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We report two weightlifters, presenting with progressive atrophy and weakness of the medial portion of pectoralis major muscle. We discuss possible differential diagnoses and propose a clinical and neurophysiological approach. Our cases highlight that the syndrome may be unilateral or bilateral, probably due to anatomical susceptibility and hyper-training. We review this rare syndrome and promote its awareness and full diagnostic assessment, aiming to its prompt recognition in weightlifters.

BackgroundThe diagnosis of dementia with Lewy bodies (DLB) is based on diagnostic clinical criteria, which were updated over the years.ObjectiveTo evaluate, through a systematic review, accuracy of the diagnostic criteria, testing a possible improvement over time.MethodsWe searched on MEDLINE and SCOPUS databases for studies reporting diagnostic parameters regarding the clinical diagnosis of DLB until October 2016. We performed meta-analysis, using a Bayesian approach, on those using pathological examination as gold standard, subclassified based on the different diagnostic criteria used.ResultsWe selected 22 studies on 1585 patients. Pooled sensitivity, specificity and accuracy were 60.2%, 93.8%, 79.7%, respectively, for criteria antecedents to McKeith 1996. For McKeith 1996-possible, pooled sensitivity, specificity and accuracy were 65.6%, 80.6%, 77.9% in early stages and 72.3%, 64.3%, 66% in late stages, respectively. For McKeith 1996-probable, pooled sensitivity, specificity and accuracy were 19.4%, 95.1%, 77.7% in early stages and 48.6%, 88%, 79.2% in late stages, respectively. McKeith criteria 2005 were evaluated only in late stages: pooled sensitivity, specificity and accuracy were 91.3%, 66.7% and 81.6%, respectively, for possible diagnosis (only one study) and 88.3%, 80.8%, 90.7% for probable diagnosis, decreasing to 85.6%, 77.1% and 81.7% if only considering clinical settings focused on dementia diagnosis and care.Conclusions and relevanceDiagnostic criteria have become more sensitive and less specific over time, without substantial change in the accuracy. Based on current data, about 20% of DLB diagnosis are incorrect. Future studies are needed to evaluate if the recently released revised consensus criteria will improve the diagnostic accuracy of DLB.

Background: The profile and degree of cognitive impairment in Multiple System Atrophy (MSA) and the impact of sleep disorders, REM sleep behavior disorder (RBD) in particular, in parkinsonism-related cognitive deficits are currently being debated. Summary: We reviewed the cognitive, affective and sleep findings in MSA and also carried out a longitudinal investigation of 10 MSA patients. At the first evaluation, 3 patients showed isolated cognitive deficits. After a mean of 16 months, these patients remained unchanged, while 1 patient worsened from a normal condition. No significant differences emerged when the cognitive, affective and video-polysomnographic findings of MSA-P and MSA-C were compared. Depression was present in half of the patients, although it did not influence their cognitive performance. Comparisons between the first and second evaluation data showed significant worsening in visual attention and in ADL/IADL and UMSARS. Key Messages: Isolated cognitive deficits are evidenced in a minority of MSA patients with the absence of a clear-cut diagnosis of dementia in the early stages of the disease. Attention and executive functions are often impaired. This study with a short follow-up period showed that RBD, although present in almost all patients affected by MSA, does not appear a clear early marker of cognitive impairment. Future longer-term studies with a larger patient sample are thus encouraged.

This section, IMAGES IN SLEEP MEDICINE, is intended to tap a relatively unique feature of sleep science: images that have great educational and conceptual content. These could be, for example, electroencephalograms, electromyograms, polysomnograms, portable devices, actigrams, scans including functional images, pathology specimens, brain slice preparations, fluorescent microscopy and other cutting edge techniques. The source may be human or nonhuman, but the clinical relevance should be clear. Illustrations must consist of clear and high-quality, blackand-white or color-digitized images. At this stage, videos can be submitted only for online publication. The message accompanying the picture may be a maximum of 500 words, with no more than 5 references. It is especially important not to try and make a case report out of these submissions, and detailed clinical (as contrasted to image) analysis should be avoided. The material should be submitted on the Sleep Medicine website (www.ees.elsevier.com/sleep). Images can be embedded within a text document such as Microsoft Word, a slide program such as Microsoft PowerPoint, or converted to Acrobat files. Authors should expect that the images will be available for use (with acknowledgement) to the general sleep community for teaching purposes, and so copyrighted/ patentable material should be avoided. In its final form, these images will be a freely available, searchable digital teaching image library. Comments regarding the images should be sent by e-mail to the section co-editors within a month of the publication date. These comments will be summarized and posted, if determined to be of educational content, with the original image on the journal’s web site, expected to be active for content soon. We hope this section will be enriched by the contributions of our colleagues who wish to offer stimulating opportunities for discussion and new insights in the field of sleep.

