Federica Provini

Publications (73)287.56 Total impact

• Article: Tobacco habits in nocturnal frontal lobe epilepsy.

  Ilaria Naldi, Francesca Bisulli, Luca Vignatelli, Laura Licchetta, Francesca Pittau, Lidia Di Vito, Barbara Mostacci, Veronica Menghi, Federica Provini, Pasquale Montagna, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: The beneficial effect of nicotine has been reported in autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) patients, but not tested in sporadic cases. Recently, a nicotine defect in the arousal pathway has been hypothesized even in sporadic NFLE patients and their relatives. This case-control family study was designed to test whether NFLE subjects were more likely to use tobacco than controls, as an indirect marker of cholinergic arousal system dysregulation. At least four relatives were included for each NFLE proband and control. Each subject was questioned about tobacco habits; 434 individuals were recruited. Moreover, we compared NFLE patients with age- and sex-matched controls to determine whether they are more likely to use tobacco. We found a slightly higher trend of tobacco use in NFLE probands compared to that in control subjects; we did not find any significant difference in the distribution of tobacco use among NFLE group compared to that in the control group.
  Epilepsy & Behavior 12/2012; · 2.34 Impact Factor
• Article: The parasomnias: Mechanisms and treatment.

  Paolo Tinuper, Francesca Bisulli, Federica Provini
  [show abstract] [hide abstract]
  ABSTRACT: Although parasomnias should be considered benign conditions without a deleterious impact on sleep quality and quantity, especially in children, it is important to recognize and properly diagnose these phenomena. Moreover, parasomnias may be misdiagnosed as epileptic seizures, in particular seizures with a predominant complex motor behavior as seizures occurring in nocturnal frontal lobe epilepsy (NFLE), leading to unnecessary and expensive investigations and prolonged and unsuccessful treatment. In this article we describe the clinical and neurophysiologic features of the most common parasomnias, giving the most reliable elements of differential diagnosis between parasomnias and epileptic nocturnal seizures, namely the typical seizures occurring in NFLE. The diagnostic value of history-taking, video-polysomnography, home video recording, and diagnostic scales is discussed. Next we describe the intriguing aspect of the frequent coexistence, in the same family and even in the same patients, of epileptic and parasomniac attacks, giving a common neurophysiologic interpretation. Finally some brief indications to the treatment of parasomnias are suggested.
  Epilepsia 12/2012; 53 Suppl 7:12-9. · 3.96 Impact Factor
• Article: Abnormal medial thalamic metabolism in patients with idiopathic restless legs syndrome.

  Giovanni Rizzo, Caterina Tonon, Claudia Testa, David Manners, Roberto Vetrugno, Fabio Pizza, Sara Marconi, Emil Malucelli, Federica Provini, Giuseppe Plazzi, Pasquale Montagna, Raffaele Lodi
  [show abstract] [hide abstract]
  ABSTRACT: Pathophysiology of restless legs syndrome is poorly understood. A role of the thalamus, specifically of its medial portion which is a part of the limbic system, was suggested by functional magnetic resonance imaging and positron emission tomography studies. The aim of this study was to evaluate medial thalamus metabolism and structural integrity in patients with idiopathic restless legs syndrome using a multimodal magnetic resonance approach, including proton magnetic resonance spectroscopy, diffusion tensor imaging, voxel-based morphometry and volumetric and shape analysis. Twenty-three patients and 19 healthy controls were studied in a 1.5 T system. Single voxel proton magnetic resonance spectra were acquired in the medial region of the thalamus. In diffusion tensor examination, mean diffusivity and fractional anisotropy were determined at the level of medial thalamus using regions of interest delineated to outline the same parenchyma studied by spectroscopy. Voxel-based morphometry was performed focusing the analysis on the thalamus. Thalamic volumes were obtained using FMRIB's Integrated Registration and Segmentation Tool software, and shape analysis was performed using the FMRIB Software Library tools. Proton magnetic resonance spectroscopy study disclosed a significantly reduced N-acetylaspartate:creatine ratio and N-acetylaspartate concentrations in the medial thalamus of patients with restless legs syndrome compared with healthy controls (P < 0.01 for both variable). Lower N-acetylaspartate concentrations were significantly associated with a family history of restless legs syndrome (β = -0.49; P = 0.018). On the contrary, diffusion tensor imaging, voxel-based morphometry and volumetric and shape analysis of the thalami did not show differences between the two groups. Proton magnetic resonance spectroscopic findings in patients with restless legs syndrome indicate an involvement of medial thalamic nuclei of a functional nature; however, the other structural techniques of the same region did not show any changes. These findings support the hypothesis that dysfunction of the limbic system plays a role in the pathophysiology of idiopathic restless legs syndrome.
  Brain 11/2012; · 9.46 Impact Factor
• Article: Cardiovascular-Sleep Interaction in Drug-Naïve Patients With Essential Grade I Hypertension.

