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BACKGROUND AND PURPOSE The aim was to assess functional and radiological outcomes after bridging therapy (intravenous thrombolysis plus mechanical thrombectomy) versus direct mechanical thrombectomy (MT) in unknown onset stroke patients. METHODS A cohort study was conducted on prospectively collected data from unknown onset stroke patients who received endovascular procedures at ≤6 h from symptom recognition or awakening time. RESULTS Of the 349 patients with a 10-point Alberta Stroke Program Early Computed Tomography Score (ASPECTS), 248 received bridging and 101 received direct MT. Of the 134 patients with 6-9-point ASPECTS, 123 received bridging and 111 received direct MT. Each patient treated with bridging was propensity score matched with a patient treated with direct MT for age, sex, study period, pre-stroke disability, stroke severity, type of stroke onset, symptom recognition to groin time (or awakening to groin time), ASPECTS and procedure time. In the two matched groups with 10-point ASPECTS (n = 73 vs. n = 73), bridging was associated with higher rates of excellent outcome (46.6% vs. 28.8%; odds ratio 2.302, 95% confidence interval 1.010-5.244) and successful recanalization (83.6% vs. 63%; odds ratio 3.028, 95% confidence interval 1.369-6.693) compared with direct MT; no significant association was found between bridging and direct MT with regard to rate of symptomatic intracerebral hemorrhage (0% vs. 1.4%). In the two matched groups with 6-9-point ASPECTS (n = 45 vs. n = 45), no significant associations were found between bridging and direct MT with regard to rates of excellent functional outcome (44.4% vs. 31.1%), successful recanalization (73.3% vs. 76.5%) and symptomatic intracerebral hemorrhage (0% vs. 0%). CONCLUSIONS Bridging at ≤ 6 h of symptom recognition or awakening time was associated with better functional and radiological outcomes in unknown onset stroke patients with 10-point ASPECTS.

Central sleep apnoea (CSA) is a lack of drive to breathe during sleep, which can occur in physiologic as well as in pathologic conditions. A particular type of CSA, defined treatment-emergent CSA (TECSA), may occur after the treatment of obstructive sleep apnoea syndrome (OSAS), either with CPAP or surgery. TECSA is transitory and seems to be related to the severity of OSAS. We describe a 51-year-old man affected by severe OSAS who developed severe, transient CSA immediately after upper airways surgery. We believe that CSA was triggered by the sudden variation in nocturnal arterial PCOApnee notturne centrali post chirurgia disostruttiva delle prime vie aeree nei pazienti affetti da OSAS.L’Apnea Centrale nel Sonno (CSA) è caratterizzata da un mancato input a compiere l’atto respiratorio durante il sonno e può verificarsi sia in condizioni fisiologiche che in condizioni patologiche. Un particolare tipo di CSA, definito come “Apnea Centrale nel Sonno Emergente dal Trattamento” (TECSA), può verificarsi dopo il trattamento della Sindrome delle Apnee Ostruttive durante il Sonno (OSAS), sia dopo ventiloterapia con CPAP che dopo intervento chirurgico. La TECSA è di solito transitoria e sembra correlata alla gravità dell’OSAS. Descriviamo un uomo di 51 anni affetto da OSAS grave, che ha sviluppato gravi e transitorie apnee centrali nel sonno, immediatamente dopo l’intervento chirurgico delle vie aeree superiori. Ipotizziamo che la CSA sia stata innescata dalla brusca variazione dei valori notturni di PCO

Background and purpose Autoimmune encephalitis (AE) represents a complex syndrome with diverse clinical manifestations and therapeutic outcomes. The aim of this study was to report the clinical characteristics and the long-term outcome of patients with paraneoplastic and idiopathic AE. Methods All patients with subacute encephalopathy admitted to the Neurology Department of our Institution from January 2012 to May 2019 were consecutively enrolled. Patients' serum and cerebrospinal fluid were tested for neural-specific autoantibodies by indirect immunofluorescence assays on mouse brain, rat neurons, cell-based assays and immunoblots. Outcome was assessed by the modified Rankin Scale score. Results From 107 adult patients with subacute encephalopathy, 50 patients were finally diagnosed with AE. Neural antibodies (Abs) were detected in 45/50 patients (90%). Leucine-rich glioma-inactivated protein 1 immunoglobulin G was the most frequent (6/50, 12%) Ab specific to neural surface antigens detected in adults with AE. Paraneoplastic encephalitis was diagnosed in 16/50 patients (32%). The presence of bilateral temporal lobe lesions on magnetic resonance imaging and cerebrospinal fluid restricted oligoclonal bands was associated with a higher probability to detect cancer at the time of AE diagnosis. All patients with Abs to neural surface antigens had a good outcome at last follow-up. Severe disability at AE onset and the lack of long-term immunosuppression predicted a poor outcome. Conclusions Leucine-rich glioma-inactivated protein 1 immunoglobulin G was the most frequent Ab detected. Patients with bilateral temporal lobe lesions and oligoclonal bands have a higher probability to harbour an occult tumour. In these patients, a strict surveillance and monitoring for cancer detection is recommended.

