The Lehigh Valley Health Network narcolepsy cohort: clinical and polysomnographic analysis of 304 cases.

Study Objective: We aimed to characterize clinical features, comorbidities and polysomnographic characteristics of a large cohort of patients with narcolepsy.

Methods: We undertook a retrospective chart and polysomnographic review of all patients with a diagnosis of narcolepsy type 1 (NT1) or narcolepsy type 2 (NT2) seen within the Lehigh Valley Health Network between 2000 and 2022.

Results: We found 304 cases with a diagnosis of narcolepsy (52 NT1, 252 NT2), based on , third edition criteria.

Cataplexy Mistaken for Seizures in a Patient With Undiagnosed Narcolepsy Type I.

Narcolepsy Type 1 is a sleep disorder, with cataplexy as its cardinal feature, characterized by sudden decrease or loss of muscle tone triggered by strong emotions. Cataplexy can be misdiagnosed as epileptic seizures given its clinical similarity to atonic seizures. The low prevalence of the disease added another layer of complexity in providing timely and accurate diagnosis.

Treatment of REM sleep behavior disorder with trazodone: report of 3 cases.

Unlabelled: Rapid eye movement sleep behavior disorder is a sleep disturbance characterized by the absence of regular paralysis during rapid eye movement sleep, accompanied by dream enactment behavior. The available pharmacotherapy options for treating rapid eye movement sleep behavior disorder are limited, and the utilization of antidepressants has yielded mixed results. We report 3 cases of isolated rapid eye movement sleep behavior disorder improved with trazodone.

Seizure control with treatment of delayed sleep-wake phase disorder in juvenile myoclonic epilepsy: A case report.

Juvenile Myoclonic Epilepsy (JME) is an idiopathic generalized epilepsy associated with a characteristic sleep/wake rhythm, with the tendency to go to bed later at night, to get up later in the morning. In the pediatric population, we have previously observed specific circadian and sleep/wake patterns of generalized seizures (6 am-12 pm) and myoclonic seizures (in wakefulness, 6 am to noon). Delayed Sleep-Wake Phase Disorder (DSWPD) is characterized by sleep initiation insomnia when attempting sleep at conventional times and difficulty waking at the required time.

Images: Irregular sleep-wake rhythm disorder in transgender individuals.

Unlabelled: Growing evidence suggests that transgender individuals face a significant health disparity and are particularly vulnerable to sleep disorders. We present two patients who developed irregular sleep-wake rhythm disorder after gender reassignment and hormone replacement therapy. The growing interest in transgender health warrants further evaluation of the effects and frequency of all sleep disorders in this population.

Perspective: A resident's role in promoting safe machine-learning tools in sleep medicine.

Unlabelled: Residents and fellows can play a helpful role in promoting safe and effective machine-learning tools in sleep medicine. Here we highlight the importance of establishing ground truths, considering key variables, and prioritizing transparency and accountability in the development of machine-learning tools within the field of artificial intelligence. Through understanding, communication, and collaboration, in-training physicians have a meaningful opportunity to help progress the field toward safe machine-learning tools in sleep medicine.

Emulsion PCR (ePCR) as a Tool to Improve the Power of DGGE Analysis for Microbial Population Studies.

To the authors' knowledge, this is the first report of the use of emulsion-Polymerase chain reaction (e-PCR) coupled with denaturing gradient gel electrophoresis (DGGE) analysis. In the present work the effectiveness of ePCR in improving the power of the DGGE technique for microbial population studies was tested. Our results indicated that ePCR results in uniform amplification of several DNA molecules, overcoming the major limitations of conventional PCR, such as preferential amplification and DNA concentration dependence.

Automating Interictal Spike Detection: Revisiting A Simple Threshold Rule.

Interictal spikes (IIS) are bursts of neuronal depolarization observed electrographically between periods of seizure activity in epilepsy patients. However, IISs are difficult to characterize morphologically and their effects on neurophysiology and cognitive function are poorly understood. Currently, IIS detection requires laborious manual assessment and marking of electroencephalography (EEG/iEEG) data.

A Case of Recurrent Hypersomnia With Autonomic Dysfunction.

We report the case of a 50-year-old man with disabling recurrent hypersomnia with autonomic instability due to catatonia in the setting of atypical bipolar disorder. Treatment with valproic acid for bipolar disorder resulted in complete resolution of symptoms.

Antioxidant properties of different products and additives in white wine.

Different winemaking products (ascorbic acid, glutathione, yeast lees and a yeast autolysate) were tested in comparison with sulphur dioxide, concerning radical scavenging activity (measured by DPPH assay), oxygen consumption capacity and ability to reduce wine colour and predisposition to browning. Trials were performed in white wines and model solution. SO2 was the most active in reducing wine colour development.

Localization of sleep spindles, k-complexes, and vertex waves with subdural electrodes in children.

Purpose: To describe for the first time in children the localization of sleep spindles, K-complexes, and vertex waves using subdural electrodes.

Methods: We enrolled children who underwent presurgical evaluation of refractory epilepsy with subdural grid electrodes. We analyzed electroencephalogram data from subdural electrodes and simultaneous recording with Cz scalp electrode.

Comparison of pediatric patients with status epilepticus lasting 5-29 min versus ≥30 min.

The most common thresholds for considering prolonged seizures as status epilepticus (SE) are 5 and 30 min. It is unknown whether these different thresholds (5 or 30 min) identify patient populations with different electroclinical characteristics. We compared the characteristics of patients with SE lasting 5-29 min (SE5-29) with those with SE lasting ≥30 min (SE≥30).

