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CHAPTER 39 disorders in neurodegenerative disorders and P. Jennum, 1 J. Santamaria Cano,2 C . B a s s e t t i ,3 P. Clarenbach, 4 B . H ö g l ,5 J. Mathis, 6 R . P o i r r i e r ,7 K. Sonka, 8 E. Svanborg,9 L . D o l e n c G r o s e l j ,10 D. Kaynak, 11 M . K r u g e r ,12 A. Papavasiliou, 13 Z. Zahariev14 1 Glostrup Hospital, University of Copenhagen, Denmark ; 2Hospital Clinic of Barcelona, Spain; 3 University Hospital Zurich, Switzerland; 4 Evangelisches Johannes- Krankenhaus, Germany; 5Medical University of Innsbruck, Austria; 6 University Hospital, Inselspital, Bern, Switerzland; 7 CHU Sart Tilman, Liè ge, Belgium; 8 Charles University of Prague, Czech Republic ; 9 Division of Clinical Neurophysiology, Link ö ping, Sweden ; 10 University Medical Centre, Ljubljana, Slovenia ; 11 Dokuz Eylu l University, Izmir, Turkey; 12 H ô pital de la Ville, Luxembourg ; 13 Palia Pendeli Children’ s Hospital, Athens, Greece; 14 High Medical School, Plovdiv, Bulgaria

Introduction The current guideline will focus on neurodegenerative disorders and stroke , with an emphasis on sleep Sleep is an active process generated and modulated by a disorders in neurological , and is an update from a complex set of neural systems located mainly in the former review [1] in accordance to European Federation , brainstem, and thalamus. Sleep is altered of Neurological Societies (EFNS) guidelines [2] . in many neurological due to several mechanisms: The review will cover three main areas: lesions of the brain areas that control sleep and wakeful- 1. (Alzheimer ’ s disease, progressive supra- ness, lesions or diseases that produce pain, reduced nuclear palsy, and corticobasal degeneration); mobility, and treatments. Excessive daytime sleepiness 2. synucleinopathies (Parkinson’ s disease, multiple (EDS), sleep fragmentation, , sleep- disordered system atrophy [MSA], and with Lewy bodies breathing (SDB), nocturnal behavioural phenomena [DLB]); such as rapid eye movement (REM) sleep behaviour dis- 3. stroke, amyotrophic lateral sclerosis (ALS), myotonic order or nocturnal , , and dystrophy, myasthenia gravis, and spinocerebellar ataxias. periodic leg movement syndrome (PLMS) are common symptoms and fi ndings in neurological disorders. Sleep disorders may precede and infl uence the disease course Search s trategy in neurological diseases, involving daytime functioning, quality of life, morbidity, and mortality. The literature search included PUBMED and the Diagnostic and treatment procedures and the treat- Cochrane Database. These were searched until 2009 or ment of sleep disorders have developed considerably in over as much of this range as possible, looking for the recent years. There are thus increased opportunities for different sleep disorders and symptoms in each of the the management of sleep disorders associated with neu- most frequent or relevant degenerative neurological dis- rological diseases. orders and stroke. Language of writing was restricted to European languages. Studies considered for inclusion were, when possible, randomized controlled trials of

European Handbook of Neurological Management: Volume 1, 2nd Edition adult patients, in any setting, suffering a neurodegenera- Edited by N. E. Gilhus, M. P. Barnes and M. Brainin tive disorder (, Parkinson ’ s disease, © 2011 Blackwell Publishing Ltd. ISBN: 978-1-405-18533-2 Alzheimer ’ s disease) or stroke. There had to be an explicit

529 530 SECTION 6 Sleep Disorders complaint of insomnia, , or in can usually be attributed to a well - defi ned circumstance, the study participants. We also included observational while chronic insomnia is often a consequence of condi- studies. Abstracts were selected by the chairmen and tional (psychophysiological) factors, is idiopathic, or is independently inspected by individual members of the found in patients with psychiatric, medical, or neurologi- task force; full papers were obtained where necessary. A cal disorders. The latter may be due to degeneration or classifi cation of the different studies according to evi- dysfunction of the central areas involved dence levels for therapeutic interventions and diagnostic in sleep regulation, or due to motor or sensory symptoms measures will be done in accordance with the guidance produced by the disease (pain, reduced nocturnal mobil- [2] . The panel will discuss what possible diagnostic tests ity, nocturnal motor activity, etc.) that lower the thresh- and health care interventions could be recommended in old for arousal from sleep. Finally, insomnia may be each particular disease. caused by the alerting effects of the drugs employed in the treatment of neurological diseases.

