seizure disorder



Overview

What is Episodic Sleep Disorder?

Definition

Episodic sleep disorder is a neurologic disorder with uncontrollable daytime sleepiness as the main clinical symptom and is a chronic sleep disorder of unknown cause. Patients often have significant functional impairment that interferes with daily life.

Typical episodic sleep disorder is a tetralogy of symptoms, i.e. daytime sleepiness, sudden collapse, hallucinations and sleep paralysis symptoms. Most patients do not have all four symptoms at the same time, and sometimes they may be accompanied by other symptoms, such as sleep disturbances, daytime naps, unconscious behaviors, and decreased academic performance.

Typing

According to the International Classification of Sleep Disorders, 3rd edition (ICSD-3) released in 2014, episodic sleep disorder can be categorized into two types:

Episodic sleep disorder type 1: This is the syndrome of secretin (Hcrt) deficiency in the lateral hypothalamic region, previously known as sudden-onset episodic sleep disorder, which is characterized by a significant decrease in the level of Hcrt-1 in the cerebrospinal fluid.

Episodic somnambulism type 2: previously known as non-sudden onset episodic somnambulism, usually characterized by no significant decrease in cerebrospinal fluid levels of Hcrt-1.

Morbidity

The global prevalence of sudden onset episodic somnolence is 0.02% to 0.18%, and the prevalence in China is about 0.033%, i.e., 3 per 1,000 people suffer from this disease in China.

The disease usually starts at the age of 10 to 30 years, with the peak age of onset being 8 to 12 years, and is rare before the age of 5 years.

Most reports say that the proportion of males with the disease is slightly higher than that of females, and the ratio of males to females is about 2:1.

Questions you may be concerned about

What is the treatment for episodic sleeping sickness?

The treatment of episodic sleep disorder is to use psychotherapy to intervene promptly and effectively with psychological symptoms, and medication to intervene with daytime sleepiness, episodes of sudden collapse, and nocturnal sleep disturbances.

At the same time, regular and sufficient night sleep should be maintained; daytime naps (naps) should be arranged in a planned manner; occupational choices should be avoided and removed from dangerous environments, such as avoiding driving, working at heights or underwater to avoid accidents.

What are the symptoms of episodic sleeping sickness?

The four clinical signs of episodic sleep disorder are drowsiness attacks, sudden collapse, sleep paralysis and hallucinations before sleep.

Most patients with episodic sleep disorder have sudden daytime sleep episodes, such as recurrent episodes of multiple naps and naps with irresistible daytime sleepiness. Patients may also experience paralysis (i.e., sudden collapse). Patients are prone to sleep paralysis (a symptom of being conscious but unable to move their limbs) when they first go to sleep or when they first wake up, and they may also experience hallucinations before falling asleep.

Can episodic sleep disorder be cured?

Clinical studies have shown that medication and psychotherapy can improve the symptoms of episodic somnambulism, but there is no cure.

Most of the disease lasts for life, and some patients may experience a gradual decrease in symptoms with age. Patients are advised to take care of their sleep and diet, avoid any form of shift work, and take two or more regular naps during the day to help maintain arousal and improve daytime functioning.

Symptoms

Main Symptoms

Episodes of drowsiness

Most patients with episodic somnolence have sudden episodes of daytime sleepiness, which are characterized by recurrent episodes of multiple dozing, napping, or short sleep intervals (often less than 1 hour). They tend to be most pronounced in monotonous, static situations and often develop insidiously, leading to irresistible daytime drowsiness.

The patient is often in a state of low arousal, aggravated in the afternoon, especially after meals or in warm environments, and each episode lasts at least a few seconds to a few hours, usually ten minutes to wake up, and can occur several times a day. Sometimes the patient tries to stay awake by forcing himself to overcome the drowsiness and trying to concentrate, but in the end, he often fails to counteract the recurrent sleep episodes that continue to occur.

Some of the disease’s sleep can be relieved by movement or emotional fluctuations, however, unlike normal sleep when a person is fatigued, it cannot be completely relieved by adequate sleep.

Sometimes patients report that they are unaware that they ever fall asleep after a brief sleep episode or confuse dreams for facts. The manifestation of refreshing after a short nap (10-30 minutes) during the hypersomnia cycle is more specific than in other daytime hypersomnia disorders, and this doubling of spirituality after a nap is of great diagnostic value.

