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tral white matter are destroyed, while most of the axis cylinders are spared A relatively selective degeneration of myelin may also occur in some small ischemic foci due to vascular occlusion or in larger con uent areas, as is the case in Binswanger disease (see Chap 34) In subacute combined degeneration (SCD) of the spinal cord associated with pernicious anemia and in tropical spastic paraparesis (TSP), a demyelinating spinal cord disease, myelin may be affected earlier and to a greater extent than axis cylinder The same is true of progressive multifocal leukoencephalopathy (PML), central pontine myelinolysis, and Marchiafava-Bignami disease Some of these disorders and several others are no longer classi ed as demyelinative because their etiology has been established Also, for reasons that will become clear in subsequent discussion, the chronic progressive leukodystrophies of childhood and adolescence (eg, globoid body, metachromatic, and adrenal leukodystrophies), while clearly diseases of myelin, are set apart and called dysmyelinative because of their unique genetic and morphologic features and are discussed in Chap 37 Occupying an uncertain place in this nosology are demyelinative lesions associated with connective tissue diseases or merely with autoantibodies directed against DNA or phospholipids Again, the central nervous system (CNS) lesions may be multiple and cannot easily be distinguished radiologically from multiple sclerosis (MS) But, as noted further on, their nature is uncertain, and some are clearly due to a vasculopathy Excluded from discussion in this chapter are demyelinative diseases of the peripheral nerves Although they share a number of pathologic features with demyelinating diseases of the CNS, they are described with diseases of the peripheral nervous system in Chap 46 In the language of neurology, therefore, the term demyelination has acquired a special meaning; if it is to retain its value, it should be used in the restricted sense indicated above and not as a synonym for any destructive process of nerve bers or cerebral white matter or for inherent and inherited defects in myelin, even though the lesions may look alike in a section stained for myelin.

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Tremor may be de ned as a more or less involuntary and rhythmic oscillatory movement produced by alternating or irregularly synchronous contractions of reciprocally innervated muscles Its rhythmic quality distinguishes tremor from other involuntary movements, and the involvement of agonist and antagonistic muscles distinguishes it from clonus Two general categories are recognized: normal (or physiologic) and abnormal (or pathologic) The former, as the name indicates, is a normal phenomenon; it is present in all contracting muscle groups and persists throughout the waking state and even in certain phases of sleep The movement is so ne that it can barely be seen by the naked eye, and then only if the ngers are rmly outstretched; in most instances special instruments are required for its detection It ranges in frequency between 8 and 13 Hz, the dominant rate being 10 Hz in adulthood and somewhat less in childhood and old age Several hypotheses have been proposed to explain physiologic tremor, a traditional one being that it re ects the passive vibration of body tissues produced by mechanical activity of cardiac origin (ballistocardiogram) Assuredly this is not the whole explanation of physiologic tremor As Marsden has pointed out, several additional factors such as spindle input, the unfused grouped ring rates of motor neurons, and the natural resonating frequencies and inertia of the muscles and other structures are probably of greater signi cance Certain abnormal tremors, namely, the metabolic varieties of postural or action tremor and at least one type of familial tremor, are considered by some workers to be variants or exaggerations of physiologic tremor ie, enhanced physiologic tremor (as discussed further on) Abnormal or pathologic tremor, which is what one means when the term tremor is used clinically, preferentially affects certain muscle groups the distal parts of the limbs (especially the ngers and hands); less often, the proximal parts; the head, tongue, jaw, or vocal cords; and rarely the trunk and is present only in the waking state The rate in most forms is from 4 to 7 Hz, or about half that of physiologic tremor In any one individual, the rate is fairly constant in all affected parts With the advent of electromyography (EMG) and mechanical recording devices, abnormal tremors were subdivided according to their rate, their relationship to posture of the limbs and volitional movement, their pattern of EMG activity (synchronous or alternating) in opposing muscle groups, and their response to certain drugs.