PACES Flashcards
What does temporalis muscle wasting indicate?
consider Lower motor neurone Facial nerve (e.g. Bells palsy)
What happens when a person with myotonic dystrophy tries to shake hands with someone?
They struggle to release the grip easily
When you present in neurology, try and be anatomical in pattern detection - give examples
Symmetrical vs asymmetrical
Proximal vs distal
Pyramidal vs extrapyramidal
Why is a symmetrical finding important to note?
It suggests there is a general pathology such as a syndrome as opposed to a nerve/nerve root
In myotonic dystrophy what would you like to do to complete the exam?
cardiovascular exam check blood sugar FH EMG genetic testing
What neuro pts tend to come to exams?
Parkinsons Charcot marie tooth Myotonic dystrophy MS Peripheral neuropathy
What is associated with Myotonic dystrophy?
- Cataracts (buzz word: loss of red reflex in both eyes)
- Dysphagia (from weakness)
- Cardiac (cardiomyopathy and heart block - may need a pacemaker)
- Diabetes mellitus
- Hypogonadism (gynaecomastia/ testicular atrophy)
What investigations would you like for myotonic dystrophy?
- Lung function tests - to check for neuromuscular respiratory insufficiency
- ECG or echo (conduction block and cardiomyopathy)
- Fasting glucose for DM
- Slit lamp test for cataracts
- SALT assessment
- EMG: may show ‘dive-bomber’ potentials (this is pathopneumonic)
What cataracts do you get with myotonic dystrophy?
Christmas tree cataracts = pathopneumonic
What is the treatment for myotonia?
Phenytoin for the myotonia
but the weakness has no treatment
(avoid statins)
What type of genetic goup does myotonic dystrophy fall into?
autosomal dominant with genetic anticipation (trinucleide repeat)
What is genetic anticipation?
When a condition presents earlier and is more severe in the later generations
Conditions with genetic anticipation
Myotonic dystrophy
Huntingdon’s chroea
Freidrick’s ataxia
What are the differentials for bilateral ptosis?
- Myaesthenia gravis
- Myotonic dystrophy
- Bilateral Horner’s and bilateral 3rd nerve palsy (although these would be very rare
- congenital
- Neurosyphillis (but isn’t really seen anymore)
What is the cause for Argyll Robetson pupil?
Syphillis
Diabetes
What is an Argyll Robertson pupil?
A pupil which does not constrict when exposed to bright light but does constrict when focusing on a near object
What is the antibody against in myaesthenia gravis?
Acetylcholine receptor on the post synaptic membrane
What tumours are associated with myasthenia gravis?
Thymoma (look for on a CT scan)
Why do pts with myasethenia struggle to chew food?
It is a repeated movement
What is the treatement for myasthenia gravis?
Pyridostigmine (Ach-esterase inhibitors)
2nd line: add immunosuppression (steroids)
- consider thymectomy
What is the diagnostic test for myasthenia gravis?
The Tensilon test
What is another name for the tensilon test?
Edrophonium test
Lambert-Eaton myasthenic syndrome (LEMS)- what is the anti body against in this condition?
pre-synaptic membrane antibody to the Ca2+ receptor
LEMS is a _____ syndroem associated with ___ cancer
Paraneoplastic
small cell lung cancer