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I. Giacomo, M.B. B.CH. B.A.O., Ph.D.

Assistant Professor, Tufts University School of Medicine

When present in the daytime, it may also represent a fragment of segmental dystonia or tardive dyskinesia. Nocturnal Enuresis Nocturnal bedwetting with daytime continence is a frequent disorder during childhood, which may persist into adult life. Approximately 1 of 10 children 4 to 14 years of age is affected, boys more frequently than girls (in a ratio of 4:3); even among adults (military recruits), the incidence is 1 to 3 percent. The intravesicular pressure periodically rises to much higher levels in the enuretic than in normal persons, and the functional bladder capacity of the enuretic is smaller than normal. Perhaps the content of this section will be more clearly understood if a few of the introductory remarks to the later section on psychiatric diseases are anticipated here. The main thesis of the neurologist is that mental and physical functions of the nervous system are simply two aspects of the same neural process. Mind and behavior both have their roots in the self-regulating, goal-seeking activities of the organism, the same ones that provide impulse to all forms of mammalian life. But in addition, the prodigious complexity of the human brain permits, to an extraordinary degree, the solving of difficult problems, the capacity for remembering past experiences and casting them in a symbolic language that can be written and read, and the planning for events that have yet to take place. The constant but sometimes meandering internal verbal experience of this ideation during waking was aptly named "stream of thought" by William James. Whether this is an emergent property of various mental functions or simply their representation cannot be answered, but any separation of the mental from the observable behavioral aspects of cerebral function is probably illusory. Biologists and psychologists have reached this view by placing all known activities of the nervous system (growth, development, behavior, and mental function) on a continuum and noting the inherent purposiveness and creativity common to all of them. The physician is persuaded of the truth of this view through daily clinical experience, in which every possible aberration of behavior and intellect appears at some time or other as an expression of cerebral disease. The reader will find that in this section of the book, Chapters 20 and 21 are concerned with common disturbances of the sensorium and of cognition, which stand as cardinal manifestations of certain cerebral diseases. The most frequent of these are delirium and other acute confusional states as well as disorders of learning, memory, and other intellectual functions. A consideration of these abnormalities - as a rule indicative of a general disturbance of cerebral function - leads naturally to an examination of the symptoms that result from focal cerebral lesions (Chap. As emphasized in the following chapters, even these disturbances often fall between the 353 Copyright © 2005, 2001, 1997, 1993, 1989, 1985, 1981, 1977, by the McGraw-Hill Companies, Inc. The psychiatric causes of disordered thinking and behavior have special qualities that make them separable from most of the conditions considered in the next several chapters. Occurring, as it often does, during an infection with fever or in the course of a toxic or metabolic disorder (such as renal or hepatic failure) or as an effect of medication, abused drugs, or alcohol, it never fails to create grave problems for the physician, nursing personnel, and family. Nurses are burdened with the need to provide satisfactory care and a safe immediate environment for the patient and, at the same time, maintain a tranquil atmosphere for other patients. The family must be supported as it faces the frightening specter of a deranged mind and all it signifies. It is our view that such patients should be admitted to a general medical or neurologic ward and not to a psychiatric unit, which often lacks the wherewithal to properly investigate and manage the great variety of medical diseases that cause the acute confusional state. Transfer of the patient to a psychiatric service is undertaken only if the behavioral disorder proves impossible to manage in a general hospital service, or, if warranted, when the underlying medical problems have been identified and a program of treatment has been started. Compounding the difficulty is the fact that the pathophysiology of the confusional states and delirium is not fully understood, and the definitions depend to some extent on their clinical causes and relationships, with all the imprecision that this entails. The following nomenclature, though tentative, has proved useful to us and is employed in this and subsequent chapters throughout this book. Its most conspicuous attributes are impaired attention and power of concentration, disorientation- which may be manifest or is demonstrated only by direct questioning, an inability to properly register immediate events and to recall them later, a diminution of all mental activity, including the normally constant inner ideation and sometimes by the appearance of bewilderment. Thinking, speech, and the performance of goal-directed actions are impersistent or abruptly arrested by the intrusion of irrelevant thoughts or by the slightest external stimulus. Reduced perceptiveness accompanied 355 by visual and auditory illusions or hallucinations and paranoid delusions (a veritable psychosis) are variable features. Confusion, as defined in this way, is an essential ingredient of the singular state called delirium (discussed further on), in which a agitation, hallucinations, and sometimes convulsions and tremor accompany the core confusional state. It should be pointed out that confusion is also a characteristic feature of the chronic syndrome of dementia, where it is the product of a progressive failure of language, memory, and other intellectual functions; here it is mainly the long-standing and progressive nature of the mental confusion that differentiates the process from the acute confusional and delirious states that carry quite different implications. Intense emotional disturbances, of either manic or depressive type, may interfere with attentiveness and coherence of thinking and thereby produce an apparent confusional state.