Background: \ud Patent foramen ovale (PFO) is a contributor to embolic stroke of undetermined source (ESUS). Subgroup analyses from previous studies suggest that anticoagulation could reduce recurrent stroke compared with antiplatelet therapy. We hypothesised that anticoagulant treatment with rivaroxaban, an oral factor Xa inhibitor, would reduce the risk of recurrent ischaemic stroke compared with aspirin among patients with PFO enrolled in the NAVIGATE ESUS trial.\ud \ud Methods: \ud NAVIGATE ESUS was a double-blinded, randomised, phase 3 trial done at 459 centres in 31 countries that assessed the efficacy and safety of rivaroxaban versus aspirin for secondary stroke prevention in patients with ESUS. For this prespecified subgroup analysis, cohorts with and without PFO were defined on the basis of transthoracic echocardiography (TTE) and transoesophageal echocardiography (TOE). The primary efficacy outcome was time to recurrent ischaemic stroke between treatment groups. The primary safety outcome was major bleeding, according to the criteria of the International Society of Thrombosis and Haemostasis. The primary analyses were based on the intention-to-treat population. Additionally, we did a systematic review and random-effects meta-analysis of studies in which patients with cryptogenic stroke and PFO were randomly assigned to receive anticoagulant or antiplatelet therapy.\ud \ud Findings: \ud Between Dec 23, 2014, and Sept 20, 2017, 7213 participants were enrolled and assigned to receive rivaroxaban (n=3609) or aspirin (n=3604). Patients were followed up for a mean of 11 months because of early trial termination. PFO was reported as present in 534 (7·4%) patients on the basis of either TTE or TOE. Patients with PFO assigned to receive aspirin had a recurrent ischaemic stroke rate of 4·8 events per 100 person-years compared with 2·6 events per 100 person-years in those treated with rivaroxaban. Among patients with known PFO, there was insufficient evidence to support a difference in risk of recurrent ischaemic stroke between rivaroxaban and aspirin (hazard ratio [HR] 0·54; 95% CI 0·22–1·36), and the risk was similar for those without known PFO (1·06; 0·84–1·33; pinteraction=0·18). The risks of major bleeding with rivaroxaban versus aspirin were similar in patients with PFO detected (HR 2·05; 95% CI 0·51–8·18) and in those without PFO detected (HR 2·82; 95% CI 1·69–4·70; pinteraction=0·68). The random-effects meta-analysis combined data from NAVIGATE ESUS with data from two previous trials (PICSS and CLOSE) and yielded a summary odds ratio of 0·48 (95% CI 0·24–0·96; p=0·04) for ischaemic stroke in favour of anticoagulation, without evidence of heterogeneity.\ud \ud Interpretation: \ud Among patients with ESUS who have PFO, anticoagulation might reduce the risk of recurrent stroke by about half, although substantial imprecision remains. Dedicated trials of anticoagulation versus antiplatelet therapy or PFO closure, or both, are warranted.\ud \ud Funding: \ud Bayer and Janssen.

Background:The pathogenesis of otitis media is related to Eustachian tube dysfunction. The tensor veli palatini muscle actively opens the Eustachian tube and promotes middle-ear ventilation. This study describes a technique for paratubal electromyography that uses a surface, non-invasive electrode able to record tensor veli palatini muscle activity during swallowing.Methods:Twenty otitis media patients and 10 healthy patients underwent tensor veli palatini electromyography. Activity of this muscle before and after Eustachian tube rehabilitation was also assessed.Results:In 78.5 per cent of patients, the electromyography duration phase and/or amplitude were reduced in the affected side. The muscle action potential was impaired in all patients who underwent Eustachian tube rehabilitation.Conclusion:This study confirmed that Eustachian tube muscle dysfunction has a role in otitis media pathogenesis and showed that muscle activity increases after Eustachian tube rehabilitation therapy.

