Hong Kong Journal of Emergency Medicine

An undiagnosed myasthenia gravis presented with acute respiratory failure

AYC Siu, TW Wong, CC Lau

Acute respiratory failure is an uncommon initial presentation of myasthenia gravis. We present a case
of unrecognised myasthenia gravis. She initially presented with dysphonia and was managed by speech
therapy and ENT surgeons for a year. The diagnosis was finally made after the patient presented with
acute respiratory failure. (Hong Kong j.emerg.med. 2001;8:106-107)

Keywords: Acute respiratory failure, myasthenia gravis, presentation

Introduction revealed that she had known history of speech

Myasthenia gravis is an autoimmune disease
affecting the neuromuscular junction. It is
characterised by the fatigability of muscle. Most of
the patients presented as weakness, ptosis and
diplopia. The disease can be easily diagnosed by
clinical examination and specific diagnostic test.
Acute respiratory failure is rare as the initial
presentation, instead it is usually precipitated by
surgery, infection and drugs. We presented a case of
previously undiagnosed myasthenia gravis presented
with acute respiratory failure.
Case history
A 15-year-old girl was found to be unresponsive and
dyspnoeic at home. She was immediately transferred
to our department. On arrival, she was triaged as
an emergency case. Blood pressure was 145/99
mmHg, pulse was 102 per minute and regular. The
respiratory rate was 18/minute and SaO2 was 84%.
Temperature (Tympanic) was 33.7°C. The initial
Glasgow Coma Scale was E4V1M1. Further history
Correspondence to:
Siu Yuet Chung, Axel, MBChB (CUHK), FRCS (Edin)
Nor th District Hospital, Accident and Emerg ency
Department, 9 Po Kin Road, Sheung Shui, N.T., Hong Kong
Email: axel@hknet.com
Pamela Youde Nethersole Eastern Hospital, Accident and
Emergency Department, Chai Wan, Hong Kong
Wong Tai Wai, MBBS (HK), FHKCEM, FHKAM (Emergency Medicine)
Lau Chor Chiu, MBBS (HK), FHKCEM, FHKAM (Emergency Medicine)
problem followed up by ENT and speech therapy
services. Her relative denied any history of substance
abuse or recent drug intake. Initial examination
found a young thin lady with poor respiratory effort.
There was no abnormal breath sound. She was not
cyanotic or pale. The cardiovascular system and the
rest of the examination were essentially normal.
Spot sugar by Haemoglucostix was 9.1 mmol/L and
Haemogloblin by Haemocue was 12.5 g/dL.
Electrocardiogram showed sinus tachycardia and the
chest X-ray was normal.
As respiratory failure was imminent, she was
promptly given supplemental oxygen and intubated
under rapid sequence induction (Intravenous
midazolam 3 mg and succinylcholine 75 mg). Her
SaO 2 rapidly improved to 100% after intubation.
However, she was unable to breathe without
mechanical support even after the effect of muscle
relaxant had worn off.
After admission, assessment by ENT surgeons did
not reveal any upper airway obstruction. She was
subsequently noticed to have bilateral ptosis and the
Tensilon test was positive. There was significant
decremental response on repetitive stimulation in
nerve conduction test and the electromyogram did
not show any myopathic change. Her condition
g r a d u a l l y i m p r o ve d w i t h p y r i d o s t i g m i n e ,
prednisolone and 6 courses of plasmapheresis. She
was also confirmed to have anti-cholinergic receptor
antibody but CT thorax did not show any thymoma
or thymus enlargement. She was successfully
extubated on D7 and finally discharged on day 21.
Siu et al./An undiagnosed myasthenia gravis
She was re-admitted about one month later for
elective thoracoscopic thymectomy. The initial post-
operative course was uneventful. However, she
developed respiratory distress and subsequent
respiratory arrest on D1 due to sputum retention,
left pneumothorax and exacerbation of myasthenia
g ravis. She was successfully intubated and
resuscitated. The prednisolone and pyridostigmine
were stepped up and she received 4 further courses
of plasmapheresis. She was finally discharged on day
22. The section of the excised thymus showed
follicular hyperplasia only. Currently she was still
followed up in our hospital with maintenance steroid
and pyridostigmine.
Discussion
Myasthenia gravis is a neuromuscular disorder of
autoimmune origin. About 10-15% of cases occur
during adolescence.1 The hallmark of the disease is
abnormal fatigability of muscle. Majority of the
patients are female.2 The symptoms depend on the
extent and the site of muscle involved. Patients
may present with diplopia, dysphagia, ptosis and
limb weakness. In Hong Kong, about 47% of
myasthenia patients had predominant ocular
manifestations. 3
Respiratory muscle involvement is less common.
About 10% of local patients had symptoms
involving respiratory muscle but less than one tenth
of them was severe.3 The majority of cases can be
diagnosed by clinical examination, Tensilon test,
electromyogram and anti-acetylcholine receptor
antibodies assay. Acute respiratory failure is not a
common initial presentation and usually occurs after
precipitation by surgery, infection and drugs.
Our case has demonstrated the rare but well-known
manifestation of myasthenia gravis. Indeed our
patient has already presented with symptoms of
dysphonia for one year, which was compatible with
myasthenia gravis. However, it was not diagnosed
until the patient presented with acute respiratory
failure. The precipitating cause was not known. As
107
the diagnosis was not known at our department,
succinylcholine was used as muscle relaxant for rapid
sequence intubation. It was well known that it may
cause prolonged paralysis in myasthenic patients.
However, our patient did not manifest any
prolonged paralysis afterwards.
Acute respiratory failure can be due to fatigue of
respiratory muscle.4 It can also be due to upper airway
obstruction. They may present with stridor.5-7 Some
authors suggested to perform flow volume loops in
all myasthenic patients as a screening test.8 The vocal
cord of our patient was also involved by the disease
as she already had speech problem.
Myasthenia gravis is not a common entity that we
encounter daily. Patients, on occasions, may present
to the emergency department because of acute
exacerbation. Though most of them are known
cases, we should be aware of some unrecognised
cases and should consider myasthenia gravis as a
differential diagnosis for patient with acute
respiratory failure.
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