Narcolepsy and cataplexy

Obstetric Management of a Patient with Narcolepsy and Cataplexy: A Case Report

Narcolepsy and cataplexy

We report the management of a patient who suffers from narcolepsy and cataplexy and presented to the clinic at 14 weeks of gestation.

Her symptoms were resistant to modafinil but controlled with clomipramine and amphetamine. Discussion concerning mode of delivery for these patients is scarce in the literature.

We discuss some issues surrounding the antenatal management and counselling regarding mode of delivery and postpartum care.

1. Introduction

Narcolepsy is a chronic neurological condition producing disruption to normal sleep pattern resulting into excessive daytime somnolence.

Approximately 80% of the patients with narcolepsy suffer from cataplexy (involuntary loss of muscle tone) [1], and cataplexy is pathognomonic for narcolepsy. There is little information on outcome or management of this disorder in pregnancy.

We report a case of successful management of pregnancy in a patient with narcolepsy and cataplexy.

2. Case Report

A 44-year-old G3 Para1 (previous normal vaginal delivery at term 24 years ago) with a past history of narcolepsy and cataplexy was referred to the combined medical/obstetrics antenatal clinic at 14 weeks of gestation.

Narcolepsy and cataplexy was diagnosed in January 2005 and since then had been under regular Neurological review. She had developed daytime sleepiness, cataplexy, sleep paralysis, and hallucination on going to sleep. Cataplexy was controlled with clomipramine, and amphetamine (dexamphetamine) was started in November 2010 when her symptoms worsened. Her symptoms were resistant to modafinil.

Antenatally the patient was managed in conjunction with the neurologist, and referral was made to the anaesthetist, and a paediatric alert was made in view of amphetamine use in early pregnancy. The pregnancy proceeded uneventfully, and the dose of dexamphetamine was reduced during the pregnancy and eventually discontinued at 24 weeks of gestation.

She was reviewed by the anaesthetist at 36 weeks of gestation due to concerns regarding option of analgesia and mode of delivery if she presents in labour.

She was also reviewed by the consultant obstetrician and after weighing the benefits and risks of elective delivery versus spontaneous labour opted for an elective Caesarean section at nearly 38 weeks of gestation with steroid cover as per unit protocol.

The Caesarean section was performed under spinal analgesia and was uneventful. A female baby weighing 2.84 Kg was delivered with Apgar score 7, 10, and 10 at 1st, 5th, and 10th minutes of life. Cord gases were pH (V) 7.383 BE-2.8, pH (A) 7.361 BE-6.1.

Baby needed only facial oxygen at birth. Neonatal abstinence syndrome (NAS) scoring was not required for baby.

3. Discussion

Narcolepsy is a chronic neurological condition producing disruption to normal sleep pattern, and this causes excessive daytime somnolence. Approximately 80% of patients with narcolepsy suffer from cataplexy (involuntary loss of muscle tone) [1], and cataplexy is pathognomonic for narcolepsy.

The cause of narcolepsy is unknown, but it is thought that both environmental and genetic factors may play apart. It may be caused by the loss of a relatively few neurons that are responsible for producing hypocretin-a neuropeptide in the CNS. It is also associated with specific HLA allele, DQB1*0602.

Possible triggers include head trauma, infection, and change in sleeping habits [2].

Diagnostic criteria including excessive daytime sleepiness, almost daily for greater than 3 months, and cataplexy triggered by emotion, confirmed by diagnosis with nocturnal polysomnography followed by a multiple sleep latency test (MSLT) and alternatively confirmed by CSF hypocretin-1 levels

Source: https://www.hindawi.com/journals/criog/2012/982039/

Understanding Narcolepsy

Narcolepsy and cataplexy

  • Narcolepsy is an important cause of chronic sleepiness, affecting about 1 in 2,000 people. It typically develops during the teen years and lasts for life.
  • Narcolepsy is a manageable condition, and people with narcolepsy can lead full and rewarding lives.

Narcolepsy is a neurological disorder that causes persistent sleepiness and additional symptoms such as brief episodes of muscle weakness known as cataplexy, vivid, dream hallucinations, brief episodes of paralysis when falling asleep or upon awakening (sleep paralysis), and fragmented nighttime sleep. Symptoms typically develop over several months and last a lifetime.

The disorder usually begins between ages 10 and 20, although sometimes it starts as late as age 40 or 50. Narcolepsy affects women and men equally, occurring in about 1 in 2,000 people.

Narcolepsy is a manageable condition, and with an array of treatment strategies, people with narcolepsy can live full and rewarding lives.

