Skip to main content
Have a personal or library account? Click to login
Post-hypoxic Myoclonus: Current Concepts, Neurophysiology, and Treatment Cover

Post-hypoxic Myoclonus: Current Concepts, Neurophysiology, and Treatment

Open Access
|Sep 2016

Figures & Tables

Table 1

Neurophysiological Findings in Cases of Acute PHM

ReferenceFindingsOutcome
Baldy-Moulinier et al.60EEG demonstrated bilaterally synchronous spikes and wave.Patient survived and developed short term memory loss.
Madison and Niedermeyer61Case 1: continuous generalized synchronous multiple spikes and spike wave like discharges associated with myoclonic jerks.
Case 2: continuous rhythmic 2 to 3 per second with a definite spike component.
Case 3: generalized synchronous sharp waves of high voltage and a small number associated with myoclonus.
Case 4: excessive diffuse slow activity.
Case 5: high voltage delta activity; no spikes were noticed.
Cases 1 and 2 survived while the other three patients died.
Van Woert et al.62EEG: diffuse slowing and occasional theta activity.This patient survived and went on to develop LAS.
Wolf63Myoclonus was associated with EEG bursts.None of the patient survived post-anoxic myoclonus.
Niedermeyer et al.64Spike activity on EEG seen with myoclonus.Patient did not regain consciousness and died.
Jumao-as et al.11EEG performed in 15 patients with anoxic encephalopathy showed burst suppression and generalized periodic complexes as the most common pattern.None of the patients regained consciousness and all died.
Young et al.5EEG performed in 6 patients with generalized myoclonus after CPR: burst suppression and alpha coma was commonly seen.None of the patients with generalized myoclonus after CPR regained consciousness.
Harper et al.65EEG showed occasional bursts of activity at 7–11 Hz.Patient survived and developed LAS.
Wijdicks et al.14Common EEG pattern seen in cases of generalized myoclonus after CPR included burst suppression, polyspiked waves, and alpha coma.None of the patients with generalized myoclonus after CPR regained consciousness.
Wijdicks66EEG: burst suppression pattern in 2 patients after hypoxia.Both patients died.
Zivkovic and Brenner67No changes in EEG with myoclonus.
Tactile stimulation led to generalized spike and wave discharges and generalized myoclonus.
Patient did not regain consciousness and died.
Hui et al.68EEG was performed in 10 patients who developed generalized myoclonus after anoxia; 6 patients had generalized polyspikes related to myoclonus while other 4 had low voltage diffuse activity.16 out of 18 patients with generalized myoclonus died while 1 remained in a persistent vegetative state and another one developed LAS.
Thömke et al.69Different patterns of EEG were described: burst suppression, continuous generalized epileptiform discharges, and alpha coma.45 patients with generalized myoclonus after CPR died while 5 remained in a permanent vegetative state.
Fernández-Torre et al.70EEG showed burst suppression pattern. Generalized spike and wave discharges were precipitated with touch.Patient did not regain consciousness and died.
Kakisaka et al.26EEG: burst suppression pattern.EMG: agonist and antagonist muscle contract simultaneously.Patient survived with significant disabilities.
Fernández-Torre et al.8EEG: periodic, regular, generalized burst of slow waves intermixed with prominent spikes, polyspikes, and sharp waves. This pattern was accompanied with eye opening movements.Patient did not regain consciousness and died.
Legriel et al.10EEG: artifacts associated with jerks.Patient did not regain consciousness and died.
Arpesella et al.56EEG: continuous spikes, polyspikes, and slow wave diffuse activity.Patient survived and developed LAS.