Method for r eaching c onsensus Sleep - d isordered b reathing ( SDB ) These disorders are characterized by disordered breath- Where there was uncertainty, further discussion was ing during sleep. A uniform syndrome recommendation sought by the panel. Data extraction and quality assess- was suggested in 1999 by the American Academy of Sleep ments were undertaken independently by the panel Medicine [4] , which is included in ICSD - 2: reviewers. 1. Obstructive sleep apnoea syndrome (OSAS) 2. Central sleep apnoea – hypopnoea syndrome (CSAHS) 3. Cheyne – Stokes breathing syndrome (CSBS) Sleep d isorders 4. Sleep - related hypoventilation/hypoxaemic syndromes (SHVS). Classifi cation of s leep d isorders For a more thorough review of SDB, see the EFNS The International Classifi cation of Sleep Disorders guideline [1]. version 2 (ICSD - 2) lists 95 sleep disorders [3] . The ISCD - 2 has eight major categories: 1. Insomnias EDS n ot d ue to a s leep - related 2. Sleep - related breathing disorders b reathing d isorder 3. not due to a sleep- related breathing Hypersomnias (EDS) is defi ned by the inability to stay disorder fully alert and awake during the day, resulting in unin- 4. sleep disorders tended lapses into sleep. EDS should be separated from 5. fatigue, which refer to physical or mental weariness. The 6. Sleep - related movement disorders most common disorders in this group are , 7. Isolated symptoms , insuffi cient sleep, and the use of 8. Other sleep disorders. sedating medication. EDS is also commonly reported in Only a selected number of the sleep disorders related to patients with neurological disease including neurodegen- neurological diseases will be mentioned in this paper. eration, post- stroke, infl ammation, tumour, injury, or brain trauma, and may be caused by degeneration of the Insomnia sleep – wake centres, sleep fragmentation, or medication. Insomnias are defi ned by a complaint of repeated diffi - Hypersomnia in a narrow sense is describing a prolonged culties with sleep initiation, sleep maintenance, duration, major sleep episode, usually beyond 10 hours. This phe- consolidation, or quality that occurs despite adequate nomenon is typical in “ ‘ idiopathic hypersomnia with time and opportunity for sleep, and that result in some prolonged sleep time’ , but is also reported in ‘ non - organic form of daytime impairment. hypersomnia’ (ISCD- 2). The diagnostic work- up should Insomnias can be divided into acute and chronic always consider the possibility of sleep insuffi ciency syn- forms. The acute form, also termed adjustment insomnia, drome or poor . CHAPTER 39 Sleep disorders in neurodegenerative disorders and stroke 531