  Daniela Grimaldi, Federica Provini, Giovanna Calandra-Buonaura, Giorgio Barletta, Annagrazia Cecere, Giulia Pierangeli, Pietro Cortelli
  [show abstract] [hide abstract]
  ABSTRACT: Lack of nighttime blood pressure (BP) reduction by 10-20% from the mean daytime values (dipping) has been described as a distinguishing feature of essential hypertension and associated, also in normotensive subjects, with increased cardiovascular (CV) risk. Mechanisms involved in the loss of the nocturnal dip are still unclear, but involvement of autonomic nervous system (ANS) activity probably plays a crucial role. Sleep is fundamental in modulating ANS activity to maintain the physiological BP circadian rhythm, and for this reason its integrity has been widely investigated in hypertension. We investigated, under controlled conditions, the autonomic control of the CV system through an autonomic reflex screen in the awake condition and by assessment of circadian rhythm-, day-night-, time-, and state-dependent changes of BP and heart rate (HR) and associated sleep parameters in patients with a recent (≤1 yr) diagnosis of essential grade I hypertension naïve of therapy. Fourteen hypertensive patients (6 males, age: 43 ± 11 yrs; body mass index [BMI]: 24 ± 3 kg/m(2)) were compared with 28 healthy controls matched for sex, age, BMI (2 controls/patient) for cardiovascular reflex and to 8 different subjects from previous controls (6 males), comparable for age and BMI, for the day-night and nighttime CV profiles during two consecutive nights. The cardiovascular reflex screen data showed increased sympathetic effect in hypertensive patients, represented by higher overshoot of BP after Valsalva maneuver. Nighttime sleep architecture during the dark period in terms of duration, representation of sleep stages, sleep fragmentation, and incidence of arousals-periodic limb movements in sleep (PLMS) and PLMS arousals-was similar in patients and controls. Hypertensive patients displayed higher 24-h BP and HR values, but their sleep-related BP decrease was significantly reduced compared with controls. The circadian rhythms of BP and HR were intact and similar in patients and controls, coupling with the expected physiological peak time. BP and HR showed normal state-dependent modulation in hypertensive patients that, however, was higher in all sleep stages compared with controls. The lowering of systolic blood pressure (SBP) during non-rapid eye movement (NREM) sleep stages 1 and 2 and REM sleep, relative to daytime wake values, was significantly attenuated in the hypertensive group, whereas it was comparable to controls during slow-wave sleep. In hypertensive patients, analysis of sleep and CV parameters in the 90 min following sleep onset and preceding morning awakening showed normal depressor effect during the first part of the night after sleep onset and significantly higher BP rise in the hours preceding morning awakening. These findings were associated with comparable sleep architecture, sleep fragmentation, incidence of arousals, and PLMS and PLMS arousals in patients and controls. Our data suggest that drug-naïve essential grade I hypertension is associated with signs of increased vascular sympathetic response to standardized stress of the Valsalva maneuver during the awake condition, and during sleep with a non-dipping BP profile plus higher BP surge preceding morning awakening, assessable only by around-the-clock ambulatory BP monitoring, both representing additional CV risk already in early-stage hypertension and, therefore, requiring proper selection of pharmacological treatment. (Author correspondence: pietro.cortelli@unibo.it ).
  Chronobiology International 10/2012; · 4.03 Impact Factor
• Source

  Available from: Paolo Allegrini

  Article: Thalamic contribution to Sleep Slow Oscillation features in humans: a single case cross sectional EEG study in Fatal Familial Insomnia.

