Central Disorders of Hypersomnolence Flashcards

1
Q

What are 6 core features of narcolepsy?

A
  1. EDS
  2. Cataplexy
  3. Sleep paralysis
  4. Hypnogogic/pompic hallucinations
  5. Fragmented sleep
  6. Automatic behavior
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2
Q

What is the definition of a SOREMP?

A

REM sleep within 15 minutes of sleep onset

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3
Q

What are the PSG and MSLT criteria for a diagnosis of narcolepsy?

A
  • Mean sleep latency of 8 minutes or less
  • 2 SOREMP in the MSLT
  • 1 SOREMP in the PSG and 1 SOREMP in the MSLT
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4
Q

What percent of narcolepsy patients have cataplexy?

A

At least 60%

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5
Q

Which has a deficiency of orexin, Type I (with cataplexy) or Type II (without cataplexy)?

A

Type I

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6
Q

What level of CSF hypocretin (orexin) is diagnostic for Type I Narcolepsy?

A

< 110 pg/mL

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7
Q

What is the most effective treatment for cataplexy, inhibition of reuptake of NE, serotonin or dopamine?

A

NE

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8
Q

If a patient can’t take sodium oxybate for cataplexy, what could be given qhs instead?

A

Clomipramine 25 to 75 mg qhs

(TCA)

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9
Q

TCAs interact with what receptors

A
  • Serotonin
  • Acetylcholine
  • NE
  • Histamine

They DON’T interact with dopamine

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10
Q

Conditions that can cause secondary narcolepsy with low hypocretin levels (6)

A
  • MS
  • Prader-Willi syndrome
  • Myotonic dystrophy
  • AD cerebellar ataxia
  • TBI
  • Mass lesion in hypothalamus
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11
Q

Positive HLA-DQB1*0602 is found in what % of the population without narcolepsy?

A

25%

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12
Q

3 medicines for cataplexy

A
  1. Sodium oxybate
  2. Venlafaxine
  3. Clomipramine
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13
Q

MSLT or Actigraphy diagnostic criteria for Idiopathic Hypersomnia

A

MSLT = 8 min with 1 or no REM periods

or

At least 660 min of sleep over a 24 hour period

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14
Q

At what age does Klein-Levin Syndrome typically occur?

A

Teenage years

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15
Q

How long does each episode of Klein-Levin Syndrome typically last?

A

Days to a few weeks

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16
Q

How many episodes in Klein-Levin Syndrome will occur each year?

A

1 to 10 typically

17
Q

What unusual symptoms typically occur in Klein-Levin Syndrome?

A
  • Hyperphagia
  • Hypersexuality
  • Abnormal behavior: Confusion, irritability, aggression and hallucinations
18
Q

In Klein-Levin Syndrome how long do the daytime sleep episodes last?

A

Up to 18 hours per 24

19
Q

How long does Klein-Levin Syndrome last in total?

A

1 to several years with an average of 4

20
Q

What medication can make Klein-Levin Syndrome worse?

A

Daytime stimulants can cause psychiatric symptoms

21
Q

What 4 medications have show some benefits for Klein-Levin Syndrome in case reports?

A
  1. Gabapentin
  2. Carbamazepine
  3. Lithium
  4. IV steroids
22
Q

What medication may reduce the frequency of episodes in Klein-Levin Syndrome?

A

Lithium

23
Q

What medication may reduce the duration of episodes in Klein-Levin Syndrome?

A

IV steroids

24
Q

What will high leptin levels result in?

A

Suppress appetite and stimulate energy production

25
Q

Modafinil to treat residual sleepiness with OSA has what change on the MSLT?

A

Increase in sleep latency on the MSLT by 1 to 2 minutes compared to baseline

26
Q

Which is more potent, modafinil or armodafinil?

A

Armodafinil is 2 times more potent when a steady state is reached

27
Q

What changes can be seen in REM sleep in a patient with MDD?

A
  • Shortened REM latency
  • Increased REM duration
  • Increased REM density (increase frequency of rapid eye movements per REM period)
28
Q

Medication used for cataplexy that is also a metabolite of GABA and is approved for use in children

A

Sodium oxybate (Xyrem)

29
Q

A MSLT with a mean sleep latency of < 8 minutes can be found in what % of the normal population?

A

Up to 30%

30
Q

Why does narcolepsy result in a rapid transition to REM sleep

A
  • Orexin projects to the REM off neurons in the ventrolateral periaqueductal grey and lateral pontine tegmentum
  • Low orexin leads to decreased excitation of REM off neurons
31
Q

Describe how a MSLT works. How long is each nap? How long will the nap continue once the patient falls asleep?

A
  • Each potential nap runs for 20 minutes and if the patient doesn’t fall asleep the nap is terminated and the latency is 20 minutes
  • If the patient falls asleep then the nap will continue for another 15 minutes to see if he will enter REM sleep
    • If REM is obtained then the nap is stopped
32
Q

What 2 actions/treatments can trigger status cataplecticus

A
  • Abrupt withdrawl of antidepressants
  • Prazosin
33
Q

What percent of the time with MSLT be falsely negative in a patient with narcolepsy?

A

Up to 30%