Unilateral Involuntary Movement Associated with Streptococcal Infection: Neurophysiological Investigation

Two boys developed rhythmic involuntary movements in the extremities on one side of the body after febrile illness. They also showed behavioral disturbances. In both patients, serum antistreptolysin-O and antistreptokinase titers were elevated in acute illness and decreased a few months later. One patient showed tremorous movement, and the other choreiform movement. In the former, a surface EMG showed short-duration (30 to 60 ms), highly frequent (6 to 8 Hz) and synchronous discharges of multiple muscles, including the antagonists, suggesting myoclonic jerk. In the latter, a surface EMG showed long-duration (0.5 to 1 s), repetitive (about 0.5 Hz) and synchronous or asynchronous discharges of the antagonists, suggesting choreoathetosis. In both patients, giant somatosensory evoked potentials and high-voltage slow EEG activities were observed predominantly in the hemisphere contralateral to the involuntary movement. In the myoclonic patient, long-latency EMG responses were enhanced and cortical potentials preceding the myoclonus were present by jerk-locked back averaging technique. The present data suggest that unilateral rhythmic involuntary movements occur secondary to streptococcal infection. The pathophysiology of the involuntary movements may be associated with sensorimotor cortex hyperexcitability.

Key words

Streptococcal infection - Syndenham chorea - Giant SEP - C reflex - Cortical reflex myoclonus - PANDAS

References

• 1 Colony H S, Malamuda N. Sydenham's chorea: a clinicopathological study.  Neurology. 1956;  6 672-676
• 2 DiFazio M P, Morales J, Davis R. Acute myoclonus secondary to group A β-hemolytic streptococcus infection: a PANDAS variant.  J Child Neurol. 1998;  13 516-518
• 3 Giedd J N, Rapoport J L, Leonard H L, Richter D, Swedo S E. Case study: acute basal ganglia enlargement and obsessive-compulsive symptoms in an adolescent boy.  J Am Acad Child Adolesc Psychiatry. 1996;  35 913-915
• 4 Husby G, Rijn van den I, Zabriskie J B, Abdin Z H, Williams R C. Antibodies reacting with cytoplasm of subthalamic and caudate nuclei neurons in chorea and acute rheumatic fever.  J Exp Med. 1976;  144 1094-1110
• 5 Kakigi R, Shibasaki H. Somatosensory evoked potentials following stimulation of the lower limb in cortical reflex myoclonus.  J Neurol Neurosurg Psychiatry. 1987;  50 1641-1646
• 6 Kienzle G D, Breger R K, Chun R WM, Zupanc M L, Sackett J F. Sydenham chorea: MR manifestations in two cases.  AJNR. 1991;  12 73-76
• 7 Kiessling L S, Marcotte A C, Culpepper L. Antineuronal antibodies in movement disorders.  Pediatrics. 1993;  92 39-43
• 8 Peterson B, Riddle M A, Cohen D J, Katz L D, Smith J C, Hardin M T, Leckman J F. Reduced basal ganglia volumes in Tourette's syndrome using three-dimensional reconstruction techniques from magnetic resonance images.  Neurology. 1993;  43 941-949
• 9 Shibasaki H, Motomura S, Yamashita Y, Shii H, Kuroiwa Y. Periodic synchronous discharge and myoclonus in Creutzfeldt-Jakob disease: diagnostic application of jerk-locked averaging method.  Ann Neurol. 1981;  9 150-156
• 10 Shibasaki H, Yamashita Y, Neshige R, Tobimatsu S, Fukui R. Pathogenesis of giant somatosensory evoked potentials in progressive myoclonic epilepsy.  Brain. 1985;  108 225-240
• 11 Shibasaki H. Electrophysiologic studies of myoclonus.  Muscle Nerve. 1988;  11 899-907
• 12 Silfverskiold B P. Rhythmic myoclonus in three girls.  Acta Neurol Scand. 1969;  38 45-59
• 13 Singer H S, Giuliano J D, Hansen B H, Hallett J J, Laurino J P, Benson M, Kiessling L S. Antibodies against human putamen in children with Tourette syndrome.  Neurology. 1998;  50 1618-1624
• 14 Swedo S E, Leonard H L, Kiessling L S. Speculations on antineuronal antibody-mediated neuropsychiatric disorders of childhood.  Pediatrics. 1994;  93 323-326
• 15 Swedo S E, Leonard H L, Garvey M, Mittleman B, Allen A J, Perlmutter S, Dow S, Zamkoff J, Dubbert B K, Lougee L. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases.  Am J Psychiatry. 1998;  155 264-271