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Neurophysiology of Myoclonus and Progressive Myoclonus Epilepsies

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Neurophysiology of Myoclonus and Progressive Myoclonus Epilepsies Journal Identification = EPD Article Identification = 0835 Date: October 5, 2016 Time: 9:48 pm Review article Epileptic Disord 2016; 18 (Suppl. 2): S11-S27 Neurophysiology of myoclonus and progressive myoclonus epilepsies Giuliano Avanzini 1, Hiroshi Shibasaki 2, Guido Rubboli 3,4, Laura Canafoglia 1, Ferruccio Panzica 1, Silvana Franceschetti 1, Mark Hallett 5 1 IRCCS Istituto Neurologico Carlo Besta, Milano, Italy 2 Department of Brain Pathophysiology, Faculty of Medicine, Kyoto, Japan 3 IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna, Italy 4 Danish Epilepsy Center, Epilepsihospital, Dianalund, Denmark 5 National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA ABSTRACT – The high temporal resolution of neurophysiological record- ings makes them particularly suited to faithfully describing the time course of rapid events such as myoclonus and to precisely measure its time rela- tionship with other related activities. In progressive myoclonus epilepsies (PMEs) polygraphy with simultaneous EMG-EEG recordings is a crucial tool for defining the characteristic of myoclonic jerks their topography over different muscles (namely antagonists), their time course and relationship with vigilance muscle activation and stimulations. Moreover on polygraphic recordings it is possible to detect EEG activities associated to myoclonic jerks and define their time relationship with myoclonus thus differentiat- ing cortical types of myoclonus from subcorticallly generated ones. Tanks to the back averaging technique non obvious time-locked EEG potentials can be detected on polygraphy , furthermore in stimulus sensitive myoclonus the analysis can include the potential evoked by the somatosensory stim- ulus (SEP). The polygraphic recording also gives information on muscle activity suppression occurring after jerk or as pure negative myoclonus. Besides the time domain analysis, techniques based on frequency analysis have been developed to evaluate EEG-EMG coherence. The neurophysio- logical techniques provide investigators and clinicians with an invaluable information to define the type of myoclonus and its generating circuitry thus substantially contributing in the diagnosis and management of PMEs. Key words: progressive myoclonus epilepsies, neurophysiology, EEG-EMG polygraphy, cortical myoclonus, coherence analysis Neurophysiological them particularly suited to faith- features associated with fully describing the time course of the shock-like muscle contractions myoclonus in progressive which characterize myoclonus. myoclonus epilepsies Moreover, the combination of elec- Correspondence: troencephalographic (EEG) and Giuliano Avanzini IRCCS Istituto Neurologico Carlo Besta Neurophysiological recordings electromyographic (EMG) record- Milano, Italy may be conducted over a rela- ings allows detection of any EEG <[email protected]> doi:10.1684/epd.2016.0835 tively long period of time, making correlates of myoclonus and high Epileptic Disord, Vol. 18, Supplement 2, September 2016 S11 Journal Identification = EPD Article Identification = 0835 Date: October 5, 2016 Time: 9:48 pm G. Avanzini, et al. FP2-F4 F4-C4 C4-P4 P4-O2 F8-T4 T4-T6 Fp1-F3 F3-C3 C3-P3 F3-O1 F7-T3 T3-T5 Fz-Cz 100 µv Cz-Pz 1 sec RWFL RWEXT LWFL LWEXT Figure 1. Patient with Unverricht-Lundborg disease. Polygraphic recording with the patient at rest showing fragmentary multifocal myoclonus without overt EEG correlate (EMG artefacts due to myoclonic jerks involving the face are superimposed onto the EEG trace). precision measurement of their time relationship to Polygraphic recordings and EEG-EMG muscle jerks. For these reasons, neurophysiological correlations in progressive myoclonus analysis is a first-line approach to myoclonic syn- dromes, both in terms of clinical characterization and epilepsy pathophysiological investigation. In epileptic disorders, polygraphy with simultaneous The first section of this article deals with the neu- recording of EEG-EMG activity can provide relevant rophysiological techniques suitable for characterizing information for defining the characteristics of a motor different types of myoclonus, while the second section manifestation and the relationship with concomitant addresses the value of neurophysiology in defining the EEG activity. Moreover, it can be useful to identify clinical presentation of some progressive myoclonus subtle and apparently subclinical manifestations, and epilepsies (PMEs) (Minassian et al., 2016). is necessary for precise investigation of the tempo- ral relationship between EEG and EMG phenomena Neurophysiological analysis (Tassinari and Rubboli, 2008). of myoclonus In PMEs, polygraphic recording can be a