Objective: To evaluate the subjective sleep disturbances such as impaired sleep quality, excessive daytime sleepiness (EDS), worry, insomnia, and Willis Ekbom Disease (restless leg syndrome) in Myasthenia Gravis. Background: Sleep impairment symptoms such as non-restorative night sleep, excessive daytime sleepiness and insomnia have been commonly observed in patients with Mysthenia Gravis. Design/Methods: This is a prospective two-site study with 58 Myasthenia Gravis patients answering six sleep related questionnaires: Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Penn State Worry Questionnaire (PSWQ), Beck Depression Inventory (BDI), Insomnia Severity Index (ISI), and International RLS (Restless Leg Syndrome) Study Group Rating Scale. Results: Thirty-one of the 58 subjects (53.4[percnt]) were female and the mean age was 54.6+/-18.1 (20-88) years. The mean duration of disease was 85.4+/-113.7 (range 3-504) months, 24 (41.4[percnt]) subjects had a thymectomy and 46 (79.3[percnt]) subjects were taking prednisone, either alone or with pyridostigmine and/ or another immunosuppressant medication. The results of the 6 sleep related questionnaires revealed: 37 (63.8[percnt]) have poor sleep quality (PSQI score>=5 ); all report having sleep disturbance (PSQI-c5 >=1); 14 (24.1[percnt]) report EDS (ESS >=10); 29 (50.0[percnt]) show moderate worry level (PSWQ >=40); 26 (44.8[percnt]) have subthreshold insomnia level (ISI > 7) while 13 (22.4[percnt]) experiences moderate or severe insomnia (ISI >= 15); and 16 (27.6[percnt]) suffer from moderate or more severe RLS symptoms. Conclusions: Two thirds of our subjects with Myasthenia Gravis had poor sleep quality. Other sleep disturbances indications such as excessive daytime sleepiness; worry; insomnia, and restless legs syndrome symptoms were also commonly recorded in approximately one quarter of the subjects. Correlation of sleep disturbances with disease severity, anthropometric characteristics and medications will be evaluated. Study Supported by the Myasthenia Gravis Foundation of California (MGFC). Drs. Wang, Goyal, Mozaffar and Chui are members of the MGFC Medical Advisory Board. Disclosure: Dr. Wang has nothing to disclose. Dr. Le has nothing to disclose. Dr. Goyal has nothing to disclose. Dr. Mozaffar received personal compensation from Genzyme Corporation and Grifols for speaking engagements and has served as an advisory board member for Amicus, Biogen idec, Biomarin, Genzyme, Idera Pharmaceuticals and Ultragenyx. Dr. Mozaffar has receive Dr. Chui has nothing to disclose. Dr. Hungs has received personal compensation for activities with EMD Serono, Pfizer and Teva as a speaker.

Sleep disorders are frequent in patients with neurologic disease. This article provides an approach to the patient with sleep complaints that can be implemented during the course of a neurologic evaluation. Recognition of a sleep complaint is the key that leads to use of appropriate scales and sleep diaries to form a differential sleep diagnosis. Choosing the correct sleep test is essential to confirm diagnosis and plan therapy. We describe the important sleep tests for practicing neurologists: polysomnography, multiple sleep latency test, and maintenance of wakefulness test, as well as actigraphy and oximetry. The approved use of limited channel home tests (also known as "out of center testing," or "portable monitoring") is reviewed and an algorithm provided to guide the approach to the sleepy patient.

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Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA HomeNew OnlineCurrent IssueFor Authors Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA journal

PURPOSE: Limited studies suggest premedication of patients undergoing initial polysomnography (PSG) for suspected sleep-disordered breathing (SDB) with a nonbenzodiazepine hypnotic agent improves study quality and efficacy of positive airway pressure (PAP) titration. The effects of ramelteon, a melatonin agonist non-controlled substance hypnotic, are unknown in this setting. We compare the effects of eszopiclone and ramelteon on diagnostic quality, sleep architecture and efficacy of PAP titration during split night PSG.

