scholarly journals O050 Hypersomnolence in children: the diagnostic dilemma

2021 ◽  
Vol 2 (Supplement_1) ◽  
pp. A21-A22
Author(s):  
A Anantharajah ◽  
G Nixon ◽  
M Davey
Keyword(s):  
Sleep Latency ◽  
Sleep Onset ◽  
Paediatric Population ◽  
Sleep Apnoea ◽  
Diagnostic Dilemma ◽  
Diagnostic Categories ◽  
Not Otherwise Specified ◽  
Obstructive Sleep ◽  
Movement Index ◽  
Multiple Sleep Latency

Abstract Background Excessive daytime sleepiness (EDS) is reported to affect up to 20% of pre-pubertal children and 50% of adolescents. EDS can be due narcolepsy and idiopathic hypersomnolence (IH). Currently diagnosis is by a multiple sleep latency test (MSLT) with protocols adapted from adults. The aim of this study was to describe the results of MSLTs in a paediatric population and assess whether a 5th nap altered diagnosis. Methods Retrospective analysis of 253 MSLTs at a single tertiary paediatric centre from May 2004 – Jan 2021 with consent obtained in 214 patients. Narcolepsy defined as a mean sleep latency (MSL) <8min with ≥2 sleep onset REM (SOREM). IH defined as a MSL < 8 minutes with <2 SOREMs. Results outside these parameters were grouped as hypersomnolence not otherwise specified (HNOS), with ambiguous HNOS defined as MSL 8–12 minutes or ≥2 SOREM. Progress to date There were 108 (50%) females, 132 (62%) >12 years old (range 3.3–19.4 years) and 17 patients had repeat studies. Narcolepsy was diagnosed in 48 (22%) and IH in 22 (10%). Criteria for ambiguous HNOS were met by 43 (20%) patients including 11 (5%) with MSL >12 minutes with ≥2 SOREMs. A 5th nap was performed in 58 (27%) MSLTs which did not change the diagnosis. Obstructive sleep apnoea was diagnosed in 48 (22%) patients and 27 (13%) had elevated periodic limb movement index. Intended outcome and impact Applying current MLST criteria in children may significantly under-estimate diagnostic categories for paediatric EDS as evidenced by the ambiguous HNOS.

scholarly journals 1253 Multiple sleep onset REM episodes in middle age woman with excessive daytime sleepiness – Is this automatically assumed narcolepsy?

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A477-A477
Author(s):  
Kamal Patel ◽  
Bianca J Lang
Keyword(s):  
Daytime Sleepiness ◽  
Sleep Latency ◽  
Sleep Onset ◽  
Sleep Time ◽  
Multiple Sleep Latency Test ◽  
Sleep Onset Latency ◽  
Onset Latency ◽  
Obstructive Sleep ◽  
Falling Asleep ◽  
Multiple Sleep Latency

Abstract Introduction Presence of sleep onset REM episodes often raises concerns of narcolepsy. However other conditions have shown to have presence of sleep on REM episodes which include but not limited to obstructive sleep apnea, sleep wake schedule disturbance, alcoholism, neurodegenerative disorders, depression and anxiety Report of Case Here we present a case of 30 year old female with history of asthma, patent foraman ovale, migraine headache, and anxiety who presented with daytime sleepiness, falling asleep while at work, occasional scheduled naps, non-restorative sleep, sleep paralysis, and hypnopompic hallucination. Pertinent physical exam included; mallampati score of 4/4, retrognathia, high arched hard palate, crowded posterior oropharynx. She had a score of 16 on Epworth sleepiness scale. Patient previously had multiple sleep latency test at outside facility which revealed 4/5 SOREM, with mean sleep onset latency of 11.5 minutes. She however was diagnosed with narcolepsy and tried on modafinil which she failed to tolerate. She was tried on sertraline as well which was discontinued due to lack of benefit. She had repeat multiple sleep latency test work up which revealed 2/5 SOREM, with mean sleep onset latency was 13.1 minutes. Her overnight polysomnogram prior to repeat MSLT showed SOREM with sleep onset latency of 10 minutes. Actigraphy showed consistent sleep pattern overall with sufficient sleep time but was taking hydroxyzine and herbal medication. Patient did not meet criteria for hypersomnolence disorder and sleep disordered breathing. Conclusion There is possibility her medication may have played pivotal role with her daytime symptoms. We also emphasize SOREMs can be present in other disorders such as anxiety in this case and not solely in narcolepsy

