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In this chapter, the disorders of the automatic, static, postural, and other less modi able motor activities of the nervous system are discussed They are believed, on good evidence, to be an expression of the extrapyramidal motor system, meaning according to S A K Wilson, who introduced this term the motor structures of the basal ganglia and certain related thalamic and brainstem nuclei In health, the activities of the basal ganglia and the cerebellum are blended with and modulate the corticospinal and corticalbrainstem-spinal systems The static postural activities of the former are indispensable to the voluntary movements of the latter The close association of the basal ganglia and corticospinal systems becomes evident in the course of many forms of neurologic disease Cerebral lesions that involve the corticospinal tracts result not only in a contralateral paralysis of volitional movements but also in a xed posture or attitude in which the arm is exed and the leg extended (predilection of Wernicke-Mann or hemiplegic dystonia of Denny-Brown) Also, as noted in Chap 3, interruption of the motor projections by a lesion in the upper pons or midbrain may release a posture in which all four limbs or the arm and leg on one side are extended and the cervical and thoracolumbar portions of the spine are hyperextended (decerebrate rigidity) In these released motor patterns, one has evidence of labyrinthine, tonic neck, and other postural re exes that are mediated through nonpyramidal bulbospinal and other brainstem motor systems Observations such as these and the anatomic data presented in Chap 3 have blurred the classic distinctions between pyramidal and extrapyramidal motor systems Nevertheless, this division remains a useful if not an essential concept in clinical work, since it compels a distinction between several motor syndromes one that is characterized by a loss of volitional movement accompanied by spasticity; a second by akinesia, rigidity, and tremor without loss of voluntary movement; a third by involuntary movements (choreoathetosis and dystonia); and yet another by incoordination (ataxia) The clinical differences between corticospinal and extrapyramidal syndromes are summarized in Table 4-1 Much of the criticism of the pyramidal-extrapyramidal concept derives from the terms themselves The ambiguity related to the term pyramidal was discussed in Chap 3, in which it was pointed out that pure pyramidal lesions do not cause total paralysis; when the latter exists, there is always some degree of involvement of other descending motor pathways as well The term extrapyramidal is equally imprecise Strictly interpreted, it refers to all the motor pathways except the pyramidal one, making this term so allembracing as to be practically meaningless The concept of an extrapyramidal motor system becomes more meaningful if it is subdivided into two parts: (1) the striatopallidonigral system (basal ganglia) and (2) the cerebellum These parts function very differently in the control of movement and posture, and disease in each 55.

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of them results in particular disturbances of movement and posture without paralysis The basal ganglia and the symptoms that result when they are diseased are described in the pages that follow The disorders of movement and posture due strictly to cerebellar disease are described in Chap 5

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ways in which the motor system can deteriorate Compulsive, repetitive movements are the most frequent: mouthing movements, stereotyped grimacing, protrusion of the tongue, side-to-side or toand-fro tremor of the head, odd vocalizations such as snif ng, snorting, and bleating In some respects these disorders resemble tics (quasivoluntary movements to relieve tension), but careful observation shows that they are not really voluntary Haloperidol and other drugs of this class have an unpredictable therapeutic effect, seeming at times to bene t the patient only by the superimposition of a drug-induced rigidity Old age is thought always to carry a liability to tremulousness, and indeed, one sees this association with some frequency The head, chin, or hands tremble and the voice quavers, yet there is not the usual slowness and poverty of movement, facial impassivity, or exed posture that would stamp the condition as parkinsonian Some instances of tremor are clearly familial, having appeared or worsened only late in life However, the relation of tremulousness to senility is sometimes open to doubt Charcot, in a review of over 2000 elderly inhabitants of the Salpetriere, could nd only about ` 30 with tremor Some cases probably represent the exaggeration or emergence of essential tremor, but many cases cannot be explained on this basis Spastic or spasmodic dysphonia, a disorder of middle and late life characterized by spasm of all the throat muscles on attempted speech, is discussed on page 428 Blepharoclonus or blepharospasm, an involuntary movement of the eyelids, is also described on page 93

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