For a one-page summary of narcolepsy and its symptoms, causes, and treatments, download What Is Narcolepsy? (PDF).

Types of narcolepsy
Clinicians now recognize two major types of narcolepsy: narcolepsy with cataplexy (muscle weakness triggered by strong emotions) and narcolepsy without cataplexy. People who have narcolepsy without cataplexy have sleepiness but no emotionally triggered muscle weakness, and generally have less severe symptoms.

There also exists another, very rare type known as secondary narcolepsy, which occurs with injury to a deep part of the brain called the hypothalamus. In addition to any of the typical narcolepsy symptoms, people with secondary narcolepsy also have severe neurological problems and require large amounts (>10 hours) of sleep.

Sleep basics
To understand the symptoms of narcolepsy, it helps to first understand how sleep happens normally. Healthy sleep includes two types of sleep: rapid-eye-movement (REM) sleep and non-REM (NREM) sleep.

REM sleep is characterized by dreams, quick eye movements, and paralysis of the limbs and trunk that prevents someone from acting out dreams and getting injured during sleep. In non-REM sleep, dreams are less common and the body is not paralyzed. In individuals without narcolepsy, REM sleep, non-REM sleep, and wakefulness are distinct states that do not mix together.

To learn more about types of sleep and sleep cycles, see Healthy Sleep.

In people with narcolepsy, the regulation of sleep is disrupted: the boundaries between wakefulness and sleep are less distinct, and elements of sleep and wakefulness can mix together.

The sleep patterns of people with narcolepsy differ in several ways from the sleep patterns of people who do not have the disorder:

People with narcolepsyPeople without narcolepsy
Feel drowsy, inattentive, and fall asleep easily Feel alert, awake, and don’t readily fall asleep
Often enter REM sleep and dream during naps Generally do not enter REM sleep or dream during naps
Hallucinations on waking common Hallucinations on waking uncommon
Sleep paralysis common Sleep paralysis uncommon
May experience cataplexy Never experience cataplexy
Spontaneously wake from sleep Generally sleep well
Can enter REM sleep within 15 minutes Start out in non-REM sleep and then take 60–90minutes to enter REM sleep

This content was last reviewed on February 21, 2018

Source: http://healthysleep.med.harvard.edu/narcolepsy/what-is-narcolepsy/understanding

Narcolepsy – NORD (National Organization for Rare Disorders)

Narcolepsy and cataplexy

TEXTBOOKS
Goswami M. Narcolepsy. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:561-2.

Fauci AS, et al., eds. Harrison’s Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill Companies, Inc.; 1998:155.

Wyngaarden JB, et al., eds. Cecil Textbook of Medicine. 19th ed. Philadelphia, PA: W.B. Saunders Company; 1992:2065-2066.

Adams RD, et al., eds. Principles of Neurology. 4th ed. New York, NY: McGraw-Hill Companies, Inc.; 1989:314-317.

JOURNAL ARTICLES
Szakacs Z, Dauvilliers Y, Mikhaylov V, Poverennova I, Krylov S, Jankovic S, et al. Safety and efficacy of pitolisant on cataplexy in patients with narcolepsy: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 2017. doi:10.1016/S1474-4422(16)30333-7.

Ruoff C, Swick TJ, Doekel R, Emsellem HA, Feldman NT, Rosenberg R, et al. Effect of Oral JZP-110 (ADX-N05) on Wakefulness and Sleepiness in Adults with Narcolepsy: A Phase 2b Study. Sleep 2016;39:1379–87. doi:10.5665/sleep.5968.

Sullivan SS, Guilleminault C. Emerging drugs for common conditions of sleepiness: obstructive sleep apnea and narcolepsy. Expert Opin Emerg Drugs 2015;20:571–82. doi:10.1517/14728214.2015.1115480.

Hallmayer J, Faraco J, Lin L, et al. Narcolepsy is strongly associated with the T-cell receptor alpha locus. Nat Genet. 2009;41:708-711.

Billiard M. Narcolepsy: current treatment options and future approaches. Neuropsychiatr Dis Treat. 2008;4:557-566.

Alaez C, Lin L, Flore A-H, et al. Association of narcolepsy-cataplexy with HLA-DRB1 and DQB1 in Mexican patients: a relationship between HLA and gender is suggested. BMC Med Genet. 2008;9:79.

Black J, Duntley SP, Bogan RK, O’Malley MB. Recent advances in the treatment and management of excessive daytime sleepiness. CNS Spectr. 2007;12:1-14.

Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007;369:499-511.