English et al.71EEG: frequent myoclonic polyspikes were observed.Patient developed LAS but eventually died of pneumonia.
Datta et al.19EEG: continuous generalized sharp and slow epileptiform activity disrupted by bursts of generalized polyspikes. This was not associated with myoclonus.Patient developed LAS but died later due to hemoptysis.
Rossetti et al.17EEG in two patients with myoclonus showed bilateral, repetitive, and diffuse sharp waves; third patient: demonstrated sharp, spike waves with stimulus induced rhythmic, periodic, or ictal discharges. All three patients had preserved N20 on SEP.
Five patients with axial myoclonus without corresponding EEG changes died.23 patients with myoclonus demonstrated ictal EEG. Only one patient survived out of 23.
2 out of 3 patients who survived cardiac arrest went on to develop LAS. They were treated with therapeutic hypothermia.
Thömke et al.31EEG: burst suppression pattern in 39 out of 60 patients and continuous generalized epileptiform discharges in the rest.59 patients died and one survived in a persistent vegetative state.
Rajamani et al.13EEG: generalized periodic discharges with no perceptible background rhythm.Patient did not regain consciousness and died.
Lee and Lee41EEG: complex spike waves bifrontally.Patient developed LAS.
Bouwes et al.253 out of 51 SEPs were giant; EEG: epileptiform activity (33%); status epilepticus (22%); generalized periodic discharges (25%); burst suppression (6%)Good outcome was observed in 12% of the patients with acute PHM. Only one patient with status myoclonus survived and made a good recovery.
Lucas et al.21Case 1: EEG: diffusely slow background; Case 2: EEG: burst suppression pattern followed by intermittent epileptiform activity; Case 3: EEG: slow and disorganized background rhythm.All three patients survived following treatment with therapeutic hypothermia (one patient later died of septic shock).
Legriel et al.72EEG: burst suppression.Patient did not regain consciousness and died.
Fernández-Torre et al.73EEG: burst suppression.Patient did not regain consciousness and died.
Shin et al.74EEG: cyclic epileptiform waves.Patient survived and developed LAS.
Mader et al.75EEG: spike and wave activity.Patient did not regain consciousness and died.
Accardo et al.20Unreactive EEG with diffuse epileptiform discharges associated with continuous generalized multifocal myoclonus.Patient developed LAS.
Tsai et al.76EEG showed diffuse cortical dysfunctionMyoclonus was observed after the discontinuation of muscle relaxant which was done for therapeutic hypothermia. Patient regained consciousness after 13th day of cardiac arrest.
van Zijl et al.12EEG in generalized myoclonus group revealed status epilepticus (64%), diffuse slowing (17%), and burst suppression (11%).
Multifocal myoclonus group had diffuse slowing (52%), low voltage (13%), and status epilepticus (13%).
Only 1 patient out of 17 with generalized myoclonus survived.
3 patients out of 43 with PHM developed LAS.34 patients out of 43 with PHM died.
Seder et al.16EEG: 27% electrographic status epilepticus; 55% epileptiform activity (electrographic seizure or periodic epileptiform discharges)Good outcome in patients when myoclonus was not associated with epileptiform activity on EEG.
Dericioglu et al.9EEG: generalized high-amplitude, spike and sharp wave complexes.
Around 50 hours after arrest, EEG showed bilateral independent discharges.
Patient did not regain consciousness and died.
Sanna et al.77Alpha coma pattern was seen on EEG.Progressed to develop LAS.
Walsh BH et al.78EEG showed suppressed background with intermittent rhythmic pattern which was later observed to be an artifact.Patient did not regain consciousness and died.

