Introduction and Definitions
Myoclonic seizures (MS) are characterized by sudden, brief (<350 ms), shock-like contractions that vary in their distribution and intensity of manifestations (Leppik 2003). They may be generalized or confined to the face and trunk, or to extremities, or individual muscles or groups of muscles. MS can occur unilaterally or bilaterally, and if bilaterally, symmetrically or asymmetrically. They may also be rhythmic or arrhythmic, and, in terms of amplitude, they can be small (e.g., no joint movement) or massive contractions (e.g., movement of extremities, trunk, and/or head) (Video 1, 2)(Leppik 2003; Browne and Holmes 2004). Myoclonic jerks may originate in the form of brief electromyographic (EMG) burst (i.e., positive myoclonus) or, more rarely, from brief interruptions of EMG activity (i.e., negative myoclonus) (Genton et al. 2005; Guerrini et al. 2005). MS may be very subtle or extremely dramatic and can be present in several different epilepsy syndromes,...
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Abbreviations
- CJD:
-
Creutzfeldt–Jakob disease
- CSTB:
-
Cystatin B
- EEG:
-
Electroencephalography
- EMG:
-
Electromyographic
- JLA:
-
Jerk-locked averaging
- LLRI:
-
Long-loop reflex I
- MERRF:
-
Myoclonic epilepsy with ragged-red fibers
- MS:
-
Myoclonic seizures
- SEPs:
-
Somatosensory-evoked potentials
- ULD:
-
Unverricht–Lundborg disease
References
Browne TR, Holmes GL (2004) Handbook of epilepsy, 3rd edn. Lippincott Williams Wilkins, Philadelphia, pp 37–38
Genton P, Roger J, Guerrini R, Medina MT, Bureau M, Dravet C, Delgado-Escueta AV (2005) History and classification of “myoclonic” epilepsies: from seizures to syndromes to diseases. Adv Neurol 95:1–14
Guerrini R, Bonanni P, Parmeggiani L, Hallett M, Oguni H (2005) Pathophysiology of myoclonic epilepsies. Adv Neurol 95:23–46
Leppik IE (2003) Classification of the myoclonic epilepsies. Epilepsia 44(Suppl 11):2–6
Marsden CD, Hallett M, Fahn S (1985) The nosology and pathophysiology of myoclonus. In: Marsden CD, Fahn S (eds) Movement disorders, 2nd edn. Butterworth Scientific, London, pp 198–248
Shibasaki H (2006) Neurophysiological classification of myoclonus. Clin Neurophysiol 36:267–269
Shibasaki H, Hallett M (2005) Electrophysiological studies of myoclonus. Muscle Nerve 31:157–174
Acknowledgment
We are grateful to Professor Salvatore Striano and Dr. Giuseppe Capovilla for revising the text and kindly contributing to the illustrations.
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Striano, P., Zara, F. (2010). Pathophysiology of Myoclonic Seizures. In: Panayiotopoulos, C.P. (eds) Atlas of Epilepsies. Springer, London. https://doi.org/10.1007/978-1-84882-128-6_31
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