Central Disorders of Hypersomnolence

Question 1

What are 6 core features of narcolepsy?

Answer 1

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

Question 2

What is the definition of a SOREMP?

Answer 2

REM sleep within 15 minutes of sleep onset

Question 3

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

Answer 3

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

Question 4

What percent of narcolepsy patients have cataplexy?

Answer 4

At least 60%

Question 5

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

Answer 5

Type I

Question 6

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

Answer 6

< 110 pg/mL

Question 7

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

Answer 7

NE

Question 8

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

Answer 8

Clomipramine 25 to 75 mg qhs

(TCA)

Question 9

TCAs interact with what receptors

Answer 9

• Serotonin
• Acetylcholine
• NE
• Histamine

They DON’T interact with dopamine

Question 10

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

Answer 10

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

Question 11

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

Answer 11

25%

Question 12

3 medicines for cataplexy

Answer 12

1. Sodium oxybate
2. Venlafaxine
3. Clomipramine

Question 13

MSLT or Actigraphy diagnostic criteria for Idiopathic Hypersomnia

Answer 13

MSLT = 8 min with 1 or no REM periods

or

At least 660 min of sleep over a 24 hour period

Question 14

At what age does Klein-Levin Syndrome typically occur?

Answer 14

Teenage years

Question 15

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

Answer 15

Days to a few weeks

Question 16

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

Answer 16

1 to 10 typically

Question 17

What unusual symptoms typically occur in Klein-Levin Syndrome?

Answer 17

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

Question 18

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

Answer 18

Up to 18 hours per 24

Question 19

How long does Klein-Levin Syndrome last in total?

Answer 19

1 to several years with an average of 4

Question 20

What medication can make Klein-Levin Syndrome worse?

Answer 20

Daytime stimulants can cause psychiatric symptoms

Question 21

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

Answer 21

1. Gabapentin
2. Carbamazepine
3. Lithium
4. IV steroids

Question 22

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

Answer 22

Lithium

Question 23

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

Answer 23

IV steroids

Question 24

What will high leptin levels result in?

Answer 24

Suppress appetite and stimulate energy production

Question 25

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

Answer 25

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

Question 26

Which is more potent, modafinil or armodafinil?

Answer 26

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

Question 27

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

Answer 27

* Shortened REM latency * Increased REM duration * Increased REM density (increase frequency of rapid eye movements per REM period)

Question 28

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

Answer 28

Sodium oxybate (Xyrem)

Question 29

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

Answer 29

Up to 30%

Question 30

Why does narcolepsy result in a rapid transition to REM sleep

Answer 30

* 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

Question 31

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

Answer 31

* 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

Question 32

What 2 actions/treatments can trigger status cataplecticus

Answer 32

* Abrupt withdrawl of antidepressants * Prazosin

Question 33

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

Answer 33

Up to 30%