[i] Abbreviations: CPR, Cardiopulmonary Resuscitation; EEG, Electroencephalography; LAS, Lance–Adams Syndrome; PHM, Post-hypoxic Myoclonus; SEP, Somatosensory Evoked Potential.

Figure 1

Neurophysiological findings in acute PHM. More than one pattern of electroencephalography was seen in the same patient during the course of myoclonus. Burst suppression was the most common pattern while alpha coma was the least common pattern observed. The highest rate of survival was seen in the diffuse slowing group and the lowest rate was seen in the spike-wave group. Multiple studies were commonly performed in the same patient.

Table 2

Neurophysiological Findings in Cases of LAS

ReferenceEEGEMGJerk-locked Back-averagingLong Latency ReflexSEP
Lance and Adams2Case 1: spike discharges at paracentral electrode.
Case 2: no abnormality.
Case 3: atypical spike and wave at 2.5–5 per second.
Spike was usually recorded in all muscles of the limb; contraction was stronger in flexors.Myoclonic jerks followed shortly after spikes; duration of negative cortical spike varied from 15–35 ms and followed by positive deflection of 10–15 ms.NANA
Erbslöh and Prüll792–4 c/sec spike and wave; polyspike and wave synchronous with the myoclonus.NAAfter a latency of 20–50 ms following the cortical spikes myoclonus was observed.NANA
Hirose et al.80Bitemporal independent random low amplitude delta slowing. No cortical spike potentials were observed.NANANANA
Tassinari et al.81Well-preserved background activity with rare bursts of low voltage sharp theta activity. During REM sleep, typical long lasting bursts of fast spikes over the midline and frontocentral regions.NANANANA
Van Woert et al.62Case 1: moderate generalized slow activity with occasional sharp waves.NANANANA
Goldberb and Dorman48Case 1: mild slowing.
Case 2: focal spikes.
Case 3: normal.
NANANANA
De Léan et al.43Case 1: Muscle artifact; no spike activity observed.
Case 2: slow activity over the left hemisphere; no spike activity.
NANANANA
Hallett et al.28Slow and fast activity over both hemispheres and very frequent spikes followed by slow waves.The duration of EMG was 10-30 msec.
Cranial nerve nuclei were activated in ascending order and arm muscles before the leg.
EEG spikes were not time locked to EMG discharges.NANormal.
Carroll and Walsh82Central intermittent single and short duration multiple spike and slow wave complexes.
Background activity was 6–7 Hz theta activity. Some discharges were associated with myoclonus.
NANANANA
Hallett et al.83Diffusely slow with mixed sharp waves.Simple waveform lasting 10 to 30 msec.
Cranial muscles activated before the arm and arm muscles before the leg.
An EEG negative transient preceded and was time locked to the myoclonus.NAGiant SEPs.
Fahn84Case 1: generalized spike and slow wave complexes that were maximum parasagittally.
Case 2: generalized spike and slow wave complexes that were maximal in frontocentral region.
Case 3: fast activity in the frontocentral region.
Case 1: bilaterally symmetrical motor unit discharges that were synchronous in all extremities.
Case 2: synchronous contraction in all muscle groups tested. There was no correlation between myoclonic jerks and epileptiform discharges.
NANACase 1: normal
Case 2: normal
Case 3: high amplitude waves
DeLisa et al.85NormalNANANANA
Bruni et al.47Frequent bisynchronous spike and polyspike and wave localized to centroparietal region. No relationship was observed between EEG and myoclonus.NANANANA
Rollinson and Gilligan86NormalNANANANA
Coletti et al.87Paroxysms of symmetrical sharp waves of large amplitude and bursts of polyspike and spike-wave over the central regions.Demonstrated simultaneous involvement of agonist and antagonist muscles.Simultaneous EEG–EMG recording did not demonstrate correlation between myoclonus and EEG.NANA
Kelly et al.88NormalNANANormalNormal
Sotaniemi89Symmetric slowing and intermittent delta activity.NANANANA
Magnussen et al.90Case 1: normal when myoclonus appears.
Case 2: 4–6 Hz activity without changes related to the myoclonus.
NANANANA
Obeso et al.91Excess of generalized theta activity.Duration of EMG discharge was 60 to 120 msec.EEG potential was not time locked to the jerk.NANot enlarged.
Chee and Poh925–6 cps theta activity. No epileptiform activity detected.NANANANA
Fahn33Case 5: EEG slow background with low voltage spikes and polyspikes bilaterally.Case 1: EMG shows synchronous and asynchronous firing of leg flexors and extensors.Case 5: jerk-locked averaging failed to show cortical spikes.NACase 5: normal.
Case 6: normal.
Hauw et al.36Case 1: EEG showed spontaneous paroxysmal crises with spike activity associated with myoclonus.