  Angelo Gemignani, Marco Laurino, Federica Provini, Andrea Piarulli, Giorgio Barletta, Paola d'Ascanio, Remo Bedini, Raffaele Lodi, David Neil Manners, Paolo Allegrini, Danilo Menicucci, Pietro Cortelli
  [show abstract] [hide abstract]
  ABSTRACT: Studying the thalamic role in the cortical expression of the Sleep Slow Oscillation (SSO) in humans by comparing SSO features in a case of Fatal Familial Insomnia (FFI) and a group of controls. We characterize SSOs in a 51-year-old male with FFI carrying the D178N mutation and the methionine/methionine homozygosity at the polymorphic 129 codon of the PRNP gene and in eight gender and age-matched healthy controls. Polysomnographic (21 EEG electrodes, two consecutive nights) and volumetric- (Diffusion tensor imaging Magnetic Resonance Imaging DTI MRI) evaluations were carried out for the patient in the middle course of the disease (five months after the onset of insomnia; disease duration: 10 months). We measured a set of features describing each SSO event: the wave shape, the event-origin location, the number and the location of all waves belonging to the event, and the grouping of spindle activity as a function of the SSO phase. We found that the FFI individual showed a marked reduction of SSO event rate and wave morphological alterations as well as a significant reduction in grouping spindle activity, especially in frontal areas. These alterations paralleled DTI changes in the thalamus and the cingulate cortex. This work gives a quantitative picture of spontaneous SSO activity during the NREM sleep of a FFI individual. The results suggest that a thalamic neurodegeneration specifically alters the cortical expression of the SSO. This characterization also provides indications about cortico-thalamic interplays in SSO activity in humans.
  Sleep Medicine 05/2012; 13(7):946-52. · 3.40 Impact Factor
• Article: Nocturnal epileptic seizures versus the arousal parasomnias

  Pasquale Montagna, Federica Provini, Francesca Bisulli, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: We review here the clinical features of Nocturnal Frontal Lobe Epilepsy (NFLE) and the arousal parasomnias, to define possible differential diagnostic criteria and their inter-observer reliability, and to determine whether both conditions share a common disturbance of arousal mechanisms. Polysomnographic recordings defined several types of seizures in NFLE: Paroxysmal Arousals (PA), Nocturnal Paroxysmal Dystonia (NPD), Epileptic Nocturnal Wanderings (ENW). There are however several difficulties with the diagnosis of NFLE, not the least being that EEG discharges during the seizures are not universally found. These diagnostic difficulties are highlighted, as well as the fact that studies of the inter-observer reliability of the video polysomnographic features of NFLE were found unsatisfactory, especially for the PA. When NFLE patients and their relatives were matched to control subjects and analysed in regard to personal and family history for the parasomnias, the arousal parasomnias were reported significantly higher by patients with NFLE and their relatives. This higher recurrence of a personal and family history for the parasomnias in NFLE patients suggests that the two conditions share a common underlying mechanism. Based on evidence from Autosomal Dominant NFLE, a genetic disorder with mutations in nicotinic acetylcholine receptors(nAchR), we hypothesise that arousal parasomnias and NFLE both, have abnormal arousal mechanisms in the dorsal cholinergic arousal branch triggering peculiar motor patterns in the medial frontal lobe regions. Fragestellung Es werden Studien bewertet, die klinische Bilder nächtlicher Frontallappenepilepsie und Arousal-Parasomnien vergleichen. Differential-diagnostische Kriterien und deren Interrater-Reliabilität werden bestimmt. Es wird untersucht, ob bei den Erkrankungen der gleiche Arousalmechanismus zugrunde liegt. Patienten und Methoden Patienten mit Frontallappen epilepsie wurden videopolysomnographisch untersucht und ihre Anfälle wurden typisiert: Paroxysmale Arousal (PA), nächtliche paroxysmale Dystonie (NPD) und epileptische nächtliche Wanderungen (ENW). Die Patienten wurden mit Kontrollpersonen gematcht und ihre persönliche und Familienanamnese bezogen auf Parasomnien erhoben. Ergebnisse Das Auftreten von Arousalparasomnien war bei Patienten mit Frontallappenepilepsie und ihren Verwandten signifikant häufiger. Die Interrater-Reliabilität der Videopolysomnographie war unbefriedigend, besonders bezogen auf PA. Schlussfolgerungen Verlässliche diagnostische Kriterien für die nächtliche Frontallappenepilepsie zur Unterscheidung von Arousal-Parasomnien werden benötigt, insbesondere in Anbetracht der oft negativen iktalen EEG-Ergebnisse. Das häufige Auftreten von Parasomnien bei Patienten mit Frontallappenepilepsie lässt vermuten, dass ähnliche Mechanismen zugrunde liegen. Aus Untersuchungen bei autosomal dominanter Frontallappenepilepsie, einer genetischen Erkrankung mit Mutationen am nikotinergen Azetylcholinrezeptor, kann abgeleitet werden, dass Arousalparasomnien und Frontallappenepilepsien den gleichen gestörten Arousalmechanismus haben, bei dem im dorsal cholinergen Arousal-Zweig bestimmte motorische Muster in der medial frontalen Region ausgelöst werden.
  Somnologie - Schlafforschung und Schlafmedizin 04/2012; 12(1):25-37.
• Article: Physiologic autonomic arousal heralds motor manifestations of seizures in nocturnal frontal lobe epilepsy: implications for pathophysiology.

