33. Behavioral Presentations of Medical and Neurologic Disorders

8/12/2019 33. Behavioral Presentations of Medical and Neurologic Disorders

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IV. Dem entia Delirium and Related Conditions

33 BEHAVIORAL PRESE NTA TIONS OF MEDICALAND NEUROLOGIC DISORDERSC. 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 bea medical or neurologic disease. Patients with m edical illness w ho present with behavioral or psy-chiatric symptoms as the major manifestation have been shown to have significant morbidity andmortality that worsens with delay in diagnosis and treatment. Illnesses as diverse as brain tumorsand renal failure may present with behavioral syndromes, and for many of the conditions there arespecific and effective therapies. Psychiatric treatment is unlikely to be effective and th e conditionmay worsen unless the primary p roblem is addressed. Hence, the timely and expeditious identifica-tion of patients with secondary or induced behavior syndromes is crucial.2 What are the typical behavioral presentations of medical and neurologic diseases?Whereas nearly every symptom , syndrome, and psychiatric d iagnostic category has been de-scribed, several presentations are particularly comm on. Confu sional states, psychosis, dep ression,and personality changes are the most frequent, with anxiety, mania, and conversion disorder occur-ring less often. AH varieties of presentation are seen. Affected patients may present with isolatedsymp toms or with multiple sym ptom s 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 nochan ge over months to years. The bad news, therefore, is that we need to consider an underlyingmedical o r neurologic problem in nearly every patient that we see. The good new s, however, is thatclinical 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 psychiatricillness and onset of symptoms after age 40 should raise the suspicion of medical or n eurologic ill-ness. A thorough review of systems may u ncover other problems that otherwise would be overlookedin the face of major behavioral disturbances. A history of headaches, syncope, seizures, head trauma,focal neur olog ic problems i.e., visual disturbance, wea kne ss, incoordination), card iopu lmo narycomplaints, incontinence, weight chan ge, or fevers should prompt further investigation. Finally, pre-senting complaints that have a higher likelihood of representing medical illness include progressiveintellectual deterioration, apathy or indifference, and visual hallucinations without accompanyingauditory hallucinations.

3 What is confusion?Because of its everyday use, confusion as a medical term has confused many clinicians. In clini-cal terms, confusion m eans the inability to maintain a coherent line of thought. Confusional states areexceedingly comm on, and most arise acutely because of a reversible toxic or m etabolic disorder withprom inent effects on the brain. The patient with an acute confusional state typically presents with im -paired attention, disorientation, incoherent thinking, hallucinations, delusions, illusions, disturbed167


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168 Behavioral Presentations of Medical and Neurologic Disorderssleep-wake cycles, and variable alterations in level of consciousness. The cardinal feature is the dis-turbance of attention; other symptoms present in varying combinations and degrees.Synonymous terms terms include delirium and metabolic or toxic encephalopathy, and each maybe used to emphasize certain aspects of the syndrome. The term acute organic brain syndrome, how-ever, is inadequate, both because it lacks specificity and because it promulgates the unlikely beliefthat some behavioral disorders do not result from brain dysfunction. This terminology has beendeleted 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 withprior brain disease or injury, postoperative or bum patients, and patients with acquired immunodefi-ciency syndrome AIDS). The list of causes for the acute confusional state is long but the morecommon disorders associated with confusion are listed below:

Common Causes of the Acute Confusional StateIntoxications-alcohol; prescription, over-the-counter, and street drugs; solvents; heavy metals;Withdrawal states-alcohol, sedative-hypnotic drugsNutritional deficiencies-thiamine Wernickes encephalopathy),vitamin B,,, folate, niacinMetabolic disorders-lectrolyte and acid-base disturbances; hepatic, renal, pancreatic diseaseInfections-pneumonia, urinary tract infection, sepsis, AIDSEndocr inopathieshypo- and hyperthyroidism, hypo- and hypergl ycemia, hypo- and

Structural brain disease-traumatic brain injury, seizure disorders, stroke, subarachnoidor parenchymalPostoperative states-anesthesia, electrolyte disturbances, fever, hypoxia, analgesics

pesticides; carbon monoxide

hyperadrenocorticismhemorrhage, epidural or subdural hematoma, encephalitis, brain abscess

5. Differentiate primary and secondary psychosis.The essence of psychosis is loss of contact with reality. This breakdown in perception, thoughtcontent, and communications takes various forms, including hallucinations, delusions, motor distur-bances, paranoia, and changes in affect. Although the typical constellation of symptoms and signs ofschizophrenia has been described in medical and neurological illness, usually other clues suggest anunderlying pathologic process.Secondary or induced psychosis often has a more abrupt onset, more prominent alterations inlevel of consciousness, and more evidence of intellectual deterioration. The character of symptomsalso may be different, with induced psychosis more likely to cause visual hallucinations without au-ditory hallucinations, and poorly defined delusions.Pr imary psychosis, due entirely to psychiatric illness, more often manifests auditory hallucina-tions, preserved level of alertness and orientation, and more complex and stable delusions.