Adenotonsillar microbiome: an update

2018 ◽  
Vol 94 (1113) ◽  
pp. 398-403 ◽  
Author(s):  
James Jordan Johnston ◽  
Richard Douglas
Keyword(s):  
Pathogenic Bacteria ◽  
Current Knowledge ◽  
Otitis Media With Effusion ◽  
Paediatric Population ◽  
Sleep Apnoea ◽  
Recurrent Tonsillitis ◽  
Surface Culture ◽  
Upper Airways ◽  
Palatine Tonsils ◽  
Obstructive Sleep

Pathogenic bacteria associated with the adenoids and tonsils cause much morbidity in the paediatric population. Hyperplasia of the adenoids is associated with otitis media with effusion and hyperplasia of the palatine tonsils is associated with both recurrent tonsillitis and obstructive sleep apnoea. Most current knowledge of the microbiology of the upper airways has been derived from culture-based studies, which usually reflect only a small fraction of the bacteria present on the mucosal surface. Culture-independent molecular surveys based on 16S ribosomal RNA sequencing are now being employed to determine the microbiota on the surface and within the tissue of adenoids and palatine tonsils. This review describes the new techniques applied in determining the microbiome and summarises the results of studies employing these techniques.

857 Too sleepy: A pediatric case of Prader Willi Syndrome and Narcolepsy

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A333-A333
Author(s):  
Elizabeth Lam ◽  
Sonal Malhotra ◽  
Daniel Glaze
Keyword(s):  
Daytime Sleepiness ◽  
Sleep Latency ◽  
Chromosome Region ◽  
Genetic Disorder ◽  
Sleep Onset ◽  
Apnea Hypopnea Index ◽  
Prader Willi Syndrome ◽  
Obstructive Sleep ◽  
Patient Reported

Abstract Introduction Prader Willi syndrome (PWS) is a genetic disorder due to deletion of the paternal copies of genes within the chromosome region 15q11-q13. Individuals with PWS are commonly seen with obesity, sleep disordered breathing, and excessive daytime sleepiness (EDS). While the exact cause of EDS in individuals with PWS is not fully understood, there have been reports of PWS with narcolepsy-like syndrome. We report a case of a patient with PWS with findings suggesting the diagnosis of Narcolepsy Type 2. Report of case(s) Our patient is a 12-year-old male with PWS and 2nd degree heart block who was evaluated in our pediatric sleep center. He has a previous diagnosis of mild obstructive sleep apnea (OSA) with an apnea hypopnea index (oAHI) of 3.2. At 12 years of age, mother and patient reported that he had increased snoring, weight gain, EDS with a Pediatric Daytime Sleepiness Score (PDSS) of 10 and frequent refreshing naps during the daytime. Patient denied cataplexy during that visit. Subsequently, 2-week actigrapghy was performed which demonstrated an average total night sleep of 8 hours and 8 minutes. Overnight PSG with Multiple Sleep Latency Test (MSLT) demonstrated an oAHI of 4.8, total sleep time of 6.88 hours. During the MSLT, the mean sleep latency was 6.2 minutes and 5 sleep onset REM periods were observed over 5 nap opportunities. At his follow-up visit, methylphenidate was initiated after clearance by his cardiologist. Patient and mother opted for medical management of his mild OSA with Fluticisone and Montelukast. At his follow-up appointment, the patient had improvement in daytime sleepiness with a PDSS of 2 despite taking his Methylphenidate at night. Patient was instructed to switch to morning Methylphenidate dosing to optimize treatment of his EDS. Conclusion EDS is a common complaint seen in patients with PWS, however the etiology of it is not entirely understood. It is thought to be centrally mediated with components of hypersomnia and narcolepsy like-symptoms. More research is needed to better understand, diagnose and adequately treat patients with PWS and EDS. Support (if any):

851 Self-Medication with Caffeine for 31 Years: A Case of Undiagnosed Childhood Narcolepsy Type II

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A331-A331
Author(s):  
Moustapha Doulaye Seydou ◽  
Christian Karime ◽  
Brenda Wyrick ◽  
Amir Khan
Keyword(s):  
Sleep Latency ◽  
Treatment Plan ◽  
Sleep Onset ◽  
Panic Attacks ◽  
Apnea Hypopnea Index ◽  
Type Ii ◽  
Obstructive Sleep ◽  
Sleep Benefit ◽  
Patient Reported ◽  
Multiple Episodes

