Curriculum standards
Knowledge guides
LG14: Central disorders of hypersomnolence
Key presentations and conditions
Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.
Less common or more complex presentations and conditions
Advanced Trainees will understand these presentations and conditions.
Advanced Trainees will understand the resources that should be used to help manage patients with these presentations and conditions.
Epidemiology, pathophysiology, and clinical sciences
Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.
Investigations, procedures, and clinical assessment tools
Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.
Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.
Important specific issues
Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.
Presentations
- Changes to appetite or weight
- Decreased energy
- Difficulty concentrating
- Difficulty waking up from sleep
- Extreme daytime sleepiness (EDS)
- Learning difficulties or behavioural problems
- Memory and speech difficulties
- Mood changes, such as anxiety and irritation
- Paradoxical hyperactivity
- School absenteeism
- Sleeping more than average
Conditions
- Hypersomnolence:
- hypersomnia associated with a psychiatric condition
- hypersomnia due to a medical condition
- idiopathic hypersomnia
- insufficient sleep syndrome
- narcolepsy type 1
- narcolepsy type 2
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- conduct an appropriate examination
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
- establish a differential diagnosis
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- plan and arrange appropriate investigations
- recognise the clinical presentation
- take a comprehensive clinical history
Manage
- advocate and liaise with school authorities
- involve multidisciplinary teams
- prescribe therapies tailored to patients’ needs and conditions
- provide evidence-based management
- recognise potential complications of disease and its management, and initiate preventative strategies
Consider other factors
- consider age-appropriate treatment options based on maturity and mental understanding
- identify individual and social factors and the impact of these on diagnosis and management
Presentations
- Disinhibited behaviours
- Hallucinations
Conditions
- Kleine–Levin Syndrome
- Menstruation-related hypersomnia
For each presentation and condition, Advanced Trainees will know how to:
Synthesise
- conduct an appropriate examination
- consider the impact of illness and disease on patients and their quality of life when developing a management plan
- establish a differential diagnosis
- identify relevant epidemiology, prevalence, pathophysiology, and clinical science
- plan and arrange appropriate investigations
- recognise the clinical presentation
- take a comprehensive clinical history
Manage
- advocate and liaise with school authorities
- involve multidisciplinary teams
- prescribe therapies tailored to patients’ needs and conditions
- provide evidence-based management
- recognise potential complications of disease and its management, and initiate preventative strategies
Consider other factors
- consider age-appropriate treatment options based on maturity and mental understanding
- identify individual and social factors and the impact of these on diagnosis and management
- Behavioural and pharmacological strategies to manage EDS and the daytime consequences of sleep disorders
- Clinical context in which limited channel sleep studies might be useful, including tests performed in the home
- Clinical context in which multiple sleep latency test (MSLT) and maintenance of wakefulness test (MWT) are indicated, and age-related limitations to these
- Common causes of hypersomnia, including:
- behavioural factors
- environmental factors
- medical
- medication use
- primary sleep disorders
- psychiatric conditions
- Common causes of persistent EDS in patients with treated obstructive sleep apnoea (OSA)
- Contents of the International Classification of Sleep Disorders (ICSD-3)
- Criteria for defining the severity of daytime sleepiness or inability to maintain wakefulness
- Daytime neurocognitive consequences of abnormalities of sleep-wake regulation
- Difference between fatigue, lethargy, tiredness, and sleepiness / drowsiness
- Essential features of a sleep study report used in clinical decision making
- Essential features of reports on tests of sleep propensity used in clinical decision making
- Genetics, presentation, and treatment of narcolepsy
- How disease states and medication use can affect sleep-wake regulation and EDS
- Impact of drowsiness / sleepiness on school and home safety
- Impact of MSLT and MWT findings on driving
- Impact of sleep disorders on quality of life and behaviour
- Important behavioural factors that influence subjective and objective sleepiness and neurocognitive function, such as chronic sleep restriction
- Important role of actigraphy and sleep diaries as part of the evaluation
- Indications for and the interpretation of common tests used in the evaluation of EDS and daytime consequences of sleep disorders
- Lifestyle implications of EDS and other sleep disorders
- Limitations of current available tests for assessment of EDS and daytime consequences of sleep disorders, and identify current research developments
- Models of sleep deprivation and sleep disruption, and the effects on daytime function
- Nature of the above tests, including details of how they are carried out
- Normal neurobiology and neuropharmacology of sleep-wake regulation
- Normative data for sleep architecture and tests of sleep propensity
- Other medical conditions that are associated with EDS, such as myotonic dystrophy and Prader–Willi syndrome
- Primary hypersomnias of central origin that are associated with EDS, including idiopathic hypersomnolence and narcolepsy
- Range of limited channel sleep studies available
- Recognise the actions of centrally acting pharmacological agents and their interactions with sleep
- The role of questionnaires and sleepiness scales in children
Investigations
- Assess severity of daytime consequences of sleep disorders
- Identify EDS or inability to maintain wakefulness based on tests of sleep propensity
- Interpret raw data from sleep studies, including:
- airflow parameters
- airway pressures
- body position
- chin electromyography
- effort parameters
- ECG
- electroencephalogram (EEG)
- electromyography (EMG)
- electro-oculogram (EOG)
- measures of CO2
- oxygen saturation
- Interpret results of investigations regarding EDS and daytime consequences of sleep disorders in the clinical context of the patient
- Perform a thorough history, examination, and sleep-specific assessment, including the use and limitations of sleepiness questionnaires with children
- Perform the relevant general physical, neurological, and respiratory examinations
- Synthesise patients’ symptoms and signs into comprehensive differential diagnoses, and plan further investigations if needed
- Take a thorough sleep history from the patient, as well as bed environment and sleep hygiene behaviour, including bedtime routine, diet, and screen time
Procedures
- Actigraphy
- MWT
- MSLT
- Polysomnography (PSG)
- Urine drug screen
- Determine optimal treatment settings from treatment sleep study parameters
- Determine the requirement for further evaluation in the event of an indeterminate limited channel sleep study
- Determine when MSLTs and MWTs might be appropriate
- Explain and manage drug misuse and withdrawal
- Explain behavioural strategies to reduce the symptoms of EDS and the daytime consequences of sleep disorders, such as sleep education and schedule modification, including the role of naps
- Explain lifestyle implications of EDS and daytime consequences of sleep disorders
- Explain public health and wider societal implications of the diagnosis of narcolepsy and related disorders, such as career choice and industrial safety
- Generate reports for diagnostic and treatment sleep studies
- Identify pathological hypersomnolence or inability to maintain wakefulness based on tests of hypersomnolence
- Interpret results, and formulate management plans
- Monitor the pharmacotherapies for central nervous system (CNS) disorders of hypersomnolence, including the surveillance of side effects and compliance
- Prescribe pharmacotherapy for REM intrusion symptoms, such as cataplexy, in NT1 patients
- Prescribe pharmacotherapy to reduce symptoms of EDS and daytime consequences of sleep disorders
- Recognise the indications for completion of a sleep diary:
- explain the completion of a sleep diary to patients, parents, or carers
- interpret sleep diaries, applying knowledge of normal sleep duration and timing according to age
- use sleep diary information to inform treatment decisions
- Recognise the limitations and clinical applicability of various types of limited channel sleep studies, including:
- cardiorespiratory sleep studies
- limited channel PSG studies
- overnight oximetry
- Recognition of wearable devices which are now widely available in the consumer market, as well as their limitations
- Surveillance of other disorders in patients with CNS disorders of hypersomnolence, such as increased risk of cardiovascular disease and appearance of obstructive sleep apnoea