Wise MS, Arand DL, Auger RR, Brooks SN, Watson NF. Treatment of narcolepsy and other hypersomnias of central origin. Sleep. 2007;30:1712-1727.

Nishino S. Clinical and neurobiological aspects of narcolepsy. Sleep Med. 2007;8:373-399.

Longstreth Jr. WT, Koepsell TD, Ton TG, Hendrickson AF, van Belle G. The epidemiology of narcolepsy. Sleep. 2007;30:13-26.

Dauvilliers Y, Carlander B, Billiard M. Narcolepsy, from Westphal to hypocretin. Presse Med. 2004;33:1593-600.

Dauvilliers Y, Carlander B, Rivier F, Touchon J, Tafti M. Successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset. Ann Neurol. 2004;56:905-8.

Mignot E. Sleep, sleep disorders and hypocretin (orexin). Sleep Med. 2004;5:S2-8.

McKenna L, McNicholas F, Childhood onset narcolepsy. A case report. Eur Child Adolesc Psychiatry. 2003;12:43-7.

Hood Bm, Harbord MG, Paediatric narcolepsy: complexities of diagnosis. J Paediatr Child Health. 2002;38:618-21.

Randomized trial of modafinil as a treatment for the excessive daytime somnolence of narcolepsy: US Modafinil in Narcolepsy Multicenter Study Group. Neurology. 2000;54:1166-1175.

Lin L, et al. The sleep disorder canine narcolepsy is caused by a mutation in the hypocretin (orexin) receptor 2 gene. Cell. 1999;98:365-376. [See comment in Cell. 1999;98:409-412.]

INTERNET
Bozorg AM, Benbadis SR. Narcolepsy. Medscape. http://emedicine.medscape.com/article/1188433-overview Updated: May 9, 2017. Accessed June 6, 2017.

National Institute of Neurological Disorders and Stroke. Narcolepsy Fact Sheet. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Narcolepsy-Fact-Sheet Accessed June 6, 2017.

Mayo Clinic for Medical Education and Research. Narcolepsy. http://www.mayoclinic.com/health/narcolepsy/DS00345 Updated September 1, 2015. Accessed March 2, 2017.

Source: https://rarediseases.org/rare-diseases/narcolepsy/

Narcolepsy – Symptoms

Narcolepsy and cataplexy

Not everyone with narcolepsy has the same symptoms. Some people have symptoms regularly, while others are less frequently affected.

Symptoms may develop slowly over a number of years, or suddenly over the course of a few weeks.

Narcolepsy is usually a long-term (chronic) condition, although some of the symptoms may improve as you get older.

You should see a GP if you think you may have narcolepsy so they can find out what's causing your symptoms.

If necessary, you'll be referred to a sleep disorder specialist, who can confirm the diagnosis.

Find out more about diagnosing narcolepsy

Excessive daytime sleepiness is usually the first sign of narcolepsy. It can have a significant impact on everyday life.

Feeling drowsy throughout the day and struggling to stay awake makes it difficult to concentrate at work or school.

People with narcolepsy may be misjudged as being lazy or rude.

Sleep attacks, where you fall asleep suddenly and without warning, are also common in people with narcolepsy. They may happen at any time.

The length of time a sleep attack lasts will vary from person to person. Some people will only have “microsleeps” lasting a few seconds, whereas others may fall asleep for several minutes.

If narcolepsy is not well controlled, sleep attacks may happen several times a day.

Most people who have narcolepsy also experience cataplexy, which is sudden temporary muscle weakness or loss of muscular control.

Typical symptoms of cataplexy are:

  • the jaw dropping
  • the head slumping down
  • legs collapsing uncontrollably
  • slurred speech 
  • double vision or finding it difficult to focus

Cataplexy attacks are usually triggered by an emotion, such as excitement, laughter, anger or surprise.

Attacks can last from a few seconds to several minutes.

Some people with narcolepsy have cataplexy attacks once or twice a year, while others have them several times a day. 

In an attempt to avoid attacks, some people may become emotionally withdrawn and socially isolated.

Some people with narcolepsy experience episodes of sleep paralysis. This is a temporary inability to move or speak that occurs when waking up or falling asleep.

The episodes can last from a few seconds to several minutes. Although sleep paralysis does not cause any harm, being unable to move can be frightening.

Narcolepsy can also cause a number of other symptoms, including:

  • hallucinations – seeing or hearing things that are not real, particularly when going to sleep or waking up; a presence in the bedroom is the most commonly reported hallucination
  • memory problems
  • headaches
  • restless sleep – for example, having hot flushes, waking up frequently, having vivid nightmares, or physically acting out dreams
  • automatic behaviour – continuing with an activity without having any recollection of it afterwards 
  • depression

Speak to a GP if you have narcolepsy and it's making you feel low or depressed.

