Upper limb neurology

Question 1

Introduction

Answer 1

Hello, my name is Cassie, I am the paediatric registrar. I have been asked to perform a developmental assessment on x, an x year old x.

Thank you for volunteering to help us today, this examination shouldn’t cause any distress or pain, however if it does let me know and I’ll alter my approach. Is it okay if we proceed? Great

In order for me to examine your upper limbs, I’ll have to have a look at your muscles! do you mind taking off your shirt? Great, I’ll just wash my hands and I’ll chat to the examiner as I go so don’t worry too much about what I’m saying.

I’m having a look around the room, looking for mobility, respiratory, or nutritional devices, of which I see none/describe.

x looks well/unwell with (pallor/jaundice/ appears dyspnoeic). xx has no distinctive facial features, and looks like his mum.

He appears small for his ag, of a disporportionate weight to height/disproportionately tall, however I would like to check this on growth charts, ideally serial growth measurements. Are these available?

Question 2

ddx of Horner’s syndrome

Answer 2

Neck pathology, check expansion on that side of the diagram

Question 3

Sign of horners syndrome

Answer 3

ptosis, small pupil, Mydriasis,

Question 4

Myopathic facies

Answer 4

Paucity of facial expression; There is bilateral ptosis. Wasting of the temporalis and masseter muscles contributes to a thin visage referred to as a hatchet face. Sternomastoid atrophy causes a pencil necked appearance.

Question 5

Myopathic facies in infant?

Answer 5

tented upper lip and hypotonia

Question 6

ddx of myopathic facies

Answer 6

myopathies, myotonic dystrophy, myasthenia gravis

Question 7

short neck ddx

Answer 7

Kllippel Fiel anomaly

Question 8

What is the Klippel Fiel anomaly?

Answer 8

Klippel-Feil syndrome is a rare disorder where two or more vertebrae in the neck are fused together from birth

Question 9

if you see short neck and suspect klippel fiel anomaly, what might you thenlook for?

Answer 9

spasticity, findings of syryngomyelia

Question 10

what are you looking at in the lower limbs?

Answer 10

posture, muscle wasting (peroneal atrophy of CMT), involuntary movements, growth arrest, foot deformities (pes cavus)

Question 11

What are you looking for on the skin?

Answer 11

stigmata of NF1, Stigmata of Tuberus sclerosis