Patients with episodic sleeping sickness are often associated with learning problems and impaired concentration, but neuropsychological testing is generally normal.

Sudden collapse disorder

Sudden collapse is a characteristic symptom of episodic sleeping sickness and its diagnostic importance in episodic sleeping sickness has long been recognized. Sudden collapse is defined as a brief sudden onset of muscle weakness in which the patient becomes paralyzed. Sudden collapse is most often triggered by emotional factors, mostly euphoric emotions such as laughter, and to a lesser extent, depressed emotions such as anger.

The patient is conscious at the onset of the attack, but may have blurred vision or ptosis, accompanied by slurred speech and muteness. In some patients, episodes of muscle weakness may last for several minutes.

Sudden collapse episodes in episodic sleep disorder often occur months to years after the onset of excessive daytime sleepiness (EDS).

Sleep paralysis

Sleep paralysis symptoms account for 20% to 50% of all patients and are usually accompanied by pre-sleep hallucinations. Sleep paralysis is defined as a brief inability to perform any activity that lasts for a few seconds or minutes just after sleep begins or ends, with the patient being conscious but unable to move his or her limbs.

Such episodes can usually be terminated by a mild stimulus, such as when someone else touches the limb or speaks to the patient. In a few cases, the seizure may last for several minutes after the patient is awake and struggling.

The symptom is often fearful, especially when it first occurs. This fearful experience is often intensified by the fact that sleep paralysis often occurs in conjunction with pre-sleep hallucinations.

Pre-sleeping hallucinations

Patients with episodic sleeping sickness often experience abnormal auditory or visual perceptions during sleep or upon waking, i.e., hallucinations that occur between sleep and wakefulness. These hallucinatory experiences are often unpleasant and are typically accompanied by feelings of fear and threat.

The patient’s hallucinations usually contain visual, auditory, and tactile components, and are often complex, vivid, and dream-like experiences, which may also be accompanied by episodes of sudden collapse and sleep paralysis, but awareness of the external environment is usually present.

Other symptoms

Between 36% and 63% of patients with episodic narcolepsy may develop automatic behaviors, which are aimless, monotonous, repetitive movements that occur when the patient appears to be awake.

Other symptoms may include disturbed sleep at night and memory loss.

Accompanying Symptoms

The disease may be accompanied by precocious puberty, obstructive sleep apnea syndrome, behavioral disorders during rapid eye movement sleep, anxiety or depression, and migraine headaches.

Obesity may occur. Patients with episodic sleeping sickness are more likely to be overweight, and weight gain may be associated with low metabolism.

Etiology

Genetic factors

There is a correlation between the development of the disease and genetic factors. One study has shown that the risk of sudden collapse in first-degree relatives of patients with episodic narcolepsy is 1% to 2%, which is 10 to 40 times higher than that of the same population.

Biological factors

Numerous studies have found a close relationship between human leukocyte antigens (HLA) and episodic sleeping sickness, which is associated with an increased susceptibility to episodic sleeping sickness.

Loss of secretin neuron specificity in the lateral hypothalamic area is a characteristic pathological change of the disease and is associated with polygenic susceptibility, environmental factors and immune response.

Insufficient or absent secretin secretion in the hypothalamus is responsible for triggering episodic somnambulism type 1.

Episodic sleeping sickness can be caused by abnormal expression of tumor necrosis factor.

A central nervous system inflammatory response may also cause episodic somnolence.

Psychosocial factors

Too much stress at work during the day, poor psychological resilience, and poor sleep quality at night predispose to the disease.

Intense psychological stress may contribute to the early onset of the disease.

Risk factors

Known risk factors for episodic sleep disorder include:

Age: Episodic sleep disorder usually begins in people between the ages of 10 and 30.

Family history: If a family member has episodic sleep disorder, the risk of developing episodic sleep disorder is 10 to 40 times higher.

Head trauma: Trauma to the head is more likely to cause episodic sleep disorder than in the normal population.

Sudden changes in sleeping habits: Sudden changes in sleeping habits may be one of the factors affecting the development of episodic sleep disorder.

