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Castel Guelfo di Bologna, Italy

Antelmi E.,University of Bologna | Provini F.,University of Bologna | Provini F.,IRCCS Institute of Neurological science
Sleep Medicine Reviews | Year: 2015

Propriospinal myoclonus (PSM) is a rare type of spinal myoclonus characterized by muscle jerks that usually start in the midthoracic segments and then slowly propagate up and down into the spinal cord, resulting in repetitive and irregular jerky flexion, or extension of the trunk, neck, knees and hips. PSM can be symptomatic, but up to 80% of reported cases appear idiopathic. PSM tends to occur especially while the subject is lying down. PSM at sleep onset was first described by experts in sleep medicine.The original electrophysiological features included fixed pattern of muscle activations, slow spinal cord conduction (5-15 m/s), electromyographic burst duration less than 1000 ms, synchronous activation of agonist and antagonist muscles and no involvement of facial muscles. PSM has been reported to be a functional (psychogenic) movement disorder in a number of cohorts. The differential diagnosis between idiopathic PSM and the functional forms is not always straightforward. A consistent polymyographically documented muscle activation pattern may be supportive but by no means sufficient and additional neurophysiological investigations are required.PSM should be differentiated from other movement disorders involving the abdomen and trunk, or occurring at sleep-wake transition.This article offers a comprehensive overview of the spectrum of PSM phenotypes. © 2014 Elsevier Ltd.

Giulioni M.,Divisions of Neurosurgery | Marucci G.,University of Bologna | Martinoni M.,Divisions of Neurosurgery | Volpi L.,Divisions of Neurology | And 8 more authors.
Journal of Neurosurgery | Year: 2013

Object. The study was performed to investigate the relation between seizure outcome after surgical treatment of mesial temporal lobe epilepsy (MTLE) and pathological findings, classified according to the recently proposed classifications of mesial temporal sclerosis (MTS), granule cell pathology (GCP), focal cortical dysplasia (FCD) and epilepsy-associated low-grade tumors (ELGT). Methods. The authors analyzed data obtained in 120 consecutive cases involving patients presenting with drugresistant MTLE, who underwent tailored anteromesial temporal lobe resection, and correlated seizure outcome with pathological findings. They identified 5 histopathological groups: Group 1-ELGT, alone or associated with other lesions (30 cases); Group 2-isolated FCD (17 cases); Group 3-MTS, with or without GCP (28 cases); Group 4- MTS associated with FCD, with or without GCP (37 cases); Group 5-other lesions (8 cases). Results. Engel Class I outcome was observed in 83% of patients with ELGT (Class IA in 63%); in 59% of patients with isolated FCD, with FCD Type II showing a better prognosis than FCD Type I; in 82% of patients with isolated MTS (Class IA in 50%), with MTS Type 1a and MTS Type 1b showing a better prognosis than MTS Type 2 and patients with MTS and GCP having better postsurgical results than those with MTS without GCP. Engel Class I outcome was also achieved in 84% of patients with FCD associated with MTS (Engel Class IA in 62%); also in this group MTS 1a and MTS 1b associated with FCD showed a better prognosis than FCD associated with MTS 2. Finally, Engel Class I was also achieved in 2 patients with vascular malformation and in 1 with a temporal pole encephalocele. Conclusions. Patients with MTLE and ELGT, MTS, or MTS associated with FCD showed the best postsurgical seizure outcome (Engel Class I in more than 80% of cases), whereas only 63% of patients with isolated FCD achieved the same type of outcome. Interestingly, the analysis of seizure outcome in histopathological subtypes of FCD and of MTS showed different prognoses in the different pathological subgroups, with worse outcomes for atypical MTS, absence of GCP, and isolated FCD Type I. © AANS, 2013.