Case 2: EEG showed bilateral delta waves and no spike activity.
Case 3: EEG did not show spike associated with myoclonus.
Case 4: EEG showed slow waves of 4–5 Hz with intermittent paroxysmal potentials.
NANANANA
Kim et al.93Slow theta to delta waves were seen in both hemispheres.NANANANormal.
Giménez-Roldán et al.454–6 Hz bitemporal runs; no activity with myoclonus observed.NANANANA
Witte et al.94Spike activity at the vertex when patient performed any movement.Simultaneous EEG–EMG showed that spikes were present before any myoclonic movement was observed.NANANormal.
Obeso et al.95NAEMG discharges were brief (less than 50 ms).NANAEnlarged.
Brown et al.30Generalized and polyphasic spikes followed by slow wave. The spikes were usually associated with myoclonus.EMG activity lasting 25–100 ms.Positive peak preceded the jerk by 10 ms.NADisorganized and of small amplitude.
Rizvi and Karetzky96NormalNANANANA
Wicklein and Schwendemann97Spike and wave activity variably related to the myoclonus.NANANANA
Yamaoka et al.98Case 1: spike discharge.
Case 2: alpha and theta waves and no spike discharge was seen.
NANANANA
Werhahn et al.997 out of 14 patients with LAS had abnormal EEG. These included: bilateral spikes, sharp waves, polyspike wave complexes sometimes associated with myoclonus.NAAll patients except one had a time-locked cortical correlate preceding myoclonus.NA2 out of 14 patients with LAS had pathologically enlarged cortical SEPs.
One patient had normal SEPs.
Lance and Adams, 2001100Simultaneous EEG–EMG recording showed spike discharges preceding myoclonus by 7 ms for occipital muscles and 32 ms for quadriceps.NANANANA
Krauss et al.50Case 1: several temporal-central spikes.
Case 2: generalized slowing but no epileptiform discharges.
NANANANA
Frucht et al.38NANAPerformed in three patients showed cortical origin of myoclonus.NANA
Polesin and Stern57Spike and polyspike discharges over the vertex region which was consistent with her myoclonic jerks.NANANANA
Zhang et al.40Case 1: low amplitude alpha waves, diffuse delta activity, and sharp and slow waves at night. No epileptiform discharge during myoclonus.
Case 2: low amplitude theta waves mixed with faster waves; no epileptiform discharge during myoclonus.
NANANANA
Datta et al.19Sporadic generalized epileptiform discharges not correlated with myoclonic jerks.NANANANA
Arpesella et al.56Diffuse alpha activity mixed with theta.NANANANA
Yamada et al.52No epileptiform discharges.Brief generalized bursts lasting 20–30 ms.NANANormal.
Huang et al.101NAShort duration (<30 ms) myoclonic bursts.EEG event 26 ms prior to the myoclonus.NANA
González de la Aleja et al.58Generalized spike and slow wave complexes with mild background slowing.EEG–EMG simultaneous recording revealed a time locked cortical discharge preceding myoclonus and myoclonus without a correlated EEG.NANANA
Accardo et al.20Normal background with isolated brief polyspike discharges associated with myoclonic jerk.NANANANA
Cho et al.102NormalNANANANA
Ferlazzo et al.37NAEEG–EMG simultaneous recording showed generalized spike and polyspike-wave discharges associated with myoclonus.NANANA
Ilik et al.103Frequent myoclonic polyspikes.NANANANA
Budhram et al.104Case 1: generalized spike and polyspike wave discharges which correlated with myoclonic jerks.
Case 2: no epileptiform activity was observed.
NANANANA
Božić et al.105Spikes and polyspikes on slow wave background activity. The EEG spikes were highly frequent and generalized.
Myoclonus and EEG abnormalities were not linked to each other.
NANANANormal.
Asahi et al.51NAShowed a cortical myoclonus pattern.NANANormal.
Park et al.39Frequent bilateral central or hemispheric spike or poly-spike discharges.NANANANA
Sanna et al.77Spike and slow wave synchronous with myoclonus.NANANANA

[i] Abbreviations: EEG, Electroencephalography; EMG, electromyography; LAS, Lance–Adams Syndrome; NA, Not Applicable; SEP, Somatosensory Evoked Potential.

Figure 2

Neurophysiological findings in CHRONIC PHM (LAS). Electroencephalography is the most common neurophysiological study followed by jerk-locked back averaging in chronic post-hypoxic myoclonus. An overwhelming majority of myoclonus in chronic PHM was found to be of cortical origin with jerk-locked back averaging. Multiple studies were commonly performed in the same patient.

DOI: https://doi.org/10.5334/tohm.323 | Journal eISSN: 2160-8288
Language: English
Page range: 409 - 409
Submitted on: May 31, 2016
Accepted on: Aug 16, 2016
Published on: Sep 17, 2016
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2016 Harsh V. Gupta, John N. Caviness, published by Columbia University Libraries/Information Services
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.