  Giovanna Calandra-Buonaura, Nicola Toschi, Federica Provini, Ivan Corazza, Francesca Bisulli, Giorgio Barletta, Stefano Vandi, Pasquale Montagna, Maria Guerrisi, Paolo Tinuper, Pietro Cortelli
  [show abstract] [hide abstract]
  ABSTRACT: This study describes changes in heart rate (HR) and HR variability (HRV) related to clinical onset of seizures in nocturnal frontal lobe epilepsy (NFLE) in order to determine whether signs of autonomic activation precede onset of seizure motor manifestations, which was selected as seizure onset (SO). Further, to clarify the nature (epileptic or physiologic) of the changes in autonomic cardiac control presumed to precede SO, time-dependent variations in HR and HRV related to physiological cortical arousals associated with motor activity (phases of transitory activation, PAT) were also investigated. HR and HRV spectral power, quantified by means of wavelet transform, were analyzed in relation to the onset of motor manifestations in 45 NFLE seizures and 45 PAT derived from whole night video-polysomnographic recordings of ten patients and of ten control subjects, respectively. Analysis of HRV showed a shift of sympathetic/parasympathetic cardiac control toward a sympathetic predominance in the 10s immediately preceding SO, while changes in HR were evident only one second before SO. This sympathetic activation was not associated with a sleep-wake transition or changes in respiratory activity, both of which occurred concurrently with SO. Similar changes in HR and HRV were observed in the 10s before the motor and electroencephalographic onset of PAT. Our study demonstrates that a similar autonomic activation precedes the motor manifestations of NFLE seizures and physiological arousal. This autonomic activation could represent part of the arousal response, which could be implicated in the occurrence of both seizure and arousal motor manifestations.
  Sleep Medicine 03/2012; 13(3):252-62. · 3.40 Impact Factor
• Article: Video-polysomnographic study of a patient with Morvan's Fibrillary Chorea.

  Bartolo Lanuzza, Debora Aricò, Filomena I I Cosentino, Federica Provini, Raffaele Ferri
  Sleep Medicine 02/2012; 13(5):550-3. · 3.40 Impact Factor
• Article: Abnormal sleep-cardiovascular system interaction in narcolepsy with cataplexy: effects of hypocretin deficiency in humans.