Sleep disordered breathings (SDB) worsens the clinical prognosis of stroke patients. Continuous positive airway pressure (CPAP) is a promising effective treatment. Unfortunately, not all patients are compliant with CPAP, suggesting that it is not appropriate for all patients with obstructive sleep apnoea (OSA) after stroke. People with the highest likelihood of benefiting have to be identified. We present a classification of cases with stroke and SDB to be adopted in order to identify the best responders to CPAP treatment. We propose to classify patients in four subgroups: (1) patients who terminate the apnoea by arousing from sleep; these cases are those affected either by an anatomical or a functional obstruction of upper airways that may precede or are the consequence of stroke; (2) cases that alternate OSA to central sleep apnoea (CSA) cause of an altered loop gain; (3) cases in whom ischemic damages have altered the sleep microstructure (CAP); (4) cases that manifest a CSA as the direct consequence of stroke on the central neuronal drive to breath. So far, no study has investigated the consequences of stroke on sleep microstructure. In order to better elucidate these relationships, when reviewing the PSG tracings of stroke patients, the microstructure of sleep should be systematically analysed.

Dittoni S, Mazza M, Losurdo A, Testani E, Di Giacopo R, Marano G,Di Nicola M, Farina B, Mariotti P, Mazza S, Della Marca G.Psychological functioning measures in patients with primary insomniaand sleep state misperception.Acta Neurol Scand 2013: DOI: 10.1111/ane.12078.© 2013 John Wiley & Sons A/SObjective – Sleep state misperception (SSM) is a term used in theInternational Classification of Sleep Disorders to indicate people whomistakenly perceive their sleep as wakefulness. SSM is a form ofprimary insomnia. The aim of this study was to record psychologicalfunctioning measures (anxiety, depression, ability to feel pleasure,obsessive–compulsive traits) in a population of patients with primaryinsomnia and to evaluate the relationship between these measures andthe patients’ perception of their sleep. Materials and Methods –Seventy-six consecutive patients with primary insomnia were enrolled:34 men and 42 women, mean age 53.9 13.1. Sleep study includedthe following: Pittsburgh Sleep Quality Index, Epworth SleepinessScale, Berlin’s Questionnaire and home-based polysomnography.Psychometric evaluation included the following: Self-AdministeredAnxiety Scale, Beck’s Depression Inventory, Maudsley’s ObsessiveCompulsive Inventory, Snaith–Hamilton Pleasure Scale, EatingAttitude Test. Results – All patients with insomnia had psychometricscores higher than the general population, but very few patients, inboth groups, had anxiety or depression scores consistent with severemood or anxiety disorders. Comparisons between subjective andobjective scores confirmed that most sleep parameters wereunderestimated. Patients with SSM had lower anxiety scores ascompared to patients without SSM. Conclusions – The study did notsucceed in identifying any predictor of sleep misperception.We speculate that a group of patients, rather than being extremelyworried by their insomnia, may have a sort of agnosia of their sleep.