Similar errors appear even more frequently when the patient is asked to explain the text, read aloud, or give an explanation in writing. As with other tests of aphasia, it may be necessary to increase the complexity of the test- from digits and simple words to complex words, phrases, and sentences- in order to disclose the full disability. The patient may be unable to repeat what is said to him, despite relatively adequate comprehension- the hallmark of conduction aphasia. Contrariwise, normal repetition in an aphasic patient (transcortical aphasia) indicates that the perisylvian area is largely intact. Preserved repetition is also characteristic of anomic aphasia and occurs occasionally with subcortical lesions. Disorders confined to naming, other language functions (reading, writing, spelling, etc. These deficits can be quantified by the use of any one of several examination procedures. The use of these procedures will enable one to predict the type and localization of the lesion in approximately twothirds of the patients, which is not much better than detailed bedside examination. Using these tests, aphasia of the Broca, Wernicke, conduction, global, and anomic types accounted for 392 of 444 unselected cases studied by Benson. Treatment the sudden onset of aphasia would be expected to cause great apprehension, but except for cases of pure or almost pure motor disorders of speech, most patients show remarkably little concern. It appears that the very lesion that deprives them of speech also causes at least a partial loss of insight into their own disability. Reassurance and a program of speech rehabilitation are the best ways of helping the patient at this stage. Whether contemporary methods of speech therapy accomplish more than can be accounted for by spontaneous recovery is still uncertain. Most aphasic disorders are due to vascular disease and trauma, and they are nearly always accompanied by some degree of spontaneous improvement in the days, weeks, and months that follow the stroke or accident. A Veterans Administration Cooperative Study (Wertz et al) has suggested that intensive therapy by a speech pathologist does hasten improvement. Also, Howard and colleagues have shown increased efficacy of word retrieval in a group of chronic stable aphasics treated by two different techniques. In an interesting personal experiment by Wender, a classicist who had become aphasic, practice of Greek vocabulary and grammar led to recovery in that language, but there was little recovery of her facility with Latin, which was not similarly exercised. As a rule, therapy is not advisable in the first few days of an aphasic illness, because one does not know how severe or lasting it will be. Also, if the patient suffers a severe global aphasia and can neither speak nor understand spoken and written words, the speech therapist is virtually helpless. Under such circumstances, one does well to wait a few weeks until one of the language functions has begun to return. Then the physician and therapist can begin to help the patient to use that function to a maximum degree. In milder aphasic disorders, the patient may be sent to the speech therapist as soon as the illness has stabilized. The methods of language rehabilitation are specialized, and it is advisable to call in a person who has been trained in this field. Frustration, depression, and paranoia, which complicate some aphasias, may require psychiatric evaluation and treatment. The developmental language disorders of children pose special problems and are considered in Chap. Prognosis and Patterns of Recovery Some aspects of prognosis have been discussed above. In general, recovery from aphasia due to cerebral trauma is usually faster and more complete than that from aphasia due to stroke. The various dissociative speech syndromes and pure word mutism tend to improve rapidly and often completely. Also, in general, the outlook for recovery from any particular aphasia is more favorable in a left-handed person than in a right-handed one.

