The levels of evidence (Class I-IV) supporting the recommendations and ratings of recommendations (A-C, Good Practice Point) are defined at the end of the "Major Recommendations" field.
Sleep Disorders Associated with Neurological Disease
Sleep disorders are commonly observed in patients with tauopathies, and there should be an increased awareness of these disorders. It is recommended to perform a detailed medical history of sleep disorders in tauopathies, i.e., insomnia, excessive daytime sleepiness (EDS), motor and dreaming activity, and sleep-disordered breathing (SDB). Polysomnography (PSG) recording, preferably with audiovisual recording, is suggested for the diagnosis, especially when rapid eye movement (REM) sleep behaviour disorder (RBD) and/or SDB are suspected disorders (Level C).
The majority of patients with synucleinopathies experience one or more sleep disorders. It is recommended to perform a detailed medical history of sleep disorders in tauopathies, i.e., insomnia, EDS, motor and dreaming activity, and SDB. PSG recording, preferably with audiovisual recording, is suggested for the diagnosis, especially when RBD and/or SDB is suspected (Level B).
Sleep disorders, especially SDB, occur often in stroke patients. Screening for SDB and other sleep disorders is recommended as part of the stroke evaluation programme, especially in ischaemic stroke patients (Level A).
Motor Neuron, Motor End Plate, and Muscle Diseases
SDB often occurs in patients with motor neuron, motor end plate, and muscle diseases, and should be considered in all patients. Minimum evaluation should include PSG eventually combined with additional carbon dioxide analysis, and eventually supplied with serial polygraphy or oximetry measures for the identification of sleep-related hypoventilation during the disease course (Level B).
Genetic Neurodegenerative Disorders
Sleep disorders occur in several genetic neurological diseases. The patients should be questioned, and further evaluation of these disorders should rely on a clinical judgement (Level C).
Management of Sleep Disorders in Neurological Diseases
- Patients with neurological diseases often have significant sleep disorders that affect sleep and daytime function, with increased morbidity and even mortality. Many of these disorders are treatable. Therefore, increased awareness should be directed toward sleep disorders in patients with neurodegenerative, cerebrovascular, and neuromuscular diseases. Despite this, there are limited number of studies with a high evidence level.
- PSG is a diagnostic minimum for the diagnoses of sleep disorders in patients with neurological diseases.
- In patients with nocturnal motor and/behavior manifestations, a full video-PSG/video-electroencephalography (EEG)-PSG is recommended.
- Respiratory polygraphy has a moderate sensitivity and specificity in the diagnosis of obstructive sleep apnoea syndrome (OSAS) without neurological diseases, but its value for the diagnosis of other SDBs or in neurological patients with suspected OSAS has not been evaluated compared to gold standard of PSG. Consequently, respiratory polygraphy may be used as a method for detecting OSAS, but the value of its use for SDB in patients with neurological diseases needs further validation.
- Oximetry has a poor sensitivity/specificity for the identification of OSAS in patients without neurological diseases. Oximetry cannot differentiate between obstructive and central sleep apnoea or is insufficient to identify stridor. Oximetry alone is not recommended for the diagnosis of SDB in neurological disorders.
- Patients with SDB, muscle weakness, and cardiac or pulmonary comorbidity may present a sleep hypoventilation syndrome that manifests early as increased carbon dioxide. Partial pressure of arterial carbon dioxide (paCO) should be measured in such cases during sleep recordings.
- Fixed pressure continuous positive airway pressure (CPAP)/auto-adjusted CPAP is the most effective treatment of OSAS. This probably also includes patients with OSAS and neurologic diseases. However, there is a need for further evaluation of the effect of CPAP in patients with OSAS and neurologic diseases.
- Bi-level/variable positive-airway pressure ventilation, noninvasive positive pressure ventilation (NIPPV) and volumetric ventilation is useful for SDBs such as central apnoeas, Cheyne-Stokes breathing, and alveolar hypoventilation.
- There is a clear need for further studies focusing on the diagnostic procedures and treatment modalities in neurological patients with sleep disorders.
Evidence Classification Scheme for a Diagnostic Measure
Class I: A prospective study in a broad spectrum of persons with the suspected condition, using a 'gold standard' for case definition, where the test is applied in a blinded evaluation, and enabling the assessment of appropriate tests of diagnostic accuracy
Class II: A prospective study of a narrow spectrum of persons with the suspected condition, or a well-designed retrospective study of a broad spectrum of persons with an established condition (by 'gold standard') compared to a broad spectrum of controls, where test is applied in a blinded evaluation, and enabling the assessment of appropriate tests of diagnostic accuracy
Class III: Evidence provided by a retrospective study where either persons with the established condition or controls are of a narrow spectrum, and where test is applied in a blinded evaluation
Class IV: Any design where test is not applied in blinded evaluation OR evidence provided by expert opinion alone or in descriptive case series (without controls)
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion
Rating of Recommendations for a Diagnostic Measure
Level A rating (established as useful/predictive or not useful/predictive) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (established as probably useful/predictive or not useful/predictive) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (established as possibly useful/predictive or not useful/predictive) requires at least two convincing class III studies.
Rating of Recommendations for a Therapeutic Intervention
Level A rating (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.
Good Practice Point Where there was a lack of evidence but consensus was clear, the task force has stated their opinion as Good Practice Points.