crucial tool for the investigation and definition of the characteristics The correlation between EEG and EMG activities asso- of myoclonic phenomena, which represent one of the ciated with myoclonus is the basis for investigating the cardinal features of this vast group of diseases. EMG pathophysiology of myoclonus as well as the clinical is usually recorded using surface electrodes placed on diagnosis of PMEs. Several signal analysis techniques the skin overlying the muscles involved in myoclonic relating to time and frequency domains, which are activity, which should be clearly identified by clinical currently employed to detect EEG correlated with examination (figure 1 figure 1). The cortical correlates myoclonus and used to investigate its pathophysiol- of myoclonus have also been analyzed using magneto- ogy, will be highlighted in this first section. encephalography (MEG), which can complement the S12 Epileptic Disord, Vol. 18, Supplement 2, September 2016 Journal Identification = EPD Article Identification = 0835 Date: October 5, 2016 Time: 9:48 pm Neurophysiology of myoclonus and progressive myoclonus epilepsies At Rest Arms Outstretched FP2-F4 F4-C4 C4-P4 P4-O2 Fz-Cz Cz-Pz Fp1-F3 F3-C3 C3-P3 P3-O1 R.Orb. Or. R. SCM R. Delt R.Wrist Flex R.Wrist Ext. R. APB R. Tib. Ant. 100µv female 27 yrs old 1sec. Figure 2. Patient with PME associated with SCARB2 mutation, at disease onset. Left panel: the patient is at rest; EEG shows preserved background activity without epileptiform abnormalities; on the EMG channels, erratic multifocal myoclonic jerks without EEG correlate are evident. Right panel: the patient keeps her arms outstretched; the EMG channels show continuous rhythmic cortical myoclonic activity at a frequency around 12-20 Hz. EEG information in terms of the cortical generators of negative myoclonus is common in the same patient. myoclonus (Mima et al., 1998). The EMG correlate of a single action myoclonus is Myoclonus is an essential and defining feature of PMEs. an EMG potential of short duration (20 to 30 ms), It can occur spontaneously or be induced or exacer- which appears synchronously in agonist and antag- bated by a variety of stimuli (such as light, sound, touch onist muscles (figure 3). It is usually followed by an and emotional strain) and active movement or posture EMG-silent period lasting 40 to 120 ms (in rare cases maintenance. At rest, in PMEs, myoclonus is commonly up to 300 ms). The myoclonic bursts and silent periods fragmentary and multifocal, and is particularly appar- ent in the musculature of the face and distal limbs (figure 2, left panel). Action myoclonus, in which move- ment (as an attempt or intention to move) initiates A jerking, is a common feature in almost all the condi- tions underlying PMEs, and can be extremely disabling. RW Flexor At rest, the EMG expression of myoclonus is a burst of myoclonic potentials of brief (100 ± 50 ms) dura- RW Extensor tion, typically occurring synchronously in agonist and antagonist muscles. If myoclonus occurs in a contract- B ing muscle, then after the myoclonus there is a brief RW Flexor (50-100 ms) suppression of muscle activity. A period of suppression of muscle activity without RW Extensor a preceding myoclonus can also produce a negative jerk, due to a sudden interruption and resumption of 1 s ongoing muscular activity. This latter phenomenon is referred to as ‘negative myoclonus’ and is related to Figure 3. EMG recording from two antagonist muscles of the a mechanism of supraspinal inhibition lasting from right wrist (RW) showing the presence of synchronous EMG 100 to 500 ms. In PMEs, a mixture of positive and bursts with an irregular (A) and quasi-rhythmic (B) course. Epileptic Disord, Vol. 18, Supplement 2, September 2016 S13 Journal Identification = EPD Article Identification = 0835 Date: October 5, 2016 Time: 9:48 pm G. Avanzini, et al. are seldom related to EEG spike and waves or polyspike by coherence and phase analysis of EEG-EMG signals, and waves (Tassinari et al., 1974). More often, the indicating a significant EEG-EMG coupling and a direct EEG correlate is small in transient amplitude, merg- corticospinal transfer (Rubboli et al., 2011). Rhyth- ing with spontaneous EEG activity. The cortical nature mic jerks in the beta band have also been described of the myoclonic event can be assumed when a time- in some PMEs associated with rare storage diseases locked spike or other EEG paroxysmal event is detected (Brown et al., 1999; Panzica et al., 2003; Canafoglia et on EEG-EMG polygraphic recordings either by visual al., 2006). inspection (figure 1) or by the back-averaging tech- Additional information can be drawn from sleep niques that will be described in detail below. Cortical recordings which should be performed in the PME myoclonus, regardless of whether it is positive or work-up whenever possible. The results
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