This type of spinal cord damage, without radiologic evidence of fracture or dislocation, is particularly common in children. That rupture of the supporting ligamentous elements has nonetheless occurred can be revealed by gentle flexion and extension of the neck under radiologic observation which demonstrates slight dislocation of the vertebra (spinal instability). Another mechanism of cord and spinal root injury, involving extremes of extension and flexion of the neck, is so-called whiplash or recoil injury, most often the result of an automobile accident. When a vehicle is struck sharply from behind, the head of the occupant is flung back uncontrollably; or, if a fast-moving vehicle stops abruptly, there is sudden forward flexion of the neck, followed by retroflexion. Under these conditions the occipitonuchal and sternocleidomastoid muscles and other supporting structures of the neck and head are affected much more often than the spinal cord or roots. Nevertheless, in rare instances, quadriparesis, temporary or permanent, results from a violent whiplash injury. The exact mechanism of neural injury in these circum- stances is not clear; perhaps there is a transient posterior dislocation of a vertebral body, a momentary buckling of the ligmentum flavum, or retropulsion of the intervertebral disc into the spinal canal. The presence of a congenitally narrow cervical spinal canal or of spinal diseases such as cervical spondylosis, rheumatoid arthritis, or ankylosing spondylitis adds greatly to the hazard of damage to the cord or roots. Also, preexisting spondylotic symptoms may be aggravated and become the source of chronic pain. There are in addition examples of spinal cord compression that result from the prolonged hyperextension of the cervical spine during a protracted period of stupor. This accounts for some cases of quadriplegia in opiate or other drug addicts following a period of sustained unresponsiveness (Ell et al). A special type of spinal cord injury, occurring most often in wartime, is one in which a high-velocity missile penetrates the vertebral canal and damages the spinal cord directly. In some cases the missile strikes the vertebral column without entering the spinal canal but virtually shatters the contents of the dural tube or produces lesser degrees of impairment of spinal cord function. Rarely, the transmitted shock wave will cause a paralysis of spinal cord function that is completely reversible in a day or two (spinal cord concussion, described further on). Acute traumatic paralysis may also be the indirect consequence of a vascular mechanism. Fibrocartilaginous emboli from an intervertebral disc that has ruptured into radicular arteries or veins of the spinal cord may cause infarction. Or, a traumatic dissecting aneurysm of the aorta may occlude the segmental arteries of the spinal cord, as in the cases reported by Weisman and Adams and by Kneisley. An analysis of 2000 cases of spinal injury collected from the medical literature by Jefferson up to 1927 showed that most vertebral injuries occurred at the levels of the first and second cervical, fourth to sixth cervical, and eleventh thoracic to second lumbar vertebrae. Impact to the head with the neck flexed or sharply retroflexed, as mentioned earlier, was the main cause of injuries to the cervical region. These are not only the most mobile portions of the vertebral column but also the regions in which the cervical and lumbar enlargements of the cord greatly reduce the space between neural and bony structures. The thoracic cord is relatively small and its spinal canal is capacious; additional protection is provided by the high articular facets (making dislocation difficult) and limitations in anterior movement imposed by the thoracic cage. The majority of the fatal cases have been associated with fracturedislocations or dislocations of the upper cervical spine (C1 to C4 vertebrae) with resultant sudden respiratory paralysis. Among nonfatal cases, fracture-dislocation of the mid and lower cervical spine is the most frequent established mechanism of spinal cord injury in civilian life. In the United States, the annual incidence of spinal cord injury is 5 cases per 100,000 population; males predominate (4:1). Each year about 3500 persons die in close relation to their injury, and another 5000 are left with complete or nearly complete loss of spinal cord function. These changes, designated as traumatic necrosis of the spinal cord, are maximal at the level of injury and one or two segments above and below it. Separation of the constituent pathologic entities- such as hematomyelia, concussion, contusion, and hematorrhachis (bleeding into the spinal canal)- is of little value either clinically or pathologically. As a lesion heals, it leaves a gliotic focus or cavitation with variable amounts of hemosiderin and iron pigment. Progressive cavitation (traumatic syringomyelia) may develop after an interval of months or years and as it extends above the main lesion, lead to a delayed central or incomplete transverse cord syndrome.