They can advise you about how to minimise the effect narcolepsy has on your daily life.

They can also put you in touch with narcolepsy organisations or support groups, such as Narcolepsy UK.

Source: https://www.nhs.uk/conditions/narcolepsy/symptoms/

About Narcolepsy

Narcolepsy and cataplexy

Narcolepsy is a chronic neurological disorder that can begin at any age and continues throughout life. It is a sleep disorder, involving irregular patterns in Rapid Eye Movement (REM) sleep, and significant disruptions of the normal sleep/wake cycle.

Onset typically occurs in pre-teens/teens or the early twenties, but can also happen later in life. Narcolepsy is believed to affect approximately 1 in 2,000 people in the United States.

It affects both sexes equally and occurs throughout the world, but is underrecognized and underdiagnosed. Once established, narcolepsy is generally stable and can most often be effectively treated.

Lifespan is not affected.

Narcolepsy with cataplexy is caused by the destruction of hypocretin-producing cells in the hypothalamus region of the brain.

Hypocretin (also known as orexin) is a neurotransmitter involved in the regulation of the sleep/wake cycle as well as other bodily functions (e.g. blood pressure and metabolism).

Narcolepsy with cataplexy is an auto-immune disorder. More research is needed to determine the exact triggers behind narcolepsy without cataplexy.

Diagnosis of narcolepsy is usually confirmed in a sleep lab through a series of tests, which typically includes an overnight polysomnogram (PSG or sleep study) to rule out other causes of EDS and detect any unusual REM patterns.

The Multiple Sleep Latency Test (MLST), or daytime nap test, follows, which measures the rapidity of sleep onset and how quickly REM sleep follows. The MLST is the most widely accepted diagnostic test for narcolepsy. A blood test is sometimes used to determine if there is a genetic predisposition towards the disorder.

Finally, some research facilities measure the level of hypocretin in the cerebrospinal fluid (CSF). This is rare and only used in certain situations.

The Diagnostic and Statistical Manual (DSM V) divides narcolepsy into Narcolepsy Type 1, or narcolepsy with cataplexy, and Narcolepsy Type 2, or narcolepsy without cataplexy.

Symptoms include:

Excessive Daytime Sleepiness (EDS) which is described as a persistent sense of mental cloudiness (brain fog), lack of energy, or extreme exhaustion.

  It includes daytime sleep attacks that may occur with or without warning and may be uncontrollable, and persistent drowsiness, which can continue for prolonged periods of time.

Microsleeps, or fleeting, involuntary moments of sleep that may intrude into the waking state, are also experienced as part of EDS for many people. Naps can help people with narcolepsy (PWNs) feel refreshed for a short period of time before EDS symptoms return.

Cataplexy, the second major symptom of narcolepsy, is nearly unique to the disease. It is a sudden loss of muscle tone, usually triggered by emotions such as laughter, surprise, fear, or anger. Cataplexy occurs while the person is awake and causes feelings of weakness and a loss of voluntary muscle control.

Cataplexy may occur more often during times of stress or fatigue. Attacks can involve only a slight feeling of weakness in one part of the body (i.e. sagging facial muscles, nodding head, buckling knees, garbled speech, etc.) or an immediate and total full body collapse.

Although someone suffering a severe cataplexy attack may appear unconscious, they are actually awake and alert. Attacks can last from a few seconds up to several minutes.

Cataplexy is related to the loss of muscle tone usually associated with dreaming or REM sleep; as a protection against acting out one’s dreams, muscles become immobile or paralyzed. In cataplexy, this protection is triggered inappropriately during wakefulness.

Disrupted or fragmented nighttime sleep is sleep disrupted by periods of wakefulness, vivid dreams, sleep talking, and movement. PWNs typically have no difficulty initially falling asleep.

Hypnagogic (during sleep onset) and hypnopompic (during waking) hallucinations are vivid, realistic, and often frightening dreams that occur on the edge of sleep and wakefulness.

Sleep paralysis is the temporary inability to move, occurring in the transition between sleep and wakefulness.

If you think you have narcolepsy, take the Epworth Sleepiness Scale and Swiss Narcolepsy Scale. These tools can be used to assess daytime sleepiness.

