1 MENTAL RETARDATION AND DEVELOPMENTAL DISABILITIESSuggestions for Lecturer -1-hour lecture -Use slides alone or to supplement your own teaching materials. --Supplement lecture with handouts, such as figures and tables from papers listed as references in the GRS chapter on mental retardation and developmental disabilities. Topic

2 OBJECTIVES Know and understand:The definition of mental retardation (MR) and its prevalence among older adults How to recognize and manage psychiatric, mental, and behavioral disorders in older adults with MR Ways to overcome barriers to diagnosis and treatment Common comorbidities found in individuals with developmental disabilities with or without MR Topic

3 TOPICS COVERED Nomenclature PrevalencePsychiatric and Mental Disorders in Aging Adults with MR Medical Disorders Social Conditions Developmental Disabilities and Comorbidity Topic

4 STANDARDS OF EVIDENCE (SOE)Rating Basis of Rating Studies Justifying Rating A Consistent and good quality patient-oriented evidence Large cohort studies for risk factors/prognosis; RCTs for diagnosis/treatment B Somewhat inconsistent or limited quality patient-oriented evidence Smaller or single cohort studies for risk factors/ prognosis; small or single RCTs or cohort studies for diagnosis/treatment; uncontrolled studies C Very inconsistent or very limited patient-oriented evidence, consensus, disease-oriented evidence, and/or case series for studies of diagnosis, treatment, prevention, or screening Single small cohort study for risk factors/prognosis; single small cohort study or RCT for diagnosis/treatment; case series D Unstudied common practice or opinion No evidence RCT = randomized controlled trial Patient-oriented evidence measures outcomes that matter to patients: morbidity, mortality, symptom improvement, cost reduction, quality of life. Disease-oriented evidence measures intermediate, physiologic, or surrogate endpoints that may or may not reflect improvements in patient outcomes (ie, blood pressure, blood chemistry, physiological function, and pathological findings). Topic

5 NOMENCLATURE Definition of MR:IQ of ~70 or below based on formal test results and Impairment in adaptive functioning before age 18 Not everyone with a developmental disability has MR This slide set focuses on individuals with MR who may or may not have a comorbidity such as cerebral palsy, epilepsy, or autism spectrum disorder The definition of MR on this slide comes from the Diagnostic and Statistical Manual, 4th Edition, Text Revision (DSM IV-TR). Unfortunately, differences over nomenclature for MR continue to exist based on historical, cultural, or geographic issues, or on perceptions of “correctness.” While this discord alone does not seem to have limited research efforts, it can cause some confusion, indicating a need to find common terms and acceptance of definitions. Criticism of the diagnostic criteria for “normal” intelligence and adaptive functioning continue but are not pertinent to this discussion. Slide 5 Topic Slide 5

6 PREVALENCE OF MR Life expectancy for people with MR has increased substantially 1930: Average age of death 15 yr for males, 22 yr for females 1990s: >40% live to at least age 60 Prevalence currently 1%–1.5% in the US For people 60 yr, this number expected to double by 2030 The number of individuals with MR surviving into old age is increasing because of generally better health care overall, including earlier detection and treatment of some conditions. It is difficult to quantify the prevalence of MR among older individuals because of methodologic considerations. Various studies have had some predictable variation in the results. It is even more problematic to consider the issues from the perspectives of other cultures and standards in areas other than Europe and North America. In general, longevity decreases with severity of intellectual impairment, certain comorbid conditions (eg, seizure disorders, Down syndrome), and the general health and wealth of the country or culture. Slide 6 Topic Slide 6