The survival of patients with Alzheimer disease is reduced to half the expected rate, mainly because of respiratory and cardiovascular causes and inanition but also for other reasons that are not entirely clear. Whether low educational attainment is a risk factor for the development of Alzheimer disease or, conversely, whether cognitively demanding occupations protect against dementia has not been settled, but some provocative data indicating that this may be so have been presented in Chap. In fewer than 1 percent of such cases there is a dominant inheritance pattern (Nee et al, Goudsmit et al; see further on, in the section on pathogenesis). Reports of substantial familial aggregations of dementia without a specific pattern of inheritance also suggest the operation of more than one genetic factor. Several studies have documented a significant increase in the risk of ostensibly sporadic Alzheimer disease among first-degree relatives of patients with this disorder. Again, this risk is disproportionately greater in females, adding to the evidence that women in general are at slightly higher risk for Alzheimer disease (Silverman et al). Li and coworkers have provided evidence that patients with an earlier age of onset of Alzheimer disease (before age 70) are more likely to have relatives with the disease than are patients with later onset. Genetic studies are difficult because the disease does not appear at the same age in a given proband. Even in identical twins, it may develop at the age of 60 years in one of the pair and at 80 years in the other. Occasionally, however, it is brought to attention by an unusual degree of confusion in relation to a febrile illness, an operation, mild head injury, or the taking of medication. Other patients may present with complaints of dizziness, mental fogginess, nondescript headaches, or other vaguely expressed and changeable somatic symptoms. Questions are repeated again and again, the patient having forgotten what was just discussed. Once the memory disorder has become pronounced, other failures in cerebral function become increasingly apparent. Comprehension of spoken words seems at first to be preserved, until it is observed that the patient does not carry out a complicated request; even then it is uncertain whether the request was not understood because of inattention or was forgotten. Almost imperceptible at first, these disturbances of language become more apparent as the disease progresses. Finally, after many years of illness, there is a failure to speak in full sentences; the finding of words requires a continuous search; and little that is said or written is fully comprehended. There is a tendency to repeat a question before answering it, and later there may be a rather dramatic repetition of every spoken phrase (echolalia). The deterioration of verbal skills has by then progressed beyond a groping for names and common nouns to an obvious anomic aphasia. Other elements of receptive and executive aphasia are later added, but discrete aphasias of the Broca or Wernicke type are characteristically lacking. In general there is a paucity of speech and a quantitative reduction in mentation. Faults in balancing the checkbook, mistakes in figuring the price of items and in making the correct change- all these and others progress to a point where the patient can no longer carry out the simplest calculations (acalculia or dyscalculia). The car cannot be parked; the arms do not find the correct sleeves of the jacket or shirt; the corners of the tablecloth cannot be oriented with the corners of the table; the patient turns in the wrong direction on the way home or becomes lost. The route from one place to another cannot be described, nor can given directions be understood. As this state worsens, the simplest of geometric forms and patterns cannot be copied. Late in the course of the illness, the patient forgets how to use common objects and tools while retaining the necessary motor power and coordination for these activities. The razor is no longer correctly applied to the face; the latch of the door cannot be unfastened; and eating utensils are used awkwardly. Ideational and ideomotor apraxia are the terms applied to the advanced forms of this motor incapacity (pages 48 and 402). As these many amnesic, aphasic, agnosic, and apraxic deficits declare themselves, the patient at first seems unchanged in overall motility, behavior, temperament, and conduct. Social graces, whatever they were, are retained in the initial phase of the illness, but troublesome alterations gradually appear in this sphere as well. Restlessness and agitation or their opposites- inertia and placidity- become evident.

