Dementia Case File
Eugene C. Toy MD, Donald Briscoe, MD, FA AFP, Bruce Britton, MD, Joel J. Heidelbaugh, MD, FA AFP, FACG
Case 32
An 83-year-old woman is brought to the clinic by her husband who was concerned with his wife's memory problems. He first noticed some memory decline a few years ago, but the onset was subtle and did not interfere with her day-to-day activities. Mainly, she has some difficulty remembering details, is repeating things, and is being forgetful. The patient's family noticed her gradually increasing memory problems, particularly over the past year. She is unable to remember her appointments and relies heavily on written notes and appointment books. Recently, she got lost while driving and was found by her family 10 hours later. She was unable to use her cell phone and was unsure about her home address and phone number. She has also become more reclusive. She does not enjoy her church activities anymore and prefers to stay at home most of the time. She does not want to cook, and she is less attentive to her housework. The patient says that she has always been forgetful. Her medical history is significant for well-controlled hypertension and a history of mastectomy secondary to breast cancer diagnosed 20 years ago. She has no significant history of tobacco or alcohol use. She is independent with all activities of daily living, but needs assistance with medication administration, banking, and transportation. She is up-to-date with her health maintenance and immunization. Her vital signs and general physical examination are normal.
⯈ What is the most likely diagnosis?
⯈ What office testing can help to determine a diagnosis?
⯈ What laboratory testing and imaging studies are indicated at this time?
ANSWER TO CASE 32:
Dementia
Summary: An 83-year-old woman is noted by her family to have increasing memory difficulties at home. She is forgetful, repeats questions, and does not remember conversations. She had the very significant episode of getting lost in her home town. She is seemingly unaware that there is a problem that is slowly and progressively worsening.
- Most likely diagnosis: Dementia of Alzheimer type.
- Office-based testing that may be beneficial: Folstein Mini Mental Status Examination (MMSE) is the most widely used instrument. Others available include the Clock Test, the Short Portable Mental Status Questionnaire, the Mini-Cog Test, and the Montreal Cognitive Assessment (MoCa). In addition, a screening test for depression should be performed.
- Laboratory testing and imaging studies: Blood count, electrolytes, glucose, calcium, liver function tests, folate, vitamin B12, thyroid-stimulating hormone (TSH), and erythrocyte sedimentation rate. Consider syphilis screening if there is a risk factor or evidence of prior infection, or if patient lives in an area of high incidence. Non contrast head computed tomography (CT) scan or magnetic resonance imaging (MRI).
ANALYSIS
Objectives
- Develop a differential diagnosis for dementia.
- Learn how to appropriately evaluate a complaint of memory loss.
- Learn about treatment of Alzheimer dementia, the most common specific diagnosis of dementia.
Considerations
This 83-year-old woman is noted by her family to have progressive decrease in cognitive function. She is forgetful, gets lost easily, and this has been slowly but steadily worsening. The most likely diagnosis is dementia; however, other conditions should be considered in the differential diagnosis such as medications, stroke, thyroid disorders, chronic syphilis, or other metabolic conditions. Depression can also present as dementia at times. The workup for this patient includes a careful history and physical examination, imaging of the brain, and selective laboratory tests such as TSH, vitamin B12 level, complete blood count (CBC), and comprehensive metabolic panel. Screening for syphilis should also be considered.
Approach To:
Dementia
DEFINITIONS
EXECUTIVE FUNCTIONS: High-level cognitive abilities that control other, more basic, abilities. Executive functions include the ability to start and stop behaviors, alter behaviors to fit circumstances, and adapt behaviors to new situations.
CLINICAL APPROACH
The essential features of the diagnosis of dementia are memory loss and impairment of executive function. Dementia is a clinical diagnosis that can go unrecognized until it is in an advanced stage. Patients rarely report memory loss; the informants are usually their family members. However, relatives may fail to recognize signs and symptoms of dementia because many have a tendency to think that memory loss can be a part of normal aging. Studies of aging have shown that nonverbal creative thinking and new problem-solving strategies may decline with age, but information, skills learned with experience, and memory retention remain intact.