6. Which disorders may present with secondary psychosis?Disorders Associated with econdmy Psychosis

Complex partial seizuresAlcohol withdrawal StrokeDrugs prescription,over-the-counter, Brain infectionsBrain neoplasmsDementia Alzheimers disease, Picks

Traumatic brain injurystreet; for example bromocriptine,levodopa, diet pills, amphetamines)thyroid disease; vitamin deficiencies) disease)Metabolic disorders hepatic, renal,Multiple sclerosis

disease, Huntingtons disease, Wilsons


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Behavioral Presentations of Medical and Neurologic Disorders I697 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 morecommon 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 picturetypical of major depression in every respect. Clues to distinguishing these patients include the ab-sence 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 N eurologic Causes o Depression

Drugs oral contraceptives, beta-blockers, Metabolic disorders thyroid disorders, adrenalopiates, benzodiazepines, barbiturates, disorders, hepatic disease, hypoglycemia,methyldopa) pancreatic and gastrointestinal cancer)Stroke Dementia Alzheimers disease, ParkinsonsSystemic lupus erythematosusBrain neoplasms NeurosyphilisTraumatic brain injuryMultiple sclerosis

disease, Huntingtons disease)

8. Which disorders may present with mania?Manic patients present with increased energy, flight of ideas, grandiosity, and impaired judg-ment against a background of an abnormally elevated or irritable mood. There may be delusions andhallucinations as well. Mania has been described as the presenting symptom of many medical disor-ders and also as a consequence of head trauma and seizure disorder. The diagnosis of secondary orinduced mania is suggested by associated neurologic signs and symptoms and initial presentationafter the age of 40Com mon Medica l and NeLirologic Causes o Mania

Drugs e.g., excessive thyroid hormone,amphetamines, cocaine, monoamineoxidase inhibitors, steroids)Multiple sclerosisDementia Huntingtonsdisease, Wilsonsdisease, Picks disease)Hyperthyroidism Herpes simplex encephalitisSeizure disorders especially complex- Neurosyphilis

Traumatic brain injuryStroke

partial) 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 knowthem when we see them. Subtle alterations in basic character and temperament often herald the onsetof neurologic illness. Comportment, motivation, affect, judgment, and impulse control may changedramatically in the face of disease or injury of the brain. Whereas nearly any illness or injury mayalter personality, the following lists provides a clinical guide.Medical and Neurologic Causes o Pe rso ndi g Change

Traumatic brain injuryDementia Picks disease, Alzheimers disease, Complex partial seizure disorderDrug and alcohol abuseInfection with human immunodeficiencyvirusHuntingtons disease, Wilsons disease, Neurosyphilisnormal pressure hydrocephalus)

Brain neoplasms H WStroke Hypo- and hyperthyroidismMultiple sclerosis


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170 Behavioral Presentations of Medical and N eurologic Disorders10. Which disorders may present with anxiety?Patients with anxiety typically display apprehen sion, fear, hyperattentiveness, trembling, rest-lessness, dizziness, dry m outh, and palpitations. These are com mon autonom ic responses to psycho-logical stress but also may represent an undiagnosed medical or neurologic il lness. As in theexam ples above, absence of related history, lack of precipitating event, and older ag e at onset sug-gest an un derlying disorder. In large series of patients, end ocrine diseases and cardiopulmonary con-ditions were m ost likely to present with anxiety.

Likely Disorders Associated with AnxietyHyperthyroidismHypoglycemiaPheochromocytomaHypoparathyroidismCardiovascular disease

Pulmonary diseaseDrugsAlcohol or sedative-hypnotic withdrawalSystemic lupus erythematosusWilsons d isease11. Why is it important to recognize conversion disorder?Many patients present with sym ptom s and signs that suggest medical or neurologic illness, butare due to unconscious manifestations of em otional conflict. The clinician needs to be able to recog-nize this pattern, both for accurate diagnosis of psychiatric illness and for isolation of hysterical clin-ical features from those that may be d ue to med ical or neurologic il lness. An im portant point toremember is that signs and symptom s of conversion disorder are common in patients with knownneurologic illness. For example, nonepileptic seizures may be encountered in patients with estab-lished seizure disord ers. Such traditional signs of conv ersion disorde r as give-way weakness,nonanatomic sensory chang es, and la belle indifference may be seen in patients with m ultiple sclero-sis and other neurologic disorders see Chap ter 31 for fur ther discussion of conversion disorder).Disorders that frequently accompany hysteria include:Multiple sclerosis Com plicated migraineSystem ic lupus erythem atosus NeurosyphilisSeizure disorders Endo crine disorders12. What is an appropriate evaluationof patients presenting with behavioral syndromes?

