General neurology Flashcards
(190 cards)
What % of those with ALS have FTD?
13%
What is the major genetic cause shared by ALS and FTD?
C9orf72
TDP-43 is found in which diseases?
FTD and ALS
What is Onuf’s nucleus responsible for?
Sphincter control
How do triptans work (in migraine)?
Activators of 5-HT receptors, 1B/1D/1F
What options are available for migraine prophylaxis?
Amitriptyline, nortriptyline, pizotifen, candesartan, propranolol, sodium valproate, topiramate, verapamil
Which nerves pass through the jugular foramen?
IX, X, XI
What passes through the cavernous sinus?
CN III, IV, V1, V2, VI
What causes autonomic dysreflexia?
Spinal cord injuries above T6.
- HTN and bradycardia
- Below T6 allows splanchnic innervation and therefore dilatation to bring down BP
What are the common side effects of phenytoin?
Gingival hyperplasia, hirsutism, rash, folic acid depletion, osteoporosis, cerebellar signs
What is the prognosis at diagnosis?
3-5 years
What does reinnervation look like on EMG?
High amplitude CMAPs
How is conduction block defined on Nerve Conduction Studies?
A reduction in CMAP area/amplitude of at least 20% compared with distal CMAP area/amplitude.
The duration of the proximal CMAP should not increase by >20% (see temporal dispersion)
Which antibody is multifocal motor neuropathy associated with?
anti-GM1
How is primary lateral sclerosis distinguishable?
Affects upper motor neurons only
How is progressive muscular atrophy distinguishable?
Affects lower motor neurons only
What are the 2 most commonly found mutations in ALS?
C9orf72 and SOD1
How is EPHA4 implicated in prognosis in ALS?
Lower EPHA4 = longer survival
Which exposure is the strongest risk factor for ALS?
Military service.
How does riluzole work in ALS?
Suppresses excessive motor neuronal firing
How does edaravone work in ALS?
Suppresses oxidative stress
What is the treatment for cluster headaches?
Acute: 100% O2, triptan
- Severe refractory => indomethacin or dihydroergotamine
Bridging: prednisone or occipital nerve block with methylpred
Prophylactic: Verapamil
What is the genetic mutation responsible for Wilson’s disease?
ATP7B mutation on chromosome 13 (autosomal recessive)
Which mutation is associated with the highest risk of multiple sclerosis?
HLA DRB1*1501
Other risk = *0801