Abstract Introduction Narcolepsy represents a relatively rare chronic neurological sleep disorder. While peak incidence occurs in adolescence and early-adulthood, reports indicate a substantial number of children under 10 remain undiagnosed or are misdiagnosed. The present case describes a female with undiagnosed narcolepsy type II self-medicating with caffeinated beverages from the age of 7. Report of case(s) A 40-year-old female presented at our clinic with excessive daytime fatigue and hypersomnolence despite adequate sleep (Epworth sleepiness scale= 16/24). The patient denied snoring, sleep paralysis, catalepsy, and hypnagogic/hypnopompic hallucinations. Symptoms began at the age of 7 and steadily worsened, with teachers reporting significant concentration difficulties and multiple episodes of unintentional sleep onset in the classroom. The patient reported heavily relying on caffeinated beverages from the age of 7 to remain awake and focused on classroom activities. Starting at the age of 7, the patient consumed on average a caffeine-equivalent of 1 espresso shot/day (64mg caffeine/day). This increased to 4–6 espresso shots/day (256-384mg caffeine/day) by the age of 12 and 5–9 espresso shots/day (320-576mg caffeine/day) by the age of 18. At the age of 25, the patient developed severe anxiety with panic attacks and episodes of suicidal ideation. With multiple episodes of sleep onset while operating a motor vehicle, a near-accident prompted medical evaluation. Diagnosed with general anxiety disorder and idiopathic hypersomnolence, escitalopram and armodafinil were started with limited effect. The patient continued self-medicating with caffeinated beverages until age 38 when she was diagnosed with narcolepsy type II. Sodium oxybate was subsequently added to her treatment plan with initial sleep benefit and caffeine reduction. A repeat mean sleep latency test confirmed narcolepsy type II (mean sleep latency= 3 minutes, mean rapid eye movement [REM] sleep latency= 3 minutes). Polysomnography was later performed due to non-resolving symptoms, revealing mild obstructive sleep apnea (REM apnea-hypopnea index= 13.5/hour). Continuous positive airway pressure was added to the treatment regime with significant sleep benefit. Conclusion We describe a case of undiagnosed childhood narcolepsy type II necessitating significant caffeine consumptions in order to maintain classroom performance. With known anxiety-provoking effects of caffeine, the case highlights the importance of addressing childhood narcolepsy. Support (if any):

Validation of Multiple Sleep Latency Test for the diagnosis of pediatric narcolepsy type 1

Neurology ◽  
2019 ◽  
Vol 93 (11) ◽  
pp. e1034-e1044 ◽  
Author(s):  
Fabio Pizza ◽  
Lucie Barateau ◽  
Isabelle Jaussent ◽  
Stefano Vandi ◽  
Elena Antelmi ◽  
...  
Keyword(s):  
Roc Curve ◽  
Sleep Latency ◽  
Pediatric Population ◽  
Sleep Onset ◽  
Biological Data ◽  
Multiple Sleep Latency Test ◽  
Roc Curve Analysis ◽  
Specificity And Sensitivity ◽  
Multiple Sleep Latency

ObjectiveTo validate polysomnographic markers (sleep latency and sleep-onset REM periods [SOREMPs] at the Multiple Sleep Latency Test [MSLT] and nocturnal polysomnography [PSG]) for pediatric narcolepsy type 1 (NT1) against CSF hypocretin-1 (hcrt-1) deficiency and presence of cataplexy, as no criteria are currently validated in children.MethodsClinical, neurophysiologic, and, when available, biological data (HLA-DQB1*06:02 positivity, CSF hcrt-1 levels) of 357 consecutive children below 18 years of age evaluated for suspected narcolepsy were collected. Best MSLT cutoffs were obtained by receiver operating characteristic (ROC) curve analysis by contrasting among patients with available CSF hcrt-1 assay (n = 228) with vs without CSF hcrt-1 deficiency, and further validated in patients without available CSF hcrt-1 against cataplexy (n = 129).ResultsPatients with CSF hcrt-1 deficiency were best recognized using a mean MSLT sleep latency ≤8.2 minutes (area under the ROC curve of 0.985), or by at least 2 SOREMPs at the MSLT (area under the ROC curve of 0.975), or the combined PSG + MSLT (area under the ROC curve of 0.977). Although specificity and sensitivity of reference MSLT sleep latency ≤8 minutes and ≥2 SOREMPs (nocturnal SOREMP included) was 100% and 94.87%, the combination of MSLT sleep latency and SOREMP counts did not improve diagnostic accuracy. Age or sex also did not significantly influence these results in our pediatric population.ConclusionsAt least 2 SOREMPs or a mean sleep latency ≤8.2 minutes at the MSLT are valid and reliable markers for pediatric NT1 diagnosis, a result contrasting with adult NT1 criteria.Classification of evidenceThis study provides Class III evidence that for children with suspected narcolepsy, polysomnographic and MSLT markers accurately identify those with narcolepsy type 1.