  Daniela Grimaldi, Giovanna Calandra-Buonaura, Federica Provini, Patrizia Agati, Giulia Pierangeli, Christian Franceschini, Giorgio Barletta, Giuseppe Plazzi, Pasquale Montagna, Pietro Cortelli
  [show abstract] [hide abstract]
  ABSTRACT: Narcolepsy with cataplexy (NC) is associated with loss of hypocretin neurons in the lateral hypothalamus involved in the circadian timing of sleep and wakefulness, and many biologic functions including autonomic control. The authors investigated whether chronic lack of hypocretin signaling alters cardiovascular control during sleep in humans. Comparison of 24-hr circadian rhythms, day-night, time- and state-dependent changes of blood pressure (BP) and heart rate (HR) in drug-free patients with NC and control subjects. University hospital. Ten drug-free patients with NC (9 men, 1 woman) and 12 control subjects (9 men, 3 women). N/A. Daytime BP was comparable in patients with NC and controls, but patients with NC displayed a nighttime nondipping BP pattern. The 24-hr circadian rhythmicity of BP and HR was normal in both groups. Systolic BP during nighttime rapid eye movement sleep was significantly increased in the NC group. The 24-hr HR was significantly higher in the NC group but the day-night and state-dependent HR modulations were intact. The nighttime BP pattern coupled in the NC group with increased sleep fragmentation and a higher prevalence of arousals, periodic limb movements in sleep (PLMS), and PLMS arousals. In an analysis of the sleep/cardiovascular interaction in the periods after sleep onset and preceding morning awakening, only PLMS were consistently associated with the blunted nighttime decrease in BP in the NC group. Hypocretin deficiency in humans may couple with an altered nighttime BP regulation that can be associated with an increased cardiovascular risk. This finding may be the result not only of the hypocretinergic deficiency per se but also of the altered sleep/wake regulation characterizing NC.
  Sleep 01/2012; 35(4):519-28. · 5.05 Impact Factor
• Article: Parasomnias and nocturnal frontal lobe epilepsy (NFLE): lights and shadows--controversial points in the differential diagnosis.

  Francesca Bisulli, Luca Vignatelli, Federica Provini, Chiara Leta, Elio Lugaresi, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: Nocturnal frontal lobe epilepsy (NFLE) is characterized by seizures with complex, often bizarre, violent behaviour arising only or mainly during sleep. These unusual seizures and their occurrence during sleep are often accompanied by normal EEG tracings and neuroradiological findings, making it difficult to distinguish NFLE seizures from other non-epileptic nocturnal paroxysmal events, namely parasomnias. NFLE was described for the first time in 1981, but, as its epileptic origin was controversial, the condition was called nocturnal paroxysmal dystonia. Even though many aspects of parasomnias and NFLE have been clarified in the last two decades, the problem of differential diagnosis remains a challenge for clinicians. This paper discusses some controversial points still under debate. The difficulties in distinguishing nocturnal epileptic seizures from parasomnias reflect just one aspect of the intriguing issue of the pathophysiological relationships between all types of paroxysmal motor behaviours during sleep.
  Sleep Medicine 12/2011; 12 Suppl 2:S27-32. · 3.40 Impact Factor
• Article: Agrypnia excitata.

  Elio Lugaresi, Federica Provini, Pietro Cortelli
  [show abstract] [hide abstract]
  ABSTRACT: The concept of Agrypnia excitata (AE) was originally proposed as a concept deriving from the clinical and anatomo-pathological observations obtained in three different diseases, Fatal familial insomnia (FFI), Delirium tremens (DT), and Morvan syndrome (MS). Agrypnia refers to a condition of severely reduced or absent sleep due to organic disorders. Excitata refers to the association of agrypnia with generalized motor and autonomic hyperactivation. AE is a syndrome that has been claimed to relate to a dysfunction in the thalamo-limbic circuits that govern sleep-wake cycles and autonomic activities.
  Sleep Medicine 12/2011; 12 Suppl 2:S3-10. · 3.40 Impact Factor
• Article: Nocturnal frontal lobe epilepsy: new pathophysiological interpretations.

