The video below is the section for Neurocognitive Disorder Due to Alzheimer’s Disease from Part 2 of TSM’s lecture series on DSM-5 and the EPPP, followed by a transcript. This lecture series aims to equip those preparing for the EPPP with everything you need to know about the impact DSM-5 will be having on the EPPP. To watch all of Part 2, click here. To watch Part 1, click here. To register for our webinar series to watch future lectures and discuss your questions with a content expert, click here.
Transcript of DSM-5 EPPP Lecture Video: Neurocognitive Disorder Due to Alzheimer’s Disease
Narrative Definition: Alzheimer’s disease is an irreversible, progressive brain disease that causes neuronal dysfunction, decrease in the interconnections among neurons, and eventual death of those brain cells, leading to impairment in cognitive function. Symptoms are insidious, and develop gradually over a period of years, and typically begin with memory problems. But all domains of cognition can be affected in the course of the disease: complex attention (selective attention, processing speed), executive function (word-finding, planning, reasoning), learning and memory (immediate memory, cued recall, autobiographical memory), language (expressive and receptive), perceptual-motor abilities (visual perception, perceptual-motor), and social cognition (recognition of emotions). Thus, the DSM-5 designates the degeneration in cognitive function as a neurocognitive disorder, in place of the term previously used, dementia.
Neurocognitive disorders are classified according to etiology (e.g., degenerative, vascular, traumatic), and described according to severity. A mild neurocognitive disorder indicates decline from a previous level of performance in one or more cognitive domains, but the cognitive impairment does not interfere with capacity for independence in activities of daily living. In contrast, when cognitive impairment worsens to the point of interfering with independence in everyday activities (e.g., requiring assistance with paying bills or managing medications), it is named a major neurocognitive disorder. The severity of symptoms varies according to how far the disease has progressed, and can generally be assessed and tracked over time by neurocognitive measures, including the mental status examination.
When clinically significant cognitive impairments are presumably caused by Alzheimer’s Disease, the diagnosis is Mild (or Major) Neurocognitive Disorder Due to Alzheimer’s Disease. The clinician will indicate Probable Alzheimer’s disease (if there is evidence of causative genetic mutation from genetic testing or family history), or Possible Alzheimer’s disease (in the absence of the genetic causative factor). Diagnostic symptoms are three: (a) clear evidence of decline in memory and learning (for Mild cases; for Major cases, there must also be at lest one other cognitive domain affected); (b) steadily progressive, gradual decline in cognition, without extended plateaus; no evidence of mixed etiology; and (c) no evidence of mixed etiology. The presumption of cause in the case of Alzheimer’s Disease is because at this time there are no effective tests to prove conclusively the etiology of the cognitive decline. Thus, the diagnosis of neurocognitive disorder due to Alzheimer’s Disease is made through a process of elimination of other verifiable etiologies (e.g., vascular disease, traumatic bran injury, Parkinson’s disease). It is encouraging to know that several biomarkers and imaging techniques are being developed to help with future early diagnosis in every day medical practice.
When coding the diagnosis, the DSM-5 has clear instructions: for probable major neurocognitive disorder due to Alzheimer’s disease, code Alzheimer’s disease first, then the neurocognitive decline, specifying whether with or without behavioral disturbance. For possible major neurocognitive disorder due to Alzheimer’s disease, code the neurocognitive disorder, but do not use an additional code for Alzheimer’s disease. When diagnosing mild neurocognitive disorder due to Alzheimer’s disease, do not use an additional code for Alzheimer’s disease. For mild disorder, there is no code for behavioral disturbances specifer, but these disturbances should be indicated in writing.
