Right-to-left shunt and obstructive sleep apnea in cluster headache

Introduction: Cluster headache (CH) is a trigeminal autonomic cephalalgia characterized by extremely painful, strictly unilateral, headache attacks accompanied by ipsilateral autonomic symptoms. Only few studies investigated a possible role of right-to-left shunt (R-to-LS) and sleep apnea (OSA) in cluster pathogenesis or expression and no prior studies were located that combined the two conditions in CH patients. Objective: To define the potential combined effect of right-to-left shunt and obstructive sleep apnea in patients with cluster headache and their possible influence on the frequency of attacks and on response to oxygen therapy of headache attacks. Methods: 33 patients with cluster headache were recruited and subsequently invited to undergo polysomnography and a transcranial doppler bubble study. Polysomnography is used for the diagnosis of obstructive sleep apnea whereas transcranial doppler bubble study can help diagnose a cardiac right-to-left shunt. Results: Transcranial doppler results demonstrated that 10 out of 31 patients in our cohort had a right-to-left shunt (RLS). Polysomnography revealed that 10 out of 32 patients had obstructive sleep apnea (OSAS). Nineteen out of 33 subjects had one of the two conditions but only one of our 33 patients had both conditions simultaneously. In this sample patients with clear seasonality to their cluster attacks had a higher frequency of obstructive sleep apnea than patients without seasonality. Also a good response to oxygen treatment of the attacks was higher in OSAS patients. Conclusion: the presence of RLS or OSAS, by their possible influence on blood oxygenation, seems to be independently able to predispose to cluster headache or to make it clinically manifest, while the hypothesizable synergistic role between them in favoring cluster headache was not put in evidence. Additionally, our study suggested that the seasonality of cluster headache, may be influenced by the seasonal nature of obstructive sleep apnea. Finally, the presence of sleep breathing alterations seems to be also able to modulate the efficacy of oxygen inhalation on cluster headache attacks

Consensus paper on the use of BIVA (Bioeletrical Impendance Vector Analysis) in medicine for the management of body hydration

Department of Emergency Medicine, Sapienza University, Post Graduate School in Emergency-Urgency Medicine, Sant'Andrea Hospital, Rome, Italy University of North Dakota School of Medicine and Biomedical Sciences Grand Forks, ND, USA Department of Medicine, University of Padova, Italy Department of Emergency Medicine, Cleveland Clinic, Cleveland, OH, USA Department of Nephrology, Dialysis and Dietology, SM della Misericordia Hospital, Rovigo, Italy Cardiovascular Department, San Filippo Neri Hospital, Rome, Italy Department of Nephrology Dialysis and Transplantation, International Renal Research Institute (IRRIV), San Bortolo Hospital, Vicenza, Italy GREAT: Global Research in Acute Conditions Team Networ

Drug withdrawal along with bridge therapy with methylprednisolone and/or diazepam for treatment of medication overuse headache. A preliminary report from the WASH-OUT study

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Chronic inflammatory demyelinating polyneuropathy as a possible novel component of autoimmune poly-endocrine-candidiasis-ectodermal dystrophy.

8We describe two unrelated boys with autoimmune poly-endocrine-candidiasis-ectodermal dystrophy syndrome (APECED) who, in addition to manifesting the most common symptoms (chronic mucocutaneous candidiasis, hypoparathyroidism and Addison's disease), developed progressive muscular weakness in both the proximal and distal limbs, sensory loss and absent tendon reflexes. Electrophysiological studies disclosed a reduction of nerve conduction velocity in both patients that was consistent with the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).This diagnosis was supported by histological demyelination in nerve biopsy specimens with patchy CD4, CD8 and CD68-positive cell infiltration in the first patient and increased protein content in the cerebrospinal fluid in the second patient. Our cases represent the first report of an association between APECED and CIDP, in which peripheral nerve demyelination may represent a novel disease component in APECED. Our findings highlight the need to explore apparently rare manifestations in patients with APECED.nonenoneValenzise M; Meloni A; Betterle C; Giometto B; Autunno M; Mazzeo A; Cao A; De Luca FValenzise, M; Meloni, A; Betterle, C; Giometto, B; Autunno, M; Mazzeo, A; Cao, A; De Luca,

Identification of the infant-type R631C mutation in patients with the benign muscular form of CPT2 deficiency.

Carnitine palmitoyltransferase 2 (CPT2) deficiency is the most common defect of mitochondrial fatty acid oxidation; three different clinical phenotypes have been described but the adult form, involving exclusively the skeletal muscle, is the most frequent. We describe herein 3 families where 4 individuals manifested with the adult form of CPT2 deficiency. CPT2 gene molecular analysis identified the homozygous R631C mutation, so far only reported in severe infantile cases. Our data evidenced that R631C mutation is not exclusively detected in the infantile form but it may be present in a wider spectrum of CPT2 phenotypes. These findings indirectly suggest that other modulators may influence clinical severity of CPT2 deficiency

Noninvasive hemodynamic monitoring in emergency patients with suspected heart failure, sepsis and stroke: the premium registry.

