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J Epilepsy
1988;1:173-195© 1988 Demos Publications
Myoclonic, Tonic, and Atonic Seizures in Children:Clinical and Electroencephalographic Features
Gregory L. Holmes
Tonic, atonic, and myoclonic seizures are among the most difficult seizures toclassify, diagnose, and treat in children. In this review, syndromes associatedwith childhood tonic, atonic, and myoclonic seizures are reviewed with particularattention to the clinical manifestations, EEG findings, and management. Key
Words:
Epilepsy--Tonic seizures--Atonic seizures--Myoclonus.The taxonomy of myoclonic, tonic, and atonicseizures is one of the most controversial and con-fusing problems in pediatric epileptology. Myoclo-nus, in particular, represents a particularly difficultproblem in classification. Clinicians have struggledwith the concept of myoclonus in an effort to define itproperly, to differentiate it from other movement dis-orders, and to elucidate its pathophysiology (1-7).No common etiological, anatomical, or physiologicalfeatures bind all types of myoclonus. Myoclonus canarise from many areas of the central nervous system,and the condition has been reported in associationwith lesions of the cortex, cerebellum, brainstem, andspinal cord. Likewise, multiple etiological agents canlead to myoclonus. As can be seen in Table 1, the etio-logies of myoclonus are quite diverse and vary con-siderably in significance. Myoclonus may be a totallynormal phenomenon, such as hypnic jerks or sleepstarts. Conversely, myoclonus may be associatedwith virtually any severe insult to the brain, whethertoxic, metabolic, infectious, traumatic, or degenera-tive. Likewise, the pathophysiology of myoclonusvaries; some types of myoclonus are nonepilepticphenomena and are classified as movement disordersFrom the Department of Neurology, Harvard MedicalSchool and The Children's Hospital, Boston, MA, U.S.A.Address correspondence and reprint requests to Dr. G.L. Holmes at Clinical Neurophysiology Laboratory, TheChildren's Hospital, 300 Longwood Avenue, Boston, MA02115, U.S.A.similar to chorea, tics, athetosis, or tremors, whereasother types of myoclonus are epileptic phenomena(8). Without EEG monitoring it may be very difficultto differentiate epileptic from nonepileptic myoclonus.In this review, only epileptic myoclonus is discussed.In the International Classification of Seizures,myoclonic seizures are classified as a generalized sei-zure disorder (9). Myoclonic seizures may be of theprimary seizure type or a component of an epilepticsyndrome (Table 2). For example, myoclonic seizuresare the primary seizures in cryptogenic myoclonicepilepsy of early childhood and infantile spasms,whereas in the Lennox-Gastaut syndrome (LGS)myoclonic seizures are just one of many seizuretypes.Unlike myoclonus, tonic and atonic seizures aresomewhat easier to diagnose and differentiate fromnonepileptic events. Furthermore, both tonic andatonic seizures rarely occur as the isolated soleseizure types and are usually seen in association withother types of seizures.
Clinical Features of Myoclonic, Tonic,and Atonic Seizures
Some authors combine myoclonic, tonic, and atonicseizures together under the rubric "minor motor sei-zures" in order to distinguish seizures with lessmotor involvement from generalized tonic-clonicseizures. However, even though the seizures some-times are difficult to differentiate from one another,
j EPILEPSY, VOL. 1, NO. 4, 1988
173
 
G. L
HOLMES
Table
1.
Classification of myoclonus
Nonepileptic myoclonusPhysiological myoclonusSleep startsBenign awake myoclonusPathophysiological myoclonusHyperexplexia (startle disease)Shuddering attacksPeriodic movements of sleep (sleep myoclonus)Restless leg syndromeBenign neonatal sleep myoclonusBenign myoclonus of early infancyBenign familial polymyoclonusSegmental myoclonusBrain stemOpsoclonusPalatal myoclonusSpinal cordEpileptic myoclonusInfectionsSubacute sclerosing panencephalitis (SSPE)Jakob-Creutzfeldt diseaseEncephalitisCongenital brain anomaliesToxinsSystemic diseasesUremiaHepatic insufficiencyOthersPostanoxicComponent of familial progressive neurological diseaseProgressive myoclonic epilepsy with Lafora's bodiesProgressive myoclonic epilepsy without Lafora'sbodiesDyssnergia cerebellaris myoclonia (Ramsay-Hunt)Metabolic disordersCeroid lipofuscinosisJuvenile form of Gaucher's diseaseSialidosis (cherry red spot-myoclonus syndrome)Gangliosidoses including GM2, GM 1Mitochondrial syndromesMyoclonus and ragged red fibers (MERRF)Mitochondrial myopathy, encephalopathy, lacticacidosis, and stroke-like episodes (MELAS)Primary epileptic myoclonusGeneralized epileptic myoclonus of early childhoodSymptomatic myoclonic epilepsyCryptogenic myoclonic epilepsyJuvenile myoclonic epilepsy of JanzEpilepsia partialis continuaFocal cortical myoclonusAs component of other seizure typesAbsence with myoclonic componentEyelid myoclonic seizures with absencesComponent of generalized tonic-clonic seizuresCentrencephalic myoclonic-astatic petit realLennox-Gastaut syndrome174
] EPILEPSY, VOL. 1, NO. 4, 1988
they are classified as distinct seizure types in theInternational Classification of Epileptic Seizure (9).
