Electromyography in CNS Disorders: Central EMG

Massachusetts

PREFACE

Electromyography and electroneurography, in which electrical activity produced by skeletal muscles and peripheral nerves is studied, have proved to be useful in investigation and understanding of a variety of neurological disorders. In most laboratories, however, these electrodiagnostic techniques have been used to help in the diagnosis of diseases that affect the peripheral nerves, neuromuscular junctions, or skeletal muscle fibers. Although major advances in electronic and computer technology have made it possible to study, quantitate, and document reflex activity in intact human subjects, most neurologists still rely on gross clinical observations and most electromyographers continue to use conventional techniques of EMG and nerve conduction studies to differentiate myopathy from neuropathy. In the past three decades, it has been shown that by using electrophysiological techniques one can record most of the reflexes (both proprioceptive and exteroceptive) commonly studied in a clinical setting. These studies, in addition to providing better insight into the underlying physiological mechanisms, provide an objective quantitative measure of function of the central and peripheral nervous systems in man.

The application of clinical neurophysiological studies using classical EMG and nerve conduction techniques to evaluate function of the central nervous system is termed central EMG.

As the president (1981–1982) of the American Association of Electromyography and Electrodiagnosis I organized an international symposium on central EMG in order to introduce practicing electromyographers to new concepts in clinical neurophysiology. Distinguished clinical neurophysiologists from different parts of the world participated in the symposium. Many physicians who attended the symposium were convinced that electromyographic techniques are useful in documenting normal and abnormal functions, not only of the peripheral neuromuscular apparatus, but also of the autonomic and central nervous systems. The purpose of this book is to introduce neurologists, physiatrists, neurosurgeons, orthopedic surgeons, clinical electromyographers, and other interested physicians to the new concept of central EMG. Since many of the techniques described here are used for studies of motor control in man, this volume will also be useful for physical therapists and occupational therapists who are involved in the treatment of patients with disorders of the central nervous system. It is hoped that this book, which has contributions from scientists whose work has been responsible for major advances in clinical neurophysiology, will stimulate interest in wider application of electrophysiological techniques for diagnosis, monitoring, and treatment of patients seen in departments of neurology, neurosurgery, rehabilitation medicine, and orthopedic surgery.

I would like to express my gratitude to my clinical neurophysiologist colleagues who came from all over the world to participate in the symposium and for writing reviews highlighting some of their work related to central EMG. My special thanks also go to my colleague Dr. Robert R. Young for his help and advice, and to Ms. Susan Wyoral for carefully reviewing the chapter manuscripts, illustrations, and references. I also wish to thank members of the American Association of Electromyography and Electrodiagnosis for their support and encouragement, and for helping me to organize the first international symposium for the association. Finally, I wish to thank the publishers for their excellent cooperation during the preparation of this book.

Bhagwan T. Shahani, M.D., D. Phil. (Oxon)

CHAPTER 1

Pyramidal and Extrapyramidal Disorders

James W. Lance

Publisher Summary

This chapter provides an overview of pyramidal and extrapyramidal disorders. Of some 20 million neurons descending from the motor cortex, only one million proceed to the pyramids of the medulla, decussating to form the lateral corticospinal tract. The remaining extrapyramidal or parapyramidal fibers are distributed to the basal ganglia, thalamus, red nucleus, pons, and the medullary reticular formation. The basal ganglia and cerebellum play a part in the planning or programming of movement as well as feeding back information through the thalamus to the motor cortex, correcting movements in progress. The final expression of extrapyramidal activity in the control of muscle tone and of coarse movements involving axial and proximal muscles is mediated by the reticulospinal and vestibulospinal pathways. The chapter also discusses the effect of lesions in the motor system. If a muscle is stretched suddenly and the stretch is sustained, the stretch reflex arc will be activated repetitively at 5–8 Hz. The chapter presents EMG studies of patients with extrapyramidal disorders. The tonic stretch reflex in Parkinson’s disease is interrupted by tremor mechanisms so that the examiner elicits cogwheel rigidity on manipulating a limb through a range of movement at a joint. The chapter further presents the distinguishing features of the upper motor neuron syndrome.

PRINCIPLES OF MOTOR CONTROL

Pyramidal and Extrapyramidal Pathways

The motor cortex used to be considered the cerebral center for the control of movement in which individual muscles or whole patterns of movement were represented. More recently, the motor cortex appears to have been relegated to a subordinate status and its direct projection to spinal motor neurons, the pyramidal tract, regarded as little more than an interneuron linking the brain with the spinal cord. The truth probably lies somewhere in between. Of some 20 million neurons descending from the motor cortex, only one million proceed to the pyramids of the medulla, decussating to form the lateral corticospinal tract. The remaining extrapyramidal, or parapyramidal, fibers are distributed to the basal ganglia, thalamus, red nucleus, pons, and the medullary reticular formation (Phillips and Porter 1977).

