Abstract
Paroxysmal dyskinesia (PxD) is a rare group of movement disorders manifesting as abnormal involuntary movements that occur episodically and last only briefly. Among various complementary studies, neurophysiological series have distinct importance. Classical neurophysiological tests such as electroencephalography, electromyography, and somatosensory/visual evoked potentials are typically performed on patients with PxD. Although in most cases the results are negative, they are nevertheless important to rule out other neurological diseases included in the differential diagnosis or to confirm concurrent diseases such as epilepsy. Another group of neurophysiological techniques including magnetoencephalography and transcranial magnetic stimulation have been used to explore the pathophysiology of PxD. Both provide strong evidence regarding inhibitory impairment in sensorimotor circuits at both cortical and spinal levels in patients with PxD.
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Abbreviations
- CNV:
-
Contingent negative variation
- EEG:
-
Electroencephalography
- EMG:
-
Electromyography
- LICI:
-
Long intracortical inhibition
- MEG:
-
Magnetoencephalography
- MEP:
-
Motor evoked potentials
- PED:
-
Paroxysmal exertion/exercise-induced dyskinesia
- PHD:
-
Paroxysmal hypnogenic dyskinesia
- PKD:
-
Paroxysmal kinesigenic dyskinesia
- PNKD:
-
Paroxysmal non-kinesigenic dyskinesia
- PxD:
-
Paroxysmal dyskinesia
- SICI:
-
Short intracortical inhibition
- SNW:
-
Slow negative wave
- TMS:
-
Transcranial Magnetic Stimulation
- β-ERS:
-
Beta event-related synchronization
References
van Rootselaar AF, Schade van Westrum S, Velis DN, Tijssen MA. The paroxysmal dyskinesias. Pract Neurol. 2009;9(2):102–9.
Erro R, Bhatia KP. Unravelling of the paroxysmal dyskinesias. J Neurol Neurosurg Psychiatry. 2019;90(2):227–34.
Perona-Moratalla AB, Argandoña L, García-Muñozguren S. Paroxysmal dyskinesias. Rev Neurol. 2009;48 Suppl 1:S7–9. Spanish.
Bhatia KP. Paroxysmal dyskinesias. Mov Disord. 2011;26(6):1157–65.
Ohmori I, Ohtsuka Y, Ogino T, Yoshinaga H, Kobayashi K, Oka E. The relationship between paroxysmal kinesigenic choreoathetosis and epilepsy. Neuropediatrics. 2002;33(1):15–20.
Hirata K, Katayama S, Saito T, Ichihashi K, Mukai T, Katayama M, et al. Paroxysmal kinesigenic choreoathetosis with abnormal electroencephalogram during attacks. Epilepsia. 1991;32(4):492–4.
Lombroso CT, Fischman A. Paroxysmal non-kinesigenic dyskinesia: pathophysiological investigations. Epileptic Disord. 1999;1(3):187–93.
Suls A, Dedeken P, Goffin K, Van Esch H, Dupont P, Cassiman D, et al. Paroxysmal exercise-induced dyskinesia and epilepsy is due to mutations in SLC2A1, encoding the glucose transporter GLUT1. Brain. 2008;131(Pt 7):1831–44.
Erro R, Bhatia KP, Espay AJ, Striano P. The epileptic and nonepileptic spectrum of paroxysmal dyskinesias: channelopathies, synaptopathies, and transportopathies. Mov Disord. 2017;32(3):310–8.
Tinuper P, Cerullo A, Cirignotta F, Cortelli P, Lugaresi E, Montagna P. Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. Epilepsia. 1990;31(5):549–56.
Scheffer IE, Bhatia KP, Lopes-Cendes I, Fish DR, Marsden CD, Andermann E, et al. Autosomal dominant nocturnal frontal lobe epilepsy. A distinctive clinical disorder. Brain. 1995;118(1):61–73.
Mehta SH, Morgan JC, Sethi KD. Paroxysmal dyskinesias. Curr Treat Options Neurol. 2009;11(3):170–8.
Erro R, Brigo F, Trinka E, Turri G, Edwards MJ, Tinazzi M. Psychogenic nonepileptic seizures and movement disorders: a comparative review. Neurol Clin Pract. 2016;6(2):138–49.
Apartis E. Clinical neurophysiology in movement disorders. Handb Clin Neurol. 2013;111:87–92.
Marelli C, Canafoglia L, Zibordi F, Ciano C, Visani E, Zorzi G, et al. A neurophysiological study of myoclonus in patients with DYT11 myoclonus-dystonia syndrome. Mov Disord. 2008;23(14):2041–8.
Ganos C, Aguirregomozcorta M, Batla A, Stamelou M, Schwingenschuh P, Münchau A, et al. Psychogenic paroxysmal movement disorders--clinical features and diagnostic clues. Parkinsonism Relat Disord. 2014;20(1):41–6.