These are summarized in Table 16-3 and their number will almost certainly expand in the next few years. As with numerous other genetic neurologic disorders, a single mutation may produce different seizure types, and a single type may be the result of one of several different mutations. This is particularly true in a group that has been termed generalized epilepsy with febrile seizures plus. This denotes various combinations of uncomplicated febrile seizures, febrile seizures persisting beyond childhood, generalized, absence, myoclonic, atonic, and temporal lobe seizures. Also notable is the low penetrance of some monogenic epileptic disorders, particularly the autosomal dominant one associated with nocturnal frontal seizures. Another group of epilepsies with mendelian inheritance has been ascribed to genetic defects that do not implicate ion channels. Most of these are primarily myoclonic disorders in which the epilepsy is symptomatic. Thus, two forms of progressive myoclonic epilepsy, Unverricht-Lundborg disease and Lafora body disease, are the result respectively of mutations in genes encoding cystatin B and a protein, tyrosine phosphatase. Other forms of myoclonic epilepsy are presumably related to primary defects that cause different forms of ceroid lipofuscinosis (see Chap. To these inherited forms of epilepsy may be added diseases such as tuberous sclerosis, which have a strong proclivity to cause seizures. A more complex genetic element is also identified in several other classic childhood seizure disorders- absence epilepsy with three-per-second spike-and-wave discharges and benign epilepsy of childhood with centrotemporal spikes- both of which are transmitted as autosomal dominant traits with incomplete penetrance or perhaps in a more complicated manner. In the partial, or focal, epilepsies (which is the form that seizures take in two-thirds of adults and almost half of the children with epilepsy), the role of heredity is not nearly so clear. Among the familial cortical epilepsies, both a temporal and frontal lobe type are inherited in a polygenic fashion or in an autosomal dominant pattern. Undoubtedly also inherited is the tendency to develop simple febrile convulsions, though the mode of inheritance is uncertain. The genetics of the epileptic disorders has been reviewed in detail by Steinlein, Delgado-Escueta and colleagues, Hirose and associates, Malafosse and Moulard, and Anderson and Hauser, whose articles are recommended. In the diagnosis of epilepsy, history is the key; in most adult cases the physical examination is relatively unrevealing. Often, in emergency departments, it is difficult to differentiate the postictal effects of an unwitnessed seizure from mild confusion following cerebral concussion or from a brief loss of consciousness produced by a subarachnoid hemorrhage. The clinical differences between a seizure and a syncopal attack are considered in Chap. The authors have erred in mistaking akinetic seizures for simple faints and vasovagal and cardiac faints for seizures. If blood is tested after the episode in question, elevation in creatine kinase (persistent for hours) and prolactin (for up to 10 min) may be helpful in the diagnosis of a convulsive seizure. Postictal confusion, incontinence, and a bitten tongue clearly bespeak seizure rather than syncope. Helpful maneuvers are to have the patient hyperventilate in order to evoke an attack or to observe the patient counting aloud for 5 min. These attacks are so variable and so often induce disturbances of behavior and psychic function- rather than obvious interruptions of consciousness- that they may be mistaken for temper tantrums, hysteria, sociopathic behavior, or acute psychosis. Verbalizations that cannot be remembered, walking aimlessly, or inappropriate actions and social behavior are characteristic. As stated above, we have placed emphasis on amnesia for the events of at least part of the seizure as a crucial criterion for the diagnosis of temporal lobe epilepsy. A mild complex partial seizure, consisting of a brief loss of consciousness and lip-smacking, may be mistaken for an absence unless it is kept in mind that the former (but not the latter) is commonly followed by a period of confusion and dysphasia when the language areas are involved. Epilepsy complicated by states of constitutional mental dullness and confusion poses special problems in diagnosis. Most epileptic patients seen in a general hospital or in office practice show no evidence of mental retardation, regardless of the type of seizure. Undoubtedly, seizures are more common in the mentally retarded, but recurrent seizures in themselves seldom cause intellectual deterioration (Ellenberg et al); when this does happen, one should suspect an underlying degenerative or hereditary metabolic disease. An exception to this statement is the patient with frequent and uncontrolled subclinical seizures (nonconvulsive status) who is drugged or in a postseizure psychotic state. Hospital admission and a systematic study of the seizure state and drug levels are necessary in the analysis of this problem.