The basal ganglia and cerebellum play a part in the planning or programming of movement as well as feeding back information through the thalamus to the motor cortex, correcting movements in progress. The final expression of extrapyramidal activity in the control of muscle tone and of coarse movements involving axial and proximal muscles (and influencing flexor and extensor synergies of the limbs) is mediated by the reticulospinal and vestibulospinal pathways. With this background of extrapyramidal activity, or complementary to it, is the ability, conferred by the monosynaptic projection from cortex to spinal motor neuron of pyramidal tract fibers, to use distal muscles discretely for precise movements of the hands and feet.

The Basal Ganglia

The caudate nucleus and putamen (neostriatum) receive afferent fibers from almost all parts of the neocortex, particularly the sensorimotor area (Denny-Brown 1962). Fibers from the neostriatum project through the globus pallidus and substantia nigra to the thalamus, where they make two important connections. The first of these is in the ventrolateral thalamic nucleus, from which efferents pass to the motor cortex, thus completing a loop between cortex and basal ganglia (Fig. 1.1). Although the ventrolateral thalamic nucleus is also a relay station between the cerebellum and motor cortex, there is little integration at this site of the output from basal ganglia and cerebellum in the modulation of cortical activity.

Figure 1.1 Cortico-strio-thalamo-cortical loop. Corticofugal fibers concerned with the programming and feedback of movement pass to the caudate nucleus (CN) and putamen (P). The caudate nucleus and putamen project to the ventrolateral nucleus of the thalamus (Th) via the globus pallidus (GP) and substantia nigra (SN) and thence to the motor cortex (MC). (ST = subthalamic nucleus; RF = reticular formation.)

The second connection made by basal ganglia fibers in the thalamus takes place in the medial nuclei, which have reciprocal connections with the midbrain reticular formation (Fig. 1.2), thought to be responsible for the control of muscle tone by the basal ganglia.

Figure 1.2 Reciprocal connections of the basal ganglia with the reticular formation. Fibers from the caudate nucleus (CN) and putamen (P) project through the globus pallidus (GP) to medial thalamic nuclei and thence to the reticular formation of the midbrain. Fibers from the substantia nigra (SN) feed into the same pathway. The reticular formation projects back through the medial thalamic nuclei to the caudate nucleus.

The discharge of cells in the caudate nucleus and putamen is regulated by a nigrostriatal pathway using dopamine as a transmitter (Fig. 1.3), while a pathway in the reverse direction from neostriatum to substantia nigra releases gamma-aminobutyric acid. The degeneration of the substantia nigra in Parkinson’s disease releases the basal ganglia from a restraining influence so that its links with the motor cortex and the reticular formation become hyperactive, causing tremor and increased muscle tone.

Figure 1.3 Reciprocal connections modulating basal ganglia function, with the subthalamic nucleus (ST) (interruption of this pathway causes hemiballismus), and with the substantia nigra (SN). Striatonigral neurons inhibit SN cells by the release of gamma-aminobutyric acid. The nigrostriatal pathway, which uses dopamine as a transmitter and degenerates in Parkinson’s disease, inhibits cells in the putamen and caudate nucleus.

Another circuit with a predominantly inhibitory influence joins the globus pallidus with the subthalamic nucleus (see Fig. 1.3). Damage to the subthalamic nucleus or its projections causes wild rotary and flinging movements of the contralateral limbs, known as hemiballismus.

The Cerebellum

The cerebellar hemispheres develop in complexity with the cerebral cortex as the phylogenetic scale is ascended, comprising 88% of the human cerebellum (Eccles 1977). The intermediate zone, which runs parallel to the vermis and lateral to it, forms a closed loop with the motor cortex (Fig. 1.4). It receives proprioceptive information from the spinal cord, as well as afferents from the cortex (by a crossed pathway synapsing in the pons), and projects back to the motor cortex through the globose and emboliform nuclei. This cortico-ponto-cerebello-cortical pathway monitors pyramidal tract activity in relation to limb position, providing online correction of movement. The circuit takes about 20 msec to complete in the human brain (Eccles 1977). In addition to this feedback function, the intermediate zone may initiate movements in response to a proprioceptive stimulus.

Figure 1.4 Neocerebellar connections, intermediate zone (NI), forming a closed loop with the motor cortex (area 4). Information is transmitted via the corticopontocerebellar pathway to the intermediate zone and returns via the nucleus interpositus (IP), which is separated into globose and emboliform nuclei in humans, and the ventrolateral nucleus of thalamus (VL). This system can be influenced by proprioceptive stimuli from the limbs as indicated by the spinocerebellar tract (SCT) connections with the paleocerebellum (P). Climbing fibers omitted for simplicity; CS, central sulcus. Reproduced from Lance and McLeod (1981) by permission of Butterworths, London.

