COMPINDIUM

ON
NARCOLEPSY,
IDIOPATHIC CENTRAL NERVOUS SYSTEM
HYPERSOMNOLENCE,
&
ATTENTION DEFICIT DISORDER
Compiled by James M. Simmons
All Rights Reserved
1996, Revised 1996, 2005, 2016

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TABLE OF CONTENTS
OVERVIEW
NARCOLEPSY
IDIOPATHIC CENTRAL NERVOUS SYSTEM HYPERSOMNIA
CLASSIFICATION OF NARCOLEPSY
SYMPTOMS OF NARCOLEPSY
SLEEP ATTACK
CATAPLEXY
SLEEP PARALYSIS
HYPNOGOGJC HALLUCINATIONS
HEADACHE
OBESITY
SLEEP ONSET RAPID EYE MOVEMENT
EMPLOYMENT & SOCIAL IMPACT OF NARCOLEPSY.
NARCOLEPSY AND MEMORY
OBJECTIVE NEUROLOGICAL FINDiNGS
NARCOLEPSY AND PSYCHOLOGICAL CHANGES
APPENDIX
Scripps Gets Grant to Study Sleep Disorder

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OVERVIEW
NARCOLEPSY
Narcolepsy afflicts more than 200,00 Americans. In most cases the first symptom of the
disease, excessive daytime sleepiness, develops during childhood or adolescence. This initial
presentation is followed by cataplexy or other auxiliary symptoms several years later. Not
infrequently, many years pass before the proper diagnosis of narcolepsy is made. Narcolepsy is a
chronic lifelong disease without periods of remission. Excessive daytime sleepiness,
inappropriate sleep attacks and the pathognomonic symptom of 9ataplexy, are diagnostic of
narcolepsy. Confirmation of the disease is made by a multiple sleep latency test. Although still
not being used for diagnostic purposes, the association between narcolepsy an the human
leukocyte group A (LA) antigen DR2 is the strongest so far described for any disease. With the
help of psychosocial support, therapeutic naps, and medications, the patient with narcolepsy may
be able to lead a normal life. Methylphenidate and imipramine are the two most widely used
drugs for the treatment of daytime somnolence and cataplexy respectively.
Key words: Sleep, REM; narcolepsy; hallucination; cataplexy; sleep disorders. [Narcolepsy,
Chaudhary & Husain - The Journal of Family Practice, Vol. 36, No. 2, 1993]
IDiOPATHIC CENTRAL NERVOUS SYSTEM HYPERSOMNIA [ICNSH]
Central nervous system hypersomnia is a disabling disease that is understood even less
than narcolepsy. Patients impairment is frequently very severe, despite the absence of obvious
neurological lesions. At times, the severity of the impairment can place lives in jeopardy.
Persons with CNS hypersomnia have sustained third degree burns during automatic behavior
episodes or have turned on gas furnaces or stoves without lighting theim..
[Principles and Practice of Sleep Medicine, Kryger, Roth, Dement.]
Central Nervous System Hypersomnia is a very disabling problem, which often leads to
permanent unemployment and responds poorly to medical treatment.
[Kryger, Roth, Dement.]
The sleep specialist must be able to convey the seriousness of this chronic disability to
the Social Security Administration and other agencies and must objectively and convincingly
document the presence of this disorder, in view of the long-term prognosis and current absence
of treatments adequate to control the symptoms.
[Kryger, Roth, Dement.]
CLASSIFICATION OF NARCOLEPSY:
From an etiological standpoint, narcolepsy can be classified into two types: idiopathic
and symptomatic. Idiopathic narcolepsy is a syndrome of unknown origin, not the consequence
of another disease. Speculations on the possible origin of this form appears to be a
neurochemical transmitter-synaptic malfunction in the brains sleep-wake system. Symptomatic
narcolepsies occur in association with brain concussions, encephalitis, and tumors in the
mesodiencephalic region.... A few believe that all cases of narcolepsy are symptomatic (Vein,
1974). Still others believe that no causal relationship has been demonstrated between brain
concussions, encephalitis, and narcolepsy.
[Sleep Disorders - Diaanosis and Treatment, 2nd Ed., Williams, Karacan, Moore.]