Source: https://narcolepsynetwork.org/about-narcolepsy/

Narcolepsy Symptoms, Treatment & Remedies

Narcolepsy and cataplexy

Narcolepsy is a sleep disorder characterized by excessive sleepiness, sleep paralysis, hallucinations, and in some cases episodes of cataplexy (partial or total loss of muscle control, often triggered by a strong emotion such as laughter). Narcolepsy occurs equally in men and women and is thought to affect roughly 1 in 2,000 people. The symptoms appear in childhood or adolescence, but many people have symptoms of narcolepsy for years before getting a proper diagnosis.

People with narcolepsy feel very sleepy during the day and may involuntarily fall asleep during normal activities. In narcolepsy, the normal boundary between awake and asleep is blurred, so characteristics of sleeping can occur while a person is awake.

For example, cataplexy is the muscle paralysis of REM sleep occurring during waking hours. It causes sudden loss of muscle tone that leads to a slack jaw, or weakness of the arms, legs, or trunk.

People with narcolepsy can also experience dream- hallucinations and paralysis as they are falling asleep or waking up, as well as disrupted nighttime sleep and vivid nightmares.

Narcolepsy with cataplexy is caused by the loss of a chemical in the brain called hypocretin. Hypocretin acts on the alerting systems in the brain, keeping us awake and regulating sleep wake cycles.

In narcolepsy, the cluster of cells that produce hypocretin—located in a region called the hypothalamus—is damaged or completely destroyed.

Without hypocretin, the person has trouble staying awake, and also experiences disruptions in the normal sleep-wake cycles.

Currently there is no cure for narcolepsy, but medications and behavioral treatments can improve symptoms for people so they can lead normal, productive lives.

Narcolepsy is diagnosed by a physical exam, taking a medical history, as well as conducting sleep studies. If you do have narcolepsy, the most effective treatment is often a combination of medications and behavioral changes.

People who are diagnosed with narcolepsy should seek counseling through educational networks and support groups. Getting a diagnosis of narcolepsy and managing the symptoms can be overwhelming and the disorder is not well understood by the general public.

It helps to learn best practices and access support through others who have the disorder.

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Source: https://www.sleepfoundation.org/articles/narcolepsy

Narcolepsy with Cataplexy in an Elderly Woman

Narcolepsy and cataplexy

A 72-year-old woman was referred for a 15-year history of brief attacks of generalized weakness that occurred when she was tense or startled. During these episodes, she squatted, closed her eyes, and had difficulty speaking, but there was no disturbance of consciousness.

The cerebrospinal fluid level of orexin/hypocretin was low (92 ng/L), leading to a diagnosis of narcolepsy with cataplexy according to the International Classification of Sleep Disorders (ICSD)-2 criteria. Cataplexy should be considered for sudden attacks of weakness lasting less than 2 minutes and with no alteration of consciousness.

Measurement of cerebrospinal fluid levels of orexin/hypocretin is recommended when the diagnosis is uncertain.

A 72-year-old woman was referred to our hospital by her primary care doctor for a 15-year history of experiencing “attacks” during which she “lost body control.” These episodes occurred one to ten times on most days, and lasted approximately 1 minute. They occurred when she was tense or startled (e.g.

telephone ringing, unexpectedly meeting an acquaintance, or being called into the clinic room by her doctor). During these episodes, she closed her eyes, squatted, and had difficulty speaking.

While she could not answer questions, she maintained consciousness and was able to hear the individuals around her and could accurately recall their conversations.

The patient reported that she often fell asleep during the day, including during meals, and that she frequently took 15-minute naps that refreshed her temporarily. She occasionally experienced hypnagogic hallucinations, but did not experience sleep paralysis.

None of her relatives had similar symptoms. The patient had a 4-year history of well-controlled diabetes, as well as hypertension and hyperlipidemia; she had no complications from her diabetes.

Medications included metformin, voglibose, glimepiride, losartan, hydrochlorothiazide, nifedipine, and atorvastatin.

The patient had experienced attacks at her physicians’ offices, and they had been witnessed by generalists, neurologists, and neurosurgeons, who had variously diagnosed essential tremor, orthostatic hypotension, epilepsy, and psychosomatic disease.

A video of an attack was recorded when she visited our clinic (see video link), which showed her experiencing paroxysmal weakness.

As can be seen in the video, when the patient was called into the clinic room, she first stood up from her chair and then assumed a half-sitting posture with her head and arms hanging down and her eyes closed.

Although she was able to temporarily stand again and walk a few steps, she subsequently became unable to support herself and had to squat down. After 1 minute, she recovered and was able to stand and move normally.

