Movement disorders (sometimes called extrapyramidal disorders) impair the regulation of voluntary motor activity without directly affecting strength, sensation, or cerebellar function. They include hyperkinetic disorders associated with abnormal, involuntary movements and hypokinetic disorders characterized by poverty of movement. Movement disorders result from dysfunction of deep subcortical gray matter structures termed the basal ganglia. Although there is no universally accepted anatomic definition of the basal ganglia, for clinical purposes they may be considered to comprise the caudate nucleus, putamen, globus pallidus, subthalamic nucleus, and substantia nigra. The putamen and the globus pallidus are collectively termed the lentiform nucleus; the combination of lentiform nucleus and caudate nucleus is designated the corpus striatum.
The basic circuitry of the basal ganglia consists of three interacting neuronal loops (Figure 11-1). The first is a corticocortical loop that passes from the cerebral cortex; through the caudate and putamen, the internal segment of the globus pallidus, and the thalamus; and then back to the cerebral cortex. The second is a nigrostriatal loop connecting the substantia nigra with the caudate and putamen. The third, a striatopallidal loop, projects from the caudate and putamen to the external segment of the globus pallidus, then to the subthalamic nucleus, and finally to the internal segment of the globus pallidus. In some movement disorders (eg, Parkinson disease), a discrete site of pathology within these pathways can be identified; in other cases (eg, essential tremor), the precise anatomic abnormality is unknown.
Basic neuronal circuitry of the basal ganglia.
TYPES OF ABNORMAL MOVEMENTS
Categorizing an abnormal movement is generally the first step toward arriving at the neurologic diagnosis. Abnormal movements can be classified as tremor, chorea, athetosis or dystonia, ballismus, myoclonus, or tics. They can arise in a variety of contexts, such as in degenerative disorders or with structural lesions. In many disorders, abnormal movements are the sole clinical features.
A tremor is a rhythmic oscillatory movement best characterized by its relationship to voluntary motor activity, that is, according to whether it occurs at rest, during maintenance of a particular posture, or during movement. The major causes of tremor are listed in Table 11-1. Tremor is enhanced by emotional stress and disappears during sleep. Tremor that occurs when the limb is at rest is generally referred to as static tremor or rest tremor. If present during sustained posture, it is called a postural tremor; although this tremor may continue during movement, movement does not increase its severity. When present during movement but not at rest, it is generally called an intention or kinetic tremor. Both postural and intention tremors are also called action tremors.
Table 11-1.Causes of Tremor.