Nowadays oral appliance therapy is recognised as an effective therapy for many patients with primary snoring and mild to moderate obstructive sleep apnoea (OSA), as well as those with more severe OSA who cannot tolerate positive airway pressure (PAP) therapies. For this reason, it is important to focus on objective criteria to indicate which subjects may benefit from treatment with a mandibular advancement device (MAD). Various anthropometric and polysomnographic predictors have been described in the literature, whereas there are still controversies about the role of drug-induced sleep endoscopy (DISE) and advancement bimanual manoeuvre as predictor factors of treatment outcome by oral device. Herein, we report our experience in treatment of mild moderate OSA by oral appliance selected by DISE. We performed a single institution, longitudinal prospective evaluation of a consecutive group of mild moderate patients with obstructive sleep apnoea syndrome who underwent DISE. During sleep endoscopy, gentle manoeuvre of mandibular advancement less than 5 mm was performed. In 30 of 65 patients (46.2%) we obtained an unsuccessful improvement of airway patency whereas in 35 of 65 patients (53.8%) the improvement was successful and patients were considered suitable for oral device application. Because 7 of 35 patients were excluded due to conditions interfering with oral appliance therapy, we finally treated 28 patients. After 3 months of treatment, we observed a significant improvement in the Epworth medium index [(7.35 ± 2.8 versus 4.1 ± 2.2 (p0.05)], in mean AHI [(21.4 ± 6 events per hour versus 8.85 ± 6.9 (p0.05)] and in mean ODI [(18.6 ± 8 events per hour to 7 ± 5.8 (p0.05)]. We observed that the apnoea/hypopnoea index (AHI) improved by up to 50% from baseline in 71.4% of patients selected after DISE for MAD therapy. In the current study, mandibular advancement splint therapy was successfully prescribed on the basis not only of severity of disease, as determined by the subject's initial AHI, but also by DISE findings combined with results of gentle mandibular advancement manoeuvre allowing direct view of the effects of mandibular protrusion on breathing spaces in obstruction sites, and showing good optimisation of selection of patients for oral device treatment.Il trattamento con dispositivi di avanzamento mandibolare (MAD) rappresenta un'efficace alternativa terapeutica per i pazienti affetti da roncopatia semplice, OSAS di grado lieve/moderato e in casi selezionati di OSAS grave con scarsa tollerabilità alla terapia ventilatoria con C-PAP. Pertanto è importante identificare dei criteri oggettivi per selezionare i pazienti che possono beneficiare del trattamento con i sistemi di avanzamento mandibolare (MAD). In letteratura sono stati descritti vari fattori predittivi sia antropometrici che polisonnografici, mentre esistono ancora controversie circa il ruolo della Sleep Endoscopy e della manovra di avanzamento mandibolare bimanuale durante lo stesso esame come fattori predittivi del successo terapeutico con MAD. In questo studio descriviamo la nostra esperienza nel management di pazienti affetti da OSAS lieve/moderata trattati con MAD e selezionati mediante "sleep endoscopy". Abbiamo eseguito una valutazione prospettica longitudinale di una serie consecutiva di pazienti giunti alla nostra osservazione con diagnosi di OSAS lieve/moderata e sottoposti a sleependoscopy. Durante il sonno indotto farmacologicamente è stata eseguita una delicata manovra di avanzamento mandibolare con escursione inferiore ai 5 mm e abbiamo riscontrato che in 30 dei 65 pazienti (46,2%) lo spazio respiratorio non migliorava in modo significativo a livello dei siti di ostruzione osservati, mentre in 35 dei 65 pazienti (53,8%) si osservava un miglioramento significativo tale da poter indicare terapia con MAD. In 7 dei 35 pazienti venivano riscontrate condizioni che ostacolavano l'applicazione del MAD per cui 28 dei 35 pazienti sono stati sottoposti a terapia con MAD. Dopo 3 mesi di trattamento abbiamo documentato un miglioramento significativo dell'indice di Epworth medio [(7,35 ± 2,8 vs 4,1 ± 2,2 (p0.05)], dell'AHI medio [(21.4 ± 6 eventi per ora verso 8,85 ± 6,9 (p0.05) ] e dell'ODI medio [(18.6 ± 8 eventi per ora versus 7 ± 5.8 (p0.05)]. Abbiamo inoltre osservato che l'AHI migliorava di almeno il 50% rispetto al basale nel 71.4% dei pazienti selezionati mediante sleep endoscopy. In questo studio, la terapia con i dispositivi di avanzamento mandibolare è stata prescritta con successo sulla base non soltanto dell'indice di apnea/ipopnea, ma anche dei reperti della sleep endoscopy e della manovra di avanzamento mandibolare, ottenendo una visione diretta degli effetti della protrusione mandibolare sullo spazio respiratorio in corrispondenza dei siti di ostruzione, e ottenendo una buona ottimizzazione della selezione dei pazienti per il trattamento con MAD.

