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
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