Clinicians should assess cognitive function whenever cognitive impairment or deterioration is suspected. These concerns may be based on direct observation, patient report, or concerns raised by family members, friends, or caretakers. Patients with dementia may have difficulty with one or more of the following:
- Learning and retaining new information (rely on lists, calendars)
- Handling complex tasks (banking, bills, payments)
- Reasoning (adapting to unexpected situations, unfamiliar environment)
- Spatial ability and orientation (getting lost driving, walking)
- Language (word finding, repetition, confabulation)
- Behavior (agitation, confusion, paranoia)
The evaluation of a patient with suspected dementia should include a mental status examination. The Folstein MMSE is the most widely used tool in the screening for dementia. The sensitivity of the MMSE for dementia is as high as 87% and the specificity is as high as 82%. The interpretation of the score depends on the patient's education level. It is most accurate in those with at least a high school education.
Another valuable test that can be used in a busy primary care setting is the Clock Test. The patient is asked to draw a clock with a specific time. The patient must then accurately draw the clock face with the " big hand" and "small hand" in the correct positions. It is quick, easy to administer, and evaluates executive function in multiple cognitive domains. Other brief cognitive screening tests, such as the Short Portable Mental Status Questionnaire, modified MMSE, MoCA, and Mini-Cog (three-item recall combined with clock drawing) can be used in the primary care setting.
In the evaluation of dementia, it is necessary to get information from people who know the patient well. Useful information can be obtained from informantbased functional tests, such as the functional activities questionnaire (FAQ), the instrumental activities of daily living (IADL), and caregiver burden assessments. This information can be important for physicians and families in making plans for long-term care. See Case 18 (Geriatric Health Maintenance) for more on functional assessment.
ALZHEIMER DISEASE
Alzheimer disease is the most common cause of dementia. Although a definitive diagnosis can only be made by the presence of neuritic plaques and neurofibrillary tangles detected on autopsy, clinical diagnostic criteria have been developed (Table 32-1). Common diagnostic criteria include the gradual onset and progression of cognitive dysfunction in more than one area of mental functioning that is not caused by another disorder.
The initial evaluation includes a detailed history, from both the patient and another informant (usually a spouse, child, or other close contact) and complete physical and neurologic examinations to evaluate for any focal neurologic deficit that may be suggestive of a focal neurologic lesion. A validated test, such as the MMSE, should be used to confirm the presence of dementia. The results of this test can also be used to follow the clinical course, as a reduction in score over time is consistent with worsening dementia.
A focused evaluation to rule out other causes of dementia must be performed as well. The physical examination should focus on neurologic deficits consistent with prior strokes, signs of Parkinson disease (eg, cogwheel rigidity and/or tremors), gait abnormalities or slowing, and eye movements. Patients with Alzheimer disease generally have no motor deficits at presentation.
Depression in the elderly can present with symptoms of memory disturbance. This is known as "pseudodementia:' As depression is common and treatable, a screening test for depression should be performed when dementia is evaluated. Similarly, hypothyroidism and vitamin B12 deficiency are common and treatable conditions that can cause cognitive problems. TSH and vitamin B12 levels should be performed as a routine part of the workup. Neurosyphilis could present in this fashion, but is such an uncommon diagnosis that routine screening would not be recommended. Evaluation for neurosyphilis would be warranted if there were identified highrisk factors, history of the disease, or if the patient lived in an area with a high
prevalence of syphilis. Neuroimaging with either a noncontrast CT scan or an MRI of the brain is recommended to rule out other confounding diagnoses. Other testing, such as positron emission tomography (PET), genetic testing, and spinal fluid analysis are not routinely recommended. Referral to neurology is appropriate when diagnosis is uncertain.
When the diagnosis of Alzheimer disease is made, a comprehensive care plan should be initiated. The management of Alzheimer disease must be directed both at the patient and at the patient's family or caregivers. The goals of therapy are to maximize the cognition, delay functional decline, and prevent or improve the behavioral disturbances.
Table 32-2 lists the medications that are primarily used in the treatment of Alzheimer disease. Family members should understand that the medications may delay the progression of the disease but may not reverse any decline that has already occurred. For that reason, the medications may be more beneficial if started earlier in the course of the disease.
Antipsychotic medications have also been used to control hallucinations and agitation in patients with Alzheimer disease. However, this is an "off-label" use of medication and data show a higher death rate associated with the use of the newer antipsychotics. The Food and Drug Administration (FDA) has placed a black box warning against the use of typical and atypical antipsychotic medications for dementia-related psychosis due to the increased risk of deaths. Herbal medications such as Ginkgo biloba and huperzine A have inconsistent evidence for efficacy, but appear to be safe alternatives. These should not be used with prescription medications due to potential interactions.
Behavioral interventions also may be beneficial. These can include scheduled toileting in an effort to reduce episodes of incontinence, writing reminder notes, keeping familiar objects around, providing adequate lighting, and making duplicates of important objects (eg, keys) in case they get lost. Caregivers also need support and may benefit from appropriate training, support groups, and periodic respite care.