Dreissen YE, Tijssen MA. The startle syndromes: physiology and treatment. Epilepsia. 2012;53(Suppl 7):3–11.
Mir P, Huang YZ, Gilio F, Edwards MJ, Berardelli A, Rothwell JC, et al. Abnormal cortical and spinal inhibition in paroxysmal kinesigenic dyskinesia. Brain. 2005;128(Pt 2):291–9.
Walter WG, Cooper R, Aldridge VJ, McCallum WC, Winter AL. Contingent negative variation: an electric sign of sensorimotor association and expectancy in the human brain. Nature. 1964;203:380–4.
Franssen H, Fortgens C, Wattendorff AR, van Woerkom TC. Paroxysmal kinesigenic choreoathetosis and abnormal contingent negative variation. A case report. Arch Neurol. 1983;40(6):381–5.
Liu YT, Chen YC, Kwan SY, Chou CC, Yu HY, Yen DJ, et al. Aberrant sensory gating of the primary somatosensory cortex contributes to the motor circuit dysfunction in paroxysmal kinesigenic dyskinesia. Front Neurol. 2018;9:831.
Hsu WY, Kwan SY, Liao KK, Chen RS, Lin YY. Altered inhibitory modulation of somatosensory cortices in paroxysmal kinesigenic dyskinesia. Mov Disord. 2013;28(12):1728–31.
Cardin JA, Carlén M, Meletis K, Knoblich U, Zhang F, Deisseroth K, et al. Driving fast-spiking cells induces gamma rhythm and controls sensory responses. Nature. 2009;459(7247):663–7.
Hsiao FJ, Hsu WY, Chen WT, Chen RS, Lin YY. Abnormal somatosensory synchronization in patients with paroxysmal kinesigenic dyskinesia: a magnetoencephalographic study. Clin EEG Neurosci. 2017;48(4):288–94.
Hsu WY, Liao KK, Tseng YJ, Kwan SY, Chen RS, Lin YY. Reduced postmovement cortical inhibition in patients with paroxysmal kinesigenic dyskinesia. Neurology. 2013;81(4):353–60.
Margari L, Perniola T, Illiceto G, Ferrannini E, De Iaco MG, Presicci A, et al. Familial paroxysmal exercise-induced dyskinesia and benign epilepsy: a clinical and neurophysiological study of an uncommon disorder. Neurol Sci. 2000;21(3):165–72.
Jones SJ, Halonen JP, Shawkat F. Centrifugal and centripetal mechanisms involved in the “gating” of cortical SEPs during movement. Electroencephalogr Clin Neurophysiol. 1989;74(1):36–45.
Cohen LG, Starr A. Localization, timing and specificity of gating of somatosensory evoked potentials during active movement in man. Brain. 1987;110(2):451–67.
Rossini PM, Caramia MD, Zarola F. Mechanisms of nervous propagation along central motor pathways: noninvasive evaluation in healthy subjects and in patients with neurological disease. Neurosurgery. 1987;20(1):183–91.
Quartarone A, Bagnato S, Rizzo V, Morgante F, Messina C, Girlanda P. Transcranial magnetic stimulation as trigger of dystonic attacks in a patient affected by paroxysmal kinesigenic dyskinesia. Neurol Sci. 2005;26(5):362–6.
Kang SY, Sohn YH, Kim HS, Lyoo CH, Lee MS. Corticospinal disinhibition in paroxysmal kinesigenic dyskinesia. Clin Neurophysiol. 2006;117(1):57–60.
Shin HW, Kang SY, Hallett M, Sohn YH. Extended surround inhibition in idiopathic paroxysmal kinesigenic dyskinesia. Clin Neurophysiol. 2010;121(7):1138–41.
Lee MS, Kim WC, Lyoo CH, Lee HJ. Reciprocal inhibition between the forearm muscles in patients with paroxysmal kinesigenic dyskinesia. J Neurol Sci. 1999;168(1):57–61.
Day BL, Marsden CD, Obeso JA, Rothwell JC. Reciprocal inhibition between the muscles of the human forearm. J Physiol. 1984;349:519–34.
Sibilla S, Ballerini L. GABAergic and glycinergic interneuron expression during spinal cord development: dynamic interplay between inhibition and excitation in the control of ventral network outputs. Prog Neurobiol. 2009;89(1):46–60.
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Palomar, F.J., Mir, P. (2021). Neurophysiology of Paroxysmal Dyskinesia. In: Sethi, K.D., Erro, R., Bhatia, K.P. (eds) Paroxysmal Movement Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-53721-0_8
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DOI: https://doi.org/10.1007/978-3-030-53721-0_8
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