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A 60-year-old man presents to the accident and emergency department complaining of gradual onset of slurred speech over many months.

Examination reveals proximal muscle weakness that improves with repetition and a bulbar palsy. He is frail and emaciated.

What is the most likely diagnosis?

(Please select 1 option)

Acute cerebrovascular event

Myasthenia gravis

Lambert-Eaton syndrome Correct

Parkinson's disease

Motor neurone disease

Explanation

Lambert-Eaton syndrome is a paraneoplastic effect of some small cell lung cancers.

It presents with proximal muscle weakness which improves with repetition and can cause bulbar palsies. Demonstrating the presence of a malignancy and detecting antibodies against the pre-synaptic calcium channels on the neuromuscular junction is key to making the diagnosis.

Acute cerebrovascular events would present with an acute focal neurological defect and not have a gradual onset or improvement with repetition.

Motor neurone disease may present with bulbar features but the lack of muscle fasciculation, distal muscle weakness and improvement with repetition make this unlikely.

Myasthenia gravis presents with proximal muscle weakness that gets worse with repetition and autoantibodies are detected against the acetylcholine receptor.

Parkinson's disease may present with monotonous speech and bulbar symptoms however the lack of tremor and symptoms which improve with repetition make this unlikely.

Reference:

Kumar P, Clark M. Kumar and Clark Clinical Medicine. 6th ed. London: Saunders; 2005.

NICE Pathways. Stroke overview.

Answer Statistics

1

1%

2

10%

3

70%

4

4%

5

16%

Times answered: 277