Unfortunately, even with the best of care, Alzheimer disease is relentless and progressive. Families may have significant difficulties and conflicts regarding issues surrounding end-of-life care and placement in assisted living or nursing homes. Resources such as local chapters of the Alzheimer Association ( www.alz.org) may provide valuable services, information, and support.
VASCULAR DEMENTIA
Vascular dementia, or multi-infarct dementia, is the second most common cause of dementia. In vascular dementia, there is neuronal loss as a consequence of one or more strokes. The symptoms are related to the amount and location of the neuronal loss. Vascular dementia can exist along with Alzheimer disease or other causes of dementia, resulting in a mixed-dementia syndrome. Unlike Alzheimer disease, which is a gradually progressive process, vascular dementia often has a sudden onset and progresses in a stepwise fashion. Patients tend to function at a certain level and then show an acute deterioration when the initial, or subsequent, infarcts occur. The risk factors include those for cerebrovascular disease (hypertension, tobacco use, diabetes, etc). There are no controlled trials showing medication effectiveness in vascular dementia, so the treatment is aimed at reducing the risk of further neurologic damage.
LEWY BODY DEMENTIA
Lewy body dementia is the third most common form of dementia. This dementia presents early on with vivid hallucinations, fluctuation in cognition, and often parkinsonian extrapyramidal signs and postural instability. Tremor is less apparent and levodopa is not very effective in these patients. Daytime drowsiness and sleeping, staring into space for prolonged periods of time, and episodes of disorganized speech can further distinguish Lewy body dementia from Alzheimer disease. Therapies are similar as those for Alzheimer disease.
FRONTOTEMPORAL LOBE DEMENTIA
Frontotemporal lobe dementia is the fourth most common form of dementia and due to the behavioral disturbances associated with this, dementia can be very distressing for the patient's family. In this form of dementia, patient's personalities can significantly change, becoming antisocial or disinhibited from social norms with poor impulse control. Patients can develop apathy, emotional blunting, and perseveration behaviors including echolalia, and stereotypical behaviors such as toe tapping and repetitive motor activity. There are little pharmacologic therapies with significant evidence for efficacy. Counseling and support of the family can mitigate the stress of caring for these patients.
OTHER ILLNESSES ASSOCIATED WITH DEMENTIA
Numerous other conditions may present with dementia or have dementia as a prominent symptom. Parkinson disease commonly has an associated dementia, especially as the overall disease advances. Huntington disease is an autosomal dominant disorder that presents with progressive dementia, depression, and choreiform movements. Dementia can be a complication of chronic alcohol abuse, reinforcing the need for a complete history of substance use. Potentially reversible forms of dementia include normal pressure hydrocephalus (the triad of dementia, gait disturbance, and urinary incontinence), chronic subdural hematoma, and depression. Many prescription and over-the-counter medications can cause memory disturbances. Chief among these are anticholinergic medications, sedatives (benzodiazepines), sleeping pills, and narcotic pain medications. As noted previously, hypothyroidism, vitamin B12 deficiency, and neurosyphilis may present as dementing illnesses. Metabolic abnormalities, such as hyponatremia or abnormal calcium levels, and other infections, such as AIDS, can also cause dementia.
DELIRIUM
Delirium is an acute change in mental status that is characterized by fluctuations in levels of consciousness. It is usually caused by an acute medical illness, the use of a medication, or the withdrawal from a drug or alcohol. Delirium affects 10% to 30% of hospitalized patients, with a higher incidence in the elderly, in those with an underlying dementia, and in those with multiple underlying medical conditions. The treatment of delirium is treatment of the condition that precipitated it. Delirium is often reversible if the underlying cause can be found and aggressively managed. Patients with delirium have significantly longer hospital stays and increased mortality rates.
CASE CORRELATION
- See Case 25 (Major Depression).
COMPREHENSION QUESTIONS
32.1 A 63-year-old man is brought in by his family because of memory loss. They have noted a worsening of his symptoms over several months. They also report that he has had multiple falls, hitting his head on one occasion, and has had frequent urinary incontinence. On examination, a gait apraxia is noted. Which of the following is the most likely diagnosis?
B. Normal pressure hydrocephalus
C. Dementia with Lewy bodies
D. Delirium
32.2 An 82-year-old woman is admitted to the hospital for altered mental status. Her family says that she has been confused and falling asleep frequently and that she has been hallucinating-talking to people who are not in the room. They report that prior to this illness, she was independent and "sharp as a tack:' On urine analysis, she is found to have a urinary tract infection (UTI). Which of the following is the most appropriate treatment?