FES is an often forgotten and underdiagnosed entity characterized by flaccid and easily everted upper lids, occurring spontaneously or with minimal manipulation. It is usually seen in overweight, middleaged males who complain of a foreign body sensation, burning, tearing and redness. Some studies have found an interesting association with OSAS [1,2]. This relationship may have both diagnostic and therapeutic implications. Nevertheless, the majority of OSAS patients, obese or non-obese, do not have FES. Furthermore, FES may also be a presenting symptom in patients with undiagnosed OSAS [2]. Although the cause remains unknown, in all probability OSAS has no real causative relationship, other than a sequence of extreme apnea, right heart failure, and increased venous pressure that worsens a propensity for this phenomenon. Additionally, treatment of obesity and OSAS may have a favourable effect on the course of FES [3]. The clinician should be aware of this association so that both underlying OSAS or FES can be detected. The condition should be suspected in any obese patient with chronic watery and red eyes. Besides weight loss and continuous positive airway pressure therapy if OSAS is suspected, treatment consists of supportive measures such as ocular lubrication, eyelid taping, or an eyeshield at bedtime to keep the eyelids closed while sleeping, and surgery to address horizontal laxity and redundant eyelid tissues.

While dreaming amputees often experience a normal body image and the phantom limb may not be present. However, dreaming experiences in amputees have mainly been collected by questionnaires. We analysed the dream reports of amputated patients with phantom limb collected after awakening from REM sleep during overnight videopolysomnography (VPSG). Six amputated patients underwent overnight VPSG study. Patients were awakened during REM sleep and asked to report their dreams. Three patients were able to deliver an account of a dream. In all dreaming recalls, patients reported that the amputated limbs were intact and completely functional and they no longer experienced phantom limb sensations. Phantom limb experiences, that during wake result from a conflict between a pre-existing body scheme and the sensory information on the missing limb, were suppressed during sleep in our patients in favour of the image of an intact body accessed during dream.

Peduncular hallucinosis (PH) consists of formed and coloured visual images, which the patient knows are unreal; it is often associated with lesions of the pons, midbrain and diencephalon. A 72-year-old man had noted the sudden onset of visual hallucinations one year before, specifying the time and body position in a 4-week, 24-h diary. Thereafter, he underwent video-polysomnography (VPSG), brain magnetic resonance imaging (MRI), angiography (MRA), proton spectroscopy ((1)H MRS), and single photon emission tomography (SPECT). Patient's diaries and VPSG showed a strong clustering of hallucinatory experiences during the evening/night time while lying in supine position, similar to hypnagogic hallucination and sleep paralysis in supine position. Repeated episodes of REM sleep behaviour disorder (RBD) occurred during the night. MRI and MRA showed an elongated and dilated left internal carotid artery displacing the left subthalamus upwards, and (1)H MRS relatively decreased N-acetyl-aspartate in the left subthalamus. Brain SPECT during PH revealed hypoperfusion in the right temporal region and hyperperfusion in the left occipital and right opercular regions (the latter possibly related to the patient's awareness of unreality). PH resolved with serotonergic (citalopram) therapy.