7 PSYCHIATRIC AND MENTAL DISORDERS IN ADULTS WITH MRAdults with MR have similar risk factors for mental illnesses as their “normal” peers, but they may have additional risks Adults who were raised in institutions or did not benefit from modern medical care are at greater than normal risk The prevalence of psychiatric disorders among adults with MR is about 5 times that of age-matched controls Prevalence rates are 10%–40% The occurrence and severity of psychiatric disturbances vary with age and comorbidities It is problematic to determine the prevalence of mental illness among people with MR. Diagnostic criteria have changed over time, health care has improved, studies have been conducted in different countries and regions, standard methodologies are lacking, and the relative numbers of institutionalized versus community-based individuals have changed. There are many reports of greater than expected rates of certain mental illnesses or behavioral disorders associated with specific physical illnesses or genetic disorders. For example, older adults with autism spectrum disorders exhibit higher rates of compulsive behaviors requiring psychiatric treatment. In people with MR, the occurrence and severity of psychiatric disturbances vary by age and comorbid conditions. For example, one study showed that individuals with Down syndrome who subsequently developed Alzheimer’s dementia were far more likely to suffer from psychologic and behavioral symptoms, with more rapid decline in functional status, than those who did not develop dementia. Conversely, some symptoms may improve as the patient ages or develops comorbid problems. For example, in adults with Down syndrome, any obsessive and compulsive symptoms may wane if memory function worsens. Slide 7 Topic Slide 7

8 DEMENTIA AND MR Individuals with MR have a higher overall prevalence of dementia than is found in age-matched controls in the general population (SOE=A) All causes of dementia are possible, but some are more likely than others Down syndrome “Pugilistic dementia” associated with repeated self-injuring blows to the head from coup/contracoup effects Slide 8 Topic Slide 8

9 INCIDENCE OF DEMENTIA IN ADULTS WITH DOWN SYNDROMEPercent of people with Down syndrome Nearly 100% of adults with Down syndrome have developed the characteristic histologic neuropathology of dementia by age 40. However, it is not typical for individuals with Down syndrome to develop overt dementia at that early an age. In a 1989 study, 49 of 96 patients with Down syndrome met criteria for dementia with an average age of onset of about 54 ± 6 yr. 50 yr 60 yr Incidence of dementia Slide 9 Topic Slide 9

10 LIFE EXPECTANCY OF ADULTS WITH DEMENTIA AND DOWN SYNDROMEMedian age of death, yr The figures on this slide come from data on nearly 18,000 individuals with Down syndrome, compiled by the Centers for Disease Control and Prevention. Based on data from death certificates, standardized mortality odds ratios (SMOR) of people with Down syndrome were more likely to show congenital heart defects (SMOR=29.1), dementia (SMOR=21.2), hypothyroidism (SMOR=20.3), or leukemia (SMOR=1.6) than those of people without Down syndrome. Apart from leukemia and testicular cancer, the risk of other malignant diseases was low in persons with Down syndrome. Most individuals with Down syndrome and dementia now die during the sixth decade of life. Slide 10 Topic Slide 10

11 DIAGNOSIS AND TREATMENT OF DEMENTIA IN PEOPLE WITH MRDementia is diagnosed according to the same criteria as in the general population: Establish cognitive and adaptive deterioration Demonstrate deficits on exam (preferably with longitudinal follow-up showing progression of deficits) Exclude other possible causes of deterioration and other mental disorders Case reports have documented tolerability of cholinesterase inhibitors and the glutamate antagonist memantine in people with MR But there is no evidence of efficacy in this population Slide 11 Topic Slide 11

12 DIFFICULTIES WITH ADAPTIVE BEHAVIORSTypes of adaptive behaviors: Conceptual (eg, speaking, reading, writing) Social (eg, rules, sense of responsibility) Practical (eg, job skills, eating, dressing) In general, the greater the severity of MR, the lower the level of adaptive abilities, but these abilities can be improved over time with behavioral supports Like any group, aging adults with MR can lose or become less adept with some adaptive behaviors Adaptive behaviors are learned social and practical skills concerned with daily functions. Limitations in these skills have a negative impact on people’s lives; however, these skill abilities are not fixed in place or easily assigned to a particular level of MR. Slide 12 Topic Slide 12