Various infections: Exposure to infections may be associated with the development of episodic sleep disorder.

Consultation

Department of Medicine

Psychiatry

If you experience excessive daytime sleepiness that occurs almost every day and lasts for at least 3 months, have muscle weakness triggered by emotional excitement, have hallucinations and sudden collapse before going to sleep, please consult a doctor promptly.

Sleep specialists

You can also visit a sleep specialist, sleep center, or sleep clinic if you experience any of the above symptoms.

Preparation for medical treatment

Preparing for the consultation: registration, preparation of documents, and frequently asked questions

Tips for medical treatment

Seizure sleep disorder can be harmful to your health if not diagnosed and treated in a timely manner. It is recommended that you consult a doctor as soon as possible for regular treatment of the symptoms, and do not use medication on your own.

Special reminder: the cause of episodic somnolence may be mental and psychological factors, it is recommended to get enough rest before seeking medical treatment to help maintain a stable mental state.

Preparation List

Symptom list

Particular attention should be paid to the time of onset of symptoms, special manifestations, etc.

Is there excessive daytime sleepiness, occurring almost daily?

Is there any muscle weakness triggered by emotional excitement?

Is there fatigue or general malaise, loss of concentration or memory during the day?

Are there hallucinations and sudden collapses before falling asleep?

Medical History Checklist

Any blood relatives with psychosomatic disorders?

Have you ever suffered from any psychosomatic disorder?

Any previous visits to other hospitals and what was the diagnosis?

Checklist

Test results in the last six months, which can be brought to the doctor’s office

Sleep examination: polysomnography, sleep electroencephalography

Imaging examination: brain CT examination, magnetic resonance imaging examination, ultrasound examination

Other tests: Psychological examination

Medication List

Medications used in the last 3 months, if available, bring the box or package with you to the doctor’s office.

Benzodiazepine sedative-hypnotics: diazepam, lorazepam, clonazepam, eszopiclone

Non-benzodiazepine sedative-hypnotics: dexzopiclone, zopiclone, zolpidem, zaleplon

Sedative antidepressants: trazodone, mirtazapine, fluvoxamine, doxepin, amitriptyline

Melatonin receptor agonists: melatonin extended-release tablets, ramelteon

Diagnosis

Diagnostic basis

The examination and evaluation of episodic sleep disorder consists of the following four areas:

Neurophysiologic examination.

Cerebrospinal fluid Hcrt-1 testing.

Genetic subtype testing.

Scale assessment.

A comprehensive neurophysiologic examination includes standard nPSG monitoring in the sleep laboratory and MSLT during the daytime on the following day. nPSG and MSLT are optional for the diagnosis of sudden onset episodic sleepiness and necessary for the diagnosis of non-sudden onset episodic sleepiness.

If your doctor suspects episodic sleep disorder, the following tests may be performed:

Multiple sleep latency test: utilizing polysomnography to test the patient’s sleep multiple times following standard procedures.

Wakefulness maintenance test: to assess the patient’s ability to remain awake during the day in an environment with minimal sensory stimulation.

Polysomnography: Simultaneous video monitoring of the patient’s electroencephalogram (EEG), electrooculogram (EEG), electromyogram (EMG), nasal pressure and oro-nasal thermal sensors to monitor respiratory airflow, snoring, chest and abdominal movements, finger-prick pulse oximetry, electrocardiogram (ECG), and limb electromyogram (EMG).

Human leukocyte antigen (HLA) typing.

Magnetic resonance imaging: to rule out possible organic pathology.

Mood assessment.

Cognitive function assessment.

Medical diagnostic criteria

International Classification of Diseases, 10th edition (ICD-10) diagnostic criteria for “non-organic narcolepsy disorder”.

Excessive daytime sleepiness or sleep episodes that cannot be explained by insufficient sleep duration; and/or prolonged transition time to a fully awakened state while awake.

Daily sleep disturbances for more than 1 month or recurrent brief episodes that cause significant distress or interfere with social or occupational functioning.

Lack of additional symptoms of episodic sleep disorder (sudden collapse, pre-sleep hallucinations, sleep paralysis) or clinical evidence of sleep apnea (nocturnal apnea, typical intermittent snoring, etc.).

