IV.

Dementia, Delirium, and Related Conditions

3 3 . BEHAVIORAL PRESENTATIONS OF MEDICAL

AND NEUROLOGIC DISORDERS

C. Alan Anderson, M.D., and Christopher M. Filley, M.D

1. Why is the identification of an underlying medical or neurologic disorder important? What initially seems to be a standard psychiatric illness on closer examination may prove to be a medical or neurologic disease. Patients with medical illness who present with behavioral or psychiatric symptoms as the major manifestation have been shown to have significant morbidity and mortality that worsens with delay in diagnosis and treatment. Illnesses as diverse as brain tumors and renal failure may present with behavioral syndromes, and for many of the conditions there are specific and effective therapies. Psychiatric treatment is unlikely to be effective and the condition may worsen unless the primary problem is addressed. Hence, the timely and expeditious identification of patients with secondary or induced behavior syndromes is crucial.

2. What are the typical behavioral presentationsof medical and neurologic diseases?
Whereas nearly every symptom, syndrome, and psychiatric diagnostic category has been described, several presentations are particularly common. Confusional states, psychosis, depression, and personality changes are the most frequent, with anxiety, mania, and conversion disorder occurring less often. AH varieties of presentation are seen. Affected patients may present with isolated symptoms or with multiple symptoms of sufficient duration and severity to meet DSM-IV criteria. The problem may be acute and progressive, or it may present as a chronic condition with little or no change over months to years. The bad news, therefore, is that we need to consider an underlying medical or neurologic problem in nearly every patient that we see. The good news, however, is that clinical clues help to identify patients at higher risk and assist in focusing the evaluation. In general, the absence of prior psychiatric problems, lack of family history of psychiatric illness, and onset of symptoms after age 40 should raise the suspicion of medical or neurologic illness. A thorough review of systems may uncover other problems that otherwise would be overlooked in the face of major behavioral disturbances. A history of headaches, syncope, seizures, head trauma, focal neurologic problems (i.e., visual disturbance, weakness, incoordination), cardiopulmonary complaints, incontinence, weight change, or fevers should prompt further investigation. Finally, presenting complaints that have a higher likelihood of representing medical illness include progressive intellectual deterioration, apathy or indifference, and visual hallucinations without accompanying auditory hallucinations.

3. What is confusion?
Because of its everyday use, confusion as a medical term has confused many clinicians. In clinical terms, confusion means the inability to maintain a coherent line of thought. Confusional states are exceedingly common, and most arise acutely because of a reversible toxic or metabolic disorder with prominent effects on the brain. The patient with an acute confusional state typically presents with impaired attention, disorientation, incoherent thinking, hallucinations, delusions, illusions, disturbed
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sleep-wake cycles, and variable alterations in level of consciousness. The cardinal feature is the disturbance of attention; other symptoms present in varying combinations and degrees. Synonymous terms terms include delirium and metabolic or toxic encephalopathy, and each may be used to emphasize certain aspects of the syndrome. The term acute organic brain syndrome, however, is inadequate, both because it lacks specificity and because it promulgates the unlikely belief that some behavioral disorders do not result from brain dysfunction. This terminology has been deleted from the DSM-IV, and we suggest that it be dropped from common medical usage as well.
4. Which disorders may present with confusion? Patients at higher risk for developing an acute confusional state include the elderly, patients with prior brain disease or injury, postoperative or bum patients, and patients with acquired immunodeficiency syndrome (AIDS). The list of causes for the acute confusional state is long but the more common disorders associated with confusion are listed below:

Common Causes of the Acute Confusional State

Intoxications-alcohol; prescription, over-the-counter, and street drugs; solvents; heavy metals; pesticides; carbon monoxide Withdrawal states-alcohol, sedative-hypnotic drugs Nutritional deficiencies-thiamine (Wernickes encephalopathy),vitamin B,,, folate, niacin Metabolic disorders-lectrolyte and acid-base disturbances; hepatic, renal, pancreatic disease Infections-pneumonia, urinary tract infection, sepsis, AIDS Endocrinopathieshypo- and hyperthyroidism, hypo- and hypergl ycemia, hypo- and hyperadrenocorticism Structural brain disease-traumatic brain injury, seizure disorders, stroke, subarachnoidor parenchymal hemorrhage, epidural or subdural hematoma, encephalitis,brain abscess Postoperative states-anesthesia, electrolyte disturbances, fever, hypoxia, analgesics

