Primary Hypersomnia

Sleep disorders

Clinicals - History

Fact Explanation
Excessive sleepiness. Prolonged sleep episodes or daytime sleepiness occurring daily at least for period of 1 month. Excessive sleepiness.
Prolonged sleep episodes or daytime sleepiness occurring daily at least for period of 1 month.
Excessive sleepiness cause clinically significant distress to the person's life It may Impair work performance and even be involved In accidents at work or while driving, impairs social functioning. Excessive sleepiness cause clinically significant distress to the person's life
It may Impair work performance and even be involved In accidents at work or while driving, impairs social functioning.
Symptoms of another sleep disorder Other sleep disorders includes, narcolepsy, breathing related sleep disorder, circadian rhythm sleep disorder, parasomnia. If other sleep disorders present with hypersomnia, they should be treated first before the diagnosis of hypersomnia is made. Symptoms of another sleep disorder
Other sleep disorders includes, narcolepsy, breathing related sleep disorder, circadian rhythm sleep disorder, parasomnia. If other sleep disorders present with hypersomnia, they should be treated first before the diagnosis of hypersomnia is made.
Symptoms of a depressive disorder Sleep disturbance commonly occurs in major depressive disorder and causes excessive day time sleepiness. Symptoms of a depressive disorder
Sleep disturbance commonly occurs in major depressive disorder and causes excessive day time sleepiness.
Sleepiness is not due to a medical disorder or due to effects of a substance Respiratory causes as obstructive sleep apnea, and structural lesions such as stroke or head injury, endocrine causes like hypothyroidism, metabolic derangements, can cause hypersomnia. Alcohol abuse, withdrawal from cocaine, caffeine can also cause excessive sleepiness. Sleepiness is not due to a medical disorder or due to effects of a substance
Respiratory causes as obstructive sleep apnea, and structural lesions such as stroke or head injury, endocrine causes like hypothyroidism, metabolic derangements, can cause hypersomnia. Alcohol abuse, withdrawal from cocaine, caffeine can also cause excessive sleepiness.
Ask for a family history of sleep disorders. individuals with primary hypersomnia are likely to have other family members affected by primary hypersomnia. Ask for a family history of sleep disorders.
individuals with primary hypersomnia are likely to have other family members affected by primary hypersomnia.
Similar previous episodes, and the duration of those symptoms Periods of excessive sleepiness lasting at least 3 days occurring several times a year at least for 2 years is known as 'recurrent hypersomnia'.
The recurrent form of hypersomnia is also known as Kleine-Levin Syndrome, often accompanies symptoms of megaphagia, hypersexuality and cognitive disturbances.
Similar previous episodes, and the duration of those symptoms
Periods of excessive sleepiness lasting at least 3 days occurring several times a year at least for 2 years is known as 'recurrent hypersomnia'.
The recurrent form of hypersomnia is also known as Kleine-Levin Syndrome, often accompanies symptoms of megaphagia, hypersexuality and cognitive disturbances.