Absence of any neurologic and medical conditions that could manifest daytime sleepiness symptoms.

International Classification of Sleep Disorders, 3rd edition (ICSD-3) diagnostic criteria for episodic sleep disorder

Diagnostic Criteria for Episodic Sleeping Disorder Type 1

Episodic Sleep Disorder Type 1 needs to be met simultaneously:

The patient has episodes of uncontrollable daytime sleepiness and sleepiness, with symptoms lasting for at least 3 months.

1 or 2 of the following conditions are met:

There are episodes of sudden collapse (meeting the essential features of the definition). Have a mean sleep latency of ≤8 min after a standard multiple sleep latency test (MSLT) examination and have ≥2 sleep onset REM sleep phenomena (SOREMPs). Nocturnal polysomnography (PSG) is recommended prior to the MSLT test.The presence of sleep onset REM sleep phenomena on PSG can be substituted for 1 sleep onset REM sleep phenomena on daytime MSLT.

Hcrt-1 concentration in cerebrospinal fluid ≤110 pg/ml or <1/3 of the normal reference value was detected by immunoreactivity assay.

Diagnostic criteria for episodic somnambulism type 2

Episodic somnambulism type 2 needs to be fulfilled simultaneously:

The patient has uncontrollable daytime sleepiness and sleep episodes with symptoms lasting for at least 3 months.

Mean sleep latency on standard MSLT examination ≤ 8 minutes and ≥ 2 SOREMPs. nPSG is recommended prior to the MSLT examination, and the presence of sleep onset REM sleep phenomena on nPSG can be substituted for sleep onset REM sleep phenomena on 1 daytime MSLT.

There were no sudden collapse episodes.

Hcrt-1 concentration in cerebrospinal fluid was not tested, or immunoreactive assay measurements were >110 pg/ml or greater than 1/3 of the normal reference value.

Narcolepsy symptoms and/or MSLT results cannot be explained by other sleep disorders such as sleep deprivation, obstructive sleep apnea syndrome (OSAS), delayed sleep phase disorders, substance use, or withdrawal.

Diagnostic Criteria for Episodic Sleep Disorder in the United States Diagnostic Manual of Mental Disorders, 5th Edition (DSM-5)

Diagnostic Criteria

A. Repeated irresistible need to sleep, fall asleep, or nod off in the same day. It must have occurred at least 3 times per week during the past 3 months.

B. Presence of at least 1 of the following symptoms:

Sudden collapse episodes, defined as (a) or (b) below, occurring at least a few times per month.

a. Brief (seconds to minutes) episodes of bilateral loss of muscle tone, but maintained wakefulness, that can be induced by laughing or joking, in individuals with a long-term illness.

b. Spontaneous episodes of grimacing or jaw detachment with tongue spitting or generalized hypotonia in children or individuals within 6 months of onset, without any apparent emotional trigger.

Hypothalamic secretin deficiency, measured using cerebrospinal fluid (CSF) with hypothalamic secretin-1 immunoreactivity values (less than or equal to one-third of the value in healthy subjects or less than or equal to 110 pg/mL using the same assay). Low levels of hypothalamic secretin-1 tested in cerebrospinal fluid were not observed in the context of acute brain injury, inflammatory reactions, or infections.

Nocturnal polysomnography presenting a rapid eye movement (REM) sleep latency of less than or equal to 15 minutes, or multiple sleep latency tests showing an average sleep latency of less than or equal to 8 minutes, and 2 or more sleep onset REM periods.

Severity Classification

Mild: infrequent sudden falls (less than 1 per week) with only 1 or 2 snoozes per day and lesser disruption of nighttime sleep.

Moderate: 1 sudden collapse per day or several days, requiring multiple snoozes per day, interfering with nighttime sleep.

Severe: multiple drug-resistant sudden collapse episodes per day with almost constant drowsiness that interferes with nighttime sleep.

Differential Diagnosis

The diagnosis of episodic sleeping sickness needs to be differentiated from the following disorders:

Idiopathic hypersomnia

Often lacks the manifestations associated with rapid eye movement sleep, such as episodic sudden collapse, sleep paralysis, and pre-sleep hallucinations, and does not have the multiple nap latency test manifestations of episodic sleeping sickness.

