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Narcolepsy With Cataplexy
Narcolepsy With Cataplexy
WITH CATAPLEXY
CASE STUDY
POLYSOMNOGRAPHY II
-Sudeep, Vibhuti
Definition:
Narcolepsy:
It is a chronic neurological disorder characterized by
excessive daytime sleepiness (EDS) despite a restful
night of sleep and sudden and uncontrollable attacks of
sleep accompanied by hallucinations and paralysis.[1]
It effects approximately 1 in 2000 people.[1]
No known cure for Narcolepsy but with aid of
medication, behavior modification, scheduled naps and
other treatments, symptoms can be managed to lead a
relatively normal life.[1]
Definition:
Cataplexy:
It is one of the symptoms of Narcolepsy.
It is a partial or complete loss of muscle tone in response
to strong emotion.[2]
Loss in muscle tone could be mild or severe.
Can cause head dropping, impaired speech, clumsiness
and in severe cases complete collapse of the body to
ground.[2]
Usually triggered by laughter, anger, fear or surprise, but
unusual things such as a particular odor can also
precipitate an episode.[2]
Symptoms of Narcolepsy:
Excessive Daytime Sleepiness
Cataplexy
Hypnagogic Hallucinations
Sleep Paralysis
Automatic Behavior
Disrupted Nocturnal Sleep
Case Study
A 43 yr old presented to the sleep lab with complains
of :
Loud snoring.
Cessation of breathing during sleep.
Difficulty falling asleep and maintaining sleep.
Crawling sensations in his legs at sleep onset, leg
kicking during sleep.
Excessive daytime sleepiness and fatigue.
Sudden weakness and difficulty breathing when angry
and laughing.
Case Study Cont…
Sleep is described as restless and he awakens in the
morning unrefreshed.
Wakes up in night gasping for air and has trouble
falling asleep.
He usually takes one nap on weekdays and two naps
on weekends for about 2 hours each.
The Epworth-Sleepiness Scale was 14.
Patient’s father has been diagnosed with Narcolepsy
Medications:
Ritalin:
Using it for 10 yrs, for ADHD.
This drug suppresses REM.
Pristiq:
Antidepressant in a group of drugs called selective
serotonin and norepinephrine reuptake inhibitors
(SNRIs).[3]
Causes Insomnia.
Has been using it for a month.
PSG:
An overnight PSG was performed, pt had trouble
falling asleep and staying asleep.
Sleep Latency was 58 min and REM Latency was 263
minutes.
Sleep efficiency was 61.7%.
The sleep stage percentages were 3.8% stage 1, 51.3%
stage 2, 35.5% stages 3 and 9.7% REM sleep.
Pt had a very fragmented sleep and would wake up
almost every hour.
PSG Cont..
Mild snoring was noted. There were 11 respiratory
events consisting of 3 apneas (3 obstructive, 0 mixed,
and 0 central) and 8 hypopneas. The apnea-hypopnea
index was 2.8. The mean oxygen saturation during the
study was 92%, with a minimum oxygen saturation of
88%.
There were 10 periodic limb movements during sleep,
resulting in a PLM-index of 3.3. 0 of these movements
was associated with arousals, resulting in a PLM-
arousal index of 0.
MSLT:
Due to patients questionable symptoms of cataplexy
and family history of Narcolepsy, the pt was advised to
undergo an MSLT.
After an overnight sleep study, MSLT was initiated and
the pt was provided with 5 nap opportunities to asses
his sleepiness during the day.
Pt fell asleep in all naps, usually reaching upto Stage II
of sleep, but would take progressively less time to fall
asleep.
MSLT Results:
The pt was diagnosed with Narcolepsy with Cataplexy
even though there were no REM sleep in naps.
The medical director of the lab felt that use of Ritalin
for a long period of time had suppressed REM.
The muscle tone in chin leads were almost at Atonia
during 3 naps.
Treatment:
Medications used in the treatment of Narcolepsy and
Cataplexy are symptom related.
Excessive Daytime Sleepiness:
All meds used for treatment for EDS suppress REM[4]
Meds include Ritalin, Dexedrine, Desoxyn. [4]
Cataplexy
All meds used for treatment of Cataplexy, suppress REM
and increase Sleep Latencies.[4]
Meds include, Vivactil, Clomipramine, Desipramine.[4]
References
1. Butkov et.al: Fundamentals of Sleep Medicine. 1st
Edition, Philadelphia: Williams & Wilkins, 2007: 159-
160.
2. Butkov et.al: Fundamentals of Sleep Medicine. 1st
Edition, Philadelphia: Williams & Wilkins, 2007: 162-163
3. http://www.drugs.com/pristiq.html