scholarly journals A Longitudinal Follow-Up Study on Multiple Sleep Latency Test and Body Mass Index of Patients With Narcolepsy Type 1 in Korea

2017 ◽  
Vol 13 (12) ◽  
pp. 1441-1444 ◽  
Author(s):  
Yoo Hyun Um ◽  
Tae-Won Kim ◽  
Jong-Hyun Jeong ◽  
Ho-Jun Seo ◽  
Jin-Hee Han ◽  
...  
2018 ◽  
Vol 27 (5) ◽  
pp. e12700 ◽  
Author(s):  
Yu-Shu Huang ◽  
Christian Guilleminault ◽  
Cheng-Hui Lin ◽  
Chia-Hsiang Chen ◽  
Wei-Chih Chin ◽  
...  

2001 ◽  
Vol 74 (1) ◽  
pp. 149-149 ◽  
Author(s):  
Robert S Lindsay ◽  
Robert L Hanson ◽  
William C Knowler

1989 ◽  
Vol 47 (1) ◽  
pp. 76-79 ◽  
Author(s):  
Rubens Reimão ◽  
Aron Diament

A case of periodic hypersomnia in an 11-year-old female with the unique features of mental deficiency, incontinentia pigmenti, acanthosis nigricans and hereditary multiple exostosis (diaphysial aclasis) is reported. The clinical, Polysomnographic and Multiple Sleep Latency test features of this case with a follow up of seven years are consistent with a diagnosis of periodic (intermittent) excessive somnolence. The unique presentation, however, does differ from Kleine-Levin syndrome and suggests a relationship between the predominantly ectodermal, congenital disorders and the sleep-wake, pattern dysfunction.


Neurology ◽  
2019 ◽  
Vol 93 (11) ◽  
pp. e1034-e1044 ◽  
Author(s):  
Fabio Pizza ◽  
Lucie Barateau ◽  
Isabelle Jaussent ◽  
Stefano Vandi ◽  
Elena Antelmi ◽  
...  

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.


2019 ◽  
Vol 32 (1) ◽  
pp. 17
Author(s):  
Sérgio Azevedo ◽  
Joana Saraiva ◽  
Francisco Caramelo ◽  
Lúcia Fadiga ◽  
Luísa Barros ◽  
...  

Introduction: The use of continuous subcutaneous insulin infusion therapy in type 1 diabetes mellitus has increased due to its benefits on glycemic control and on the lifestyle flexibility. The aim of this study was to assess the impact of continuous subcutaneous insulin infusion therapy on glycemic control, body mass index, total daily dose of insulin and complications associated with this therapy, during 20 years of experience in Centro Hospitalar e Universitário de Coimbra.Material and Methods: This retrospective study included patients with type 1 diabetes mellitus who started continuous subcutaneous insulin infusion therapy up until 2005, followed at Centro Hospitalar e Universitário de Coimbra. Glycated hemoglobin A1c, body mass index, total daily dose of insulin and acute complications associated with continuous subcutaneous insulin infusion therapy were evaluated immediately prior to initiation of continuous subcutaneous insulin infusion therapy with follow-up at six months, one year, five, 10, 15 and 20 years. The frequency of acute complications associated with this type of therapy was also evaluated.Results: This study included 20 patients (seven males, 13 females) with mean disease duration up to the start of continuous subcutaneous insulin infusion therapy of 16.1 ± 7.9 years, mean age of onset of continuous subcutaneous insulin infusion therapy of 31.1 ± 8.4 years and follow-up during 13.2 ± 2.3 years. The reasons for initiating pump therapy were: inadequate metabolic control in 15 patients, history of asymptomatic or severe hypoglycemia in four patients, and pregnancy/pregnancy planning in one patient. The previous median of glycated hemoglobin A1c was 9.3% (6.5 – 16.0) and, at six months, decreased to the minimum value of 7.2% (5.3 – 9.8); p < 0.0125. The reduction of glycated hemoglobin A1c remained statistically significant in the first 10 years of follow-up. There was a statistically significant difference in the body mass index variation at 10 years with continuous subcutaneous insulin infusion therapy compared to previous body mass index; 24.7 kg/m2 (18.9 – 31.8) vs 25,5 kg/m2 (18.9 – 38.9), p < 0.0125. Daily insulin requirements were reduced from 56.5 U (32.0 – 94.0) to 43.8 U (33.0 – 64.0) (p < 0.0125) at six months and no statistical differences were found in the remaining follow-up. There were two severe episodes of hypoglycemia (incidence 0.0095/patient/year), five episodes of diabetic ketoacidosis (0.0238/patient/year) and no infections at the site of catheter insertion.Discussion: This study shows that continuous subcutaneous insulin infusion therapy improved glycemic control, especially during the first 10 years of follow-up and allowed a significant decrease in total daily dose of insulin in the first six months. The rate of acute complications was low.Conclusion: Treatment with continuous subcutaneous insulin infusion therapy seems effective in achieving metabolic control in selected patients with type 1 diabetes mellitus.


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