Oro-pharyngeal dysphagia is frequently present during the acute phase of stroke. The aim of the present study was to evaluate whether the recording of surface EMG using a nasopharyngeal (NP) electrode could be applied to evaluation of pharyngeal muscle activity in acute stroke patients and if this neurophysiological measure is related with clinical assessment of swallowing. Patients were examined and clinical severity was assessed with the National Institute of Health Stroke Scale (NIHSS) score; dysphagia was evaluated through bedside screening test using the Gugging Swallowing Scale (GUSS). Extension of the ischaemic lesion was measured by quantitative score, based on CT scan [Alberta Stroke Programme Early CT Score (ASPECTS)]. We analysed 70 patients; 50 were classified as dysphagic (Dys+), and 20 as non-dysphagic (Dys-). Each participant underwent a surface NP EMG recording performed with a NP electrode, made of a Teflon isolated steel catheter, with a length of 16 cm and a tip diameter of 1.5 mm. The electrode was inserted through the nasal cavity, rotated and positioned approximately 3 mm anteroinferior to the salpingo-palatine fold. At least four consecutive swallowing-induced EMG bursts were recorded and analysed for each participant. Swallowing always induced a repetitive, polyphasic burst of activation of the EMG, lasting around 0.25 to 1 sec, with an amplitude of around 100-600mV. Two parameters of the EMG potentials recorded with the NP electrode were analyzed: duration and amplitude. The duration of the EMG burst was increased in Dys+ patients with a statistically significant difference compared to Dys- patients (p0.001). The amplitude was slightly reduced in the Dys+ group, but statistically significant differences were not observed (p = 0,775). Nevertheless, the burst amplitude showed a significant inverse correlation with NIHSS [r(48) = -0.31; p0.05] and ASPECTS scores [r(48) = -0.27; p0.05], meaning that the burst amplitude progressively reduced with an increase of clinical severity (NIHSS) and topographic extension of brain lesions in CT (ASPECTS). These results suggest that NP recordings can give a semi-quantitative measure of swallowing difficulties originating from pharyngeal dysfunction, in fact, electromyographic findings suggest reduced pharyngeal motility.La disfagia orofaringea è spesso presente durante la fase acuta di un ictus. Lo scopo di questo lavoro è stato quello di valutare se la registrazione elettromiografica di superficie tramite un elettrodo nasofaringeo può essere impiegata per testare l’attività muscolare del faringe nei pazienti con ictus acuto e se queste misurazioni elettrofisiologiche possono essere correlate con la valutazione clinica della deglutizione. Dal punto di vista clinico la severità del quadro è stata valutata mediante l’utilizzo della scala del National Institute of Health Stroke (NIHSS); la disfagia è stata valutata mediante il test di screening Gugging Swallowing Scale (GUSS); l’estensione della lesione ischemica alla TAC è stata misurata attraverso l’Alberta Stroke Programme Early CT Score (ASPECTS). Abbiamo valutato 70 pazienti di cui 50 disfagici (Dys+), e 20 non disfagici (Dys–). Ciascun partecipante è stato sottoposto a un’elettromiografia di superficie registrata mediante un elettrodo NP costituito da un catetere di Teflon isolato in acciaio (lungo 16 cm e con un diametro in punta di 1,5 mm). L’elettrodo è stato inserito attraverso la cavità nasale, ruotato e posizionato approssimativamente 3 mm antero-inferiormente rispetto alla volta salpingo-palatina. Per ogni partecipante sono state registrate ed analizzate le risposte elettromiografiche di almeno quattro deglutizioni volontarie ripetute. La deglutizione induce sempre all’elettromiografia burst ripetitivi e polifasici di durata compresa fra 0,25 e 1 secondo, con un’ampiezza intorno ai 100-600mV. I disfagici hanno mostrano una maggiore durata del burst rilevato all’elettromiografia rispetto ai non disfagici, con una differenza statisticamente significativa (p0,001), ma non hanno mostrano differenze in termini di ampiezza del burst stesso (p = 0,775); quest’ultima invece era inversamente correlata con lo NIHSS score [r(48) = –0,31; p0,05)] e con lo ASPECTS score [r(48) = –0,27; p0,05]. Questi risultati suggeriscono che le registrazioni nasofaringee possono rappresentare un indice semi-quantitativo delle difficoltà deglutitorie secondarie a disfunzione faringea ed in particolare, i risultati dell’elettromiografia sarebbero indicativi di una ridotta motilità faringea durante la fase acuta di un ictus.

Cluster headache (CH) is a primary headache with a close relation to sleep. CH presents a circa-annual rhythmicity; attacks occur preferably during the night, in rapid eye movement (REM) sleep, and they are associated with autonomic and neuroendocrine modifications. The posterior hypothalamus is the key structure for the biological phenomenon of CH. Our aim is to describe a 55-year-old man presenting a typical episodic CH, in whom we performed a prolonged sleep study, consisting of a 9-week actigraphic recording and repeated polysomnography, with evaluation of both sleep macrostructure and microstructure. During the acute bout of the cluster we observed an irregular sleep-wake pattern and abnormalities of REM sleep. After the cluster phase these alterations remitted. We conclude that CH was associated, in this patient, with sleep dysregulation involving the biological clock and the arousal mechanisms, particularly in REM. All these abnormalities are consistent with posterior hypothalamic dysfunction.

Background and purpose The detection of antibodies binding neural antigens in patients with epilepsy has led to the definition of ‘autoimmune epilepsy’. Patients with neural antibodies not responding to antiepileptic drugs (AEDs) may benefit from immunotherapy. Aim of this study was to evaluate the frequency of autoantibodies specific to neural antigens in patients with epilepsy and their response to immunotherapy. Methods Eighty-one patients and 75 age- and sex-matched healthy subjects (HS) were enrolled in the study. Two groups of patients were included: 39 patients with epilepsy and other neurological symptoms and/or autoimmune diseases responsive to AEDs (group 1) and 42 patients with AED-resistant epilepsy (group 2). Patients' serum and cerebrospinal fluid were evaluated for the presence of autoantibodies directed to neural antigens by indirect immunofluorescence on frozen sections of mouse brain, cell-based assays and a radioimmunoassay. Patients with AED-resistant epilepsy and neural autoantibodies were treated with immunotherapy and the main outcome measure was the reduction in seizure frequency. Results Neural autoantibodies were detected in 22% of patients (18/81), mostly from the AED-resistant epilepsy group (P = 0.003), but not in HS. Indirect immunofluorescence on mouse brain revealed antibodies binding to unclassified antigens in 10 patients. Twelve patients received immunotherapy and nine (75%) achieved >50% reduction in seizure frequency. Conclusions A significant proportion of patients with AED-resistant epilepsy harbor neural-specific autoantibodies. The detection of these antibodies, especially of those binding to synaptic antigens, may predict a favorable response to immunotherapy, thus overcoming AED resistance.