13 MALADAPTIVE BEHAVIORSExamples: withdrawal, self-injury, stereotypy Severe or frequent in up to 50%–60% of adults with MR Can persist for years The proportion decreases with age for various reasons Exception 1: In Down syndrome the proportion is higher and the incidence of behavioral problems increases with the degree of MR Exception 2: Aggression is similarly frequent in all age groups and has an extremely variable presentation Maladaptive behaviors are observable phenomena that are counter-productive or disruptive for the individual. They can be common in individuals with MR and can be either a learned response or an impulsive response to a stressor. Various other terms are sometimes used to describe these acts, including target behaviors (behaviors targeted for extinction) or challenging behaviors. Slide 13 Topic Slide 13

14 BARRIERS TO DIAGNOSIS AND TREATMENT (1 of 2)Limited self-awareness Estimate the degree to which the patient is aware of his or her problem, condition, or feelings Avoid complex questions and high-level vocabulary Consult collateral sources of information, such as family or caregivers who can provide histories and other data Limited communication ability Receptive and expressive abilities can be comparable or quite different Differences in these abilities is a characteristic feature of some conditions There are many diagnostic and treatment challenges for clinicians when seeing patients who have MR, with or without comorbid issues of mental illness or challenging behaviors. The best treatment requires an accurate, or at least the most likely, diagnosis, and that requires obtaining the best history. Unfortunately, all too often the patient’s history and his or her subjective reporting are limited or unavailable. Barriers to communication can exist for both the clinician and patient. It is up to the clinician to try to be as effective as possible by recognizing the limitations of the patient. Slide 14 Topic Slide 14

15 BARRIERS TO DIAGNOSIS AND TREATMENT (2 of 2)Diagnostic (aka comorbid) overshadowing The presence of MR itself makes it difficult to diagnose and treat mental illness or challenging behaviors Diagnostic overshadowing is a barrier to critical thinking, and clinicians should remain aware of it when presented with a difficult situation Diagnostic criteria are written for general population May need to adapt DSM IV-TR and ICD-9 criteria to the various levels of MR for each diagnosis Slide 15 Topic Slide 15

16 DIAGNOSIS OF MENTAL DISORDERS IN OLDER ADULTS WITH MRFollow the same principles of history-taking and examination that apply for the general population, with the caveats discussed on the previous 2 slides Mental disorders often present as behavioral changes The reports of family or other caregivers are extremely important Consider a change in staff, residential or vocational setting, or family health as a precipitating factor for behavioral changes It is important not to over-diagnose and therefore over-treat an individual’s presentation. Because insight, judgment, and adaptive or coping skills are limited, individuals with MR are more likely to “act out,” which may be incorrectly perceived as a serious symptom of illness when, in fact, it may just be frustration. Medication may not be called for in a situation in which supportive therapy and time could lead to resolution. Medication can be used, however, to create a window of opportunity to make behavioral supports or strategies more effective. A changes in staff, residential or vocational setting, or family health should be reported and considered as a precipitating factor for all behavioral changes. The concepts of applied behavioral analysis are important tools to use in determining cause and effect of problem behaviors. Slide 16 Topic Slide 16

17 TREATMENT OF MALADAPTIVE BEHAVIORS IN MRThe appropriate treatment or response might be instructional or behavioral Preferred behavior programs reward good behavior Pharmacologic intervention may be necessary for the safety of the patient or those nearby Very few medications are approved for the most common and challenging behaviors, and prescribing medications off-label is common Slide 17 Topic Slide 17

18 PRINCIPLES OF MEDICATION MANAGEMENTTreating major mental illness in older adults with MR is similar to treating the general population Change only one medication at a time Start new medications at a low dosage and monitor the results (“start low, go slow”) If possible, taper dosages of all medications, ultimately discontinuing pharmacotherapy Avoid antipsychotic medications Do not use second-generation antipsychotic medications for sleep or “anxiety” It is beyond the scope of this slide set to discuss the various treatment options for such a diverse patient group. Autism spectrum disorders probably represent the largest diagnostic group among aging individuals for whom medication management is common and difficult. Self-injurious behaviors are certainly the most common reason medications are considered. Self-injurious behaviors include several potentially life-threatening behaviors that can cause damage to the brain, eyes, and ears, as well as the potential for systemic infections. Slide 18 Topic Slide 18

