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Case #8 Weakness

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Urology

Version Jan 2023

A 27-year-old polish gentleman who normally works in a shop and is otherwise fit and healthy had a recent viral illness 3 weeks ago and was off work for a week. He made a good recovery and has been back at work.
However, over the past 4 days, he has noted increasing weakness which started in his feet and tingling in both legs and some mild back and leg pain. On examination, he has absent ankle and knee jerks on both sides and power is 2/5. Tone is reduced and there are some fasciculations and there is also distal sensory loss. He now has some tingling in his fingers.

1. Where could the lesion be based on the clinical findings

The findings of distal lower limb areflexia and paralysis suggest a lower motor neurone lesion, so the disease is distal to the anterior horn cell in the spinal cord. So this involves either nerve roots and/or the peripheral nerves. The extent shows that the disease is symmetrical and bilateral and ascending (starts in feet). This is a problem in the peripheral nervous system. Usually it is due to demyelination and sometimes due to axonal damage.

2. What is the likely diagnosis and differentials

Distal areflexia and ascending paralysis sounds very much like Guillain-Barre syndrome which is an Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most commonly encountered variant.

3. What are the most useful tests

If the presentation is classical then investigations can simply be the clinical history and exam and confirmatory nerve conduction studies. An LP to measure CSF protein which is often elevated can also be done. However Nerve conduction and CSF can be normal if done early. If there is any concern about a cord lesion then an MRI is done. It may be reasonable to check HIV serology. If there was a history of diarrhoea then stool cultures. GBS often comes on several weeks after a viral or gastrointestinal illness classically due to campylobacter.

4. He continues to have ongoing weakness - what tests would you watch closely and what treatment should be started

The concerns is that the weakness affects his respiratory muscles and this can lead to respiratory failure. He needs to have his FVC measured several times a day. If this falls significantly, he needs to be moved to HDU and even considered for intubation and ventilation.
Indication for Intubation

FVC <20 mL/kg
Maximal inspiratory pressure > -30 cmH2O (negative inspiratory force)
Maximal expiratory pressure is <40 cmH2O

Standard treatment would be IV Immunoglobulins given over several days. An alternative would be plasma exchange. The disease is felt to be due to antibodies attacking the nerves and this will help to mop up the antibodies. There is no role for steroids in Acute GBS.

5. What are the differentials here

Often the presentation is classical but if not then the diagnostic causes can widen and one should consider the possibility

Acute spinal cord disease with flaccid weakness
Acute intermittent porphyria
Myasthenia gravis
Botulism - progressive weakness with no sensory symptoms with normal CSF
Tick paralysis, Diphtheria, Poliomyelitis, Porphyria

6. Before starting IVIG what other test would you do

Patient needs screen for IgA deficiency before IVIG given. If he has IgA deficency and is given IVIG there might be a risk of anaphylaxis.

7. What other important issues are there

With paralysed legs he will need good skin care and regularly turned. He may need a catheter. He needs good VTE prophylaxis with paralysed legs. Patients often take months for recovery and need passive and then active physiotherapy. Some patients need ventilated for weeks.

7. Question

Answer

References

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