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SYMPTOMS OF NARCOLEPSY
The criteria for the diagnosis of Narcoleptic syndrome are is called the Narcoleptic
Tetrad:
1.
2.
3.
4.

The occurrence of daytime sleep attacks;

Cataplexy;
Sleep paralysis;
Hypnagogic hallucinations; [Williams, Karacan, Moore.]

SLEEP ATTACK
...The term sleep attack is misleading because it implies the narcoleptic patient is well
awake and alert during the day, except for a few irresistible sleep episodes. This is not the case.
There is, in general, a deteriorated condition of wakefulness, which itself, may be overcome by
sleep at almost any time. A person developing narcolepsy might start by falling asleep at times a
normal person would think unremarkable: during a concert, after lunch, or watching television.
Patients with advanced narcolepsy often faIl asleep at totally inappropriate times: while walking,
driving, engaged in sports, teaching, or even during sexual intercourse.
[Williams, Karacan, Moore.]
CATAPLEXY
Cataplexy, or abrupt occurrences of muscular weakness and hypotonia, is the second
most common symptom of narcolepsy and is often triggered by the change in emotion associated
with laughter, fear, surprise, or rage. These sudden losses or reductions of voluntary muscle
control last only a short time (ordinarily five seconds to two minutes) and the patient is fully
aware of what is happening. Cataplexy occurs in 65 to 75% of all patients with narcolepsy. It is
more frequent in idiopathic narcolepsy than in the symptomatic forms, in which it is present in
only 24 to 40% of the cases.
[Williams, Karacan, Moore.]
SLEEP PARALYSIS
Sleep paralysis, also paroxysmal, is a state similar to cataplexy, but it is not triggered by
emotion. However, it can be easily ended by external stimuli, alterations of consciousness and
hallucinations can occur, and the paralysis lasts longer than 30 seconds. Usually an occurrence
lasts for only one to ten minutes and happens only two or three times a month. Sleep paralysis
occurs in 10 to 50% of the cases of narcolepsy. Indeed, sleep paralysis is not a rare independent
phenomenon, occurring occasionally 2.7 to 6.1% of the general population.
[Williams, Karacan, Moore.]
HYPNOGOGIC HALLUCINATIONS
Hypnogogic hallucinations are auditory or somatosensory perceptions, having no basis in
sensory information from the subject~ s environment, that occur between the states of sleep and
consciousness. By nature, these hallucinations are dreamlike and are often accompanied by sleep
paralysis. Because consciousness is frequently clouded, patients not critical of their perceptions
may experience intense feelings of anxiety.
[Williams, Karacan, Moore.]

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HEADACHE
Headache is a common complaint of patients with narcolepsy. The headaches are usually
not migraines and often disappear with a good sleep. Their occurrence may be due to the
patients constant need to fight off sleep.
[Williams, Karacan, Moore.]
OBESITY
Obesity, usually slight to moderate, is seen in up to one-third of the patients with
narcolepsy. [Williams, Karacan, Moore.]
SLEEP ONSET RAPID EYE MOVEMENT
Characteristics of REM sleep regulation in narcoleptics is an abnormal transition from
wakefulness to REM sleep or sleep onset REM periods (SOREMPs). This occurs during the
diurnal periods, and follows awakenings from REM to NREM in the nocturnal period.
[Williams, Karacan, Moore.]
One of the chief differences between narcoleptic patients and normal subjects can be
seen in the transition from wakefulness to sleep. Normal adults enter NREM sleep from
wakefulness and do not enter REM sleep until about 70 minutes of NREM sleep have elapsed.
Narcoleptic patients pass directly into REM wakefulness, or after an abbreviated NREM period
of sleep. This sharp passage to REM sleep is thus called SOREMP. It should be understood that
the patient with narcolepsy can also fall asleep normally.... [Patients] with narcolepsy can have
SOREMPs either during [the afternoon] or at the onset of nocturnal sleep if conditions are right.
[Williams, Karacan, Moore.]
EMPLOYMENT & SOCIAL IMPACT OF NARCOLEPSY
Narcolepsy remains a very disabling neurologic illness that is poorly or incompletely
controlled by treatment. It leads to a variety of complications when it goes undiagnosed, such as
traffic and industrial accidents. Narcolepsy is a major employment problem for its victims owing
to many employers unwillingness to allow short (15-minute) naps two or three times during the
day. It is often responsible for job discrimination, job dismissal, early retirement, and depression
secondary to these circumstances.
[Sleep Disorders Medicine: Basic Science, Technical Considerations, and Clinical
Aspects,
Edited by Sudhansu Chokroverty]
Narcolepsy leads to a variety of complications: for example, ... difficulties at work
responsible for disability, forced retirement, or job dismissal...
[Kryger, Roth, Dement.]
Undoubtedly, narcolepsy is a disabling disorder, leading, in many instances, to loss of
gainful employment because of daytime sleepiness and automatic behavior. it is also a disorder
poorly understood by patients, family members and peers that can result in rejection from
families and other social entities, in divorces, in loss of self-esteem, and in depressive reactions.
For these reasons, and in consideration of age of onset, it is important to put narcoleptic patients
in contact with support groups and to help with the creation of regional narcolepsy associations
and patient groups.
[Kryger, Roth, Dement, pg. 344]

