26th August 2024

A 56-year-old woman is referred to your clinic complaining of weakness. She describes an approximately two-year history

A 56-year-old woman is referred to your clinic complaining of weakness. She describes an approximately two-year history of progressive difficulties getting up from chairs and has had to trade in her car for a 4×4 as she was unable to get out of the low seat. Alongside this, she has noticed her hands becoming weaker with difficulty opening jars and, more recently, with using her car keys. She has also noticed difficulties with swallowing and has now been avoiding red meat for the last 6 months due to her fear of choking.

On examination, you note 4/5 strength in her hip and knee flexors and her knee extensors bilaterally and normal power in the rest of the lower limb. In the upper limb, she has normal strength in her upper arm but 2/5 strength in her wrist and finger flexion and 4/5 in other movements of the hand and wrist. The rest of her neurological exam is normal.

What is the most likely diagnosis?

Select the SINGLE most appropriate option from the list below. Select ONE option only.