Circadian r hythm d isorders b. abnormal REM sleep behaviours documented Circadian rhythm disorders are defi ned as a misalign- during (PSG) monitoring; ment between the patient’ s sleep pattern and the pattern • an absence of electroencephalographic (EEG) epilepti- that is desired or regarded as the societal norm. Most of form activity during REM sleep unless RBD can be the conditions observed in this group are associated with clearly distinguished from any concurrent REM sleep - external factors like shift work, or social habits, but related disorder; in relation to neurological diseases, conditions that • the sleep disturbance not being better explained by destruct the neural input to the suprachiasmatic nucleus another , medical or , (e.g. complete bilateral retinal, optic nerve, chiasm, or mental disorder, or medication use or substance use hypothalamic lesions) may induce a condition that disorder. resembles circadian disorders. The patient and those sharing the can be injured. RBD is observed in the majority of patients with MSA, Parasomnias in DLB, and in a signifi cant proportion of patients with Parasomnias are undesirable sensorimotor events that Parkinson ’ s disease. RBD is also commonly observed in appear exclusively during sleep. Parasomnias are disor- diffuse Lewy body (DLB) and Machado – Joseph disease ders of arousal, partial arousal, and sleep stage transition. [8 – 19] . Patients with isolated RBD have a signifi cant risk These disorders do not primarily cause a complaint of of developing Parkinson ’ s disease, DLB, or MSA, espe- insomnia or excessive sleepiness, but frequently involve cially if other brainstem manifestations such as changes abnormal behaviours during sleep. Many of the disorders reduced smell, , mild cognitive impairment, or are common in children, but some are also present in incontinence are present [6, 10, 20] . The occurrence of adults. Parasomnias are subdivided into the following hallucinations in Parkinson’ s disease is related to the groups: presence of RBD [8] . Reduced striatal dopamine trans- 1. disorders of arousal from non - REM sleep: confu- porters have been observed in these patients [21] . RBDs sional arousal, sleep walking, and sleep terror; are strongly linked to narcolepsy with , espe- 2. parasomnias usually associated with REM sleep: REM cially in patients with hypocretin defi ciency [22] , and are sleep behaviour disorder (RBD), recurrent isolated sleep further observed in a number of other diseases, for paralysis, and disorder; example stroke and . A confi dent diag- 3. Other parasomnias, such as enuresis, sleep- related nosis relies on a full PSG recording, preferably with a groaning (), , sleep - synchronized audiovisual recording. related hallucinations, and eating disorders. There is a need for further clarifi cation of the EMG Of these parasomnias, RBD has a particular relation- abnormalities in RBD, with special emphasis on muscle ship to neurodegenerative diseases. tone, motor activity (number of movements, duration, and intensity), and their relation to sleep stages. These REM s leep b ehaviour d isorder (RBD ) are is currently undergoing clarifi cation and evaluation. This disorder is characterized by vigorous movements occurring during REM sleep associated with an abnormal Sleep - related m ovement d isorders absence of the physiological muscle atonia and with Sleep - related movement disorders are characterized by increased phasic electromyographic (EMG) activity relatively simple, usually stereotypic movements that during REM sleep [5 – 7] . Diagnostic criteria are: disturb sleep to complex movements of different inten- • the presence of REM sleep without atonia: the EMG sity, duration and periodicities or lack of it. Periodic limb fi nding of excessive amounts of sustained or intermittent movements (PLM), restless legs syndrome (RLS), elevation of fragmented EMG tone, or excessive phasic , leg cramps, rhythmic movement disorders and submental or (upper or lower extremity) EMG other sleep- related movement disorders are classifi ed twitching; under this group. Of these RLS and PLM are of particular • one or both of the following: interest in patients with neurodegenerative disorders. Of a. sleep - related injuries, potentially injurious, or dis- special focus is subdivision into different types of stage ruptive behaviours in the history; dependent movements during sleep, as observed in the 532 SECTION 6 Sleep Disorders majority of neurodegenerative disorders, RBD and in • treatment - related nocturnal disturbances (e.g. insom- patients with narcolepsy with cataplexy. This area is cur- nia, confusion, hallucinations, and motor disturbances); rently undergoing clarifi cation and evaluation. • sleep - related symptoms such as hallucinations and vivid (), insomnia (sleep fragmenta- Sleep d isorders a ssociated with tion and diffi culties maintaining sleep), , psy- n eurological d isease chosis, and panic attacks; • EDS, sleep attacks, and episodes of during Tauopathies waking hours; Patients with progressive supranuclear palsy, Alzheimer ’ s • sleep - related disorders including RBD, RLS, PLMS, disease, and corticobasal degeneration may complain of nocturnal dystonic movements, cramps, and SDB. The signifi cant sleep- related circadian disturbances, as well as presence of RBD in Parkinson’ s disease is associated with sleep – wake and daytime problems [5, 23 – 29] . cognitive and autonomic changes; • Sleep/wake disturbances and disruption are commonly • laryngeal stridor and obstructive sleep apnoea, which observed in Alzheimer’ s disease, with daytime sleep, sleep are commonly observed in patients with MSA and are attack and episodes of microsleep. associated