  Paolo Tinuper, Francesca Bisulli, Federica Provini, Pasquale Montagna, Elio Lugaresi
  [show abstract] [hide abstract]
  ABSTRACT: Since the first descriptions of Nocturnal Frontal Lobe Epilepsy (NFLE) many theories have been proposed to explain its pathophysiological mechanisms. The aim of this paper is to formulate a tentative hypothesis designed to unify the clinical, anatomo-physiological, and genetic aspects underlying this condition. According to this hypothesis, NFLE is due to a disorder in the thalamocortical circuit involved in the arousal mechanism. Other cortical-networks involving the limbic system may explain, for instance, primitive behaviors. The role of the cholinergic system and related pathways in the pathogenesis of nocturnal seizures and parasomnias is also discussed.
  Sleep Medicine 12/2011; 12 Suppl 2:S39-42. · 3.40 Impact Factor
• Article: Arousal disorders.

  Federica Provini, Paolo Tinuper, Francesca Bisulli, Elio Lugaresi
  [show abstract] [hide abstract]
  ABSTRACT: Arousal Disorders (AD) are motor behaviours arising from NREM sleep. They comprise a spectrum of manifestations of increasing complexity from confusional arousal to sleep terror to sleepwalking. AD usually appear in childhood with a low frequency of episodes and spontaneously disappear before adolescence. The advent of video-polysomnography disclosed the existence of other phenomena alongside AD, in particular nocturnal frontal lobe seizures, requiring a differential diagnosis from AD. History-taking is usually sufficient to establish a correct diagnosis of AD even though viewing the episodes is essential for the clinician to distinguish the different motor events. Videopolysomnographic recording in a sleep laboratory is not always necessary and homemade video-recordings are useful to capture events closest to real life episodes.
  Sleep Medicine 12/2011; 12 Suppl 2:S22-6. · 3.40 Impact Factor
• Article: Oneiric stupor: the peculiar behaviour of agrypnia excitata.

  Pietro Guaraldi, Giovanna Calandra-Buonaura, Rossana Terlizzi, Pasquale Montagna, Elio Lugaresi, Paolo Tinuper, Pietro Cortelli, Federica Provini
  [show abstract] [hide abstract]
  ABSTRACT: Agrypnia excitata (AE) is a syndrome characterized by the inability to sleep associated with a generalized motor and autonomic over-activation. AE is caused by a thalamo-limbic system dysfunction and comprises three different conditions: Fatal Familial Insomnia (FFI), Delirium Tremens (DT), and Morvan Syndrome (MS). Oneiric Stupor episodes (OS) are the peculiar motor behaviour of AE. During OS patients perform simple automatic gestures mimicking daily-life activities. This paper is the first description of the different characteristics of OS in two patients with MS and another with FFI, emphasizing the specific clinical features that reliably differentiate OS from REM sleep behaviour disorders.
  Sleep Medicine 12/2011; 12 Suppl 2:S64-7. · 3.40 Impact Factor
• Article: Diagnostic accuracy of a structured interview for nocturnal frontal lobe epilepsy (SINFLE): a proposal for developing diagnostic criteria.

  Francesca Bisulli, Luca Vignatelli, Ilaria Naldi, Francesca Pittau, Federica Provini, Giuseppe Plazzi, Carlotta Stipa, Chiara Leta, Pasquale Montagna, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: To measure the accuracy of anamnestic features collected during clinical history for the diagnosis of nocturnal frontal lobe epilepsy (NFLE). A case-control diagnostic study. Participants included a case group of people with ascertained target disease (NFLE group) and a control group of people with sleep disorders potentially confounding for NFLS (NOT-NFLE group), defined by means of a consensus procedure among experts (panel diagnosis as reference standard). Two major clinical patterns defining the semeiology of the epileptic event (i.e. dystonic, DP, and/or hyperkinetic pattern, HP), and 13 additional minor features were identified, formulated as questions, and telephonically administered to NFLE and NOT-NFLE groups by a trained doctor blinded to the final diagnosis. The diagnostic accuracy of each characteristic was tested against the reference standard. Out of 262 selected subjects, 101 were recruited; 42 were NFLE and 59 NOT-NFLE. A positive history of DP or HP had a sensitivity of 59.5% and a specificity of 91.5%, irrespective of the other minor anamnestic features. The anamnestic model improved, with a sensitivity of 59.5% and specificity of 96.6%, if at least one of the following four minor anamnestic features was added: (a) duration less than two minutes, (b) unstructured vocalization during the episode, (c) experience of an aura preceding the motor attack, and (d) a history of tonic-clonic seizures during sleep. The present study disclosed two major anamnestic patterns and four minor features that we called SINFLE, with unsatisfactory sensitivity but high specificity. These patterns could be the basis for developing future NFLE diagnostic criteria and to quantify the diagnostic accuracy of elements usually collected in the clinical history.
  Sleep Medicine 12/2011; 13(1):81-7. · 3.40 Impact Factor
• Article: Morvan chorea and agrypnia excitata: when video-polysomnographic recording guides the diagnosis.