19 MEDICAL DISORDERS Adults with MR have more medical problems than age-matched individuals About 5 medical conditions per person People with severe MR have even more About 2/3 of community-dwelling people with MR have chronic conditions or major physical disability, and 50% of these conditions go undetected Visual or hearing impairments are particularly common in people with MR They increase with age and affect approximately 25% Some behaviors or conditions can worsen with age through various processes. Individuals with MR have no special immunity from the disorders of aging, and their coping mechanisms may be reduced. For example, they may be more affected by chronic pain conditions or by vision or hearing loss. Slide 19 Topic Slide 19

20 PROBLEM BEHAVIOR CAN SIGNAL PHYSICAL ILLNESS IN ADULTS WITH MRIn people who are lower-functioning or have an expressive communication disorder, a new behavioral concern can be a sentinel sign of a physical disorder As a general rule, before determining that a new problem behavior should be treated with a psychotropic medication or intervention, physical causes should be excluded New-onset self-injurious behavior can be a particularly important clue to occult illness Self-injurious behavior to the ears can be a sign of otitis externa or media. Self-injurious behavior to the eyes can be a clue to vision loss or changes. Delaying attention for a treatable vision condition, eg, presbyopia, can cause permanent loss through self-injury, such as a detached retina or corneal scarring. Slide 20 Topic Slide 20

21 LIFE EXPECTANCY Life expectancy for adults with MR is ~65 yrDecreases with increasing severity of MR Decreases with comorbidities such as inability to ambulate, lack of feeding skills, and incontinence The most common causes of death are CVD, respiratory disorders, cancer, and dementia (particularly in Down syndrome) Slide 21 Topic Slide 21

22 SOCIAL CONDITIONS At least 80% of adults with MR are cared for at home by family members About 40% of eligible individuals are not served by the formal service system Can lead to crisis when the family can no longer provide care or manage a behavioral problem About half of developmentally disabled adults with a behavior problem eventually need a different living arrangement In a typical system, more than half of families have not made plans for future care Not surprisingly, the degree of MR, physical health, and functional skills of the aging individual correlate with the degree of parental stress and burden. Even so, maternal and family characteristics such as education and income are more correlated with overall life satisfaction and maternal well-being. Slide 22 Topic Slide 22

23 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (1 of 5)System/condition Change with Developmental Disabilities Management Strategies Mental retardation Two-thirds of patients with developmental disabilities suffer from MR, many in the mild-to-moderate range. Evaluation and referral to specialized services to maximize intellectual potential Growth retardation Usually found in patients with moderate to severe disabilities; it may present as short stature, inability to gain weight, lack of sexual development, or failure to thrive. Medical evaluation for treatable causes Sensory impairment Nearly 90% of patients have impairments in hearing, vision, and speech. Strabismus is common, as is dysarthric speech. Regular evaluation of hearing, vision, and speech; correction of deficits Some developmental disabilities may not cause an intellectual disability but nonetheless can contribute to other morbidities and challenging behaviors, resulting in reduced quality of life. Among these disabilities are cerebral palsy, seizure disorders, and a host of genetic disorders too numerous to mention. Some genetic disorders have significant variability in their impact on cognitive functioning, such as William’s syndrome. Some genetic disorders once thought to affect only a single generation or gender are now thought to have broader implications, such as fragile X syndrome. In general, as the degree of cognitive impairment increases, the risk of morbidity due to physical causes increases. This slide and the next 4 slides show the approximate prevalence and severity of some common conditions found in individuals who have a developmental disability, with or without MR. Slide 23 Topic Slide 23