with a poorer prognosis. Continuous positive • Insomnia (sleep fragmentation and diffi culties main- airway pressure (CPAP) ventilation may improve respira- taining sleep) is common, as are nocturnal wandering, tion and prognosis (Class III). nocturnal confusion, ‘ sundowning ’ psychosis, and nocturia. • EDS, sleep attacks, and episodes of microsleep during the daytime may be associated with cognitive problems. Recommendations • Sleep - related disorders such as RBD, RLS, PLMS, noc- The majority of patients with synucleinopathies experience turnal complex and dystonic movements, and cramps one or more sleep disorders. It is recommended to perform a may occur in progressive supranuclear palsy and cortico- detailed medical history of sleep disorders in tauopathies, i.e. insomnia, EDS, motor, and dreaming activity, and SDB PSG basal degeneration, but are rare in Alzheimer ’ s disease. recording, preferably with audiovisual recording, is suggested • Sleep breathing disorders are common in Alzheimer ’ s for the diagnosis, especially when RBD and/or SDB is disease and are associated with disease progression and a suspected (Level B). poorer prognosis; however, the clinical signifi cance of diagnosing and treating them in this group of patients is questionable. Stroke Patients with , primarily infarctions, may suffer Recommendations from several sleep disorders and disturbances. Their Sleep disorders are commonly observed in patients with occurrence and manifestations may vary depending on tauopathies, and there should be an increased awareness of the specifi c neurological defi cits [39 – 50] : these disorders. It is recommended to perform a detailed medical history of sleep disorders in tauopathies, i.e. • SDB, especially OSAS and nocturnal oxygen desatura- > insomnia, EDS, motor and dreaming activity, and SDB. PSG tions, have commonly ( 50%) been found in patients recording, preferably with audiovisual recording, is suggested with acute stroke as well as after neurological recovery. for the diagnosis, especially when RBD and/or SDB are OSAS is a risk factor for stroke, and co - existing OSAS in suspected disorders (Level C). stroke patients may increase the risk of a further stroke. The presence of SDB, especially OSAS, may worsen the Synucleinopathies prognosis and increase the stroke re- occurrence risk. SDB Parkinson ’ s disease, MSA, and DLB are often associated may be provoked by stroke, especially, after damage to the with major sleep – wake disorders [13, 17, 28, 30 – 38] : respiratory centres in the brainstem or bulbar/ • Parkinson ’ s disease - related motor symptoms including pseudobulbar paralysis due to brainstem . Pre - existing nocturnal akinesia, early- morning , painful sleep apnoea prior to stroke may present a risk factor for cramps, , and diffi culties turning in bed; stroke, with comorbid obesity, , coronary artery CHAPTER 39 Sleep disorders in neurodegenerative disorders and stroke 533 disease and hypertension, and other cerebrovascular risk primary SDB in patients with ALS – as in other neuro- factors. There are several haemodynamic changes in sleep muscular diseases – is therefore a sleep hypoventilation apnoea that may play a role in the pathogenesis of stroke syndrome (SHVS), whereas OSAS is rare [53] . development. Stroke and SDB are both common and are Management of these patients should therefore include associated with signifi cant morbidity and mortality. relevant questions regarding symptoms suspicious for • CPAP treatment for OSAS in may reduce the risk of SDB. Common symptoms of nocturnal hypoventilations cardio- and cerebrovascular complications (Class I) and include insomnia, , and daytime potential re - occurrence of stroke, but the compliance is [59] . poor to moderate compared with the compliance in Oximetry has been suggested for the identifi cation of OSAS patients who have not had a stroke (B). and screening for sleep- related hypoventilation in • Sleepiness and fatigue are commonly reported in patients with ALS, but its value is limited to identifying patients after stroke and are often disabling symptoms. nocturnal desaturations that may occur during non- • Other sleep disorders, such as insomnia, RBD, and REM and REM sleep [57, 60, 61]. Care should be taken PLMS, may be observed as part of or after stroke. because p CO may increase before desaturations are observed, especially in patients with additional chronic obstructive lung disease. Nocturnal oximetry has been suggested as valuable for screening and for evaluating the Recommendations treatment effect [53, 57] . There has been no validation of Sleep disorders, especially SDB, occur often in stroke the diagnostic yield between a full PSG, respiratory patients. Screening for SDB and other sleep disorders is polygraphy, and nocturnal oximetry in these patients. recommended as part of the stroke evaluation programme, It is, however, important to identify early symptoms especially in ischaemic stroke patients (Level A). of respiratory failure in sleep, as these patients are able to compensate their during for a long time. This is the at which one should bring in a regular measurement of respiratory parameters Motor n euron, m otor e nd p late, and [62 – 66]. m uscle d iseases SDB is observed in several neuromuscular diseases, including muscular dystrophy, , myasthenia gravis, ALS, and post- polio syndrome. Recommendations Although there may be differences, some general obser- SDB often occurs in patients with motor neuron, motor end vations can be made. Hypoxaemia, especially during plate, and muscle diseases, and should be considered in all REM sleep, is commonly found. Severity is correlated to patients. Minimum evaluation should