Brain, Vol 121, Issue 7 1195-1212, Copyright © 1998 by Oxford University Press
A Berardelli, JC Rothwell, M Hallett, PD Thompson, M Manfredi and CD Marsden
Co-contraction and overflow of EMG activity of inappropriate muscles are
typical features of all dystonic movements whether voluntary or
involuntary. Voluntary movements are slow and more variable than normal,
and there is particular difficulty switching between component movements of
a complex task. Reduced spinal cord and brainstem inhibition is common to
many reflex studies (long-latency reflexes, cranial reflexes and reciprocal
inhibition). These reflex abnormalities may contribute to the difficulties
in voluntary movements but cannot be causal as they can occur outside the
clinically involved territory. Clinical and neurophysiological studies have
emphasized the possible role of sensory feedback in the generation of
dystonic movements. Abnormalities of cortical and basal ganglia function
have been described in functional imaging and neurophysiological studies of
patients with dystonia and in animal models of primary dystonia. Studies of
cortical function have shown reduced preparatory activity in the EEG before
the onset of voluntary movements, whilst magnetic brain stimulation has
revealed changes in motor cortical excitability. Functional imaging of the
brain in primary dystonia has suggested reduced pallidal inhibition of the
thalamus with consequent overactivity of medial and prefrontal cortical
areas and underactivity of the primary motor cortex during movements. These
findings are supported by preliminary neuronal recordings from the globus
pallidus and the thalamus at the time of stereotaxic surgery in patients
with dystonia. All this evidence suggests that primary dystonia results
from a functional disturbance of the basal ganglia, particularly in the
striatal control of the globus pallidus (and substantia nigra pars
reticulata). This causes altered thalamic control of cortical motor
planning and executive areas, and abnormal regulation of brainstem and
spinal cord inhibitory interneuronal mechanisms.
REVIEWS
The pathophysiology of primary dystonia
Department of Neurological Sciences, Universita di Rome La Sapienza and Mediterranean Neurological Institute, Neuromed, Pozzilli (IS), Italy.
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