Passmed Flashcards
What is migraine with aura?
Migraine with aura (seen in around 25% of migraine patients) tends to be easier to diagnose with a typical aura being progressive in nature and may occur hours prior to the headache. Typical aura include a transient hemianopic disturbance or a spreading scintillating scotoma (‘jagged crescent’). Sensory symptoms may also occur
Auras may occur with or without headache and:
are fully reversible
develop over at least 5 minutes
last 5-60 minutes
What are the typical aura symptoms
The following aura symptoms are atypical and may prompt further investigation/referral; motor weakness double vision visual symptoms affecting only one eye poor balance decreased level of consciousness.
What is a dystrophinopathy
X linked progressive hereditary degenerative disease of the skeletal muscle, caused by absence or deficiency of dystrophin
What is dystrophin?
Part of a large membrane associated protein in muscle which connects the muscle membrane to actin
What happens in duchenne muscular dystrophy?
There is a frameshift mutation resulting in one or both of the binding sites being lost, leading to a severe form
What isbeckers
Gfhg
What are the various patterns of motor neuron disease?
Amyotrophic lateral sclerosis
Progressive muscular atrophy
Bulbar palsy
What clues would point you towards a diagnosis of MN disease?
Fasciculations
Absence of sensory signs/symptoms
Mixture of LMN and UMN signs
Wasting of the small hand muscles/tibialis anterior is common
Doesn’t affect the external ocular muscles
No cerebellar signs
Abdominal reflexes are preserved and sphincter dysfunction if present is a late feature
How do you diagnose MN disease?
The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a reduced number of action potentials with increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy
A 40-year-old female presents with a six-hour history of painful visual loss of her right eye. She feels well otherwise and reports no nausea or vomiting. On examination, the patient’s right eye pupil does not constrict when exposed to torchlight and exhibits paradoxical dilatation on the swinging light test consistent with a relative afferent pupillary defect.
The patient’s temperature is 36.8ºC, BP 128/72mmHg, heart rate 80 beats/min.
What is the most appropriate investigation for the underlying cause of her symptoms?
MRI with contrast- to view demyelinating lesions
Acute optic neuritis is a common initial presentation of multiple sclerosis. MRI with contrast is the gold standard for investigating demyelinating lesions.
How would you investigate MS?
MRI
CSF
VEP s