B. Start an alerting agent such as modafinil (Provigil) for symptomatic treatment of her hypersomnia.
C. Start an antibiotic for treatment of her infection and optimize management of any other medical conditions.
D. Give her a dose of ziprasidone (Geodon) for her hallucinations.
32.3 A 77 -year-old man is brought to your office by his wife, who states that he has been having mental difficulties in recent months, such as not being able to balance their checkbook or plan for his annual visit with the accountant. She also tells you that he has reported seeing animals in the room with him that he can describe vividly. He takes frequent naps and stares blankly for long periods of time. He seems almost normal at times, but randomly appears very confused at other times. He has also been dreaming a lot and has fallen down more than once recently. He currently takes aspirin, 81 mg/d. On examination, the patient walks slowly with a stooped posture and almost falls when turning around. He has only minimal facial expressiveness. No tremor is noted and the remainder of the examination is normal. He is able to recall three words out of three, but clock drawing is abnormal. Laboratory studies are normal and a CT of the brain shows changes of aging. What type of dementia does this patient most likely have?
B. Alzheimer disease
C. Frontotemporal dementia
D. Vascular dementia
E. Dementia of Parkinson disease
32.4 A 66-year-old woman is brought in by her family because of difficulty with memory and disorientation that has worsened over the past 6 months. A careful history and physical examination is performed. Which of the following tests is most appropriate in this patient?
A. Head CT or MRI
B. Lumbar puncture
C. Rapid plasma reagin (RPR)
D. Electroencephalogram (EEG)
ANSWERS
32.1 B. Normal pressure hydrocephalus classically causes dementia, incontinence, and gait disturbance. All of the other listed conditions may cause memory disturbance, but the constellation of these three symptoms is most consistent with normal pressure hydrocephalus.
32.2 C. This scenario is one that is commonly seen in elderly patients and is consistent with delirium. The patient is elderly and has an infection, causing both an acute change in her mental status and a fluctuating level of consciousness. The treatment is to treat the underlying infection and any associated medical conditions.
32.3 A. This patient has dementia with Lewy bodies, which is the third most common type after Alzheimer disease and vascular dementia. He demonstrates typical signs and symptoms, including well-formed hallucinations, vivid dreams, fluctuating cognition, sleep disorder with periods of daytime sleeping, frequent falls, deficits in visuospatial ability (abnormal clock drawing), and rapid eye movement (REM) sleep disorder (vivid dreams). In Alzheimer disease, the predominant early symptom is memory impairment without the other symptoms found in this patient. In dementia of Parkinson disease, extrapyramidal symptoms such as tremor, bradykinesia, and rigidity precede the onset of memory impairment by more than 1 year. Frontotemporal dementia presents with behavioral changes, including disinhibition, or language problems such as aphasia.
32.4 A. A noncontrast head CT or MRI is recommended by the American Academy of Neurology for the routine evaluation of dementia. All of the other tests may be appropriate if there is a finding on the history or examination that calls for further testing (an exposure to syphilis, episodes suggestive of seizures, or symptoms of normal pressure hydrocephalus for which a spinal tap may be performed).
CLINICAL PEARLS
⯈ The presentation of acutely altered mental status (delirium) should prompt an aggressive workup for an underlying cause, as treatment may result in correction of the mental status.
⯈ Alzheimer disease is a disease of the family, not just the individual. It is critical to treat the patient while giving support to the caregivers.
REFERENCES
Alzheimer's Association website: www.alz.org. Accessed November 7, 2015.
American Geriatric Society website: www.americangeriatrics.org. Accessed November 7, 2015.
Cardarelli R, Kertesz A, Knebl JA. Frontotemporal dementia: a review for primary care physicians. Am Fam Physician. 2010 Dec 1;82(11):1372-1377.
Neef D, Walling AD. Dementia with Lewy bodies: an emerging disease. Am Fam Physician. 2006:73(7):1223-1230.
Seeley WW, Miller BL. Dementia. In: Kasper D, Fauci A, Hauser S, et al., eds. Harrison's Principles of Internal Medicine. 19th ed. New York, NY : McGraw-Hill; 2015. Available at: http:// accessmedicine. mhmedical.com. Accessed May 25, 2015.
Simmons BB, Hartmann B, Dejoseph D. Evaluation of suspected dementia. Am Fam Physician. 2011 Oct 15:84(8):895-902.
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