Physical exam results, including vital signs, cardiopulmonary, and neurological findings, were normal. Her body mass index was 32 (162 cm, 72 kg). Basic lab values were within normal limits, including hemoglobin A1C, which was stable at 6.8 %.

The patient underwent monitoring during these episodes. Blood glucose, Holter electrocardiogram, interictal electroencephalogram, and brain magnetic resonance imaging results were normal.

During the course of her evaluation, cataplexy was considered, as her bilateral atonic attacks seemed to occur when she was tense or startled, and she continued to maintain consciousness.

Since excessive sleepiness was suggested by medical history, we performed an evaluation to make a definite diagnosis of narcolepsy with cataplexy. Polysomnography did not detect sleep-onset rapid eye movement periods (SOREMPs).

A lumbar puncture was subsequently performed, and measurement of orexin/hypocretin in the cerebrospinal fluid (CSF) revealed a low level of 92 ng/L, leading to a diagnosis of narcolepsy with cataplexy according to the International Classification of Sleep Disorders–Second Edition (ICSD-2) criteria.

After treatment for cataplexy with paroxetine at a daily dose of 10 mg, her attacks of generalized weakness were reduced to two episodes every 2 weeks. When the dose of paroxetine was increased to 20 mg, the attacks were further reduced to fewer than one per month, and she has remained on this regimen. Since our patient could deal with her sleepiness by taking naps, she did not require medication to promote wakefulness.

Cataplexy is one of the classic tetrad of symptoms found in patients with narcolepsy; the remaining symptoms are excessive daytime sleepiness, hypnagogic hallucinations, and sleep paralysis. Individuals with cataplexy display transient, typically bilateral muscle weakness, most often provoked by strong emotion, and with consciousness fully preserved.

1 The most common triggers of cataplexy are laughter (92 % of patients), anger (70 %), and startle reaction (55 %).2 Muscular weakness that resolves immediately after an attack is a cardinal feature of cataplexy, and often affects the jaw or face rather than the lower limbs.

2 Jerking or twitching of various body parts, including the arms and face, can also occur.2

The differential diagnosis of paroxysmal weakness depends on the pattern of symptoms. Transient ischemic attacks and seizures are the major causes of unilateral paroxysmal weakness, followed by hemiplegic migraine and hypoglycemia.

In contrast, bilateral paroxysmal weakness suggests orthostatic hypotension, cardiogenic cerebral ischemia, hypoglycemia, or seizures, most of which are associated with alteration of consciousness.

Paroxysmal bilateral weakness without disturbance of consciousness which occurred in our patient suggests periodic paralysis (which lasts for several hours) or cataplexy (which resolves within 2 minutes).

Although our patient had consulted numerous physicians over a 15-year period, and some had witnessed her attacks, her narcolepsy with cataplexy remained undiagnosed, reflecting the difficulty in recognizing this condition. One of the reasons for delayed diagnosis may be that narcolepsy is uncommon, with a prevalence of 0.16 % in Japan and 0.02 % to 0.05 % in Europe.

3 Because the majority of patients tend to develop symptoms in the second decade of life,4 patients who have later onset, such as our patient, may elude diagnosis. Another barrier to diagnosing narcolepsy in our patient was that, while she experienced attacks when startled, she had never experienced an episode when laughing, which is the most common trigger of cataplexy.

Table 1 Diagnostic criteria for narcolepsy

The Table 1 shows the simplified diagnostic criteria for narcolepsy from ICSD-2.5 If a patient has a history of cataplexy and daytime sleepiness, a definite diagnosis of narcolepsy with cataplexy can be made by polysomnography and a multiple sleep latency test (MSLT) or by measuring the CSF level of orexin/hypocretin.

Polysomnography is recommended for differentiating narcolepsy from other disorders causing daytime sleepiness or to confirm the coexistence of multiple disorders, as narcolepsy often coexists with other sleep disorders, including obstructive sleep apnea syndrome, periodic limb movements in sleep, REM sleep behavior disorder, and nocturnal eating disorder.

3 The detection of at least two SOREMPs during an MSLT is a useful criterion for confirming a diagnosis of narcolepsy with cataplexy. In elderly populations, however, false-negative MSLT results occur more frequently.6 In addition, the results of one community-based study revealed that false-positive rates would be high bacause 13.1 % of men and 5.6 % in women met the criterion.

7 As such, caution should be exercised in the interpretation of positive MSLT scores.

Orexin A and B (also called hypocretin 1 and 2) play an important role in narcolepsy with cataplexy. These two neurotransmitters are released from the dorsolateral hypothalamus and are involved in regulating the sleep–wake cycle and promoting wakefulness.