Kasteleijn-Nolst Trenite D.,University of Rome La Sapienza | Rubboli G.,IRCCS Institute of Neurological science | Hirsch E.,Hopitaux Universitaires Of Strasbourg | Martins Da Silva A.,Hospital Santo Antonio | And 8 more authors.
Epilepsia | Year: 2012

Intermittent photic stimulation (IPS) is a common procedure performed in the electroencephalography (EEG) laboratory in children and adults to detect abnormal epileptogenic sensitivity to flickering light (i.e., photosensitivity). In practice, substantial variability in outcome is anecdotally found due to the many different methods used per laboratory and country. We believe that standardization of procedure, based on scientific and clinical data, should permit reproducible identification and quantification of photosensitivity. We hope that the use of our new algorithm will help in standardizing the IPS procedure, which in turn may more clearly identify and assist monitoring of patients with epilepsy and photosensitivity. Our algorithm goes far beyond that published in 1999 (Epilepsia, 1999a, 40, 75; Neurophysiol Clin, 1999b, 29, 318): it has substantially increased content, detailing technical and logistical aspects of IPS testing and the rationale for many of the steps in the IPS procedure. Furthermore, our latest algorithm incorporates the consensus of repeated scientific meetings of European experts in this field over a period of 6 years with feedback from general neurologists and epileptologists to improve its validity and utility. Accordingly, our European group has provided herein updated algorithms for two different levels of methodology: (1) requirements for defining photosensitivity in patients and in family members of known photosensitive patients and (2) requirements for tailored studies in patients with a clear history of visually induced seizures or complaints, and in those already known to be photosensitive. © 2011 International League Against Epilepsy.

Antelmi E.,University of Bologna | Antelmi E.,University College London | Ferri R.,Ic Oasi Institute Irccs | Iranzo A.,CIBER ISCIII | And 8 more authors.
Sleep Medicine Reviews | Year: 2016

The states of being are conventionally defined by the simultaneous occurrence of behavioral, neurophysiological and autonomic descriptors. State dissociation disorders are due to the intrusion of features typical of a different state into an ongoing state. Disorders related to these conditions are classified according to the ongoing main state and comprise: 1) Dissociation from prevailing wakefulness as seen in hypnagogic or hypnopompic hallucinations, automatic behaviors, sleep drunkenness, cataplexy and sleep paralysis 2) Dissociation from rapid eye movement (REM) sleep as seen in REM sleep behavior disorder and lucid dreaming and 3) Dissociation from NREM sleep as seen in the disorders of arousal. The extreme expression of states dissociation is characterized by the asynchronous occurrence of the various components of the different states that prevents the recognition of any state of being. This condition has been named status dissociatus. According to the underlying disorders/diseases and to their severity, among status dissociatus we may recognize disorders in which such an extreme dissociation occurs only at night time or intermittently (i.e., autoimmune encephalopathies, narcolepsy type 1 and IgLON5 parasomnia), and others in which it occurs nearly continuously with complete loss of any conventionally defined state of being, and of the circadian pattern (agrypnia excitata).Here, we render a comprehensive review of all diseases/disorders associated with state dissociation and status dissociatus and propose a critical classification of this complex scenario. © 2015 Elsevier Ltd.

Serafini A.,University of Udine | Rubboli G.,IRCCS Institute of Neurological science | Rubboli G.,Danish Epilepsy Center | Gigli G.L.,University of Udine | And 3 more authors.
Epilepsy and Behavior | Year: 2013

Juvenile myclonic epilepsy (JME) can be firmly diagnosed by a careful interview of the patient focusing on the seizures and by the EEG with the help, if necessary, of long-term video-EEG monitoring using sleep and/or sleep deprivation. Background activity is normal. The interictal EEG shows diffuse or generalized spike-wave (SW) and polyspike-wave (PSW) discharges. In some patients, non-specific changes or misleading features such as focal changes are found. Changes are mostly seen at sleep onset and at awakening. Provoked awakenings are more likely to activate interictal paroxysmal abnormalities than spontaneous awakenings. The presence of a photoparoxysmal response with or without myoclonic jerks (MJ) is common (30% of the cases). Myoclonic jerks are associated with a discharge of fast, irregular, generalized PSWs that predominate anteriorly. Myoclonic jerks appear to be associated with rhythmic EEG (spike) potentials at around 20. Hz. These frequencies are in the range of movement-related fast sensorimotor cortex physiological rhythms. The application of jerk-locked averaging technique has provided findings consistent with a cortical origin of MJ. Paired TMS (transcranial magnetic stimulation) studies showed a defective intracortical inhibition, due to impaired GABA-A mediated mechanisms. In this review, we present the EEG characteristics of JME with particular emphasis on the pathophysiology of MJ and on the role of sleep deprivation on interictal and ictal changes. © 2012 Elsevier Inc.

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