include PSG eventually combined with additional analysis, and respiratory strength, and sleep- related hypoventilation is eventually supplied with serial polygraphy or oximetry usually non- obstructive [51, 52]. measures for the identifi cation of sleep - related Patients with ALS and other severe motor neuron dis- hypoventilation during the disease course (Level B). eases have progressive motor deterioration with progres- sive respiratory insuffi ciency. This may manifest primarily during sleep, where the motor drive is reduced. This is especially true for patients with the bulbar form of ALS Genetic n eurodegenerative d isorders or involvement of C3– C5 in the anterior horn [53, 54]. Other neurodegenerative disorders of genetic cause may The prognosis is closely related to respiratory muscle present several sleep disturbances. Subjects with SCA - 3 strength [55] . Of note, sudden nocturnal death often (Machado– Joseph disease) may also complain of RLS, occurs during sleep. Respiratory indices such as low noc- periodic leg movements, vocal cord paralysis, and RBD turnal oxygen saturation are associated with a poorer [9, 11, 18, 67, 68] . In patients with Huntington ’ s disease, prognosis [56, 57]. Patients with diaphragmatic involve- the involuntary movements tend to diminish during ment may have signifi cantly reduced REM sleep [58] . The sleep [69] . Sleep disturbances, including disturbed sleep 534 SECTION 6 Sleep Disorders pattern with an increased latency, reduced improves nocturnal respiratory abnormalities, daytime sleep effi ciency, frequent nocturnal awakenings, and function, and cognitive problems [73 – 78] (Class I). more time spent awake with less slow wave sleep, have There is no signifi cant difference regarding treatment been reported. These abnormalities correlate in part with effect or changes in subjective variables between fi xed- the duration of illness, severity of clinical symptoms, and pressure CPAP and auto - adjusted CPAP [79, 80] degree of atrophy of the caudate nucleus [70] . The sleep (Class I). phenotype of Huntington ’ s disease may also include CPAP and bi- level ventilation insomnia, advanced sleep phase, periodic leg movements, is potentially useful in patients with SDB in stroke [40] , RBDs, and reduced REM sleep, but not narcolepsy. despite negative reports [81] . The evidence on whether Reduced REM sleep may precede . Mutant hun- this infl uences quality of life, daytime symptoms, reha- tingtin may exert an effect on REM sleep and motor bilitation, morbidity, and mortality is, however, limited, control during sleep [71] . However, other studies have which needs further clarifi cation [49] (Class II). not reported specifi c sleep disorders in Huntington Severe SDBs, including laryngeal stridor in patients patients [72] . with MSA, may be treated with CPAP/bi- level CPAP. Recent studies suggest that treatment with CPAP for MSA patients with laryngeal stridor showed high CPAP Recommendations tolerance, no recurrence of stridor, no major side effects, and a subjective improvement in sleep quality, and that Sleep disorders occur in several genetic neurological diseases. The patients should be questioned, and further evaluation of there is an increased survival time for MSA patients these disorders should rely on a clinical judgement (Level C). without stridor [31, 82] . CPAP is therefore an effective, non- invasive, long- term therapy for nocturnal stridor (Level C) and may prevent worsening of stridor under increasing dopaminergic dosages. Management of s leep d isorders in In some patients, for example those with neuromus- n eurological d iseases cular disorders, CPAP may be diffi cult to accept, and bi - level positive airway pressure ventilation may be used Diagnostic t echniques in [83] (Class IV). s leep d isorders There is evidence suggesting that oral appliance use Diagnostic procedures for sleep diagnosis include PSG, improves subjective sleepiness and SDB compared with partial time PSG, partial polygraphy (or respiratory controls in patients with OSAS without neurological polygraphy), and limited channel polygraphy: oximetry disease (Level B). Nasal CPAP is apparently more effective determining arterial oxygen saturation/pulse and actim- in improving SDB than oral appliance use (Level B). etry. Daytime sleepiness may be evaluated with the There are no data regarding the use of oral appliances in Multiple Sleep Latency Test (MSLT) or Maintenance patients with neurological diseases, so caution should be of Wakefulness Test (MWT). Many of the tests are applied concerning the use of oral appliances in patients increasingly easy to perform in or outside hospital due to with OSAS [84, 85] (Level C). technological advantages. Consequently, diagnostic pro- Surgical treatment has a limited effect on OSA [86, 87] cedures may be more easily performed as part of the (Class III). There are no studies suggesting that surgery diagnostic program for neurological patients. An over- in the upper airway has any effect on OSAS in patients view of these tests is presented in table 39.1 . with neurological diseases. Drug treatments have no positive effect on OSAS [88] Treatment of SDB in (Class II). There is no study available indicating that n eurological d iseases medication has any treatment effect for OSAS in patients with neurological diseases Treatment of OSAS Although some patients with OSAS present an CPAP is a well - documented treatment for moderate and increased weight and a negative lifestyle profi le (in terms severe OSAS (apnoea – hyperpnoea index ≥ 15/h) and of tobacco, alcohol, and physical activity), no controlled CHAPTER 39 Sleep disorders in neurodegenerative disorders and stroke 535