5. Differentiate primary and secondary psychosis. The essence of psychosis is loss of contact with reality. This breakdown in perception, thought content, and communications takes various forms, including hallucinations, delusions, motor disturbances, paranoia, and changes in affect. Although the typical constellation of symptoms and signs of schizophrenia has been described in medical and neurological illness, usually other clues suggest an underlying pathologic process. Secondary o r induced psychosis often has a more abrupt onset, more prominent alterations in level of consciousness, and more evidence of intellectual deterioration. The character of symptoms also may be different, with induced psychosis more likely to cause visual hallucinations without auditory hallucinations, and poorly defined delusions. Primary psychosis, due entirely to psychiatric illness, more often manifests auditory hallucinations, preserved level of alertness and orientation, and more complex and stable delusions.
6. Which disorders may present with secondary psychosis? Disorders Associated with Secondmy Psychosis
Complex partial seizures Alcohol withdrawal Drugs (prescription,over-the-counter, street; for example bromocriptine, levodopa, diet pills, amphetamines) Metabolic disorders (hepatic, renal, thyroid disease; vitamin deficiencies) Multiple sclerosis Traumatic brain injury Stroke Brain infections Brain neoplasms Dementia (Alzheimers disease, Picks disease, Huntingtons disease, Wilsons disease)

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7. Which disorders may present with depression? The depressed patient presents with low mood, psychomotor retardation, apathy, and anhedonia, plus the vegetative signs of decreased appetite, diminished libido, and sleep disturbance. The more common concern is overlooking functional depression while searching for medical and neurologic illness, but the reverse situation also occurs. Systemic illnesses can present with a clinical picture typical of major depression in every respect. Clues to distinguishing these patients include the absence of previous psychiatric problems or family history, no precipitating event, older age at onset, and associated medical and neurological signs and symptoms.
Frequent Medical and Neurologic Causes of Depression
Drugs (oral contraceptives, beta-blockers, opiates, benzodiazepines, barbiturates, methyldopa) Stroke Systemic lupus erythematosus Brain neoplasms Traumatic brain injury Multiple sclerosis Metabolic disorders (thyroid disorders, adrenal disorders, hepatic disease, hypoglycemia, pancreatic and gastrointestinal cancer) Dementia (Alzheimersdisease, Parkinsons disease, Huntingtons disease) Neurosyphilis

8. Which disorders may present with mania? Manic patients present with increased energy, flight of ideas, grandiosity, and impaired judgment against a background of an abnormally elevated or irritable mood. There may be delusions and hallucinations as well. Mania has been described as the presenting symptom of many medical disorders and also as a consequence of head trauma and seizure disorder. The diagnosis of secondary or induced mania is suggested by associated neurologic signs and symptoms and initial presentation after the age of 40. Common Medical and NeLirologic Causes of Mania
Drugs (e.g., excessive thyroid hormone, amphetamines, cocaine, monoamine oxidase inhibitors, steroids) Hyperthyroidism Seizure disorders (especially complexpartial) Traumatic brain injury Stroke Multiple sclerosis Dementia (Huntingtonsdisease, Wilsons disease, Picks disease) Herpes simplex encephalitis Neurosyphilis Brain neoplasms

9. Which disorders may result in personality change? Personality changes are like good art: they are hard to describe and categorize, but we know
them when we see them. Subtle alterations in basic character and temperament often herald the onset of neurologic illness. Comportment, motivation, affect, judgment, and impulse control may change dramatically in the face of disease or injury of the brain. Whereas nearly any illness or injury may alter personality, the following lists provides a clinical guide.

Medical and Neurologic Causes o f Persondig Change

Traumatic brain injury Dementia (Picks disease, Alzheimers disease, Huntingtons disease, Wilsons disease, normal pressure hydrocephalus) Brain neoplasms Stroke Multiple sclerosis Complex partial seizure disorder Drug and alcohol abuse Neurosyphilis Infection with human immunodeficiencyvirus (HW Hypo- and hyperthyroidism

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10. Which disorders may present with anxiety? Patients with anxiety typically display apprehension, fear, hyperattentiveness, trembling, restlessness, dizziness, dry mouth, and palpitations. These are common autonomic responses to psychological stress but also may represent an undiagnosed medical or neurologic illness. As in the examples above, absence of related history, lack of precipitating event, and older age at onset suggest an underlying disorder. In large series of patients, endocrine diseases and cardiopulmonary conditions were most likely to present with anxiety.
Likely Disorders Associated with Anxiety
~

Hyperthyroidism Hypoglycemia Pheochromocytoma Hypoparathyroidism Cardiovascular disease

Pulmonary disease Drugs Alcohol or sedative-hypnotic withdrawal Systemic lupus erythematosus Wilsons disease

