NEJM-Narcolepsy Flashcards

1
Q

Usually begins with what ages

A

10-20

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2
Q

OSA vs Nacrolepsy

A

OSA not refreshed after full nights sleep

Narcoleptic refreshed but has to sleep 1-2 hours later

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3
Q

Characteristic of REM sleep in narcolepsy

A

REM intrudes into wakeful states resulting in cataplexy which is a process that progresses over seconds and includes weakness in the face/neck then into the trunk and limbs.

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4
Q

What is partial cataplexy ?

A

slurred speech and sagging face resulting in slumping and possibly immobility but fully conscious state for 1-2 minutes

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5
Q

What type of hallucinations are rare in narcolepsy as opposed to psychotic disorders ?

A

Auditory

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6
Q

What % of the population is estimated to experience hypnagogic hallucinations ?

A

20%

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7
Q

What other symptoms often accompanies narcolepsy ?

A

weight gain, 15% above average BMI in adults

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8
Q

other sleep disorders that are more prevalent in the context of narcolepsy

A

PLMD,sleepwaling, REM sleep behavior disorder, OSA, depression

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9
Q

Distinguishing characteristics of delayed sleep phase disorder

A

alert at night and sleepy in the morning

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10
Q

Diagnosis of narcolepsy

A

Clinical but can be confirmed with a multiple sleep latency test preceded by a sleep study.

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11
Q

Before confirming narcolepsy what must be done ?

A

REM suppressing medications must be d/ced for up to 3 week and must get 6 hours during the sleep test

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12
Q

CSF finding in type 1 narcolepsy

A

low orexin A

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13
Q

98% of patients with narcolepsy have the HLA ______ ?

A

HLA-DQB1*06:02 (strongest association known currently)

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14
Q

Inheritance of narcolepsy

A

sporadic, monozygotic twin has 30% chance only

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15
Q

After the onset of narcolepsy what Ab has been shown to correlate ?

A

ASO, vaccines (pandemrix used in scandinavia) and flu viruses have been temporally linked

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16
Q

Putative pathogenesis underlying narcolepsy ?

A

t-cell response in a genetically susceptible person results in damage to lateral hypothalamus through molecular mimicry.

17
Q

Other disease states that could result in Narcolepsy ?

A

Sarcoidosis,demyelination,stroke,tumor,paraneoplastic disorders.

18
Q

Orexins inhibit …..

A

REM sleep

19
Q

Pathogenesis behind cataplexy

A

strong positive (usually) emotion relayed through medial prefrontal cortex and amygdala activate circuits in the pons that are disinhibited due to orexin producing neuron loss.

20
Q

Orexins do what ?

A

increase sympathetic tone, increase metabolism, and reward behaviors

21
Q

6 (first line to last line) medical tx for excessive daytime sleepiness.

A

Modafinil (provigil-inhibits D uptake),armodafinil,methylphenidate,dextroamphetamine,amph-dextro,sodiumoxybate

22
Q

4 possible treatments for cataplexy

A

Venlafaxine(Effexor),fluoxetine (Prozac),clomipramine (Anafranil) ,sodium oxybate

23
Q

two types and describe

A

type 1 - loss of lateral hypothalamic neurons that produce orexin A/B (hypocretins)
type 2 - same symptoms but unknown cause