Kleine-Levin syndrome

An unexplained form of adolescent narcolepsy bulimia. Periodic episodes of excessive sleepiness, excessive sleep duration, which may last from several days to several weeks, are often accompanied by psychiatric symptoms such as awakening euphoria, restlessness, and impulsivity; accompanied by hunger and hyperphagia. Seizures can be up to 3 to 4 times per year, starting mostly at the age of 10 to 20 years, more often in males, and can be self-resolving in adulthood.

Complex partial seizures

Since automatic behavior and forgetfulness can be present in about 50% of patients with episodic sleeping sickness, it can be easily misdiagnosed as epilepsy. Epilepsy does not have uncontrollable sleep and sudden collapse seizures, and polysomnography facilitates identification.

Secondary episodic somnolence

It needs to be differentiated from secondary episodic somnolence, such as hypoglycemic responsive episodic somnolence, hypokalemic episodic somnolence, and episodic somnolence due to brainstem tumor.

Self-assessment scale

The most commonly used scales for clinical assessment of excessive daytime sleepiness are:

Epworth Sleepiness Scale (ESS)

The Epworth Sleepiness Scale is also known as the Epworth Daytime Hypersomnia Scale. It is used to assess excessive daytime sleepiness. Sleepiness can be assessed semi-objectively with this scale. There are a total of 24 points, of which a score of >6 indicates drowsiness; a score of >11 indicates excessive drowsiness; and a score of >16 indicates dangerous drowsiness.

If a patient presenting to the hospital has unexplained drowsiness or fatigue, he or she should go to a sleep specialist or neurological, respiratory, or psychiatric department for further examination to clarify the diagnosis and treatment measures. However, changing jobs and not getting enough total sleep for any reason can also affect this score.

Stanford Sleepiness Scale (SSS)

The Stanford Sleepiness Scale (SSS) is a quick and easy tool for assessing alertness by recording “sleepiness” at various times throughout the day to detect patterns of alertness in patients. The scale is divided into 7-point scales, which are rated by the patient according to his/her own perception.

It is simple to administer and can be repeated. Studies have shown that higher scores on the scale indicate a more severe form of the disease, and the SSS can be used for initial diagnosis, as well as for further diagnosis of the patient.

Treatment

Medication

Medications for the treatment of episodic sleep disorder include 3 main areas: central stimulants to treat daytime sleepiness, antidepressants to improve symptoms of sudden collapse, and sedative-hypnotic medications to treat nocturnal sleep disturbances.

Central stimulants

New type of central stimulants

They mainly act on catecholaminergic neurons in the posterior hypothalamus that are involved in the arousal mechanism to prevent sleep.

The drug does not cause hyperexcitability or rebound hypersomnia, so it does not interfere with nighttime sleep and does not produce tolerance.

If a rash develops, the drug should be discontinued immediately; avoid alcohol during use.

Traditional central stimulants

Representative drugs: Methylphenidate, Amphetamine, Sildenafil and so on.

These drugs can promote the release of presynaptic monoamine transmitters and inhibit reuptake, long-term application is easy to produce drug tolerance and addiction, need to pay attention.

Antidepressants

Selective 5-hydroxytryptamine and norepinephrine reuptake inhibitors (SNRI) such as venlafaxine, and selective norepinephrine reuptake inhibitors (NERIs) such as reboxetine, have a certain arousal-promoting effect.

Treatment of sudden collapse is rapidly effective, but symptom rebound occurs after discontinuation of the drug.

Sedative-hypnotic drugs

Short half-life benzodiazepines can be used.

Medication reminder: psychotropic drugs are prescription drugs and need to be purchased with a doctor’s prescription and should be used strictly according to medical advice. Do not engage in driving, operating dangerous machinery or precision instruments during the use of medication to avoid bodily harm.

Psychotherapy

Psychotherapy mainly refers to treatment through conversation and communication, using different treatment methods for different problems. For patients with psychosocial stress factors, it is often necessary to combine psychotherapy with drug treatment.

Supportive Psychotherapy

Through listening, comforting, explaining, guiding and encouraging, etc. to help patients correctly recognize and treat their own disease, so that patients can actively cooperate with the treatment. It is usually carried out by doctors or other professionals. Treatment focuses on:

Active listening.