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The aim of treating a narcoleptic patient is to relieve the symptoms that interfere with
day-to-day functioning and to reassure the patient and family. With the help of psycho-social
counseling, therapeutic naps, and medication, the patient may be able to perform at an optimal
level.
Narcolepsy is frequently misunderstood.3138 Parents, teachers, spouses, and coworkers
often assign motivational causes [for symptomatic behavior], further dampening patient selfesteem. It must be explained to the patient and his or her family members, friends, and
coworkers that narcolepsy is a physical illness which the patient cannot voluntarily control.
Family and group therapy may be valuable in helping patients accept the illness and establish
reasonable expectations.
[Narcolepsy, Chaudhary & Husain - The Journal of Family Practice, Vol. 36, No. 2,
1993, pg.
210]
NARCOLEPSY AND MEMORY
About 50% of narcoleptic patients have automatic behavior with complete amnesia.32
There may be brief lapses in the middle of conversation or during more complex behavior such
as walking, driving, or continuing routine work for a period of time. ...
Approximately one half of the patients with narcolepsy have memory problems,
particularly loss of recent memory.3738 It is hypothesized that rnicrosleep occurring during the
daytime interferes with perception and acquisition of information.39 Objective evaluations have
shown that narcoleptics do have more difficulty in maintaining attention, although the results on
concentration and memory are not significantly different from those of controls.40 However,
normal results on concentration and memory tests have been attributed to the use of stimulants
by an estimated 77% of narcolepsy patients.
[Narcolepsy, Chaudhary &; Husain - The Journal of Family Practice, Vol. 36, No. 2,
1993, pg. 208]
OBJECTIVE NEUROLOGICAL FINDINGS
Patients with narcolepsy do not appear to have a true organic short-term or long-term memory
deficit revealed by memory tests. Medication (including methylphenidate and tricyclics) had no
significant effect on memory in the patients with narcolepsy studied by Broughton et al. (1981).
The authors concluded that, challenged with motivating memoty tests in a laboratory
environment, individuals with narcolepsy can perform at normal levels. Everyday life is another
matter. The patient with narcolepsy has transitory decreases in vigilance during which
information received might be poorly recalled, if at all. Thus memory tests results can be similar
to those of performance tests. The results in both cases can be related to the presence or absence
of drowsiness.
[Sleep Disorders: Diagnosis and Treatment, Williams, Karacana, Moore, pg 92.]
NARCOLEPSY AND PSYCHOLOGICAL CHANGES
Even though the majority of patients with narcolepsy are psychologically normal,
psychological problems occur with many patients. Depression has been described in patients
with narcolepsy by several researchers (Fement, et al., 1973; Cave, 1931; Sours, 1963) This ~s
not difficult to understand considering the misery narcolepsy brings to the patients life. As a
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group, patients with narcolepsy tend to be deficient in many areas of their family, social, and
emotional life (Walsh, McMahan, Sexton, & Smitson, 1982).
[Sleep Disorders: Diagnosis and Treatment, Williams, Karacana, Moore, pg 998.]
The psychological quirks and neuroses of narcoleptics are difficult to assess. Surely the
symptoms place an incredible strain upon a personss life and self-control. A person who is
overcome by a resistless sleep attack in the midst of making love, or who drops to the steet if he
listens to a funny joke and laughs, might be expected to develop some unusual behavior in
reaction. However, the fact that emotion can trigger the sleep attack has intrigued people who
have ned to understand its etiology. A survey of the literature indicates that many narcoleptics
suffered their first attack after a trauma that occurred in early adolescence. Many of these
children of about 13 to whom a death in the family caused sudden and intense emotional upset.
This may be precisely th ekind of emotional jolt that Richter postulated when he predicted that
trauma might cause the normally out-of-phase metabolic cycles to come into phase-producing
strange symptoms in a periodic fashion.
[Sleep and Dream Researci, Staff of Reasearch and Education Association, 1982, pg. 97]