Table 39.1 Methods for the diagnosis of sleep disorders in neurological disease.

Type of PSG Defi nition Indication Advantage/disadvantage

Routine PSG Multi - channel EEG, EOG, submental Routine screening for sleep Gold standard. May be performed EMG, ECG, , + / − tibial disorders: SDB, PLMS, chronic in or outside hospital. Standard EMG insomnia method Extended PSG Routine PSG + extra physiological Special indications: oesophageal Moderately expensive, time- channels, e.g. EMG, intraoesophageal refl ux, myoclonias, etc. consuming, staff- demanding pressure, carbon dioxide Depends on selected channels Video - PSG PSG + video recording Motor and behavioural A video signal is present. Full phenomena during sleep physiological recording. Includes audiovisual channels Full EEG– PSG Full 10– 20 EEG + PSG Motor and behavioural Full diagnostic procedures are disturbances for the obtained. The difference of between the methods is primarily the number of EEG channels. Expensive, time- consuming, staff- demanding MSLT Multiple ( ≥ 4) trials per day of PSG Central hypersomnias including Supportive for the diagnosis of determination of sleep latencies of narcolepsy, distinction hypersomnia and narcolepsy/ intended sleep between tiredness and EDS. sensitive to foregoing sleep loss Supportive for EDS in and discontinuation of REM neurological diseases sleep - inhibiting drugs MWT Multiple ( ≥ 4) trials per day of PSG Determination of ability to stay Supportive for wakefulness determination of sleep latencies of awake capabilities, useful for driving intended sleep inhibition ability and treatment effects