8 A deficiency in orexin/hypocretin leads to abnormalities in REM sleep (causing cataplexy, hypnagogic hallucinations, and sleep paralysis) and excessive daytime sleepiness.

8 The measurement of orexin/hypocretin in the CSF is a specific test, as low levels are found in only a few diseases apart from narcolepsy with cataplexy, such as Guillain-Barré syndrome, Ma2-positive paraneoplastic syndrome, and multiple sclerosis,9 which are easily distinguishable their clinical manifestations. CSF levels of orexin/hypocretin are low (

Source: https://link.springer.com/article/10.1007/s11606-015-3277-7

What is Narcolepsy? Symptoms, Treatment, Cataplexy, Causes & Drugs

Narcolepsy and cataplexy

Alerting medications are used for the treatment of excessive daytime sleepiness.

Amphetamines (for example, dextroamphetamine [Dexedrine], methamphetamine hydrochloride [Desoxyn], amphetamine and dextroamphetamine [Adderall]) and methylphenidate (Ritalin) are generalized central nervous system stimulants. These medications are used in narcolepsy to decrease sleepiness and improve alertness.

However, they can also produce undesirable side effects including elevation of blood pressure, nervousness, irritability, and rarely, paranoid reactions. Alerting medications can also lead to drug dependency due to the feeling of euphoria they can cause.

However, drug dependency has rarely been described in individuals with narcolepsy.

Pemoline (Cylert) is used as an alerting medication but it is less effective than traditional stimulants. This drug has the potential risk of toxic side effects on the liver and liver blood tests need to be monitored frequently.

Modafinil (Provigil) has alerting effects similar to those of the traditional stimulant. Modafinil is not a general CNS stimulant amphetamines, but the precise way it works is unknown.

This drug has a much lower risk for high blood pressure and mental side effects because it acts in a different way than classic stimulants. It does not have significant effects on the sympathetic nervous system and does not cause mood changes, euphoria, or dependence.

Furthermore, modafinil does not become ineffective with prolonged use. Headache and nausea are the most commonly reported side effects, and they are usually mild and temporary.

These side effects can be reduced by a slow increase from a low initial dose up to the desired dose. This medication does not affect cataplexy and other REM sleep symptoms.

Modafinil is usually used in a single daily dose. Switching patients from amphetamines to modafinil may cause the reappearance of cataplexy in patients previously well controlled. Increasing the dose or adding an anti-cataplectic medication usually solves this problem.

Armodafinil (Nuvigil) is an oral drug used to promote wakefulness. It is similar to modafinil (Provigil).Armodafinil promotes wakefulness by stimulating the brain; however, the exact mechanism of action of armodafinil is unknown.

Armodafinil may work by increasing the amount of dopamine (a chemical neurotransmitter that nerves use to communicate with each other) in the brain by reducing the reuptake of dopamine into nerves. The most common side effect of ararmodafinil is headache.

Other side effects including anxiety, dizziness, diarrhea, dry mouth, insomnia, nausea, fatigue, and rash may occur.The drug is recommended for single daily dosing, either in the morning, or one hour prior to a work shift.

Monoamine oxidase inhibitors (MAOIs): A class of antidepressants called monoamine oxidase inhibitors (MAOIs) can also be used for treatment of excessive daytime sleepiness. This includes phenelzine (Nardil) and selegiline (Eldepryl).

Anticataplectic medication is the general name for drugs that are used to treat cataplexy. These drugs may also be used for the other REM related symptoms, such as hypnagogic hallucinations and sleep paralysis.

Tricyclic antidepressants (TCAs), used in lower than antidepressant doses, are often effective in controlling cataplexy.

These medications act on neurotransmitter systems to produce suppression of REM sleep and consequently improve the symptoms of cataplexy.

In some cases, the side effects may limit the use of TCAs, although in most cases the side effects are temporary. The most frequent side effects are called “anticholinergic side effects,” including dry mouth, dry eyes, blurred vision, urine retention, constipation, impotence, increased appetite, drowsiness, nervousness, confusion, restlessness, and headache.

Some of the TCAs may increase periodic limb movements in sleep, which could further disrupt already disturbed nighttime sleep in narcoleptic patients. If TCAs are abruptly discontinued, a significant worsening of the cataplexy and other REM related symptoms could occur.

This “rebound phenomenon” may appear in 72 hours after discontinuation of the medication and peak in approximately 10 days from the withdrawal.