Hypoactive-hypoalert behavior (HHB), also termed “psychic akinesia,” is characterized by loss of psychic autoactivation, apathy, and stereotyped activity that are reversed by external stimulation.1 Lesions in the brainstem, basal ganglia, or frontal lobes can underlie HHB.1 We report three patients presenting with HHB due to spontaneous intracranial hypotension (SIH) syndrome. Two men aged 49 and 57 years and a woman aged 60 years were referred by relatives because of a 2-, 5-, and 2-year history of a chronic state of decreased behavioral initiative, persistent somnolence, impaired attention, and stereotyped motor activity. The patients would lie inactive for whole days if left unstimulated, but would perform adequately whenever incited to. They were scarcely concerned about their ensuing family and job problems and did not complain of mood depression, but came to our observation only because urged to by the alarmed relatives. Neurologic examination showed diffuse deep tendon hyperreflexia variously combined with limitation of vertical and vergence eye movements, lateral gaze-evoked nystagmus, and right extensor plantar response. All patients displayed simple motor perseverations such as buttoning or unbuttoning the shirt, rubbing the face, blowing the nose, hand wringing, washing, rubbing or beating the legs or the trunk, or clearing the throat. They denied orthostatic headache. They fell asleep when left undisturbed but could be easily aroused by external stimulations. Neuropsychological tests showed normal findings …

We describe a patient with a paraproteinemic anti-myelin-associated glycoprotein (anti-MAG) antibody polyneuropathy and concomitant axial myoclonic jerks. Neurophysiological investigation revealed that axial jerks were asymmetrical and exaggerated by lying in bed. They disappeared during mental arousal and sleep. Analysis of axial myoclonus showed that the first activated muscle was the left rectus abdominis with subsequent rostral and caudal propagation of a propriospinal type. Plasmapheresis substantially reduced the frequency and intensity of axial myoclonic jerks. In our patient, propriospinal myoclonus was associated with anti-MAG polyneuropathy, but the causal relationship remains unclear.

Objective We present two patients, a 52-year-old man and a 56-year-old woman, with rapid eye movement sleep behaviour disorder (RBD) since the age of 50. Method In both the patients RBD was videopolysomnographically documented. Results Both patients developed, with time, autonomic and motor symptoms consistent with the diagnosis of multiple system atrophy. During the course of the disease RBD episodes diminished in frequency but the patients’ sleep became even more abnormal, with nearly continuous motor and verbal abnormal behaviours and ambiguous and rapid oscillations of state-determining polysomnographic variables, now consistent with status dissociatus (SD). Conclusion If SD represents the evolution and most extreme form of RBD it should be investigated longitudinally in neurodegenerative diseases.

Central pattern generators (CPGs) are genetically determined neuronal aggregates in the mesencephalon, pons and spinal cord subserving innate motor behaviours essential for survival (feeding, locomotion, reproduction etc.). In higher primates CPGs are largely under neocortical control. We describe how certain motor events observed in parasomnias and epileptic seizures could have similar features and resemble motor behaviours, which can be the expression of the same CPG. Both epilepsy and sleep can lead to a temporary loss of control of neomammalian cortex that facilitates through a common platform (arousal) the emergences of stereotyped inborn fixed action patterns. Therefore we suggest that, independently from the nature of the trigger, be it a seizure or a parasomnia, the same CPGs can be involved, “caught up”, leading to a common motor semiology (the “Carillon theory”).

A “body image” is an internal construct of a unitary corporeal self that endures in space and time, even after partial amputations of the physical body. A body image provides the substrate for the feeling of “residual” experiences of the missing body part, that is the “phantom” phenomenon.1 Restless legs syndrome (RLS) is characterised by uncomfortable sensations, usually affecting the legs, associated with an urge to move, worsening at rest, in the evening, and at night, and which increase during attempts to remain still.2 We report the case of a patient who developed painful “phantom” phenomena after the amputation of both legs and the upper right forearm. One year later, in the absent portions of his extremities, there began symptoms that he had never experienced before the amputations, and that he clearly distinguished from the “phantom.” These symptoms were congruent with a diagnosis of RLS. A 56-year-old man began haemodialysis at age 39 due to a chronic renal failure secondary to glomerular nephropathy. Thereafter he developed a severe arteriosclerotic obliterative arteriopathy that led to gangrene at the age of 51, necessitating bilateral above-knee and right below-elbow amputations. A few days after the amputations, “phantom” phenomena began consisting of attacks of …