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These conditions are easily understood if one keeps in mind that, on assuming the erect posture, the pooling of blood in the lower parts of the body is normally prevented by (1) re ex arteriolar and arterial constriction, through alpha- and beta-adrenergic effector mechanisms; (2) re ex acceleration of the heart by means of aortic and carotid re exes, as described above; and (3) muscular activity, which improves venous return Lipsitz has pointed out that aging is associated with a progressive impairment of these compensatory mechanisms, thus rendering the older person especially vulnerable to syncope However, even in some younger persons, after the blood pressure has fallen slightly and stabilized at a lower level, the compensatory re exes may also fail suddenly, with a precipitant drop in blood pressure The diagnosis of orthostatic hypotension from autonomic failure is established by measuring the blood pressure in the supine and then in the standing position and noting a substantial drop accompanied by symptoms of dizziness or syncope It should be emphasized that the bedside testing of orthostatic blood pressure is best performed by having the patient stand quickly and taking readings immediately and again at 1 and at 3 min, rather than using the lying-sitting-standing sequence Postural Orthostatic Tachycardia Syndrome ( POTS ) The authors have limited experience with this syndrome and are uncertain about its validity as an entity apart from an aversion of the patient to assume an upright posture and the tendency to become weak and tremulous As described by Low et al, it consists of intolerance of the standing position accompanied by tachycardia up to 120 beats per minute or more, but without orthostatic hypotension Dyspnea, fatigue, and tremulousness and a complaint of dizziness accompany the assumption of an upright posture, and the same constellation of symptoms may be brought out by upright tilting There is a frequent association with fatigue and with exercise intolerance The situation is comparable to orthostatic intolerance in the chronic fatigue syndrome (page 435), with which POTS shares many features An impairment of cerebral autoregulation has been hypothesized; others consider it to be a limited form of dysautonomia In any case, it seems a valid entity Goldstein and associates recently compared a cohort of POTS patients with a group that experienced recurrent postural near-syncope and found that in the former group there was increased myocardial epinephrine release from intact cardiac sympathetic nerves The basis for this is not known, although the researchers did exclude the possibility of defects in the cardiac norepinephrine transporter membrane and in norepinephrine synthesis Primary Autonomic Insuf ciency (Idiopathic Orthostatic Hypotension) This presents in two forms In one form (Bradbury-Eggleston), there is probably a selective degeneration of neurons in the sympathetic ganglia with denervation of smooth muscle vasculature and adrenal glands The pathology has not been fully delineated, but lesions in other parts of the nervous system are not evident In the second type (Shy-Drager), there is a degeneration of preganglionic neurons in the lateral columns of gray matter in the spinal cord, leaving postganglionic neurons isolated from spinal control The latter lesion is often associated with degeneration of other systems of neurons in the CNS Three such system degenerations have been identi ed, occurring singly or in combination: (1) degeneration of the substantia nigra and locus ceruleus (Parkinson disease), (2) striatonigral degeneration, and (3) olivopontocerebellar degeneration In the rst two syndromes, orthostatic.

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hypotension is combined with a parkinsonian syndrome; in the third, it is associated with cerebellar ataxia Nonetheless, there are cases on record in which neuronal degeneration is limited to the sympathetic neurons of the intermediolateral cell columns These forms of degenerative disease have their onset in adult life, and the associated hypotension and syncope are usually part of a more widespread autonomic dysfunction that includes other features such as a xed cardiac rate, vocal cord paralysis, a loss of sweating in the lower parts of the body, atonicity of the bladder, constipation, and impotence in the male As indicated on page 925, the symptoms of Parkinson disease, autonomic failure, and cerebellar atrophy are often associated in various combinations and degrees a distinctive combination that has been subsumed under the term multiple system atrophy

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