Case Studies
Case 1 - A middle aged lady with progressive weakness
You are called to see a 43-year-old lady in casualty with a 5-day history of increasing weakness of all four limbs with lower back pain and sensory symptoms in her hands and feet. She has no significant previous medical or family history. She has recently been on holiday to the Canary Islands where she had a diarrhoeal illness.
On examination she has some slight facial weakness, a poor cough and weakness of the arms and legs with no reflexes and a loss of feeling to light touch and pin-prick to the wrist and ankle.
Your Answer:
Correct answer: Guillain-Barré syndrome
Your Answer:
Correct answer: In the early stages of the disease the tests are often normal. However nerve conduction studies should show evidence of slowing of nerve conduction as the condition is an acute demyelinating inflammatory neuropathy affecting all peripheral nerves as well as some of the cranial nerves.
Your Answer:
Correct answer: This is normally an inflammatory response against components in the peripheral nerve/myelin typically after some previous illness such as campylojejuni gastroenteritis or some viral infection. Thus one sees inflammation in the nerve which leads to the stripping off of the myelin around the nerve which gives the weakness and sensory loss as the nerve cannot conduct impulses normally.
Your Answer:
Correct answer: It is possible that this lady will now plateau out with her clinical condition and slowly improve. However there is a concern in someone with this degree of weakness that they will go on to develop worsening bulbar and respiratory failure. They should thus be managed on a high dependency unit where there respiratory function can be assessed using forced vital capacity and they should probably be nil by mouth, given the poor cough and risk of aspiration pneumonia. If her respiration were to get worse she would require ventilatory support. Also a significant number of people with Guillain-Barré syndrome develop autonomic involvement and she could develop unstable blood pressures as well as possible cardiac arrhythmias and thus she should be monitored for this.
Your Answer:
Correct answer: It is very unlikely that this lady has myasthenia gravis given the absent reflexes, sensory deficit and back pain. Myasthenia gravis affects the neuromuscular junction and presents with weakness which is typically fatigable in nature. Motor neurone disease presents with a motor disorder but this is very unlikely to be the diagnosis in this case given the rapid onset, sensory symptoms, as well as the involvement of the facial musculature. Finally spinal cord compression is a possibility given the back pain but again this could not cause the patient to have facial weakness and a poor cough.
Your Answer:
Correct answer: The information about the holiday may be relevant as many cases of Guillain-Barré syndrome have a preceding illness of which one of the commonest is campylojejuni gastroenteritis. This can be tested for and it might well prove positive in this lady.
Your Answer:
Correct answer: The treatment that should be offered to this lady, apart from the supportive therapy mentioned above, is some form of immunotherapy. This typically takes the form of either plasma exchange or intravenous immunoglobulin.