  Federica Provini, Sara Marconi, Marcello Amadori, Pietro Guaraldi, Giulia Pierangeli, Pietro Cortelli, Elio Lugaresi, Pasquale Montagna, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: Morvan chorea is an antibody-mediated limbic encephalopathy characterized by severe insomnia, mental confusion, hallucinations, enacted dreams, hyperhidrosis, and neuromyotonia. In a 78 years old man presenting with progressive insomnia apathy and depression, a video-polysomnogram documented enacted dreams mimicking daily life activity (oneiric stupor). This finding led us to perform a search for serum antibodies to voltage-gated K+ channels, which was positive. A diagnosis of Morvan chorea was done. The patient underwent plasma exchange with complete resolution of the clinical picture. Oneiric stupor may represent a useful precocious diagnostic marker in Morvan chorea.
  Sleep Medicine 12/2011; 12(10):1041-3. · 3.40 Impact Factor
• Article: Breathing instability in Joubert syndrome.

  Margherita Fabbri, Roberto Vetrugno, Federica Provini, Marcello Bosi, Margherita Santucci
  Movement Disorders 11/2011; 27(1):64. · 4.51 Impact Factor
• Source

  Available from: Mauro Ursino

  Article: Slow eye movements distribution during nocturnal sleep.

  Fabio Pizza, Margherita Fabbri, Elisa Magosso, Mauro Ursino, Federica Provini, Raffaele Ferri, Pasquale Montagna
  [show abstract] [hide abstract]
  ABSTRACT: To assess the distribution across nocturnal sleep of slow eye movements (SEMs). We evaluated SEMs distribution in the different sleep stages, and across sleep cycles in nocturnal recordings of 10 healthy women. Sleep was scored according to standard criteria, and the percentage of time occupied by the SEMs was automatically detected. SEMs were differently represented during sleep stages with the following order: wakefulness after sleep onset (WASO): 61%, NREM sleep stage 1: 54%, REM sleep: 43%, NREM sleep stage 2: 21%, NREM sleep stage 3: 7%, and NREM sleep stage 4: 3% (p<0.0001). There was no difference between phasic and tonic REM sleep. SEMs progressively decreased across the NREM sleep cycles (38%, 15%, 13% during NREM sleep stage 2 in the first three sleep cycles, p=0.006), whereas no significant difference was found for REM, NREM sleep stage 1, slow-wave sleep and WASO. Our findings confirm that SEMs are a phenomenon typical of the sleep onset period, but are also found in REM sleep. The nocturnal evolution of SEMs during NREM sleep stage 2 parallels the homeostatic process underlying slow-wave sleep. SEMs are a marker of sleepiness and, potentially, of sleep homeostasis.
  Clinical neurophysiology: official journal of the International Federation of Clinical Neurophysiology 02/2011; 122(8):1556-61. · 3.12 Impact Factor
• Article: FULL-LENGTH ORIGINAL RESEARCH: Increased frequency of arousal parasomnias in families with nocturnal frontal lobe epilepsy: A common mechanism?