NOTES ON ADULT ATTENTION DEFICIT DISORDER

SUGGESTED DIAGNOSTIC CRITERIA FOR ATTENTION DEFICIT DISORDER IN
ADULTS.
NOTE: Consider a criterion met only if the behavior is considerably more frequent than that of
most people of the same mental age.

A.

A chronic disturbance in which at least twelve of the following are present:

1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.

A sense of underachievement, of not meeting ones goals (regardless of how

much has actually accomplished).
Difficulty getting organized.
Chronic procrastination or trouble getting organized.
Many projects going simultaneously; trouble with follow-through.
A tendency to say what comes to mind without necessarily considering the timing
or appropriateness of the remark.
A frequent search for high stimulation.
An intolerance of boredom.
Easy distractibility, trouble focusing attention, tendency to tune our or drift away
in the middle of a page or a conversation, often coupled with an ability to hyperfocus at times.
Often creative, intuitive, highly intelligent.
Trouble in going through established channels, following proper procedure.
Impatient; low tolerance of frustration.
Impulsive, either verbally or in action, as in impulsive spending of money;
changing plans, enacting new schemes or career plans, and the like, hot tempered.

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13.
14.
15.
16.
17.
18.
19.
20.

A tendency to worry needlessly, endlessly; a tendency to scan the horizon,

looking for something to worry about, alternating with inattention to or disregard
for actual dangers.
A sense of insecurity.
Mood swings, mood lability, especially when disengaged from a person or a
project.
Physical or cognitive restlessness.
A tendency toward addictive behavior.
Chronic problems with self-esteem.
Inaccurate self-observation.
Family history of ADD or manic-depressive illness or depression or substance
abuse or other disorders of impulse control or mood.

B.
Childhood history of ADD. (It may not have been formally diagnosed, but in reviewing
the history, one sees that the signs and symptoms were there.)
C.
Situation not explained by other medical or psychiatric condition. [Driven to Distraction,
Edward M. Halloway, M.D., and John J. Ratey, M.D., 1994]
The Basic Symptons
1. Inattention/Distractibility
Adults with ADD will often still find themselves having trouble concentrating on a number of
things. They may find they have trouble staying on task when they are at work, with the result
that they do not finish as much as they would like to. This kind of thing can also affect them
around the house, where they can go from project to project without ever seeming to finish
anything. Many ADD homemakers find that they cant seem to stay on top of household chores,
and the day feels like one endless series of frustrations.
Inattentiveness also frustrates Attention Deficit adults in social situations, where they can have
considerable difficulty focusing on conversations with others. Someof them find big parties or
family get-togethers frustrating because so many conversations are going on at the same time,
and they keep getting distracted from the one they are supposed to be paying attention to. This
makes them run the risk of some embarrassment, if it becomes apparent to other people that they
have lost the train of thought. They may also look to others as if they are bored, because they
appear restless and do not always maintain eye contact.

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2. lmpulsivity
This sympton in adults may still be there, but it will be much more restrained than it is in ADD
children. By the time they are adults, many of these people have been burned enough by past
impulsive actions that they exercise more self-control. This may be especially true in social
situations whre they dont know teh other people well. In fact, some ADD adults can be
downright quiet when confronted with strangers!
3. Difficulty delaying gratification.
Impulsivity and difficulty with delay are similar problems. Impulsivity refers to action taken
without thought and without waiting. Difficulty with delay is the sense of impatience and
frustration stimulated by having to wait and think. [ADD adults] may have trouble finding the
patience for academic, schoolish like tasks such as balancing a check book, filing a tax return,
or paying bills.
[Driven to Distraction, Edward M. Halloway, M.D., and John J. Ratey, M.D., 1994]

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