24 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (2 of 5)System/condition Change with Developmental Disabilities Management Strategies Dental/oral conditions Poor dentition and oral health are very common. Oral hygiene and tooth brushing; regular dental visits Thyroid problems Thyroid problems can be a cause or a result of developmental disability. Regular testing and treatment as indicated Spinal deformities Kyphosis, scoliosis, and lordosis are common among patients with muscle weakness and spasticity. Monitoring of body habitus; physical therapy Seizure disorders Half of patients may suffer from some type of seizure disorder. Diagnosis; anticonvulsant medications Slide 24 Topic Slide 24

25 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (3 of 5)System/condition Change with Developmental Disabilities Management Strategies Degenerative joint disease Chronic muscle spasticity and mobility limitations often lead to osteoarthritis and joint disease. Strength and functional status may be prematurely impaired. Physical therapy, occupational therapy, pain management Osteopenia and osteoporosis Lack of weight bearing leads to these chronic conditions in patients who are unable to ambulate. Promotion of mobility (physical therapy); adequate calcium and vitamin D supplementation Chronic pain syndromes Muscle abnormalities and associated spinal deformities often result in chronic pain syndromes. Sensory abnormalities can result in the inability to describe the type, location, and source of the pain. Regular monitoring of function and behavior to detect possible painful conditions; pain management Slide 25 Topic Slide 25

26 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (4 of 5)System/condition Change with Developmental Disabilities Management Strategies Functional decline Aging patients with cerebral palsy and other similar conditions often develop fatigue, pain, weakness, and overuse syndromes that result in premature loss of function. This is referred to as post-impairment syndrome and often requires a reduction in work hours, increase in assistance or use of adaptive devices, and/or nursing-home placement. Physical therapy, occupational therapy, pain management Cardiac and pulmonary conditions Patients with cerebral palsy and other similar physical disabilities typically require 3−5 times the energy level of unimpaired adults, predisposing patients to premature conditions of aging, such as hypertension, heart failure, and coronary artery disease. Monitoring for hypertension, shortness of breath, angina; risk factor management Slide 26 Topic Slide 26

27 DEVELOPMENTAL DISABILITIES AND COMORBIDITY (5 of 5)System/condition Change with Developmental Disabilities Management Strategies GI conditions Gastroesophageal reflux disease and constipation are common; constipation can be chronic and severe. Monitoring; medications; fiber-rich diet; exercise Incontinence Many patients are incontinent of bowel and bladder from childhood, but others develop these problems with age. Screening for treatable causes; identifying functional impairments that can limit toileting Depression and mood disorders Patients with cerebral palsy are 4 times more likely to develop depression as age-compared other adults. The stress associated with multiple disabilities is a risk factor, as is the premature decline in functional status associated with the disorder. Regular screening; counseling and/or medications Slide 27 Topic Slide 27

28 SUMMARY (1 of 2) An increasing number of individuals with MR are surviving into adulthood and old age. Maladaptive behaviors, as well as difficulties in learning and retaining new skills of coping and adaptation, are significant problems for adults with MR and, consequently, for their caregivers. Impairments in receptive and expressive communication and coexisting cognitive limitations can contribute to difficulties in the diagnostic and treatment of medical, psychiatric, and behavioral problems. Topic

29 SUMMARY (2 of 2) In individuals with MR, disease states and physiologic changes related to age can exacerbate or attenuate maladaptive behaviors. Therapeutic interventions for maladaptive behaviors or psychiatric illnesses that coexist with MR can include medications and behavioral therapies. The term “developmental disability” can describe a variety of medical conditions that are not defined by MR. However, these conditions can contribute to maladaptive behaviors and affect an individual’s quality of life. Topic

30 CASE 1 (1 of 4) A 66-yr-old man who lives in a residential care facility is brought to the office to establish care, because his internist is no longer comfortable managing his needs. He has severe MR; its cause is unknown. The patient is nonverbal except to yell when excited or agitated. His caregivers say his agitation and need for one-on-one attention contribute to high staff turnover. Most nights he has discontinuous sleep, and his behavior deteriorates the next day. He slaps his head, always in the same spot, apparently to communicate needs, seek attention, or provide self-stimulation. Topic