Idiopathic hyperCKemia is characterized by persistently increased serum creatine kinase (CK) encountered in healthy individuals with no evidence of neuromuscular diseases.1 They may complain of fatigue, cramps, and myalgia; these nonspecific symptoms overlap with the wide spectrum of limb discomfort reported by patients with restless legs syndrome (RLS). Nevertheless, hyperCKemia is not a laboratory finding in RLS. We describe 7 patients with idiopathic hyperCKemia in whom the diagnostic workup revealed the presence of RLS with periodic limb movements in sleep (PLMS). ### Methods. Seven patients (6 men, age 22–69 years; table) referred to a center for neuromuscular diseases for severe, persistent myalgia in the lower limbs, fatigue, and cramps were included. Laboratory tests revealed hyperCKemia, confirmed in at least 3 consecutive samples, at 30-day intervals, at rest. No patient reported assumption of statins or exposure to toxins. All showed lower limbs muscle hypertrophy, particularly of quadriceps and calves (figure e-1 on the Neurology ® Web site at www.neurology.org). Patients underwent an …

Snoring is a prerequisite for obstructive sleep apnoea (OSA) and is known to run in families. Recent studies have provided sufficient evidence for a familial predisposition to OSA. In our study, 492 monozygotic and 284 dizygotic twins were contacted by telephone and asked to attend an interview which included questions of life habits, medical history, sleep habits and disorders, with particular emphasis on snoring. Our study showed that the probandwise concordance rate for habitual snoring was higher in monozygotic twins than in dizygotic ones, but the difference was not significant. The comparison of concordant pairs for habitual snoring vs. concordant pairs for non-snoring confirmed that habitual snoring is significantly associated with older age, male gender, higher body mass index (BMI), smoking and respiratory diseases. The multivariate analysis in the discordant groups confirmed that BMI is more strongly associated to habitual snoring in dizygotic twins than in the monozygotic ones. Our logistic analysis showed that other variables, such as smoking and respiratory diseases, are associated with habitual snoring in dizygotic pairs, but not in monozygotic ones. These findings suggest a genetic predisposition to habitual snoring.

A 72-year-old right-handed man with a history of hypertension and past smoking, taking low-dose aspirin therapy (100 mg once a day) for cardiovascular prevention, presented with sudden onset of right hemiparesis and confusion. The right hemiparesis improved during the following days, but the patient was still confused. He arrived at the emergency department 6 days after the onset of symptoms. On neurological examination, the patient showed mild disorientation both in time and in place; no other neurological signs could be elicited. Blood pressure was 160/90 mm Hg. Blood count, prothrombin time, activated thromboplastin time, ionogram, C-reactive protein, troponin Ic, and creatine kinase were normal. ECG revealed normal sinus rhythm (76 bpm). Extracranial …

Objective: A loss of inhibition in central motor circuits resulting in abnormal motor control is the hypothesised cause of dystonia. So far, changes in inhibitory function of cerebral cortex in dystonia, have been revealed only indirectly by recording muscle responses evoked by transcranial magnetic stimulation (TMS) of the brain. The aim of present study was to evaluate more directly cerebral cortex changes in dystonia. We had the almost unique opportunity to record directly motor cortex output after brain stimulation, in a dystonic patient who had epidural electrodes implanted in the upper cervical cord. Methods: We evaluated descending activity evoked by single and paired pulse TMS together with the inhibitory effects produced by afferent stimuli on TMS evoked activity, and compared the results with those obtained in thirteen subjects with no central nervous system abnormality who also had cervical spinal electrodes. Results: The intrinsic inhibitory activity produced by paired TMS of the motor cortex, and the inhibitory effects produced by afferent inputs, were suppressed in the patient with dystonia. Conclusions: These findings provide a direct evidence of the abnormality in motor cortex inhibitory systems in dystonia. Significance: The abnormality in cortical inhibitory system might have a role in the pathophysiology of dystonia.

We describe the case of a 13-year-old boy, in good health, with transient occipital MRI abnormalities just after one generalized seizure and the appearance of macropsia. The EEG showed a 3–5 Hz sub-continuous left occipital activity, with sporadic sharp waves. Macropsia disappeared in 2 years. This case may suggest that the presence of transient MRI abnormalities does not exclude a favorable prognosis.

