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ralysis of conjugate gaze Usually there is some combination of these abnormalities (see Chap 14) Next to nystagmus, the most frequent ocular abnormality is lateral rectus weakness, which is bilateral but not necessarily symmetrical and is accompanied by diplopia and internal strabismus With complete paralysis of the lateral rectus muscles, nystagmus is initially absent in the abducting eyes, but it becomes evident as the weakness improves The palsy of conjugate gaze varies from merely a nystagmus on extreme gaze to a complete loss of ocular movement in that direction This applies to both horizontal and vertical movements, abnormalities of the former being somewhat more frequent Paralysis of downward gaze is an unusual manifestation, but a pattern that simulates internuclear ophthalmoplegia has been seen In advanced stages of the disease there may be a complete loss of ocular movements, and the pupils, which are otherwise usually spared, may become miotic and nonreacting Ptosis, small retinal hemorrhages, involvement of the near-far focusing mechanism, and evidence of optic neuropathy occur occasionally, but we and our colleagues have never observed papilledema in this disease Although the aforementioned ocular signs are highly characteristic of Wernicke disease, disappearance of nystagmus and an improvement in ophthalmoparesis within hours or a day or two of the administration of thiamine con rms the diagnosis Ataxia Essentially the ataxia is one of stance and gait; in the acute stage of the disease, it may be so severe that the patient cannot stand or walk without support Lesser degrees are characterized by a wide-based stance and a slow, uncertain, short-stepped gait; the mildest degrees are apparent only in tandem walking In contrast to the gross disorder of locomotion is a relative infrequency of limb ataxia and intention tremor; when present, they are more likely to be elicited by heel-to-knee than by nger-to-nose testing Dysarthric, scanning speech is present only rarely Disturbances of Consciousness and Mentation These are present in some form in all but 10 percent of patients Several related types of deranged mentation and consciousness can be recognized By far the most common disturbance is a global confusional state The patient is apathetic, inattentive, and indifferent to his surroundings Spontaneous speech is minimal and many questions are left unanswered, or the patient may suspend conversation and drift off to sleep, although he can be aroused without dif culty Such questions as are answered by the patient betray disorientation in time and place, misidenti cation of those around him, and an inability to grasp the immediate situation Many of the patient s remarks are irrational and lack consistency from one moment to another If the patient s interest and attention can be maintained long enough to ensure adequate testing, one nds that memory and learning ability are also impaired In response to the administration of thiamine or an adequate diet, the patient rapidly becomes more alert and attentive and more capable of taking part in mental testing Then the most prominent abnormality is one of retentive memory (Korsakoff amnesic state) About 15 percent of patients show the signs of alcohol withdrawal, ie, hallucinations and other disorders of perception, confusion, agitation, tremor, and overactivity of autonomic nervous system function These symptoms are evanescent in nature and usually mild in degree Although drowsiness is a common feature of the global confusional state, stupor and coma are rare as initial manifestations of Wernicke disease However, if the early signs of the disease are not recognized and the patient remains untreated, there occurs a progressive depression of the state of consciousness, with stupor,
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coma, and death in a matter of a week or two, just as occurred in Wernicke s original cases Autopsy series of Wernicke disease are heavily weighted with cases of the latter type, often undiagnosed during life (Harper; Torvik et al) Some patients are alert and responsive from the time they are rst seen and already show the characteristic features of the Korsakoff amnesic state In a small number of such patients, the amnesic state is the only manifestation of the syndrome, and no ocular or ataxic signs (other than possibly nystagmus) can be discerned The memory disorder, which constitutes the chronic and truly crippling feature of the Wernicke-Korsakoff syndrome, is described below The Amnesic State As indicated in Chap 21, the core of the amnesic disorder is a defect in learning (anterograde amnesia) and a loss of past memories (retrograde amnesia) The defect in learning (memorization) can be remarkably severe The patient may be incapable, for example, of committing to memory three simple facts (such as the examiner s name, the date, and the time of day) despite countless attempts; the patient can repeat each fact as it is presented, indicating that he understands what is wanted of him and that registration is intact; but by the time the third fact is repeated, the rst may have been forgotten However, certain subtle learning may take place; for example, with repeated trials, the patient may learn complex tasks such as mirror writing or how to negotiate a maze, despite no recollection of ever having been confronted with these tasks Anterograde amnesia is always coupled with a disturbance of past or remote memory (retrograde amnesia) The latter disorder is usually severe in degree, though rarely complete, and covers a period that antedates the onset of the illness by several years Characteristically, a few isolated events and information from the past are retained, but these are related without regard for the intervals that separated them or for their proper temporal sequence Usually the patient telescopes events; sometimes the opposite occurs This aspect of the memory disorder becomes prominent as the initial (global confusional) stage of the illness subsides and some improvement in memory function occurs; this may account for certain instances of confabulation (see below) It is probably true that memories of the recent past are more severely impaired than those of the remote past (Ribot s rule); language, computation, knowledge acquired in school, and all habitual actions are preserved This is not to say that all remote memories are intact These are not as readily tested as more recent memories; the two are therefore dif cult to compare It is our impression that there are gaps and inaccuracies in memories of the distant past in practically all cases of the Korsakoff amnesic state and serious impairments in many It should be emphasized that the cognitive impairment of the Korsakoff patient cannot be explained in terms of memory loss alone, even though this is the most critical functional loss Psychologic testing discloses that certain cognitive and perceptual functions that depend little or not at all on retentive memory are also impaired The most consistent failure is with the digit symbol task and, to a lesser degree, with arithmetic and block design Also as a rule, the Korsakoff patient has no insight into his illness and is characteristically apathetic and inert, lacking in spontaneity and initiative and indifferent to everything and everybody around him However, the patient has a relatively normal capacity to reason with data immediately before him Confabulation has generally been considered to be a speci c feature of Korsakoff psychosis The validity of this view depends
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