As with all medical disciplines, it is wise to start with a detailed history, paying close attentionto onset and course of sym ptom s, past and present medical and surgical history, and comp lete reviewof medications and drugs prescription, over-the-counter, borrow ed, stolen, or obtained on thestreet). Th e family history should b e reviewed for both m edical and psychiatric illness. A com pletereview of systems also is necessary. At this point, we suggest going where the money is and re-viewing medications and drugs again.A detailed general physical examination, including neuro logic and m ental status testing isnext. Laboratory evaluations should includ e a com plete bloo d coun t, urinalysis, thyroid functionstudies, and toxicology screen. For example, you may encounter delirium due to hypog lycemia, orpsychosis related to hyperth yroid ism . Pulse oximetry or arterial blood ga s studies, lumbar punc-ture, syph ilis serology, HIV testing, B 12 and folate levels, vasculitis screen ing, and measurem entsof heavy m etals, copper, ceruloplasmin, and porphy rins may be indicated. Consider these testswhen sign s and sym ptoms sugg est part icular organ system involvement or the presence of re-versible d isorders.

Additional tests include electroencephalography EE G) and neuroimaging studies. EE G pro-vides information abou t 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 doc-umented in seizure disorders, but it often is useful in the diagnosis of acute confusional states, de-mentia, and focal brain lesions.Both comp uterized tomography CT ) and magnetic resonance imaging M RI) generate detailedanatomic information, and M RI in particular shows elegant views of brain regions that may b e im-plicated in the pathogenesis of behavioral and psychiatric disorders. Because of high cost, however,


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Dementia 171the ind ica t ions fo r ob ta in ing such scans have been cont rover s ia l . Severa l behaviora l p r esen ta t ionsshould general ly prompt a neuro imaging scan , inc lud ing acu te confus iona l s ta te or dement ia of un-kno wn c ause, the ini t ia l episode of undiagno sed psychosis , an d the f irst presentation of personalitychang e a f te r age 40 Other ind ica t ions inc lude foca l neuro logic f ind ings , movem ent d i sorder s , in -cont inence , o r ev idence of increased intracranial pressure suc h as headache, nause a, vomit ing, andpapi l l edema o n funduscopic examinat ion .

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2.3.4.56.7.89.

10.11.12.13.

BIBLIOGRAPHYCum mings JL: Organic delusions: Phenomenology, anatom ical considerations, and review. Br J PsychiatryCummings JL: Psychosis in neurologic disease: Neurobiology and pathogenesis. NeuropsychiatryCummings JL, Miller BL: Visual hallucinations:Clinical occurrence and use in differential diagnosis. West JGorman DG, C umm ings JL: Organic delusional synd rome. Semin Neurol 10:229-238, 1990.Gould R, Miller BL, Goldberg MA, Benson DF: The validity of hysterical signs and sym ptoms. Nerv MentLarson E W Organic causes of mania. Ma yo Clin Proc 63:906-912, 1988.Ma ckenzie TB, Popkin M K: Organic anxiety syndrom e.Am J Psychiatry 140:342-344, 1983.Skuster DZ, Digre KB, Corbett JJ: N eurologic c onditions presenting as psychiatric d isorde rs. Psychiatr ClinStrub RL: Mental disorde rs in brain disease. In F rederik s JA ed): Handbook of Clin ical Neurology, Vol 2.Taylor D, Lewis S: Delirium. Neu rol Neurosurg Psychiatry 56742 -751, 1993 .Filley CM , Kleinschmidt-DeM asters BK: Neurobehavioral presentations of brain neoplasm s. West J MedLyoo IK, Seol HY Byun HS, Renshaw PF: Unsuspected m ultiple sc lerosis in patien ts with psychia tric disor-Yudofsky SC, Hales RE eds): Neuropsychiatry, 2nd ed. Washington, DC , .4mencan Psyc hiatric Press, 1992.

146:184-197, 1985.Neuropsychol Behav Neu rol5 : 144-150, 1992.Med 146:46-51, 1987.

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NorthAm 15:311-333, 1992.Am sterdam, Elsevier, 1985, pp 413-441.

163:19-25, 1995.ders:A magnetic resonance imaging study. J Neuropsychiatry Clin Neurosci 8 3 -5 9 , 1996.

34 DEMENTIARoberta M khardsovt, M.D.

1. Define dementia.Dem entia is an imp airmen t in inte llectual funct ioning in a t leas t two spheres . O ne of the spheres

i s memory; the second may be any o the r a r ea of cognit ion .Cognitive unctions That M ay Be Im paired in DementiaLanguageVisuospatial ability semia utom atic tasksPersonality AbstractionJudgment CalculationObject recognition Inform ation synthesis

Ability to dress and do other

Problem solving

In contrast to delir ium, th e deficits of dem entia are relatively stable over a t leas t a few mo nths .In contrast to me ntal retardation, the deficits are acquired. Me mory dis turbance is an ear ly feature .I t may b e evidenced b y inabi l ity to learn new m ater ia l or loss of abili ty to recall previously lear nedmaterial.

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33. Behavioral Presentations of Medical and Neurologic Disorders