Pathophysiology of chorea and bradykinesia in Huntington's disease

Alfredo Berardelli, Johannes Noth, Philip D. Thompson, Eduard L E M Bollen, Antonio Currà, Gunther Deuschl, J. Gert Van Dijk, Rudolf Töpper, Michael Schwarz, Raymund A C Roos

Research output: Contribution to journalArticle

Abstract

This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median-nerve somatosensory-evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long-latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia-thalamus-cortical motor circuit.

Original languageEnglish
Pages (from-to)398-403
Number of pages6
JournalMovement Disorders
Volume14
Issue number3
DOIs
Publication statusPublished - 1999

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Chorea
Hypokinesia
Huntington Disease
Basal Ganglia
Arm
Contingent Negative Variation
Hyperkinesis
Blinking
Stretch Reflex
Muscles
Somatosensory Evoked Potentials
Transcranial Magnetic Stimulation
Caudate Nucleus
Median Nerve
Movement Disorders
Motor Cortex
Eye Movements
Thalamus
Hand
Maintenance

Keywords

  • Huntington's disease
  • Motor system
  • Neurophysiology

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Berardelli, A., Noth, J., Thompson, P. D., Bollen, E. L. E. M., Currà, A., Deuschl, G., ... Roos, R. A. C. (1999). Pathophysiology of chorea and bradykinesia in Huntington's disease. Movement Disorders, 14(3), 398-403. https://doi.org/10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F

Pathophysiology of chorea and bradykinesia in Huntington's disease. / Berardelli, Alfredo; Noth, Johannes; Thompson, Philip D.; Bollen, Eduard L E M; Currà, Antonio; Deuschl, Gunther; Van Dijk, J. Gert; Töpper, Rudolf; Schwarz, Michael; Roos, Raymund A C.

In: Movement Disorders, Vol. 14, No. 3, 1999, p. 398-403.

Research output: Contribution to journalArticle

Berardelli, A, Noth, J, Thompson, PD, Bollen, ELEM, Currà, A, Deuschl, G, Van Dijk, JG, Töpper, R, Schwarz, M & Roos, RAC 1999, 'Pathophysiology of chorea and bradykinesia in Huntington's disease', Movement Disorders, vol. 14, no. 3, pp. 398-403. https://doi.org/10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F
Berardelli, Alfredo ; Noth, Johannes ; Thompson, Philip D. ; Bollen, Eduard L E M ; Currà, Antonio ; Deuschl, Gunther ; Van Dijk, J. Gert ; Töpper, Rudolf ; Schwarz, Michael ; Roos, Raymund A C. / Pathophysiology of chorea and bradykinesia in Huntington's disease. In: Movement Disorders. 1999 ; Vol. 14, No. 3. pp. 398-403.
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