Neurology Flashcards

1
Q

What causes Lambert-Eaton myasthenia syndrome?

A

Small cell lung cancer - antibodies target and damage voltage-gated calcium channels in the presynaptic terminals of the neuromuscular junction. Less acetylcholine is released

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2
Q

What are the symptoms of Lambert-Eaton myasthenia syndrome?

A

Slow development of proximal muscle weakness. Can also affect eyes, speech and swallow, autonomic.
Reduced reflexes - with post-titanic potentiation

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3
Q

What is the treatment for Lambert-Eaton syndrome?

A

Investigate for small-cell lung cancer
Amifampridine - allows more acetylcholine to be released in the neuromuscular junction synapses
Also: immunosuppression or IVIG

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4
Q

What is the genetics for Huntington’s?

A

Autosomal dominant trinucleotide repeat disorder of CAG in huntingtin gene on chromosome 4

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5
Q

Anticipation is a feature of what type of genetic disorder?

A

Trinucleotide repeat disorders - successive generations have more repeats in the gene

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6
Q

What medications are used in Huntington’s to suppress the disordered movement?

A

Antipsychotics
Benzodiazepines
Dopamine-depleting agents

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7
Q

What is Charcot-Marie-Tooth Disease?

A

Genetic - usually autosomal dominant
Affects peripheral motor and sensory nerves

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8
Q

What are classical features of CMT?

A

High foot arches (pes caves)
Distal muscle wasting causing “inverted champagne bottle legs”
Weakness in the lower legs, particularly loss of ankle dorsiflexion
Weakness in the hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss

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9
Q

What is the ABCDE of causes of peripheral neuropathy?

A

A - alcohol
B - B12 deficiency
C - Cancer and CKD
D - Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin
E - every vasculitis

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10
Q

What is tuberous sclerosis?

A

Genetic
Hamartomas (benign neoplastic growths of tissue) usually in skin brain, lungs, heart, kidneys, eyes

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11
Q

What mutations in genes causes tuberous sclerosis?

A

TSC1 gene on chromosome 9 (codes for Hamartin)
TSC2 gene on chromosome 16 (tuberin)
Hamartin and tuberin interact with each other to control the size and growth of cells

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12
Q

What are the skin signs of tuberous sclerosis?

A

Ash leaf spots (depigmented)
Shagreen patches (pigmented)
Angiofibromas on nose and cheeks
Sublingual fibromata (nail bed)
Cafe-au-lait spots
Poliosis - isolated patch of white hair on the head, eyebrows, eyelashes or beard

Often present with epilepsy and skin changes

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13
Q

What causes seizures?

A

Either too little inhibition of neuronal activity (too little GABA) or too much excitation (too much glutamate)

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14
Q

What receptors does glutamate bind to in the synaptic cleft?

A

AMPA which causes Na+ entry
NMDA which causes Ca2+ entry
Propagates the signal

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15
Q

How does GABA inhibit signal propagation?

A

It binds to GABA-A receptors on the excitatory neurons causing Cl- to enter

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16
Q

How is GABA removed from the synaptic cleft?

A

GABA transporter 1
It is then degraded by the enzyme gamma-aminobutyric acid aminotransferase (GABA-T)

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17
Q

Which antiepileptics block calcium channels as well as sodium channels?

A

Lamotrigine
Zonisamide
Topiramate
Valproic acid

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18
Q

How do Gabapentin and Pregabalin work?

A

High voltage calcium channel blockers

19
Q

How does levetiracetam work?

A

Binds to SV2A on walls of glutamate vesicles impairing its release

20
Q

How do benzodiazepines and barbiturates work?

A

Bind to GABA-A receptor prolonging the opening of channel, increasing Cl- influx

21
Q

How does Tiagabine work?

A

Selective inhibitor of GAT1, blocking FGABA reuptake

22
Q

How does Vigabatrine work?

A

Irreversibly inhibits GABA-T, preventing catabolism of GABA, increasing levels of GABA in the brain

23
Q

Which antiepileptics can cause hyponatraemia?

A

Carbemazepine and oxycarbemazepine

24
Q

What antiepileptic can cause visual field loss?

A

Vigabatrin

25
Q

Which antiepileptics can cause double vision?

A

Lamotrigine and Phenytoin

26
Q

Which antiepileptic can cause gingival hypoplasia?

A

Phenytoin

27
Q

Which antiepileptic can cause hirsuitism?

A

Phenytoin

28
Q

Which antiepileptic can cause cognitive problems?

A

Topiramate
Zonisamide

29
Q

Which antiepileptic can cause weight loss?

A

Topiramate

30
Q

Which antiepileptics can cause weight gain?

A

Valproic acid
Gabapentin
Pregabalin

31
Q

Which antiepileptics can cause peripheral oedema

A

Gabapentin and Pregabalin

32
Q

Which antiepileptics can cause liver toxicity?

A

Valproic acid and Felbamate

33
Q

Which antiepileptic can cause aplastic anaemia?

A

Felbamate

34
Q

How does felbamate work?

A

blocks NMDA receptor, delaying signal propagation

35
Q

What are the two types of cholinergic receptors?

A

Muscarinic and nicotinic

36
Q

What ion change do M1 and M3 muscarinic receptors cause when activated?

A

Increase in calcium levels

37
Q

What changes do M2 muscarinic receptors cause when activated?

A

Increase in potassium and decrease in HR

38
Q

What are three direct acting cholinergic agonists?

A

Acetylcholine
Carbachol
Pilocarpine

39
Q

What are direct acting cholinergic agonists often used for?

A

Reducing intraocular pressure

40
Q

How do indirect acting cholinergic agonists work?

A

Act on the enzyme that breaks acetylcholine down (acetylcholine esterase)

41
Q

What class of drug is Pyridostigmine and what is it used in?

A

Reversible indirect acting cholinergic agonist
Used in myaesthenia gravis

42
Q

What condition are Donepizil, Rivastigmine, Galantamine used?

A

Alzheimers to enhance cholinergic effects

43
Q

What are cholinergic adverse effects? (DUMBBELS)

A

Diarrhoea
Urination
Miosis/muscle weakness
Bronchorrea
Bradycardia
Emesis
Lacrimation
Salivation

44
Q

What are the questions in 6CIT?

A

What year is it? 0/4 pts
What month is it? 0/3 pts
Address + rpt 0/2/4/6/8/10 pts
What time to closest hr? 0/3 pts
Count backwards 20-1 0/2/4 pts
Months in reverse 0/2/4 pts