Alzheimer’s Disease is the most common cause of degenerative neurocognitive function, but the disease is not well understood. The greatest known risk factor for developing Alzheimer’s Disease is increase in age. U.S. Census data estimates that approximately 7% of individuals diagnosed with Alzheimer’s Disease are between ages 65 and 74 years; 53% are between ages 75 and 84; and 40% are 85 and older. Family history and genetics are considered a risk factor, but less than 5% of the time Alzheimer’s is caused by specific genetic changes. In most cases, it appears that genetic, lifestyle, and environmental factors combine to affect the brain over time. Other risk factors include being female (in part because women live longer), history of head trauma, lack of exercise, high blood pressure, poorly controlled diabetes, lack of fruit and vegetables in diet, and a life with limited mental and social stimulation (having little or no stimulation in one’s job, limited social interaction, not engaging in mentally stimulating activities such as reading or playing a musical instrument).
The course of illness is protracted. Damage to the brain starts about a decade before problems become clinically evident, with neurocognitive decline appearing insidiously, progressing gradually, sometimes with brief plateaus, through severe dementia to death. The mean duration of survival after diagnosis is approximately 10 years, but some can live as long as 20 years with the diagnosis, depending on age of onset. Onset of symptoms is usually in the 8th or 9th decades of life, with early onset forms seen in the 5th or 6th decades of life and are often caused by genetic mutations. Differential diagnosis must be done with other causes of neurocognitive disorders, other neurological or systemic illness, and major depressive disorder. The older the individual, the more medical comorbidities may exist, which cloud the diagnosis and complicate treatment. The younger the age of onset, the more likely the individual will survive the full course of disease, with eventual death due to aspiration, after a period of being mute and bedbound.
Currently, confirmation of diagnosis is made postmortem. Brain size and densitivy is decreased due to massive death of neurons, and loss of connections among the surviving cells. Histopathological examination reveals the presence of two characteristic markers of the disease: beta-amyloid plaques and tau tangles. Plaques consist of deposits of protein fragments called beta-amyloid, which in normal brains are mostly removed, although small amounts may collect as people age. In individuals with Alzheimer’s disease there are large amounts of these relatively insoluble plaques, deposited in spaces between brain cells, and that can damage to cell membranes. Another part of the disease process affects microtubules responsible for transportation of nutrients and other cell components within the neuron. With tau proteins attached to them, normal microtubules become twisted and entangled, and unable to function properly.
In early-stage Alzheimer’s disease (i.e., within the first three years after symptom onset), the individuals demonstrate forgetfulness, a propensity for misplacing items, decreased interest in usual activities, lower levels of energy, reduced awareness of current events, diminished ability to perform complex tasks (e.g., higher-level mathematical operations, managing finances), deficits in new learning, anomia, sadness, and a detached or muted effect when placed in mentally or socially demanding environments. Alterations in, or loss of, cholinergic neurons have also been found in the early stages. Although deficits in episodic memory are most apparent in this stage, slight impairments in semantic memory may occur.
In mid-stage Alzheimer’s disease (i.e., three to ten years after symptom onset), individuals with the disorder often need help with activities of daily living (e.g., choosing weather-appropriate clothing, dressing themselves, toileting), are unable to perform simple mental arithmetic (e.g., counting backward from 20 by twos), demonstrate personality changes (e.g., may become suspicious or fearful), experience delusions or hallucinations, wander off, suffer from aphasia (e.g., forgetting common words or making unusual word substitutions), demonstrate increased restlessness, and exhibit increased mood lability, including increased irritability. It is during this stage that individuals begin to have difficulty remembering the names of significant people in their lives, although they are generally able to distinguish familiar from unfamiliar faces. Symptoms may include impulsive behaviors, in addition to hallucinations, delusions, and/or paranoia.
In late-stage Alzheimer’s disease, damage has spread throughout the brain, and brain tissue has shrunk significantly. Individuals are no longer able to meaningfully respond to their environment and are usually incapable of producing recognizable speech. Basic activities such as eating and toileting require assistance. Motor coordination is impaired to the point that they are unable to walk or sit upright without assistance; limb rigidity and flexion posture are common. The disease will eventually render the person bedridden. During late-stage Alzheimer’s disease, seizures may develop. The time of onset to death is generally about ten years, although there have been reports of death due to the disorder in as little as four years to as many as 20 years after symptom onset.