Clinicals - Examination

Fact Explanation
Thorough and complete neurological examination. Structural brain lesions and stoke can cause similar clinical conditions.
Individuals with Kleine-Levin syndrome may show non-specific neurological signs like depressed deep tendon reflexes, dysarthria, nystagmus.
Thorough and complete neurological examination.
Structural brain lesions and stoke can cause similar clinical conditions.
Individuals with Kleine-Levin syndrome may show non-specific neurological signs like depressed deep tendon reflexes, dysarthria, nystagmus.
Respiratory system examination: barrel shape chest, increased resonance on percussion, rhonchi on auscultation. Chronic obstructive pulmonary disease (COPD) can give rise to disturbed nocturnal sleep and cause increase day time sleepiness. Respiratory system examination: barrel shape chest, increased resonance on percussion, rhonchi on auscultation.
Chronic obstructive pulmonary disease (COPD) can give rise to disturbed nocturnal sleep and cause increase day time sleepiness.
Features of hypothyroidism: thyroid goiter, coarse skin, cold intolerance, bradycardia. Hypothyroidism can cause extreme somnolence which can mimic hypersomnia. Features of hypothyroidism: thyroid goiter, coarse skin, cold intolerance, bradycardia.
Hypothyroidism can cause extreme somnolence which can mimic hypersomnia.
Mental state examination (MSE) In patients with hypersomnia alone, MSE should be normal. However, look for sings of depression, and substance abuse. Mental state examination (MSE)
In patients with hypersomnia alone, MSE should be normal. However, look for sings of depression, and substance abuse.
Mental State Examination: Appearance Crumpled clothes, self neglect suggests alcoholism. Exclude co-morbid conditions like depression where vertical furrows in the brow, hunched shoulders, turned down face can be seen. Mental State Examination: Appearance
Crumpled clothes, self neglect suggests alcoholism. Exclude co-morbid conditions like depression where vertical furrows in the brow, hunched shoulders, turned down face can be seen.
Mental State Examination: Speech Normal. May speak slowly if the patient has co morbid depression. Mental State Examination: Speech
Normal. May speak slowly if the patient has co morbid depression.
Mental State Examination: Mood Depressed mood. Mental State Examination: Mood
Depressed mood.
Mental State Examination: Thinking Suicidal thoughts in severe depression. Nihilistic delusions, defamatory or accusatory auditory hallucinations in severe depressive disorder. Mental State Examination: Thinking
Suicidal thoughts in severe depression. Nihilistic delusions, defamatory or accusatory auditory hallucinations in severe depressive disorder.
Mental State Examination: Perception Transient hallucinations of vision and hearing in heavy alcohol drinkers usually during withdrawal. Mental State Examination: Perception
Transient hallucinations of vision and hearing in heavy alcohol drinkers usually during withdrawal.
Mental State Examination: Cognitive functions Attention and concentration and memory can be impaired in depression. Mental State Examination: Cognitive functions
Attention and concentration and memory can be impaired in depression.
Mental State Examination: Insight May be lost in severe depressive disorders otherwise should be good. Mental State Examination: Insight
May be lost in severe depressive disorders otherwise should be good.

Investigations - Diagnosis

Fact Explanation
Brain Imaging (MRI) To exclude strokes, head injury, space occupying lesions in the brain that can give rise to similar symptoms. Brain Imaging (MRI)
To exclude strokes, head injury, space occupying lesions in the brain that can give rise to similar symptoms.
Thyroid function tests (TSH, T4) To exclude hypothyroidism that could be the cause of extreme somnolence. Thyroid function tests (TSH, T4)
To exclude hypothyroidism that could be the cause of extreme somnolence.
Polysomnogram- multiple sleep latency test (MSLT) Objectively measures Extreme daytime somnolence. Mean sleep latency <8 minutes is pathological and <5 minutes is severe. Polysomnogram- multiple sleep latency test (MSLT)
Objectively measures Extreme daytime somnolence. Mean sleep latency <8 minutes is pathological and <5 minutes is severe.
Maintenance Wakefulness Test (MWT) An alternative to the MSLT. The patient sits in bed and attempts to remain awake.Latency <19 minutes is considered as abnormal. Maintenance Wakefulness Test (MWT)
An alternative to the MSLT. The patient sits in bed and attempts to remain awake.Latency <19 minutes is considered as abnormal.

Management - Supportive

Fact Explanation
Advice on lifestyle changes Patient education on avoiding dangerous activities such as driving or operating machinery, and possible dangerous situations and suitable precautions. Advice on lifestyle changes
Patient education on avoiding dangerous activities such as driving or operating machinery, and possible dangerous situations and suitable precautions.
Explanation of the diagnosis and nature of disorder. Explain that the extreme sleepiness is part of the disorder. In patients with Kleine-Levin syndrome (recurrent hypersomnia) explain the behavioral symptoms that can accompany the episodes of hypersomnia. Explanation of the diagnosis and nature of disorder.
Explain that the extreme sleepiness is part of the disorder. In patients with Kleine-Levin syndrome (recurrent hypersomnia) explain the behavioral symptoms that can accompany the episodes of hypersomnia.