  Francesca Bisulli, Luca Vignatelli, Ilaria Naldi, Laura Licchetta, Federica Provini, Giuseppe Plazzi, Lidia Di Vito, Simona Ferioli, Pasquale Montagna, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: Purpose:  Retrospective observations disclosed an overlap between parasomnias and nocturnal frontal lobe epilepsy (NFLE) not only in patients but also in their relatives, suggesting a possible common pathogenetic mechanism. This study aimed to verify whether relatives of patients with NFLE have a higher frequency of parasomnias, namely arousal disorders, and thereby shed light on the still unknown pathophysiologic mechanisms underlying NFLE. Methods:  We undertook a case-control family study in which we recruited NFLE probands and healthy controls, matched for age, sex, education, and geographic origin. At least four relatives were included for each proband and control. Each subject underwent a standardized interview, with application of the International Classification of Sleep Disorders-Revised (ICSD-R 2001) minimal criteria to diagnose the lifetime prevalence of the main parasomnias. Results:  Four hundred fifty-eight individuals were recruited: 33 NFLE probands, 200 relatives of probands, 31 controls, and 194 control relatives. All NFLE probands but one have sporadic NFLE. The lifetime prevalence of the following parasomnias differed in proband relatives versus control relatives: arousal disorders [odds ratio (OR) 4.7, 95% confidence interval (CI) 2.0-11.6; p < 0.001] and nightmares (OR 2.6, 95% CI 1.6-4.2; p < 0.001) were more frequent among NFLE proband relatives. In the secondary analysis comparing NFLE probands to controls, arousal disorders (OR 6.3, 95% CI 1.3-31.7; p = 0.023) and bruxism (OR 5.4, 95% CI 1.3-21.7; p = 0.017) were more frequent among NFLE probands. Discussion:  The higher frequency of arousal disorders in NFLE families suggests an intrinsic link between parasomnias and NFLE and an abnormal (possibly cholinergic) arousal system as a common pathophysiologic mechanism.
  Epilepsia 09/2010; 51(9):1852-60. · 3.96 Impact Factor
• Article: Increased frequency of arousal parasomnias in families with nocturnal frontal lobe epilepsy: a common mechanism?

  Francesca Bisulli, Luca Vignatelli, Ilaria Naldi, Laura Licchetta, Federica Provini, Giuseppe Plazzi, Lidia Di Vito, Simona Ferioli, Pasquale Montagna, Paolo Tinuper
  [show abstract] [hide abstract]
  ABSTRACT: Retrospective observations disclosed an overlap between parasomnias and nocturnal frontal lobe epilepsy (NFLE) not only in patients but also in their relatives, suggesting a possible common pathogenetic mechanism. This study aimed to verify whether relatives of patients with NFLE have a higher frequency of parasomnias, namely arousal disorders, and thereby shed light on the still unknown pathophysiologic mechanisms underlying NFLE. We undertook a case-control family study in which we recruited NFLE probands and healthy controls, matched for age, sex, education, and geographic origin. At least four relatives were included for each proband and control. Each subject underwent a standardized interview, with application of the International Classification of Sleep Disorders-Revised (ICSD-R 2001) minimal criteria to diagnose the lifetime prevalence of the main parasomnias. Four hundred fifty-eight individuals were recruited: 33 NFLE probands, 200 relatives of probands, 31 controls, and 194 control relatives. All NFLE probands but one have sporadic NFLE. The lifetime prevalence of the following parasomnias differed in proband relatives versus control relatives: arousal disorders [odds ratio (OR) 4.7, 95% confidence interval (CI) 2.0-11.6; p < 0.001] and nightmares (OR 2.6, 95% CI 1.6-4.2; p < 0.001) were more frequent among NFLE proband relatives. In the secondary analysis comparing NFLE probands to controls, arousal disorders (OR 6.3, 95% CI 1.3-31.7; p = 0.023) and bruxism (OR 5.4, 95% CI 1.3-21.7; p = 0.017) were more frequent among NFLE probands. The higher frequency of arousal disorders in NFLE families suggests an intrinsic link between parasomnias and NFLE and an abnormal (possibly cholinergic) arousal system as a common pathophysiologic mechanism.
  Epilepsia 09/2010; 51(9):1852-60. · 3.96 Impact Factor