Post-infectious encephalomyelitis (PIE), an inflammatory demyelinating disorder, usually begins days to weeks after virus (usually measles, Epstein–Barr virus [EBV], cytomegalovirus [CMV], or varicella zoster virus [VZV]) or mycoplasma infection, and after vaccination with hepatitis B virus,1 Bordetella pertussis , and possibly influenza virus.2 The mechanism by which the infectious agent triggers demyelination is uncertain. Two days after varicella infection, a 3-year-old girl developed fever, confusion, lethargy, and status epilepticus. CSF was acellular with normal protein and glucose, without oligoclonal bands (OGBs) or amplifiable VZV DNA, and no VZV IgM or IgG antibody in serum or CSF. Brain CT revealed diffuse white matter edema. After IV treatment with ceftriaxone, acyclovir, clonazepam, and phenobarbital, seizures stopped and mental status was normal. Neurologic signs included increased DTRs, greater on the left, and ankle clonus bilaterally. Twenty days later, she became febrile. Neurologic signs consisted of ataxia with dysmetria bilaterally and intention tremor in the arms. Serum contained VZV IgM antibody, and VZV IgG was 6,800 international units (IU) per liter (normal < 50 IU/L). There was no amplifiable VZV DNA in blood. CSF was acellular, protein 31 mg%; …

Primary headaches are closely related to sleep. Modifications in the patterns of arousal during sleep have been reported in migraine, especially in the nights preceding a headache attack. We aimed at evaluating the pattern of arousal from sleep in a group of patients affected by sleep-related migraine. We enrolled 10 consecutive patients, three males and seven females, aged between 20 and 62 years, who presented frequent attacks of migraine without aura (more than five per month), closely related to sleep (more than one-half of the attacks occurred during sleep, causing an awakening). A control group was studied, matched for age and sex. Patients and controls underwent a full-night polysomnographic study, following adaptation; arousal pattern was studied by the scoring of the high-frequency EEG arousal and by the cyclic alternating pattern (CAP). Migraineurs showed a lower CAP rate in non-rapid eye movement (NREM) sleep and, in particular, a lower number of A1 phases (low-frequency, high-amplitude EEG bursts) compared with the controls. Migraineurs also showed a lower index of high-frequency EEG arousals during rapid eye movement (REM) sleep. The reduction in the CAP rate indicates a lower level of arousal fluctuation in NREM sleep. The reduced arousal index in REM suggests a dysfunction in neural structures involved in both the control of REM sleep and the pathophysiology of migraine, such as the hypothalamus and the brainstem.

Objective Unilateral or bilateral paramedian infarction in the region of the thalamus and upper midbrain may lead to hypersomnia. To determine whether unilateral infarction of the paramedian thalamus leads to changes in excitability of ipsilesional primary motor hand area (M1). Methods We describe a patient with chronic stroke of the right dorsomedian and intralaminar thalamic nuclei, who suffered from mild persistent hypersomnia. We studied the excitability of the right and left M1 with transcranial magnetic stimulation (TMS) in the patient, and in 10 healthy controls. Results In contrast to healthy controls, contralateral electrical stimulation of the median nerve failed to induce short-latency afferent inhibition (SAI) in the ipsilesional M1. Other measures of corticomotor excitability and somatosensory evoked potentials were normal. Conclusions The selective loss of ipsilateral SAI in a patient with paramedian thalamic stroke suggests that during wakefulness, the intact paramedian thalamus facilitates the excitability of intracortical inhibitory circuits, which process thalamocortical sensory inputs in the ipsilateral M1. This preliminary finding suggests that measurements of SAI may provide a means of probing the integrity of some neural pathways, which are involved in the control of wakefulness and arousal. Significance In addition to the established role of the paramedian thalamus in arousal and memory, our observation suggests that thalamocortical projections from the paramedian thalamus contribute to the integration of sensory input at the cortical level during wakefulness.

The pathophysiology of human narcolepsy is still poorly understood. The hypoactivity of some neurotransmitter systems has been hypothesised on the basis of the canine model. To determine whether narcolepsy is associated with changes in excitability of the cerebral cortex, we assessed the excitability of the motor cortex with transcranial magnetic stimulation (TMS) in 13 patients with narcolepsy and in 12 control subjects. We used several TMS paradigms that can provide information on the excitability of the motor cortex. Resting and active motor thresholds were higher in narcoleptic patients than in controls and intracortical inhibition was more pronounced in narcoleptic patients. No changes in the other evaluated measures were detected. These results are consistent with an impaired balance between excitatory and inhibitory intracortical circuits in narcolepsy that leads to cortical hypoexcitability. We hypothesise that the deficiency of the excitatory hypocretin/orexin-neurotransmitter-system in narcolepsy is reflected in changes of cortical excitability since circuits originating in the lateral hypothalamus and in the basal forebrain project widely to the neocortex, including motor cortex. This abnormal excitability of cortical networks could be the physiological correlate of excessive daytime sleepiness and it could be the substrate for allowing dissociated states of wakefulness and sleep to emerge suddenly while patients are awake, which constitute the symptoms of narcolepsy.