The most frequently used TCAs for the treatment of cataplexy and other REM related symptoms are protriptyline (Vivactil), imipramine (Tofranil), clomipramine (Anafranil), desipramine (Norpramine), and amitriptyline (Elavil). Sedating TCAs such as clomipramine, amitriptyline, and imipramine, are usually prescribed for evening use, whereas the alerting ones (protriptyline and desipramine) are recommended for use during the day.

Selective serotonin reuptake inhibitors (SSRIs) are also useful in treating cataplexy at doses that are comparable to those used to treat depression.

The most frequently used SSRIs for treatment of cataplexy and REM related symptoms are fluoxetine (Prozac), paroxetine (Paxil), sertraline (Zoloft), citalopram (Celexa), and venlafaxine (Effexor). The SSRIs may not be as effective as the TCAs, but they have fewer side effects.

The most frequently reported side effects are dizziness, lightheadedness, nausea, and mild tremor. Rarely, mild constipation or diarrhea may occur. Fluoxetine (Prozac) given late in the day may cause insomnia.

Sodium oxybate (Xyrem), also known as gamma-hydroxybutyrate or GHB, is approved by the FDA to treat cataplexy and excessive daytime sleepiness (EDS). This drug is usually administered in two doses; the first is given at bedtime and the second four hours later.

It unifies sleep and improves the disturbed nighttime sleep characteristic of narcolepsy. This nighttime benefit may help decrease daytime drowsiness and cataplexy. Sodium oxybate is unrelated to drugs that are known to be sleep-inducing (hypnotic) and is not used for insomnia.

It can cause drowsiness and should only be taken at night.

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Insomnia is a condition characterized by difficulty falling or staying asleep. There is no set definition of insomnia in terms of hours of sleep, and insomnia can have many forms.

Some people with insomnia may have no trouble falling asleep but wake up too soon. Other people may have the opposite problem, or they have problems with falling asleep as well as staying asleep.

The common factor is poor-quality sleep that doesn't leave you feeling refreshed when you wake up.

Temporary insomnia lasts anywhere from one night to a few weeks. This can involve a single episode of poor-quality or unrefreshing sleep or recurring episodes of insomnia separated by periods of normal sleep.

On the following slides, we offer some suggestions and tips intended to help overcome temporary insomnia and maximize your chance for getting a healthy night's sleep:

Keep the room pleasant, comfortable, and get rid of clutter and distractions. Be sure to select the right bed and mattress for your needs. An old mattress or the wrong mattress for you can contribute to musculoskeletal problems and sleep disturbances.

Avoid use of the bed for TV, working, eating, or any other activities; use the bed only for sleeping and sex. If you to use the bed for a bit of nighttime reading, read only books in bed that promote relaxation and enjoyment.

“Reconditioning” is often recommended as part of the treatment plan for insomnia. This means that you are “reconditioned” to associate the bed with sleep. If you are not able to sleep at all, get bed and move to another room, so that you do not associate the bed with wakefulness.

Typically, if you are not sleeping after 20-30 minutes in bed, you should get bed and return when you are tired. During the time bed, you should not do anything that may stimulate or increase your wakefulness and you should avoid turning on the TV, computer, cell phone, or bright lights and avoid looking at the clock.

Return to bed when you feel drowsy.

Establishing a regular sleep-wake cycle can help people who suffer from insomnia. By doing so, the body will learn to set its internal clock to your schedule, eventually responding to internal cues to become sleepy at a given time and to awaken at a given time. Getting up at the same time every morning, even on weekends, is a good way to establish this regular cycle.

An afternoon nap can make falling asleep at night even harder, no matter how tired you may be. “Extra” sleep on weekends can also throw off your sleep schedule and make midweek insomnia even worse. Naps in the afternoon should be limited and short (around 20 minutes).

Limit your consumption of caffeine in the afternoon and evening. Don’t forget that chocolate, hot cocoa, and colas also are sources of caffeine.

Excessive consumption of of alcohol at any time in the day can also disrupt sleep patterns and lead to unsatisfying sleep. Don't drink any alcoholic beverages in the few hours prior to going to bed. Cigarette smoking can also worsen insomnia.

Try to fit in some exercise during the day, but avoid strenuous exercise right before bedtime. Exercise 4-5 hours before bed is preferred.

Heavy eating in the evening or eating just prior to bedtime can disrupt your sleep.

It can be helpful to establish a “winding down” ritual just prior to bedtime. The goal is to free your mind of distracting or troublesome thoughts and engage in a relaxing, enjoyable activity reading, watching a pleasant film, or listening to music.

Source: https://www.medicinenet.com/narcolepsy/article.htm

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