Both Alzheimer’s disease and Korsakoff’s syndrome share similar symptoms during the early stages of the disease, such as anterograde amnesia for declarative speech. Alzheimer’s disease can be differentiated symptomatically from Korsakoff’s syndrome by the presence of cognitive deficits beyond the amnesia.
Research has shown that the symptomatology for depression associated with Alzheimer’s disease is slightly different than other types of mood disorders. The first stage of depression in individuals with Alzheimer’s is marked by anomia, irritability, and anger. The second stage is characterized by paranoia and labile mood; at the third stage, the patient may exhibit apathy and emotional blunting. Most of these symptoms are similar to those needed for a Major Depressive Disorder diagnosis; it should be noted that a characteristic symptom of depression with Alzheimer’s disease is decreased positive affect or pleasure in response to social contacts and usual activities.
Alzheimer’s disease (dementia of the Alzheimer’s type)
A gradual-onset, progressive course, neurodegenerative disease, afflicting growing percentages of the population after 50 years of age.
- a. At age 65: 0.6 percent of males and 0.8 percent of females diagnosed
- b. By age 95: 36 percent of males and 41 percent of females diagnosed
Memory impairments are a hallmark, along with agnosia, apraxia, aphasia and deficits in executive functions
Stages of progression:
a. Early (within three years from onset): Marked by forgetfulness, low energy, misplaced items
b. Middle (from three up to 10 years from onset): Marked by personality changes, decreased ability to perform daily living activities, aphasia, restlessness, may wander off, difficulty remembering names of loved ones
c. Late: Person is bedridden, grave cognitive degeneration, decerebration, unable to speak meaningfully, need for total care; death may ensue eight to 10 year from onset, up to 15 years from first symptoms
Diagnoses of Alzheimer’s disease are often made through a process of elimination after testing for other types of dementia with neuropsychological batteries
Definitive diagnosis made only at autopsy, on basis of characteristic neurofibrillary tangles, amyloid plaques, and cholinergic dysfunction
a. Early stage Alzheimer’s has similar symptoms as Korsokoff’s syndrome, but Alzheimer’s will include cognitive deficits beyond amnesia.
Depression can mimic or exist comorbidly with Alzheimer’s; characteristic symptom of depression with Alzheimer’s disease is decreased positive affect or pleasure in response to social contacts and usual activities
Declarative memory more affected than procedural memory during initial phases of depression that is comorbid with Alzheimer’s
Risk factors include age, having Down Syndrome and a history of brain trauma. Women are also slightly more likely to become ill. By age 95, disease has 36 percent prevalence in males, and 45 percent in females
Treatment involves cholinergic medications, support for the family, optimizing living environment.
Gina, who has had Alzheimer’s Disease for 3 and half years, believes and feels like she has bugs crawling all over her body, is constantly restless, and is not able to teach her grandchildren basic addition problems. The symptoms Gina is experiencing is
A. Early-Stage Alzheimer’s Disease
- B. Late-Stage Alzheimer’s Disease
C. Mid-Stage Alzheimer’s Disease
D. Post-Stage Alzheimer’s Disease
Rationale: The correct answer is C. In mid-stage Alzheimer’s disease (i.e., three to ten years after symptom onset), individuals with the disorder often need help with activities of daily living (e.g., choosing weather-appropriate clothing, dressing themselves, toileting), are unable to perform simple mental arithmetic (e.g., counting backward from 20 by twos), demonstrate personality changes (e.g., may become suspicious or fearful), experience delusions or hallucinations, wander off, suffer from aphasia (e.g., forgetting common words or making unusual word substitutions), demonstrate increased restlessness, and exhibit increased mood lability, including increased irritability