Chronic myeloid leukemia with sleep-related painful erections as a first symptom: a case report.

BACKGROUND: Sleep-related painful erections are characterized by deep penile pain that occurs during erections in the rapid eye movement stage of sleep. CASE PRESENTATION: This case presents a 43-year-old Chinese Han patient with sleep-related painful erections. Turgid painful erections (4-5 episodes of tumescence) during the sleep hours caused pain. Further, blood testing revealed an abnormal increase in white blood cells (123 × 109/L). The patient was diagnosed with chronic myeloid leukemia by bone marrow biopsy, BCR::ABL1 fusion gene testing, and Philadelphia chromosome. However, the sleep-related painful erections have dramatically decreased in frequency of erectile pain after chemotherapy for Chronic myeloid leukemia in our case. CONCLUSION: We considered that the occurrence of sleep-related painful erections was related to chronic myeloid leukemia and the case might be secondary sleep-related painful erections.

Differences in polysomnographic, nocturnal penile tumescence and penile doppler ultrasound findings in men with stuttering priapism and sleep-related painful erections.

Men with Stuttering Priapism (SP) and sleep-related painful erections (SRPE) experience bothersome nocturnal painful erections resulting in poor sleep. The aim of this study is to observe common features and differences between men with SP and SRPE based on polysomnography, nocturnal penile tumescence (NPT), and penile doppler ultrasound (PDU). This is a prospective cohort study of 20 participants divided into two groups (Group 1 = SP [n = 12]; Group 2 = SRPE [n = 8]) with bothersome painful nocturnal erections. All participants were referred to the sleep disorder clinic to be assessed and consented for overnight polysomnography with simultaneous NPT recording and to complete validated sleep, sexual dysfunction and health-related quality of life questionnaires. Unstimulated PDU was also performed. Abnormal Polysomnographic findings (reduced sleep efficiency, total sleep time, and awake after sleep onset) were identified in both groups suggesting poor sleep. Men with SP had significantly longer erections (60.0 vs 18.5; p = 0.002) and took longer to detumesce once awake (25.7 vs 5.4 min; p = 0.001) than men with SRPE. They also had significantly higher peak systolic and end diastolic velocities on unstimulated PDU with an abnormal low resistance waveform identified. No sleep pathology was identified in men with SP. This implies a local (penile) etiology in men with SP. Men with SRPE had a normal resting PDU and abnormal sleep architecture with REM awakenings and significantly more Periodic limb movements (p = 0.04) than men with SP suggesting a central (sleep-related) cause in men with SRPE. Sexual dysfunction and poor HR-QoL was identified on validated questionnaires in both groups. SP and SRPE are rare entities that share similar symptoms (painful nocturnal erections and poor sleep) but dissimilar features of nocturnal erection onset, duration and resolution with different polysomnographic features which may allude to a different pathophysiology.

Sexsomnia y trastorno de alimentación relacionado con el sueño secundario a oxibato sódico / Sexsomnia and sleep eating secondary to sodium oxybate consumption

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Sleep-Related Painful Erections in a Patient With Obstructive Sleep Apnea Syndrome.

Sleep-related painful erection (SRPE) is a rare sleep disorder characterized by recurrent, painful penile erections occurring when awakening from rapid eye movement sleep, while erections are painless during wakefulness. Almost 35 cases have been reported worldwide, and only two of them had an associated obstructive sleep apnea syndrome (OSAS). We report a new case of a 61-year-old man suffering from SRPE associated with OSAS. The adequate treatment of respiratory events with continuous positive airway pressure did not alleviate the SRPE symptoms and excessive daytime sleepiness. The SRPE diagnosis was made by polysomnography coupled with video surveillance when the patient was referred to the sleep laboratory for residual excessive daytime sleepiness. The patient had 2-4 episodes of SRPE/night. Beta-blocker did not alleviate the SRPE, but a transient improvement was noted when the patient was treated with paroxetine. In contrast with the two previously published cases of SRPE plus OSAS, continuous positive airway treatment did not improve SRPE symptoms in our patient.

A case of sleep-related painful erections with chronic daytime genital discomfort.

Sleep-related painful erections (SRPE) are an uncommon condition characterized by recurrent nocturnal penile tumescence accompanied by penile pain without penile pathology, which occurs during the rapid eye movement (REM) sleep stage. A report of a 59-year-old patient with SRPE is described. Turgid painful erections (five to seven episodes of tumescence) during the sleep hours caused pain together with burning and tingling sensations in the penis and perineal zone during the daytime hours. Swelling of the pubic and perineal area was recurrent. Sleep loss, chronic fatigue, mild anxiety, lack of concentration and decreased work occurred along with this condition. Polysomnographic findings indicated REM sleep fragmentation. Attempts to treat this condition with muscle relaxants or anxiolytics did not prompt an improvement of this disorder, but a single daily dose of gabapentin 300 mg in combination with 1 mg clonazepam at bedtime improved total sleep time and reduced full sleep erections.