Objective : High-frequency oscillations (HFOs) evoked by upper limb stimulation reflect highly synchronised spikes generated in the somatosensory human system. Since acetylcholine produces differential modulation in subgroups of neurons, we would determine whether cholinergic drive influences HFOs. Methods : We recorded somatosensory evoked potentials (SEPs) from 31 scalp electrodes in 7 healthy volunteers, before and after single administration of rivastigmine, an inhibitor of central acetylcholinesterase. Right median nerve SEPs have been analysed after digital narrow bandpass filtering (500–700 Hz). Raw data were further submitted to Brain Electrical Source analysis (BESA) to evaluate the respective contribution of lemniscal, thalamic and cortical sources. Lastly, we analysed by Fast Fourier transform spectral changes after drug administration in the 10–30 ms latency range. Results : Rivastigmine administration caused a significant increase of HFOs in the 18–28 ms latency range. Wavelets occurring before the onset latency of the conventional N20 SEP did not show any significant change. A similar increase concerned the strength of cortical dipolar sources in our BESA model. Lastly, we found a significant power increase of the frequency peak at about 600 Hz in P3-F3 traces after drug intake. Conclusions : Our findings demonstrate that the cortical component of HFOs is significantly enhanced by cholinergic activation. Pyramidal chattering cells, which are capable to discharge high-frequency bursts, are mainly modulated by cholinergic inputs; by contrast, acetylcholine does not modify the firing rate of fast-spiking GABAergic interneurons. We thus discuss the hypothesis that cortical HFOs are mainly generated by specialised pyramidal cells.

A 71-year-old man presented recurrent transient episodes of anterograde and retrograde amnesia, since age 60 years, with a frequency of 10 per month. The attacks, preceded by vague abdominal discomfort, lasted from a few minutes to 2–3 hours. History was unremarkable except for a head trauma; he took no drugs. MRI showed a left meningoencephalocele, likely posttraumatic, in the …

Objectives : We report on a case of ESES/CSWS observed in a patient of 21 years and still persisting at the age of 25. Cases of ESES/CSWS have never been previously described in adult patients. ESES/CSWS is considered to be related to the degree of maturation of the central nervous system, and therefore strictly age-related. Methods : Our case of ESES/CSWS was observed in a 21 year old woman referred for cognitive and behavioral disorders. She had previously had epileptic fits, but was seizure free at that time. The patient underwent a full-night polygraphic recording, which showed a typical ESES/CSWS pattern, with a Spike-and-Wave Index >85%. Polysomnography was repeated 9 times in a 4 year follow-up, during which the ESES/CSWS condition persisted, despite the pharmacological treatments. The patient also underwent cerebral magnetic resonance imaging and fludeoxyglucose F 18 positron emission tomography (PET). Results : The PET study revealed reduced metabolic activity within the lower gyrus of the right parietal lobe, but no significant difference between subcortical structures and cortical mantle was seen. MRI scans were normal. Conclusions : This observation suggests that ESES/CSWS might not always be an age-related condition. Sleep EEG recordings should always be performed in patients with behavioral disorders and a history of epileptic fits.

The startle reflex is a motor response that originates in the lower brainstem.1 Abnormal symptomatic startle can be secondary to lesions in the startle pathway, involving brainstem and spinal cord.2 A 56-year-old woman developed an acute demyelinating lesion of unknown origin in medulla oblongata (figure, A–D), causing dizziness …

Attempts to analyse the sleep structure of patients with eating disorders have so far led to conflicting results. Polygraphic findings suggest that patients with bulimia nervosa are not easily distinguishable from age-matched controls, whereas anorexic patients show some abnormalities in sleep efficiency and sleep architecture. Nevertheless, both bulimic and anorexic patients complain of poor quality sleep. The aim of this study was to evaluate the microstructure of sleep in anorexia and bulimia by analysing arousal (following the rules of the American Sleep Disorders Association) and the cyclic alternating pattern (CAP). The results confirmed the presence of sleep disturbances in eating disordered patients: an increase in arousal length and the CAP rate. They also seem to confirm the findings of previous studies suggesting that altered sleep in eating disordered patients may be related to their body mass index (BMI) and psychopathological status.

Since 1998, two research groups independently reported the discovery of two novel hypothalamic neuropeptides, called hypocretin-1 and hypocretin-2, respectively many studies have been carried out about their possible functions. One group named these new peptides orexins (A and B) after the Greek word for appetite, since they found that central administration of orexins potently increased food intake in rats. However hypocretins/ orexins are involved in various hypothalamic mechanisms, such as energy homeostasis, neuroendocrine functions, appetite and satiety regulation, vigilance and defence behaviour, sleep and wake regulation. Here is a review of the recent literature, showing some recent discoveries about the link between orexin system, sleep regulation and appetite regulation.