The lateral zone of the cerebellar hemispheres receives afferents from area 6 and from sensorimotor association areas, such as 5 and 7 (Fig. 1.5), that play a part in the planning of movement. Some follow the cortico-ponto-cerebellar route and end on Purkinje cells as mossy fibers, while others synapse in the inferior olive (which introduces a delay of about 10 msec into the system) before ending as climbing fibers. The mode of interaction between mossy and climbing fibers is not fully understood. The lateral zones of the cerebellar hemispheres project back to the sensorimotor cortex through the ventrolateral thalamus, and are thought to determine the sequence and timing of muscular contraction required to execute a planned movement in response to an afferent inflow from association areas.

Figure 1.5 Neocerebellar connections, lateral zone (NL), forming an open loop with the sensorimotor cortex. Information is transmitted from the premotor region (area 6) and sensory association areas (5, 7) via the corticopontocerebellar pathway and returns to the motor cortex (area 4) via the dentate nucleus (DN) and ventrolateral nucleus of thalamus (VL). Climbing fibers (interrupted lines) arise from the inferior olive (IO). Some fibers from the dentate nucleus end in the reticular formation around the red nucleus (R); CS, central sulcus. Reproduced from Lance and McLeod (1981) by permission of Butterworths, London.

Intracerebellar recordings in conscious monkeys have shown that a visual signal causes activity in cells of the lateral zone and dentate nucleus before there is activity in cells of the motor cortex (Thach 1978). In contrast, a proprioceptive stimulus to one limb causes activity in cells of the intermediate zone and nucleus interpositus before activity occurs in cells of the motor cortex. In both cases, the ascending impulses from the cerebellum project (with those from the basal ganglia) to the ventrolateral nucleus of the thalamus before transmission to the motor cortex.

The Spinal Cord

Lateral corticospinal (pyramidal) tract fibers make mainly monosynaptic contacts with motor neurons in the human spinal cord and facilitate those neurons innervating abductor and extensor muscles in the upper limbs and flexor muscles of the lower limbs, as well as activating those neurons responsible for discrete movements of the digits. The pyramidal tract itself has little effect on human muscle tone, but the cortico-reticulospinal fibers that shadow it throughout its course exert a tonic inhibitory influence on motor neurons (Ashby et al. 1972). For this reason, upper motor neuron lesions in man (which almost invariably involve the cortico-reticulospinal pathway as well as the pyramidal tract) are characterized by hyperactive motor neurons, with resulting increase in muscle tone and tendon jerks.

The facilitory reticulospinal and vestibulospinal tracts appear to be independent of cortical control (Andrews, Knowles, and Lance 1973) and to have a tonic excitatory effect on motor neurons (Gillies, Burke, and Lance 1971), which becomes obvious when the balancing action of the cortico-reticulospinal pathway is removed (Magoun and Rhines 1948; Lance and McLeod 1981).

The action of the pyramidal and extrapyramidal pathways is expressed partly through reflex pathways in the spinal cord.

The Stretch Reflex.

Group Ia afferents from primary muscle spindle endings synapse on motor neurons in the anterior horn to cause contraction of the surrounding muscle fibers in response to a dynamic stretch stimulus. The sensitivity of the stretch reflex is regulated from cortex and brain stem by the mechanism just described. The Ia afferents inhibit the motor neurons of antagonistic muscles (reciprocal inhibition).

The Disynaptic Inhibitory Pathway.

Group Ib afferents from Golgi tendon organs respond to muscle tension and inhibit motor neurons. This system is active only during contraction of the muscle fibers with which the tendon organs are in series, and thus ceases to sustain inhibition once the muscle relaxes (Houk and Henneman 1967).

The Flexor Reflex.

Flexor reflex afferents (FRA) include group II fibers from secondary endings on muscle spindles as well as smaller muscle and cutaneous afferent fibers conducting pain impulses. Group II fibers respond to static stretch; that is, they signal increasing muscle length. Their central effect is to facilitate flexor responses and inhibit extensor responses in the lower limbs of human subjects as well as in spinal animals. Their action can be altered by bulbospinal pathways (Lundberg 1975) and, in the decerebrate state, may even be reversed, so that group II afferent input reinforces the extensor contraction that characterizes the decerebrate state (McGrath and Matthews 1973). The dorsal reticulospinal tract, which regulates flexor reflexes (Fig. 1.6), runs in the dorsolateral quadrant of the spinal cord. Lesions in this region can convert the extensor rigidity of the decerebrate state to the clasp-knife phenomenon of the spastic state by releasing the extensor-inhibitory action of group II afferents (Burke et al. 1972), and possibly smaller afferent fibers as well (Rymer, Houk, and Crago 1979). Release of flexor reflex afferents can also cause flexor spasms in paraplegic