Desarrollos recientes en la evaluación del trastorno de conducta del sueño REM / No disponible

El trastorno de conducta del sueño REM (TCREM) es una alteración caracterizada por la ausencia de atonía muscular durante esta fase del sueño y la emergencia de conductas motoras asociadas a ensoñaciones de contenido generalmente desagradable. Entre estas conductas se incluyen acciones como gritar, saltar, agitar los brazos o dar patadas y puñetazos, que corresponden con el correlato motor de la actividad onírica, de ahí su caracterización como “actuación de los ensueños”. La necesidad de que se requiera evidencia polisomnográfica para confirmar el diagnóstico de TCREM hace que la evaluación de este problema resulte muy costosa, ya que la polisomnografía consume mucho tiempo y recursos. Por ello, el desarrollo de instrumentos de evaluación y, más particularmente, de instrumentos de cribado (screening) que permitan una detección precoz de los individuos afectados por esta patología y que, por consiguiente, podrían requerir una intervención temprana, es una de las líneas más actuales de investigación en este campo. El propósito de este trabajo es analizar los desarrollos recientes en el ámbito de la evaluación del TCREM, presentando las diversas herramientas actualmente disponibles y aportando datos acerca de sus garantías científicas(AU)

Rapid eye movement (REM) sleep behaviour disorder (RBD) is a problem characterized by loss of muscle atonia during this sleep phase and the emergence of motor behaviours generally associated to unpleasant dreams. These behaviours include yelling, jumping, arms waving, kicking and punching, working as a motor correlate for the oneiric activity, hence its characterization as “acting out” their dreams. Polysomnography is necessary to diagnose RBD and this procedure is costly. For this reason, one current research lines is the development of assessment tools and, more specifically, of screening instruments to identify patients with RBD that may require early intervention as early as possible. The aim of this study was to analyse recent developments in the clinical assessment of this disorder, presenting the various tools currently available and providing data about their scientific guarantees(AU)

Sleep-related painful erections associated with obstructive sleep apnea syndrome.

Sleep-related painful erection is a rare syndrome recognized by reports of painful nocturnal erection, an association between REM sleep and pain, and the absence of pain during wakeful sexual activity. Approximately 30 cases have been reported in the literature. We add two more cases, each of which seemed to be associated with severe sleep apnea. Treatment of the apnea with Continuous Positive Airway Pressure device lessened the symptom in both men. Implications of this association are discussed.

Does abnormal non-rapid eye movement sleep impair declarative memory consolidation?: Disturbed thalamic functions in sleep and memory processing.

Non-rapid eye movement (NREM) sleep has recently garnered support for its role in consolidating hippocampus-based declarative memories in humans. We provide a brief review of the latest research on NREM sleep activity and its association with declarative memory consolidation. Utilizing empirical findings from sleep studies on schizophrenia, Alzheimer's disease, and fibromyalgia, we argue that a significant reduction of slow-wave sleep and sleep spindle activity contribute to the development of deficits in declarative memory consolidation along with concomitant sleep disturbances commonly experienced in the aforementioned disorders. A tentative model is introduced to describe the mediating role of the thalamocortical network in disruptions of both declarative memory consolidation and NREM sleep. The hope is to stimulate new research in further investigating the intimate link between these two very important functions.

Caso clínico interactivo. Trastorno de comportamiento durante el sueño en paciente joven / No disponible

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Asociación de la rigidez peneana nocturna con testosterona, síndrome metabólico y otras variables: un estudio piloto prospectivo transversal / Association of Nocturnal Penile Rigidity with Testosterone, Metabolic Syndrome, and Other Variables: a Prospective Cross-Sectional Pilot Study

Introducción: El objetivo fue estudiar la relación entre la rigidez peneana nocturna (RPN) con el síndrome metabólico (SM) y la testosterona en varones que consultan por trastornos de erección (DE). Material y método: Se incluyeron 234 varones en un estudio piloto prospectivo y transversal. Se midieron los niveles séricos de testosterona total y biodisponible y otros parámetros bioquímicos relacionados con el SM y con las RPN. Los pacientes se agruparon según la rigidez de las erecciones: normales (alta rigidez, componente predominante psicológico de la disfunción) o anormales (baja rigidez, posible componente orgánico o físico de la DE) y por la presencia o ausencia de SM. Resultados: El modelo de regresión logística para la rigidez del pene como variable dependiente demostró que el riesgo de rigidez anormal es menor en individuos con mayor testosterona total (OR=0,96; 95% CI=0,92-0,99) o biodisponible (OR=0,91; 95% CI=0,84-0,99). Pacientes con niveles de testosterona entre 8 y 12 mmol/L presentaron un riesgo cuatro veces mayor de tener rigidez anormal comparados con aquellos con niveles superiores a 12 mmol/L (OR=3,96; 95% CI=1,89, 8,31). Si se consideraban únicamente aquellos varones sin SM, solo la edad y el índice de masa corporal (IMC) aparecían como factores de riesgo asociados a la rigidez anormal. La edad aumentó el riesgo de rigidez anormal en un 8% (OR=1,08; 95% CI=1,03-1,13) y el IMC lo aumentó en un 18% (OR=1,18; 95% CI=1,01-1,38). Conclusión: La asociación de niveles de testosterona con la rigidez del pene fue baja y desaparece si se asocia con SM (AU)