Partial channel polygraphy Respiratory Monitoring of respiration + arterial OSAS Easy, inexpensive. Moderate to polygraphy oxygen saturation + / − cardiac good sensitivity and specifi city measures, e.g. pulse for OSAS; the validity for other SDBs is not known Oximetry Monitoring of arterial oxygen saturation Monitoring or screening for Easy, inexpensive. Low sensitivity severe SDB and specifi city for SDB. Exclusion of SDB not possible Determination of motor activity Sleep – wake disturbances Inexpensive. Limited clinical (days – months) usefulness

ECG, electrocardiography; EOG, Electrooculography. See text for other abbreviations.

studies have evaluated the effect of intervention against due to the rareness of the disease, there are no random- these factors [89] (Class IV). No studies have addressed ized studies regarding CSAHS and treatment. Drug the effect of lifestyle interventions on OSAS in patients treatment with acetazolamide and theophylline has with neurological diseases. furthermore been suggested [93] , but the evidence for their use is poor (Class IV). Treatment of CSAHS Case series have shown that CPAP treatment does not Treatment of CSBS infl uence the carbon dioxide response in CSAHS, despite Initially, CPAP was used in patients with central apnoea/

a reduction in apnoeas, an increase in p aO 3 , and a reduc- CSBS and cardiac insuffi ciency [94 – 97] , but in recent tion in subjective sleepiness [90 – 92] (Class IV). Probably years adaptive ventilation has been found to be effective, 536 SECTION 6 Sleep Disorders probably via an increased preload in patients with signifi - cussed early in the course of the disease. It is important cant cardiac failure, and to reduce the respiratory to clarify the limitations of the treatment, and the discus- abnormalities, although the long- term prognosis is not sion should include careful debate regarding whether known [98, 99] (Class IV). A recent randomized con- such treatment should be offered, its initiation, the need trolled study suggests that the use of non- invasive adap- for , whether invasive ventilation should be tive ventilation may improve daytime function and offered, and discontinuation [106, 107] . respiratory and cardiac measures [100] (Class II). The experience with the use of adaptive ventilation, CPAP or Drug t reatment bi - level CPAP in patients with Cheyne – Stokes respiration due to central respiratory failure, for example brainstem Treatment of EDS in n eurological d iseases lesions, is sparse, and the evidence level is poor (Class C). Several groups of patients with neurological diseases commonly complain of EDS. The aetiology may be sec- Treatment of s leep h ypoventilation s yndrome ondary to the neurological disease or its medication Treatment includes nasal intermittent positive - pressure (dopaminergic or drugs), or the conse- ventilation (NIPPV) with bi- level positive airways pres- quence of concomitant sleep disorders such as sleep sure (variable positive airways pressure), non - invasive apnoea, nocturnal motor phenomena, etc. In patients in volumetric ventilation, and eventually invasive ventila- whom these factors cannot be modifi ed, such tion, under the control of nocturnal respiratory param- as or modafi nil may be used as symp- eters [101] (Class IV). CPAP is not the primary treatment, tomatic therapy. Modafi nil was primarily introduced to as the motor effort is mostly reduced in these patients, treat EDS in narcolepsy [108 – 113] . Case studies [114, which may lead to worsening of the SDB. NIPPV may 115] and double- blind controlled studies [116, 117] reduce sleep disturbances, increase cognitive function, suggest that modafi nil reduces EDS in Parkinson ’ s and prolong the period to tracheostomy [102, 103] (Class patients (Class B- II ) despite the fact that Ondo et al . ’ s IV). Current evidence about the therapeutic benefi t of study did not prove the long - term effect of modafi nil in mechanical ventilation is weak but consistent, suggesting Parkinson’ s disease [118] . Modafi nil has also been sug- alleviation of the symptoms of chronic hypoventilation gested in ALS [119] and post- stroke depression [120, in the short term. Evidence from a single randomized 121], but no controlled studies are available (Class IV). trial of non - invasive ventilation with a limited number Furthermore, modafi nil has been used for the treatment of participant suggests a prolonged survival and improved of residual EDS in OSAS undergoing CPAP treatment quality of life in people with ALS, especially among those without neurological comorbidity [122] . There is some with minor bulbar involvement, but not in patients with evidence that other centrally acting drugs such ass meth- severe bulbar impairment [104, 105] (Class III). ylphenidate may have similar effects [123] , but there have been no comparisons between modafi nil and methylphe- Follow - u p nidate. EDS in Parkinson’ s disease was successfully Although there is no evidence on when and how the reduced by [124] (Class II). follow- up of treatment with CPAP and NIPPV should be executed, we recommend regular follow- up of the treat- Other d rug and n on - p harmacological t reatment ment with control of compliance and treatment effect of s leep d isorders in n eurological d iseases (Class IV). Treatment of sleep disorders in neurodegenerative dis- eases is often complex and may involve different strate- Ethical a spects gies. Parkinson’ s disease- related motor symptoms can be Treatment of patients with severe neurological diseases treated with long- acting DA agonists to obtain continu- such as ALS and MSA with NIPPV includes medical and ous DA receptor stimulation during the night. On the ethical problems that should be addressed. Adequate other hand, nocturnal disturbances may be related to involvement of the patients and family, and the treat- treatment, and therefore continued monitoring of treat- ment, its use, and its limitations, should be carefully dis- ment effect should offered. CHAPTER 39 Sleep disorders in neurodegenerative disorders and stroke 537