Myoclonic Seizures
In epileptic myoclonus, the seizures are character-ized by sudden, brief (<350 ms), shock-like contrac-tions that may be generalized or confined to the faceand trunk or to one or more extremities, or even toindividual muscles or groups of muscles (7). Myoclo-nic seizures result in short bursts of synchronizedelectromyographic (EMG) activity, which often in-volves simultaneous activation of agonist and antago-nist muscles.Massive, generalized myoclonic jerks are usuallyeasily diagnosed. However, subtle myoclonic seizuresmay resemble tics, chorea, or tremors. Differentiatingmyoclonus from tics may be difficult. In children, ticsinvolve primarily the head, neck, and shoulders, andconsist of complex movements such as facial grim-acing, eye blinking or rolling, head nodding or turn-ing, and shrugging of the shoulders. Tics can usuallybe suppressed, at least temporarily, by an effort ofwill, whereas myoclonus cannot. In chorea, the move-ments occur randomly, usually in multiple musclegroups, whereas myoclonus is usually characterizedby repetitive, stereotyped movements affecting thesame muscle groups. Myoclonus does not have thecharacteristic continuous flow of movements that isso distinctive in chorea. Tremors can usually bedifferentiated from myoclonus by the smooth to-and-fro movements compared with myoclonus, which ismore abrupt and has distinct intervals between eachmovement.
Tonic Seizures
These seizures are characterized by periods oftonic contraction of muscle groups accompanied byaltered consciousness. While tonic seizures may bebrief, lasting only seconds, they are invariably longerthan myoclonic seizures. At times, however, it maybedifficult to distinguish myoclonic seizures from brieftonic seizures, since absolute criteria regarding maxi-mum duration of a myoclonic seizure have yet to beestablished. Like myoclonic seizures, EMG activity isdramatically increased in tonic seizures.Gastaut and colleagues (10) divided tonic seizuresinto four types: axial, axorhizomelic, global, andasymmetrical. Axial tonic seizures begin with a toniccontraction of the neck muscles, leading to fixation of
 
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MYOCLONIC, TONIC, AND ATONIC SEIZURES IN CHILDREN
the head in an erect position, widely opened eyes,and jaw clenching or mouth opening. Contraction ofthe respiratory and abdominal muscles follows, andthis may lead to a high-pitched cry and brief periodsof apnea. Tonic axorhizomelic seizures begin with asequence similar to the axial type, but then the toniccontractions extend to the proximal musculature ofthe upper limbs, elevating the shoulders and abduct-ing the arms. In global tonic seizures, the tonic con-tractions extend to the periphery of the limbs. Thearms are pulled upward to a semiflexed position infront of the head, and the fists are clenched, produc-ing a body position similar to that of a child defendinghimself against a facial blow. Involvement of thelower extremities can also occur, leading to falls if thechild is in a standing position. Asymmetrical tonicseizures vary from a slight rotation of the head to atonic contraction of all the musculature of one side ofthe body. Occasionally, tonic seizures terminate witha clonic phase.In a study of epileptic falls in children, Ikeno et al.(11) described two types of tonic seizures. The firsttype, termed the "tonic type," was characterized byexcessive flexion or extension of fingers, forced flex-ion of hand joints, jaw protrusion, shoulder elevation,upper arm abduction, and tonic flexion of the trunk.This hypertonic state continued unchanged evenafter the patient fell down. The second type, termed"flexor spasms," was differentiated from the tonictype by the different distribution of hypertonicity. Itwas characterized by forward flexion of the head,elevation of shoulders, abduction of the upper armsin which the arms are flung outward and forward, andflexion of the thighs at the hip. Ur~like what was seenin the tonic type, fingers, hand joints, and elbowsremained neither tonic nor atonic: The flexor spasmswere noted by the authors to resemble infantilespasms.Egli et al. (12) described tonic drop seizures as"~xial spasms." They consisted of a uniform patternof movement consisting of a moderate flexion of thehips, upper trunk, and the head lasting 0.5-0.8 s. Thearms were almost always abducted, elevated, and in asemiflexed position. The fall was provoked by therapidity and violence of the flexion in the hips.The period of impaired consciousness during tonicseizures has an average duration of around 10 s andranges from a few seconds to a minute. Postictalimpairment, if present, is usually brief.On occasion, breath-holding attacks in childrenmay be difficult to differentiate from tonic seizures (7,13-15). The usual clinical sequence in breath-holding attacks is crying--frequently after sustaininga mild head injury--followed by breath-holding,
j EPILEPSY, VOL 1, NO. 4, 1988
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