Introduction: The aim was to study whether nocturnal penile rigidity (NPTR) correlates with metabolic syndrome (MetS) and testosterone in men consulting for erectile dysfunction (ED). Material and methods: 234 men were included in a prospective, cross-sectional pilot study. Serum total and bioavailable testosterone and other biochemical constituents were measured and compared with NPTR. Patients were classified by normal or low/abnormal penile rigidity (abnormal meaning predominant organic component of ED) and presence or absence of MetS to test the hypothesized correlations. Results: Application of the logistic regression model to rigidity as the dependent variable showed the risk of low penile rigidity to be significantly lower for patients with higher total (OR=0.96, 95% CI=0.92-0.99) or bioavailable testosterone (OR=0.91, 95% CI=0.84-0.99). Patients with testosterone levels between 8 and 12 mmol/L had a quadrupled risk of low penile rigidity compared with patients with higher levels (>12 mmol/L) (OR=3.96, 95% CI=1.89-8.31). Considering men without MetS, age and body mass index were associated as significant factors for low penile rigidity: age increased risk by 8% (OR=1.08, 95% CI=1.03-1.13) and BMI increased it by 18% (OR=1.18, 95% CI=1.01-1.38). Conclusion: Testosterone levels are weakly associated with penile rigidity and disappear when associated with MetS (AU)

Validity of the Mattis Dementia Rating Scale to detect mild cognitive impairment in Parkinson's disease and REM sleep behavior disorder.

BACKGROUND/AIMS: Mild cognitive impairment (MCI) is frequent in Parkinson's disease (PD) and idiopathic REM sleep behavior disorder (iRBD). However, only a few studies have evaluated the validity of brief cognitive measures to detect MCI in PD or iRBD using standard diagnostic criteria for MCI. Our aim was to evaluate the validity of the Mini-Mental State Examination (MMSE) and the Mattis Dementia Rating Scale (DRS-2) to detect MCI in PD and iRBD. METHODS: Forty PD patients and 34 iRBD patients were studied. Receiver operating characteristic curves were created for both tests to assess their effectiveness in identifying MCI in PD and iRBD. RESULTS: In PD, a normality cutoff of 138 on the DRS-2 yielded the best balance between sensitivity (72%) and specificity (86%) with a correct classification of 80%. In iRBD, the optimal normality cutoff was 141 on the DRS-2, with a sensitivity of 90%, a specificity of 71% and a correct classification of 82%. No cutoff for the MMSE was found to have acceptable sensitivity or specificity. CONCLUSION: The DRS-2 has satisfactory validity to detect MCI in PD or iRBD. The MMSE proved to be invalid as a screening test for MCI in both populations.

REM sleep behaviour disorder (RBD) and its associations in young patients.

STUDY OBJECTIVES: To retrospectively examine the characteristics of a population of patients <50 years of age with clinical and polysomnographic features diagnostic for RBD. METHODS: Review of our sleep centre's database for patients with RBD diagnosed over the last 7 years. Ninety-one patients were separated into two groups according to their age at the time of diagnosis (<50 y and 50 y). Clinical and polysomnographic data were reviewed. RESULTS: Sixty-two were male; mean age was 52+/-19 y. Thirty-nine were <50 y. In the group <50 y there was a male predominance but in a smaller proportion (M:F=1.4:1) compared with the group 50 (M:F=3:1). Seventy-six patients complained of abnormal behaviour (AB) during sleep, 12 with narcolepsy complained of excessive daytime sleepiness (EDS) with the AB being elicited only during consultation, and three complained of both EDS and AB. All patients, except one in the group 50, described AB related to vivid dreams with violent content. The majority of the patients had the idiopathic form of RBD in both groups (51.2% group <50, 63.4% group 50). The secondary form was associated with narcolepsy in 38.4% of patients in the group <50 y and with a synucleinopathy in 28.8% of patients in the group 50. A strong association was noted between RBD and non-REM parasomnias. CONCLUSIONS: In a population of patients with RBD presenting to a regional sleep laboratory, more than one-third of patients were <50 y at time of diagnosis. The commonest associated disorder was narcolepsy in patients <50 y, and synucleinopathy in those 50 y. The coexistence of RBD with a NREM parasomnia was not uncommon in cases of idiopathic RBD affecting patients <50 y.