Some sleep disorders, such as RLS and PLMS, may be Patients with often present circadian distur- controlled by DA agents, and others, such as insomnia bances that may be relieved by and light and EDS, may be improved by reducing dopaminergic therapy [126 – 142] (Class IV). stimulation (Class IV). In selected cases, treatment with hypnotics are men- Clonazepam or donepezil, possibly prescribed with tioned to be useful, but the evidence is limited and care melatonin, has been suggested based on case series for should be undertaken in terms of chronic use, the risk of the treatment of RBD. No controlled studies are available falls, daytime sedation, confusion, and the risk of worsen- [33, 125]. ing of SDB in the elderly.

Recommendations 1. Patients with neurological diseases often have signifi cant 5. Oximetry has a poor sensitivity/specifi city for the sleep disorders that affect sleep and daytime function, with identifi cation of OSAS in patients without neurological increased morbidity and even mortality. Many of these diseases. Oximetry cannot differentiate between obstructive disorders are treatable. Therefore, increased awareness and central sleep apnoea and is insuffi cient to identify should be directed toward sleep disorders in patients with stridor. Oximetry alone is not recommended for the neurodegenerative, cerebrovascular, and neuromuscular diagnosis of SDB in neurological disorders. diseases. Despite this, there are limited number of studies 6. Patients with SDB, muscle weakness, and cardiac or with a high evidence level. pulmonary comorbidity may present a sleep hypoventilation 2. PSG is a diagnostic minimum for the diagnoses of sleep syndrome that manifests early as increased carbon dioxide. disorders in patients with neurological diseases. p aCO should be measured in such cases during sleep 3. In patients with nocturnal motor and/behaviour recordings. manifestations, a full video- PSG/video- EEG – PSG is 7. Fixed - pressure CPAP/auto - adjusted CPAP is the most recommended. effective treatment for OSAS. This probably also includes 4. Respiratory polygraphy has a moderate sensitivity and patients with OSAS and neurological diseases. However, specifi city in the diagnosis of OSAS without neurological there is a need for further evaluation of the effect of CPAP diseases, but its value for the diagnosis of other SDBs or in in patients with OSAS and neurological diseases. neurological patients with suspected OSAS has not been 8. Bi - level/variable positive- airway pressure ventilation, NIPPV, evaluated compared with the gold standard of PSG. and volumetric ventilation are useful for SDBs such as Consequently, respiratory polygraphy may be used as a central apnoeas, Cheyne- Stokes breathing, and alveolar method for detecting OSAS, but the value of its use for hypoventilation. SDB in patients with neurological diseases needs further 9. There is a clear need for further studies focusing on the validation. diagnostic procedures and treatment modalities in neurological patients with sleep disorders.

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