31 CASE 1 (2 of 4) The caregivers note hard feces and constipation, but no other medical concerns. History includes cerebral palsy. The patient takes amitriptyline 25 mg at night for sleep and quetiapine 25 mg q12h. He is on no other medications, and this regimen has not changed for >2 yr. He ambulates only with assistance. He can eat, drink, and take medication by mouth with caution. He is restless and seems uncomfortable in his wheelchair. Recent laboratory evaluation and ECG were normal. Slide 31 Topic Slide 31

32 CASE 1 (3 of 4) Which of the following is the most appropriate next step? Increase amitriptyline to 50 mg before bed. Stop amitriptyline and begin zolpidem 5 mg. Increase quetiapine schedule to 25 mg q8h. Decrease quetiapine schedule to 25 mg before bed. Educate caregivers regarding effect of sleep disorders on behavior. Topic

33 CASE 1 (4 of 4) Which of the following is the most appropriate next step? Increase amitriptyline to 50 mg before bed. Stop amitriptyline and begin zolpidem 5 mg. Increase quetiapine schedule to 25 mg q8h. Decrease quetiapine schedule to 25 mg before bed. Educate caregivers regarding effect of sleep disorders on behavior. ANSWER: B Lack of sleep seems to worsen this patient’s disruptive behaviors. The use of amitriptyline as a sleep aid is ineffective for him and may contribute to his constipation. A reasonable first step would be to stop amitriptyline and initiate a sleep medication that will not contribute to his constipation. This intervention alone may improve his sleep, increase his comfort, and decrease disruptive behavior, allowing his caregivers to respond with appropriate behavioral interventions (SOE=D). Increasing the dose of amitriptyline at night may improve sleep but would also increase adverse events. The increased dose is likely to worsen constipation and other anticholinergic effects, and it has potential for serious adverse cardiac problems. Lingering next-day lethargy might also worsen health or problem behaviors. Decreasing the dosage of quetiapine may improve the patient’s constipation but is unlikely to improve sleep. In this case, education of caregivers on medication and behavioral management issues would be a reasonable option if that alone would solve the patient's problems. Inconsistent or inappropriate responses by caregivers to difficult behaviors can create or perpetuate problems. However, the disrupted sleep and constipation are prominent problems for this patient that can be addressed promptly. Further changes in management should await results of this intervention. Slide 33 Topic Slide 33

34 CASE 2 (1 of 4) A 76-yr-old man is brought to the office by his older sister to establish care after his doctor retired. The patient has significant autism. He lives with his sister, who reports that over the past 3 mo he has been rocking, pacing, and yelling more often, and has hit himself twice. The behaviors have slowly increased in frequency and intensity to a degree that she has not seen for several years. His sleep is disrupted and he often paces during the night. His sister reports that his care is increasingly difficult for her. Slide 34 Topic Slide 34

35 CASE 2 (2 of 4) The patient’s sister recently moved them from their family home to an apartment, and they are getting out less frequently for walks and shopping. The patient has no history of significant illnesses and is on no routine medications. The patient is slightly underweight. His skin is intact, and there are no bruises. He rocks and hums nearly constantly during the office visit. Otherwise, the examination is essentially normal. Slide 35 Topic Slide 35

36 CASE 2 (3 of 4) Which of the following is the most appropriate initial step in managing the patient’s behaviors? Begin quetiapine 25 mg. Begin zolpidem 5 mg at bedtime. Begin mirtazapine 15 mg. Reevaluate in 2 wk if the behavior persists. Begin methylphenidate 5 mg. Slide 36 Topic Slide 36