Management - Specific

Fact Explanation
Maintaining a regular sleep- wake pattern Relieves daytime drowsiness. The combination of planned daytime naps and maintaining regular nocturnal sleep times produces a significant reduction in the severity of daytime sleepiness in treatment of hypersomnia. Maintaining a regular sleep- wake pattern
Relieves daytime drowsiness. The combination of planned daytime naps and maintaining regular nocturnal sleep times produces a significant reduction in the severity of daytime sleepiness in treatment of hypersomnia.
Advice on regular daytime napping Relieves daytime drowsiness. Advice on regular daytime napping
Relieves daytime drowsiness.
Pharmacotherapy: CNS stimulants Amphetamines are derivatives of catecholamines, that are made more lipophilic so that they enter the central nervous system easily.
Side effects of amphetamines are peripheral release of norepinephrine, resulting in cardiac stimulation and vasoconstriction, Increased heart rate and blood pressure, palpitations, sweating, Increased anxiety in predisposed patients, may precipitate psychosis. Monitoring of pulse and blood pressure needed.
Contra indications to their administration are cardiovascular disease including moderate to severe hypertension, advanced arteriosclerosis, structural cardiac abnormalities.

Methylphenidate is a dopamine transporter (DAT) reuptake inhibitor. It is more lipophilic than Amphetamine with increased central penetration. Contra indications- Anxiety or agitation, severe depression, suicidal ideation, drug or alcohol dependence, psychosis.
Side effects - Abdominal pain, nausea, vomiting, dry mouth, anorexia, palpitations, tachycardia, arrhythmia, tics ( very rarely Tourette syndrome).
Pharmacotherapy: CNS stimulants
Amphetamines are derivatives of catecholamines, that are made more lipophilic so that they enter the central nervous system easily.
Side effects of amphetamines are peripheral release of norepinephrine, resulting in cardiac stimulation and vasoconstriction, Increased heart rate and blood pressure, palpitations, sweating, Increased anxiety in predisposed patients, may precipitate psychosis. Monitoring of pulse and blood pressure needed.
Contra indications to their administration are cardiovascular disease including moderate to severe hypertension, advanced arteriosclerosis, structural cardiac abnormalities.

Methylphenidate is a dopamine transporter (DAT) reuptake inhibitor. It is more lipophilic than Amphetamine with increased central penetration. Contra indications- Anxiety or agitation, severe depression, suicidal ideation, drug or alcohol dependence, psychosis.
Side effects - Abdominal pain, nausea, vomiting, dry mouth, anorexia, palpitations, tachycardia, arrhythmia, tics ( very rarely Tourette syndrome).
Pharmacotherapy: Non amphetamine wakefulness promoting medication Modafinil and Armodafinil.
Increases wakefulness through dopamine reuptake inhibition and also acts as a unique compound in the treatment of excessive daytime sleepiness.
Monitor blood pressure and heart rate in hypertensive patients.
Contra indications- Moderate to severe uncontrolled hypertension, arrhythmia, cor pulmonale.
Side effects- dry mouth, appetite changes, gastro intestinal disturbances,, tachycardia, vasodilatation.
Pharmacotherapy: Non amphetamine wakefulness promoting medication
Modafinil and Armodafinil.
Increases wakefulness through dopamine reuptake inhibition and also acts as a unique compound in the treatment of excessive daytime sleepiness.
Monitor blood pressure and heart rate in hypertensive patients.
Contra indications- Moderate to severe uncontrolled hypertension, arrhythmia, cor pulmonale.
Side effects- dry mouth, appetite changes, gastro intestinal disturbances,, tachycardia, vasodilatation.
Pharmacotherapy: Sodium Oxybate Is a rapidly acting sedative. Reduces daytime somnolence.
May act via GABA-B or specific GHB receptors. Reduces (Dopamine) DA release.
Side effects- nausea, vomiting , abdominal pain, anorexia, hypertension, dizziness, headache.
Contra indications- pregnancy.
Pharmacotherapy: Sodium Oxybate
Is a rapidly acting sedative. Reduces daytime somnolence.
May act via GABA-B or specific GHB receptors. Reduces (Dopamine) DA release.
Side effects- nausea, vomiting , abdominal pain, anorexia, hypertension, dizziness, headache.
Contra indications- pregnancy.
Pharmacotherapy: Tricyclic antidepressants Contra indications- in the immediate recovery period of myocardial infarction, in arrhythmia, in severe liver disease, in the manic period of bipolar disorder.
Side effects- arrhythmia, heart block, postural hypotension.
Pharmacotherapy: Tricyclic antidepressants
Contra indications- in the immediate recovery period of myocardial infarction, in arrhythmia, in severe liver disease, in the manic period of bipolar disorder.
Side effects- arrhythmia, heart block, postural hypotension.

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