diagnostic – neostigmine or edrophonium. Anti-acetylcholine receptor antibodies are 100% specific (Padua L, Clin Neurophysiol, 2000).Demyelination, i.e. multiple sclerosis.Traumatic ophthalmoplegia or ophthalmoplegic migraine.
Answers
Published in John D Firth, Professor Ian Gilmore, MRCP Part 2 Self-Assessment, 2018
John D Firth, Professor Ian Gilmore
A history of dysphagia to both liquids and solids is fairly typical for achalasia. The barium meal findings are consistent, though not diagnostic, and endoscopy has ruled out a peptic oesophageal stricture. Confirmation of the diagnosis is by oeosphageal manometry, which shows a high resting lower oesophageal sphincter pressure that fails to relax on swallowing. Anti-Ro/La antibodies are positive in scleroderma, which may affect the oesophagus causing dysmotility. Oesophageal candidiasis may occur in achalasia or diabetes, but the latter should not cause a dilated oesophagus. The Tensilon test is a diagnostic test for myasthenia gravis, which may present with difficulty swallowing.
Neuro-Ophthalmic Literature Review
Published in Neuro-Ophthalmology, 2018
David Bellows, Noel Chan, John Chen, Hui-Chen Cheng, Peter MacIntosh, John H. Pula, Michael Vaphiades, Konrad P Weber
In this case report and review of the literature, the authors present a patient with bladder cancer treated with transurethral resection followed by six weeks of intravesical Bacillus Calmette-Guerin (BCG). Four days after his final injection, he developed ptosis and diplopia. Testing was significant for a positive Tensilon test and positive anti-acetylcholine receptor anitbodies, consistent with myasthenia gravis (MG). His response to pyridostigmine was incomplete, but he had good response to mehtylprednisone 1g/d for 3 days, after which his symptoms resolved and have not recurred in 1 year of follow up.
A new refined technique for myogenic ptosis correction with resection of myotarsal flap by conjunctival approach
Published in Orbit, 2018
Tensilon test was carried out to exclude myasthenia gravis. All patients gave informed consent when the decision for surgery was explained to them. Surgery was under LA. The details of the perioperative management are given below. The vast majority of patients underwent surgery under LA as day cases – discharged home 1–2 hours after surgery.