Is REM sleep behaviour disorder (RBD) a risk factor of dementia in idiopathic Parkinson's disease?

OBJECTIVE: To study the sequence of occurrence of REM-sleep behaviour disorder (RBD) and dementia and their frequency among a population of patients with idiopathic Parkinson's disease (PD). METHODS: We performed a cross-sectional study on 65 PD patients seen in a movement disorder clinic and their bed partner, and asked them to complete the validated Mayo Sleep Questionnaire for RBD and sleep disorders. The diagnosis of PD with dementia (PD-D) was based on a clinical diagnosis of dementia; following DSM-IV criteria and MMSE score less than 25 and a battery of cognitive tests. RESULTS: From the 65 patients that completed the study, twenty-four met the clinical diagnosis of RBD. Ten of the 24 (42%) RBD patients met the clinical criteria of PD-D, whereas the remaining 14 patients were non-demented at the time of the study. The frequency of RBD was significantly higher in the PD-D group (n = 10, 77%) compared to the PD-ND group (n = 14, 27%, chi squared test: p = 0.0008). PD non-RBD had a lower occurrence of dementia (7.3%, 3 of 41) compared to those suffering from RBD (42%, 10 of 24). Of the 65 PD patients, 13 were diagnosed with PD-D and the remaining 52 were non-demented PD (PD-ND) patients. PD with RBD showed a faster decline in the number of dementia-free patients compared to the non-RBD patients (Log Rank test: p < 0.001). RBD preceded, coincided or followed the onset of the motor symptoms. CONCLUSION: This study shows that RBD and dementia have a significant coincidence in the course of PD, and RBD not only precedes or coincides with the motor signs, but can occur during the course of the progression of the PD, suggesting a degenerative process of the dopaminergic and cholinergic neurons of the brainstem nuclei, progressing at a different pace in each patient.

Síndrome de ingesta nocturna relacionada con el sueño con respuesta al topiramato / Nocturnal sleep-related eating disorder that responds to topiramite

El síndrome de ingesta nocturna relacionada con el sueño es una parasomnia de sueño no REM, asociadaa otros trastornos del sueño, en especial al sonambulismo, crónica, no remitente y que consiste en episodios de ingesta compulsiva de alimento durante la noche con amnesia parcial o completa del episodio. Este cuadro debe ser diferenciado del síndrome de la cena durante el sueño, que es mucho más frecuente y se asocia a trastornos endocrinos y psiquiátricos, y deotros trastornos de la conducta alimentaria durante el sueño. Caso clínico. Varón de 28 años, con un cuadro diario de, al menos, 10 años de duración, consistente en episodios nocturnos de ingesta compulsiva en un estado de semisomnolencia, con amnesia del suceso a la mañana siguiente. El paciente no tenía historia de patología psiquiátrica o de otro trastorno de la alimentación,pero sí un descanso nocturno pobre, sobrepeso y antecedentes familiares y personales de otros trastornos del sueño.No respondió a otros tratamientos, por lo que se probó el topiramato con casi total desaparición de los episodios, excelente tolerancia y mantenimiento de la eficacia durante dos años de seguimiento. Conclusiones. Revisamos en este artículo lostrastornos de la conducta alimentaria durante el sueño, el síndrome de ingesta nocturna relacionada con el sueño y sus posibilidades terapéuticas, señalando la utilidad del topiramato en este cuadro

Nocturnal sleep-related eating disorder is a non-REM sleep parasomnia that is associated to othersleep disorders, especially sleepwalking. It becomes chronic, is not remitting and consists in episodes of compulsive eating during the night, which are then partially or completely forgotten by the patient. This condition must be differentiated fromnight-eating syndrome, which is far more common and is linked to endocrinological and psychiatric disorders, as well as to other disorders involving eating behaviour during sleeping hours. Case report. A 28-year-old male who had suffered from the clinical picture every day for 10 years; this condition consisted in nocturnal episodes of binge eating in a state of semisleepiness,with no remembrance of what had happened the next morning. The patient had no history of psychiatric pathologies or any other eating disorder, but he did not rest adequately at night, was overweight and had a family and personal history of other sleep disorders. Since he did not respond to other treatments, we decided to try therapy with topiramate; as a result, the episodes disappeared, tolerance was excellent and effectiveness was maintained throughout the two years’ follow-up.Conclusions. In this paper we review eating disorders that occur during sleep, nocturnal sleep-related eating disorder and its therapeutic possibilities, while highlighting the usefulness of topiramate to treat this condition