Guiding the release of emotions.

Disease health education.

Encouragement to increase confidence.

Cognitive-behavioral intervention therapy

Research has shown that cognitive-behavioral interventions can be effective in improving sleep and improving symptoms by changing irrational perceptions of sleep and correcting faulty sleep habits. Methods such as:

Stimulus control therapy: based on the principle of conditioned reflex, instruct patients to establish correct reflex links between sleep and bed and bedroom environment, and establish stable sleep-wake patterns.

Sleep restriction therapy: Reduce the time of waking up in bed at night, and at the same time prohibit daytime napping, so that the bedtime is as close as possible to the actual sleep time.

Relaxation training.

Re-establish sleep-related beliefs.

Sleep hygiene education: Reduce or eliminate various conditions that interfere with sleep through guidance on sleep habits and sleep hygiene knowledge in order to improve sleep quality.

Traditional Chinese Medicine (TCM) Treatment

Chinese medicine is rich in evidence-based treatments for sleep disorders. In recent years, many practitioners have explored the mechanism of its occurrence from the clinical reality and proposed new ideas of evidence-based treatments. However, there is no clinically proven effective treatment for episodic sleep disorder, and some Chinese medicines have a regulating effect, which needs to be treated under the guidance of doctors.

Prognosis

Cure

Most of the disease lasts for life, and some patients’ symptoms may gradually decrease with age.

Clinical studies have shown that medication and psychotherapy can improve symptoms, and there is currently no complete cure.

Hazards

Because episodic sleeping sickness often begins in childhood or adolescence and continues throughout life, it may cause serious functional impairment and disability.

Sudden episodes of sleep may cause the individual to become unalert in an unsafe environment.

Sleep attacks may cause physical harm to the person with episodic narcolepsy, and a car accident is more likely to occur if the attack occurs while driving. There is a greater risk of cuts and burns if they are permitted to fall asleep while cooking.

People with episodic narcolepsy are more likely to be overweight and obese.

Daily

Personal Care

Psychological care

Relax yourself, relieve stress and develop more hobbies and interests.

Pay attention to mood changes in daily life, take measures to control stress and enhance psychological resilience.

Seek support from family and friends when encountering stressful events, or seek help from a counselor.

Life Care

Avoid any form of shift work.

Take 2 or more regular naps during the day to help maintain arousal and improve daytime functioning.

Avoid and get out of dangerous environment to avoid accidents.

Maintain a calm mind, combine work and rest, and exercise appropriately.

Sleep and diet care

Pay attention to dietary hygiene and nutritional balance.

Avoid overeating.

Eat more grains and vegetables.

Avoid excessive consumption of highly carbonated beverages such as Sprite and Coke.

Do not eat spicy and cold food to avoid gastrointestinal discomfort.

Avoid smoking and alcohol, and avoid intake of strong tea, coffee and other foods that have an effect on sleep.

Reasonable dietary arrangement, avoid over-eating or not eating, and adjust the dietary structure reasonably.

Adopt good habits of work and rest, and arrange rest time reasonably.

Special precautions

Take medication as prescribed by the doctor, do not increase or decrease medication or stop medication at will.

Observe and test your condition and changes in feeling, communicate with your doctor anytime you feel unwell, and fully cooperate with your doctor’s treatment.

Home Care

Effective treatment of episodic sleeping sickness requires regular and structured night sleep and regular daytime naps. Encourage patients to sleep 8 hours or more a day at night, and regulate the sleeping time and waking time.

Maintain a good state of mind. Family members should be more understanding and comforting to help patients build up confidence in overcoming the disease.

Help patients to adjust their diet and reduce the intake of excitatory foods, such as coffee and strong tea. Those who are weak after suffering from somatic diseases are prone to this disease. Those who are anemic due to blood loss should be given better nutrition.

Prevention

Generally difficult to prevent, especially difficult to recognize in children patients.

Regulate your emotions A balance of work, life, and recreation, and regulation of one’s emotions can reduce the occurrence of episodic sleeping sickness.

Enhance physical fitness during the winter months to prevent the onset of colds and flu.

Start with environmental factors and avoid more stressful jobs and any kind of shift work.