INTRODUCTION: Noninvasive hemodynamic (HD) assessments in the emergency department (ED) might assist in the diagnosis, therapeutic plan development and risk stratification of acutely ill patients. This multinational observational study was designed to initiate noninvasive HD measurements prior to any ED patient therapeutic interventions and broadly evaluate them for potential diagnostic, therapeutic and predictive value. METHODS: We enrolled patients with suspected acute heart failure (AHF), sepsis or stroke. Continuous noninvasive HD monitoring was begun using the Nexfin finger cuff device (Edwards LifeSciences, BMEYE, Amsterdam, Netherlands). Beat-to-beat HD measurements were averaged for the initial 15 minutes, prior to therapeutic intervention. We performed suspected disease group comparisons and evaluated HD predictors of 30-day mortality. RESULTS: Of 510 patients enrolled: 185 (36%) AHF, 194 (38%) sepsis and 131 (26%) stroke. HD variables were significantly different (p<0.05) amongst the groups. Cardiac output and index and stroke volume index (SVI) were highest in sepsis (6.5, 3.5, 36), followed by stroke (5.5, 2.7, 35.8), and lowest in AHF (5.4, 2.7, 33.6). The in-group HD standard deviations and ranges measurements were large, indicating heterogeneous underlying HD profiles. Presenting SVI predicted 30-day mortality for all groups. CONCLUSION: Presenting ED noninvasive HD data has not been previously reported in any large patient population. Our data suggest a potential role for early noninvasive HD assessments aiding in diagnosing of patients, individualizing therapy based on each person's unique HD values and predicting 30-day mortality. Further studies and analyses are needed to determine how HD assessments should be best used in the ED

Chronic migraine in the first COVID-19 lockdown. the impact of sleep, remote working, and other life/psychological changes

Aims: The objective of this study was to evaluate the impact of the first Italian COVID-19 lockdown on patients with chronic migraine (CM). Material and methods: The study was based on an e-mail survey addressed to CM patients of our headache center. The survey evaluated demographic, life style, sleep, psychological, and migraine features during the COVID-19 lockdown period and the month before. The outcomes were migraine impact on daily life and variation in attack frequency, attack duration, migraine pain intensity, migraine symptomatic drugs use per week, and efficacy. Results: Ninety-two patients completed the survey. During the lockdown period, attack frequency was stable in 40,2%, increased in 33,7%, and reduced in 26,1% of patients; attack duration was stable in 55,4%, increased in 23,9%, and reduced in 20,7%. Migraine pain was stable or reduced in 65,2% and increased in 34,8%; number of symptomatic drugs per week was stable in 50%, reduced in 29,3%, and increased in 20,7%; migraine drug efficacy was stable in 73,9%, reduced in 17,4%, and increased in 8,7%. Patients had a HIT-6 score of 64,63 ± 8,81. Significant associations were found with remote working, smoke, education, discontinuation of the therapy performed within headache center, migraine familiarity, sleep, anxiety, perceived stress, concern about future, and COVID-19. Conclusion: During the lockdown, approximately half of the patients had a clinical stability, a quarter an improvement, and another quarter a worsening. We identified different migraine-influencing elements; in particular, the remote working could represent an easy way to ameliorate migraineurs’ life

ConsensusPaper on the use of BIVA (BioelectricalImpedanceVector Analysis) in medicine for the managment of bodyhydratation.

Hydration status assessment remains a challenge overall in sub- clinical hyper/dehydration status. Biompedance Vector Analysis (BIVA) is a non invasive technique to estimate body mass and water composition by bioelectrical impedance measurements, resistance and reactance. The BIVA method is a unique non invasive technique that allows the combined evaluation of hydration and mass of soft tissues in any clinical condition without knowledge of body weight. This tool can aid physicians to make a fast and correct assessment in facilitating the management of hydration status in critical ill patients in different medicine settings (Cardiology, Nephrology,Gastroenterology, Internal Medicine, Geriatrics and Emergency Medicine). In Nephrology BIVA contribute to the appropriate diuretic prescriptions in chronic kidney diseases and to the identification of the adequate volume of fluid removed during hemodialysis (reducing hypotension and cramp episode, preventing nocturnal pulmonary edema, ameliorating blood pressure control and maintaining an effective diuresis for long time). In heart failure patients the combined use of BIVA, biomarkers and bedside ultrasonography allows to: a faster and more accurate diagnosis, distinguish cardiogenic and non cardiogenic dyspnea, support decisions about diuretic therapy, an accurate risk stratification of patients. Ultrafiltration is increasingly recognized as a crucial area of patients with advanced heart failure and fluid overload. The key management goal is careful monitoring of fluid status with early treatment and application of a systematic approach to managing patient during UF The role of combined technology in assessing. Hydration has to be defined, but BIVA seems to be of potential value for detecting adequately changes in hydration. In critical patients arriving at ED, earlier treatment is associated with decreased mortality .BIVA represents a new technology with the potential to objectively define congestive status in ED patients with AHF Further, it may have utility in identifying patients who require volume overload reduction therapy, as well as serving as an aid to ED disposition. Future studies aimed to better elucidate the role of BIVA in the management of dehydrated patients should be performed to standardize the use of this technique in these critical patients