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Bulbar myasthenia presenting as acute respiratory failure--a case report.
Bulbar Myasthenia usually presents as difficulty in speaking, swallowing, respiratory stridor and nasal regurgitation.[1] There are very few case reports of bulbar myesthenia presenting as acute respiratory failure; hence report of this case. A 46 year old lady was brought to the emergency with complaints of dyspnoea, chest pain and abdominal pain of 3 days duration. Her symptoms became worse in the emergency and she had a generalised convulsion followed by cardio respiratory arrest. Cardio pulmonary resuscitation (CPR) was done and she was intubated and admitted to the intensive care unit (ICU) as acute respiratory failure. History later revealed that she was repeatedly seen in ENT clinic for respiratory stridor with no definite cause, for over 2 years. She had no focus of malignancy and was not on any medication. No past history of muscle weakness or family history of muscle disorder was available. On examination, her blood pressure was 140/90mm Hg and Pulse-100/min. Rest of general physical examination, respiratory system, cardiac examination and abdomen were normal. Examination of central nervous system revealed a conscious and oriented patient with old left lower motor 7th cranial nerve palsy. Other cranial nerves were intact. Patient was moving all the limbs. Deep tandon jerks were brisk. Planters were downgoing. Routine investigations and liver and renal function tests were normal. LDH was 587 and CK was 115. X-ray chest revealed a nonhomogenous opacity over left upper zone. There was no evidence of thymoma. She was started on antibiotics. Her chest infection improved. As wearing off the ventilator was not possible, myasthenia was suspected and investigated. History taken later from the relatives revealed that patient was quite well till 2 years ago, when she started to feel difficulty in chewing, regurgitation of food from her nose and difficulty in swallowing, with nasal speech. Symptoms progressively increased in severity to the extent that she was unable to eat and speak properly. She was worse by the end of the day. She denied any other muscle weakness or joint pain. There was no history of diplopia or change in vision. Three days prior to admission she started to complain difficulty in breathing associated with productive cough and whitish sputum, sweating and hoarseness of voice. There was no associated fever. CT scan chest did not show any evidence of thymoma. Test for HIV and hepatitis were negative. Anti acetyl choline antibody was positive (1:1200u). She was diagnosed as bulbar myasthenia and was treated with IV neostagmine 2mg, 3hrly, tab mastinon 60mg TID and prednisolone 60mg per day. She improved gradually in the bulbar muscle power. However, it was not possible to extubate her even after 48 hours. Plasmapheresis[2] was started and she showed improvement. On 6th day she was extubated. On the 10th day she was able to swallow solid food and liquids without difficulty and was able to speak properly. She was discharged with the advise to continue mastinon 60mg TDS. Steroids were tapered gradually. Bulbar myasthania is a neurological emergency and when suspected and treated, the patient can get back to normal respiratory functions with adequate medical treatment alone. Patients presenting with history of myasthenia, are not difficult to diagnose or treat; but when they present in atypical way to other specialists, the diagnosis is delayed.
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