11. Why is it important to recognize conversion disorder? Many patients present with symptoms and signs that suggest medical or neurologic illness, but are due to unconscious manifestations of emotional conflict. The clinician needs to be able to recognize this pattern, both for accurate diagnosis of psychiatric illness and for isolation of hysterical clinical features from those that may be due to medical or neurologic illness. An important point to remember is that signs and symptoms of conversion disorder are common in patients with known neurologic illness. For example, nonepileptic seizures may be encountered in patients with established seizure disorders. Such traditional signs of conversion disorder as give-way weakness, nonanatomic sensory changes, and la belle indifference may be seen in patients with multiple sclerosis and other neurologic disorders (see Chapter 3 1 for further discussion of conversion disorder). Disorders that frequently accompany hysteria include: Multiple sclerosis Complicated migraine Systemic lupus erythematosus Neurosyphilis Seizure disorders Endocrine disorders 12. What is an appropriate evaluation of patients presenting with behavioral syndromes? As with all medical disciplines, it is wise to start with a detailed history, paying close attention to onset and course of symptoms, past and present medical and surgical history, and complete review of medications and drugs (prescription, over-the-counter, borrowed, stolen, or obtained on the street). The family history should be reviewed for both medical and psychiatric illness. A complete review of systems also is necessary. At this point, we suggest going where the money is and reviewing medications and drugs again. A detailed general physical examination, including neurologic and mental status testing is next. Laboratory evaluations should include a complete blood count, urinalysis, thyroid function studies, and toxicology screen. For example, you may encounter delirium due to hypoglycemia, or psychosis related to hyperthyroidism. Pulse oximetry or arterial blood gas studies, lumbar puncture, syphilis serology, HIV testing, B 12 and folate levels, vasculitis screening, and measurements of heavy metals, copper, ceruloplasmin, and porphyrins may be indicated. Consider these tests when signs and symptoms suggest particular organ system involvement or the presence of reversible disorders. Additional tests include electroencephalography (EEG) and neuroimaging studies. EEG provides information about the physiology of the brain and is safe and readily available at modest cost. Another advantage is that it can be performed at bedside if necessary. The utility of EEG is best documented in seizure disorders, but it often is useful in the diagnosis of acute confusional states, dementia, and focal brain lesions. Both computerized tomography (CT) and magnetic resonance imaging (MRI) generate detailed anatomic information, and MRI in particular shows elegant views of brain regions that may be implicated in the pathogenesis of behavioral and psychiatric disorders. Because of high cost, however,

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the indications for obtaining such scans have been controversial. Several behavioral presentations should generally prompt a neuroimaging scan, including acute confusional state or dementia of unknown cause, the initial episode of undiagnosed psychosis, and the first presentation of personality change after age 40. Other indications include focal neurologic findings, movement disorders, incontinence, or evidence of increased intracranial pressure such as headache, nausea, vomiting, and papilledema on funduscopic examination.

BIBLIOGRAPHY
1. Cummings JL: Organic delusions: Phenomenology, anatomical considerations, and review. Br J Psychiatry

146:184-197, 1985. 2. Cummings JL: Psychosis in neurologic disease: Neurobiology and pathogenesis. Neuropsychiatry Neuropsychol Behav Neurol5: 144-150, 1992. 3. Cummings JL, Miller BL: Visual hallucinations:Clinical occurrence and use in differential diagnosis. West J Med 146:46-51, 1987. 4. Gorman DG, Cummings JL: Organic delusional syndrome. Semin Neurol 10:229-238, 1990. 5. Gould R, Miller BL, Goldberg MA, Benson DF: The validity of hysterical signs and symptoms. J Nerv Ment Dis 174593-597, 1986. 6. Larson E W Organic causes of mania. Mayo Clin Proc 63:906-912, 1988. 7. Mackenzie TB, Popkin MK: Organic anxiety syndrome.Am J Psychiatry 140:342-344, 1983. 8. Skuster DZ, Digre KB, Corbett JJ: Neurologic conditions presenting as psychiatric disorders. Psychiatr Clin NorthAm 15:311-333, 1992. 9. Strub RL: Mental disorders in brain disease. In Frederiks JA (ed): Handbook of Clinical Neurology, Vol 2. Amsterdam, Elsevier, 1985, pp 413-441. 10. Taylor D, Lewis S: Delirium. 3 Neurol Neurosurg Psychiatry 56742-751, 1993. 11. Filley CM, Kleinschmidt-DeMasters BK: Neurobehavioral presentations of brain neoplasms. West J Med 163:19-25, 1995. 12. Lyoo IK, Seol HY, Byun HS, Renshaw PF: Unsuspected multiple sclerosis in patients with psychiatric disorders: A magnetic resonance imaging study. J Neuropsychiatry Clin Neurosci 83-59, 1996. 13. Yudofsky SC, Hales RE (eds): Neuropsychiatry, 2nd ed. Washington, DC, .4mencan Psychiatric Press, 1992.

34. DEMENTIA
Roberta M.Rkhardsovt, M.D.

1. Define dementia.
Dementia is an impairment in intellectual functioning in at least two spheres. One of the spheres is memory; the second may be any other area of cognition.

Cognitive Functions That May Be Impaired in Dementia

Language Visuospatial ability Personality Judgment Object recognition Ability to dress and do other semiautomatic tasks Abstraction Calculation Information synthesis Problem solving

In contrast to delirium, the deficits of dementia are relatively stable over at least a few months. In contrast to mental retardation, the deficits are acquired. Memory disturbance is an early feature. It may be evidenced by inability to learn new material or loss of ability to recall previously learned material.