37 CASE 2 (4 of 4) Which of the following is the most appropriate initial step in managing the patient’s behaviors? Begin quetiapine 25 mg. Begin zolpidem 5 mg at bedtime. Begin mirtazapine 15 mg. Reevaluate in 2 wk if the behavior persists. Begin methylphenidate 5 mg. ANSWER: C This patient’s changes in residence and familiar activities are the likely causes of his increased anxiety. The best choice among these options would be to initiate a second-generation antidepressant at an appropriate dosage and carefully monitor the results. A significant amount of clinical experience supports the off-label use of second-generation antidepressants to decrease the intensity and frequency of self-injury (SOE=D). In the choice between a relatively safe, well-studied, second-generation antidepressant with anxiolytic effects, such as mirtazapine, and a second- generation antipsychotic agent, such as quetiapine, the antidepressant offers better risk/benefit potential. If the patient’s behaviors were markedly more dangerous to himself or to others, urgency might make the antipsychotic agent the wiser option. A sedative is unlikely to produce much benefit in this case. The patient’s sleep is probably disturbed by anxiety related to changes or frustrated compulsive needs. It is better to treat the anxiety that causes the behavior than to sedate an older patient. Waiting to reevaluate the patient while both he and his sister are at risk of injury is not a good option. Because this patient has no evidence of attention deficit/hyperactivity disorder, methylphenidate is not an appropriate option. Finally, the healthcare provider should ask the patient’s sister about the extent of formal and informal social support available. Slide 37 Topic Slide 37

38 CASE 3 (1 of 4) A 55-yr-old man with Down syndrome is brought to the office by his caregivers. Over the past year he has become confused and forgetful and has needed increasingly more frequent prompts for his assigned tasks. Although they do not have specific written records, the caregivers think that his speech and movement have slowed, that he is more socially withdrawn, and that he is having more outbursts with peers and staff. In the last 4 mo, he has twice lost his balance and fallen. Slide 38 Topic Slide 38

39 CASE 3 (2 of 4) The patient has been physically healthy overall and is on no medications. His last routine physical examination was 8 mo ago, at which time there were no indications of any problem. During the visit, the patient seems shy and anxious and has a congruent affect. He has no complaints. His expressive abilities are limited and consistent with his low-moderate level of MR. Slide 39 Topic Slide 39

40 CASE 3 (3 of 4) Which of the following is the most appropriate next step? Order head CT. Perform office-based, appropriate cognitive testing. Begin fluoxetine. Begin donepezil and, if well tolerated, add memantine. Refer patient to a psychiatrist who specializes in neurodevelopmental disabilities. Slide 40 Topic Slide 40

41 CASE 3 (4 of 4) Which of the following is the most appropriate next step? Order head CT. Perform office-based, appropriate cognitive testing. Begin fluoxetine. Begin donepezil and, if well tolerated, add memantine. Refer patient to a psychiatrist who specializes in neurodevelopmental disabilities. ANSWER: B This patient has symptoms consistent with dementia in Down syndrome. Early symptoms with or without Down syndrome include more short-term than distant memory loss, forgetfulness, and confusion. Patients with Down syndrome also have earlier frontal lobe symptoms, such as slowing of speech, language, and motor activities; social withdrawal; and problems with sleep, balance, and emotional regulation (SOE=B). This patient displays many of the symptoms of dementia, but diagnostic tests and additional history are needed before determining an appropriate treatment strategy. The cognitive testing necessary in this situation is available through psychology and psychiatry services and does not require consultation with a specialist in neurodevelopmental disabilities. Despite the caregivers’ report of falls, brain imaging is not likely to yield additional useful information. Some of the symptoms suggest depression, but more information is needed before making a diagnosis of depression. The degree of severity and patient values should guide treatment for dementia. The patient’s presentation suggests mild rather than moderate or severe dementia. A medication regimen that combines two agents is not an appropriate first choice. In addition, cognitive assessment must be performed first. Slide 41 Topic Slide 41

42 © Copyright 2010 American Geriatrics SocietyACKNOWLEDGMENTS GRS Chapter Author: Mark H. Fleisher, MD GRS Question Writer: Mark H. Fleisher, MD Medical Writers: Beverly A. Caley Faith Reidenbach Managing Editor: Andrea N. Sherman, MS © Copyright 2010 American Geriatrics Society Slide 42 Topic Slide 42