Use of propidium monoazide for the enumeration of viable Brettanomyces bruxellensis in wine and beer by quantitative PCR.

Brettanomyces bruxellensis is a current problem in winemaking all over the world, and the question if B. bruxellensis has a positive or negative impact on wine is one of the most controversial discussions in the world. The presence of live B.

Central sleep apnea and complex sleep apnea in patients with epilepsy.

Purpose: We sought to examine the prevalence of central sleep apnea (CSA) and complex sleep apnea (CompSA) in patients with epilepsy and to examine their clinical profile, with respect to epilepsy type, etiology, medication use, and EEG abnormalities.

Methods: We undertook a retrospective analysis of 719 consecutive patients with epilepsy who underwent polysomnography (PSG) at our institution between 2004 and 2011. Of the 458 patients with complete data, we excluded 42 patients with congestive heart failure or left ventricular ejection fraction <40 %.

Use of propidium monoazide for the enumeration of viable Oenococcus oeni in must and wine by quantitative PCR.

Malolactic fermentation is an important step in winemaking, but it has to be avoided in some cases. It's carried out by lactic acid bacteria belonging mainly to the genus Oenococcus, which is known to be a slow growing bacterium. Classical microbiological methods to enumerate viable cells of Oenococcus oeni in must and wine take 7-9 days to give results.

Insomnia and epilepsy: a questionnaire-based study.

Study Objectives: Although disturbed sleep has been frequently reported in patients with seizures, little is known about insomnia and epilepsy. The aims of this study were (1) to analyze the prevalence and degree of insomnia in patients with epilepsy, (2) to examine the clinical features and correlates of insomnia in these patients, and (3) to evaluate the impact of poor sleep on their quality of life.

Methods: One hundred-fifty-two patients with epilepsy (mean age 46 years) completed the following questionnaires: Insomnia Severity Index, Pittsburgh Sleep Quality Index, Beck Depression Inventory-II, Quality of Life in Epilepsy Inventory-31.

Clinical evolution of seizures: distribution across time of day and sleep/wakefulness cycle.

Seizures can evolve sequentially into different clinical phases. For example, a seizure may start as an aura (first phase), then evolve into a tonic seizure (second phase), and evolve further into a generalized tonic-clonic semiology (third phase). It is currently unknown whether specific seizure evolutions cluster at particular times of the day and/or during sleep/wakefulness.

Dacrystic seizures: demographic, semiologic, and etiologic insights from a multicenter study in long-term video-EEG monitoring units.

Purpose: To provide an estimate of the frequency of dacrystic seizures in video-electroencephalography (EEG) long-term monitoring units of tertiary referral epilepsy centers and to describe the clinical presentation of dacrystic seizures in relationship to the underlying etiology.

Methods: We screened clinical records and video-EEG reports for the diagnosis of dacrystic seizures of all patients admitted for video-EEG long-term monitoring at five epilepsy referral centers in the United States and Germany. Patients with a potential diagnosis of dacrystic seizures were identified, and their clinical charts and video-EEG recordings were reviewed.

Circadian patterns of generalized tonic-clonic evolutions in pediatric epilepsy patients.

Objective: To investigate the sleep/wake, day/night, and 24-h periodicity of pediatric evolution to generalized tonic-clonic seizures (GTC).

Methods: Charts of 407 consecutive patients aged 0-21 years undergoing continuous video-EEG monitoring for epilepsy were reviewed for the presence of GTC evolution. Seizures were characterized according to 2001 ILAE terminology.

Effect of treatment of obstructive sleep apnea on seizure outcomes in children with epilepsy.

A retrospective review of children with epilepsy and obstructive sleep apnea, treated surgically for their obstructive sleep apnea from January 2008-October 2010, was performed for age, sex, type of epilepsy, antiseizure medications, sleep-study data, and changes in seizure frequency. Twenty-seven subjects (median age, 5 years) with no adjustment to their medications around their time of surgery were identified. Three months after surgery, 10 (37%) patients became seizure-free, three (11%) demonstrated >50% seizure-reduction, and six (22%) exhibited an amelioration of seizure frequency.

Diurnal and sleep/wake patterns of epileptic spasms in different age groups.

Purpose: Epileptic spasms are seizures that occur predominantly in children and are characterized by clusters of brief axial movements. Epileptic spasms may occur in the context of a variety of syndromes. Previous research has found that epileptic spasms occur in a sleep/wake and diurnal rhythm.

Outcomes of epileptic spasms in patients aged less than 3 years: single-center United States experience.

Retrospective review was performed of children aged <3 years with epileptic spasms at our center from 2004-2010. Short-term (<6 months) and long-term (≥6 months) outcomes were assessed. We included 173 children (104 boys; median age of onset, 6.

Analysis of EEG patterns and genotypes in patients with Angelman syndrome.

We prospectively analyzed EEGs from participants in the ongoing NIH Rare Diseases Clinical Research Network Angelman Syndrome Natural History Study. Of the one-hundred-sixty enrolled patients (2006-2010), 115 had complete data (58 boys, median age 3.6 years).

Effect of continuous positive airway pressure treatment on seizure control in patients with obstructive sleep apnea and epilepsy.

In patients with epilepsy, improvement in seizure control with treatment of coexisting obstructive sleep apnea (OSA) has been reported, but there is lack of data on the effect of continuous positive airway pressure (CPAP) compliance on seizure control in these patients. We examined the variability in seizure frequency in patients who were CPAP compliant and those who were not CPAP compliant. We undertook a retrospective review of clinical and polysomnographic data of adult patients with OSA and epilepsy seen at the Boston University Medical Center Epilepsy and Sleep Clinics between 2000 and 2010.