Talk To This Lady And Proceed

This was the station 3 stem when I took my PACES in London. Short stem but luckily I was well prepared for what was in store;)

This was the last station of the day, and trust me... the adrenaline surge was quite intense. The pair of nice gentlemen had me ushered in the room with a lady sitting on a couch. I immediately noticed a walking stick beside her. She was of medium built. I looked hard for clues of clinical syndrome such as dystrophia mytonica or FSH but I'd found none.

Pondering the station stem, I had these possibilities outlined in my mind:

1. Cerebellar syndrome - young lady with walking stick, must consider gait abnormalities. Differentials to consider: FA, SCA, MS, ?Wilson and other infiltrative disorders
   
2. Bulbar or pseudobulbar palsy - Differentials: MND, ?young stroke
3. Parkinsonism - unlikely PD in young lady. Differentials: drug use (neuroleptics), ?substance abuse

I proceeded to talk to the patient. To be honest, it didn't strike me to be of anything abnormal for a moment. Luckily, I stayed with the locals for 3/52 prior to the exam and hence could appreciate the subtle slurring and staccato'ing. It wasn't very clear even with phrases like "British Constituition" or "Hippopotamus"!

At this jucture, the examiners interrupted me: "So what do you think?"

I gathered my thoughts and quickly said: "I've noticed very subtle scanning speech and staccato speech. I would like to proceed to examine the cerebellar system fully."

"Please do."

I proceeded to elicit every cerebellar sign that I could. The lady had obvious past-pointing, more on the right side and dysdiadochokinesia. However, truncal stability was relatively preserved. There was sustained nystagmus on looking to the right. She displayed broad-based gait with failure to perform tandem walk. The Rhomberg's sign was negative.

I was happy to conclude that she had ample of signs to suggest a cerebellar syndrome, with the possible underlying etiologies being MS (young lady and in temperate region), and hereditary ataxias such as SCA or FA. I would complete the examination by looking into her fundi for optic atrophy as well as to examine the lower limbs for long tract signs.

"If you are given an opportunity to ask one question, what would you ask?"

"I would elicit history of similar illness running in the family."

"Please do."

I proceeded to ask the nice lady with the question and I found out that her mother and the elder brother had the same condition.

"So what do you think?"

"The inheritance pattern is likely of autosomal dominant type, and hence it cannot be Friedrich ataxia. I would think this patient has spinocerebellar ataxia of autosomal dominant type."

The examiners nodded and smiled: They tossed me a few more questions on the other possible causes of cerebellar signs in a young lady before concluding the station.

"Now let's get on to the cardiovascular station."

Verdict: 4/4