We performed a retrospective chart review on 53 muscle‐specific kinase antibody (MuSK‐Ab)‐positive myasthenia gravis (MG) patients at nine university‐based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9–79 years. Twenty‐seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long‐term (≥3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG‐related death. This survey reinforces several cardinal features of MuSK‐Ab‐positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long‐term outcome is favorable in about 60% of cases. Muscle Nerve, 2009

PURPOSE: The effects of nonbenzodiazepine hypnotics on the quality of polysomnography (PSG) in patients with suspected sleep-disordered breathing (SDB) is unclear. We compare the effects of eszopiclone and ramelteon on PSG quality and efficacy of continuous positive airway pressure (CPAP) titration.

Antonios Michalos, Rajarsi Gupta, Christopher O Olopade, Daniel L Picchiett, Marcel Hungs, and Enrico Gratton. Cerebrovascular responses in OSA patients during CPAP therapy measured in the frontal lobes of the brain bilaterally, simultaneously, and non-invasively using frequency-domain near-infrared spectroscopy. International Conference of the American Thoracic Society. May 15–20, 2009. San Diego, CA. Am J Respir Crit Care Med. 2009; 179, A3554. OSA is associated with severe cardio/cerebrovascular morbidity. Our goal is the development and introduction of Frequency−Domain Near−Infrared Spectroscopy (FD−NIRS) in Clinical Diagnostics and Sleep Medicine to identify patients at risk. FD−NIRS allows non−invasive, continuous, lengthy, and real−time measurements of cerebral tissue oxygenation and hemodynamics by measuring absolute hemoglobin (oxygenated, deoxygenated, and total) concentrations. We performed FD−NIRS concomitantly to conventional polysomnography in 135 subjects. Cerebrovascular autoregulatory responses were evaluated with an Absolute NIRS Brain Oximeter (OxiplexTS, ISS, Champaign, IL) in the frontal lobes bilaterally. After rigorous NIRS screening criteria, 40 controls and 40 OSA patients were considered. The reported findings are from a specific Severe OSA Cohort (N=5 OSA, age range 49−55, 4 males/1 female, AHI 55−90) within the total OSA group, where the subjects serve as their own controls. We are reporting quantitative measurements of cerebral tissue hemodynamic variables during the initial 3−6 hours of diagnostic PSG and subsequently, during 3−6 hours of CPAP titration. Our results (4 NIRS variables/hemisphere/second/3−6hrs/subject) suggest that there is a wide spectrum of inter− and intra−hemispheric hemodynamic responses which are unique to each OSA patient before and after CPAP and non−uniform within the total OSA cohort. Our future work will focus on a larger sample and evaluation of mechanisms that may explain the variability in cerebral tissue hemodynamics after restoration of ordered breathing and peripheral hypoxemia achieved by CPAP therapy. This abstract is funded by: NIH/NINDS R44NS040597.

BACKGROUND/OBJECTIVE:Spinal angiolipoma (SAL) is an uncommon clinico-pathological entity.DESIGN:Single case report.METHODS:Retrospective data analysis.FINDINGS:An obese woman with a 1-year history of progressive spastic paraparesis and acute deterioration underwent magnetic resonance imaging of the thoracic spine, the results of which suggested a tumor compressing the thoracic spinal cord. The histopathological examination of the completely resected tumor revealed an epidural angiolipoma.CONCLUSIONS:This case report offers a reminder that SAL should be considered in the differential diagnosis of long-standing, slowly progressive paraparesis. It remains unclear whether an increased body mass index might be a contributing factor to the development of SAL.

The findings in a stillborn female fetus of 31 weeks' gestation with congenital Gaucher disease, nonimmune hydrops/erythroblastosis, infantile arterial calcification, and neonatal hepatitis/fibrosis are presented, the first report of this complete constellation. Prior reports describe two similar patients. One lacked the hepatocellular features of giant cell hepatitis although manifesting hepatic fibrosis; the second lacked hepatic pathology. The diagnosis of Gaucher disease herein was established by microscopic examination of the proband, enzymatic analysis of trophoblast, and enzymatic and genetic study of the parents. The father was heterozygous for a recombinant glucocerebrosidase gene; the mother demonstrated a unique frame shift mutation. Thus the fetus is a compound heterozygote for a null and a severe mutation. Studies of parental DNA were negative for the D409H mutation of type IIIc Gaucher disease. Genetic studies were not performed of the ENPP1 gene, mutations of which are associated with idiopathic infantile arterial calcification.