The Assessment of ‘Sleepiness’ in Human Drug Trials: A New Perspective

2019 ◽  
Vol 8 (1) ◽  
pp. 5-26
Author(s):  
Murray Johns
Keyword(s):  
Sleep Latency ◽  
Sleep Onset ◽  
Face Validity ◽  
Drug Trials ◽  
Efficacy And Safety ◽  
Short Term ◽  
Maintenance Of Wakefulness Test ◽  
New Perspective ◽  
The Relationship ◽  
Multiple Sleep Latency

The investigation of the efficacy and safety of drugs requires assessments of their effects on alertness/sleepiness. Unfortunately, there is confusion about the nature of ‘sleepiness’, the factors which influence it, and how it can be measured under different circumstances. This review aims to clarify these matters and to offer some suggestions about how current difficulties might be overcome. Different meanings of the word ‘sleepiness’ are examined initially. Methods that purport to measure ‘sleepiness’ are then examined, including their testretest reliability and the relationship between the results of different measurements within the same subjects. Some objective methods are found not to be as reliable as was initially reported. Information about the reliability of several other methods is either inadequate or nonexistent. One assumption which underlies two frequently used objective methods for measuring ‘sleepiness’ (the Multiple Sleep Latency Test and the Maintenance of Wakefulness Test) is that the ‘sleepier’ a person is, the quicker they will fall asleep. While this assumption has face validity, other assumptions about these tests are re-examined and are found wanting, at least sometimes. The difficulty arises in part because it is not always clear when the sleep onset process begins and ends. ‘Sleepiness’ is found to be influenced much more by short-term factors, such as the subject’s posture at the time and during the preceding few minutes, than has been acknowledged previously. Some possible solutions to these difficulties are suggested, including a new conceptual model of sleep-wake control, with implications for the design of drug trials.

scholarly journals 0738 Advance Taper of Antidepressants Prior to Multiple Sleep Latency Testing Increases the Number of Sleep-Onset Rapid Eye Movement Periods and Reduces Mean Sleep Latency

SLEEP ◽  
2020 ◽  
Vol 43 (Supplement_1) ◽  
pp. A281-A281
Author(s):  
B Kolla ◽  
M Jahani Kondori ◽  
M Silber ◽  
H Samman ◽  
S Dhankikar ◽  
...  
Keyword(s):  
Eye Movement ◽  
Sleep Latency ◽  
Sleep Onset ◽  
Initial Assessment ◽  
Rapid Eye Movement ◽  
Regression Analyses ◽  
Practice Parameters ◽  
The Impact ◽  
Multiple Sleep Latency ◽  
Multiple Sleep Latency Testing

Abstract Introduction Patients presenting with excessive sleepiness are frequently on antidepressant medication(s). While practice parameters recommend discontinuation of antidepressants prior to multiple sleep latency testing (MSLT), data examining the impact of tapering these medications on MSLT results are limited. Methods Adult patients who underwent MSLT at Mayo Clinic Rochester, Minnesota, between 2014-2018 were included. Clinical and demographic characteristics, medications, including use of rapid eye movement suppressing antidepressants (REMS-AD) at assessment and during testing, actigraphy and polysomnography data were manually abstracted. The difference in number of sleep-onset rapid eye movement periods (SOREMS), proportion with ≥2 SOREMS and mean sleep latency (MSL) in patients who were on REMS-AD and discontinued prior to testing versus those who remained on REMS-AD were examined. At our center, all antidepressants are discontinued 2 weeks prior to MSLT wherever feasible; fluoxetine is stopped 4 weeks prior. Regression analyses accounting for demographic, clinical and other medication-related confounders were performed. Results A total of 502 patients (age=38.18±15.90 years; 67% female) underwent MSLT; 178 (35%) were on REMS-AD at the time of assessment. REMS-AD were discontinued prior to testing in 121/178 (70%) patients. Patients tapered off REMS-AD were more likely to have ≥2 SOREMS (OR-12.20; 95%CI=1.60-92.94) compared to patients who remained on REMS-AD at the time of the MSLT. They also had shorter MSL (8.77±0.46 vs 10.21±0.28; p>0.009) and higher odds of having ≥2 SOREMS (OR=2.22; 95%CI=1.23-3.98) compared to patients not on REMS-AD at initial assessment. These differences persisted after regression analyses accounting for confounders. Conclusion Patients who taper off REMS-AD prior to MSLT are more likely to demonstrate ≥2SOREMs and have a shorter MSL. Pending further prospective investigations, clinicians should preferably withdraw REMs-AD before an MSLT. If this is not done, the test interpretation should include a statement regarding the potential effect of the drugs on the results. Support None

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