Neurocognitive Disorders (including Dementia)

Diagnoses of different types of “neurocognitive disorders” include:

• Delirium
• Dementia (Major Cognitive Disorder)
• Mild Neurocognitive Disorder
• Neurocognitive Disorder due to Alzheimer’s Disease
• Frontotemporal Neurocognitive Disorder
• Neurocognitive Disorder with Lewy Bodies
• Vascular Neurocognitive Disorder
• Neurocognitive Disorder due to Traumatic Brain Injury
• Substance/Medication-Induced Neurocognitive Disorder
• Neurocognitive Disorder due to HIV Infection
• Neurocognitive Disorder due to Prion Disease
• Neurocognitive Disorder due to Huntington’s Disease
• Amnestic Disorder (Amnesia)
• Neurocognitive Disorder due to Another Medical Condition (e.g., multiple sclerosis, cerebrovascular diseases, chronic alcoholism)
• Neurocognitive Disorder due to Multiple Causes
• Unspecified Neurocognitive Disorder

Most often occurring in elderly adults, delirium is an impairment of consciousness, marked by a disturbance in attention and awareness. A life-threatening yet potentially reversible disorder of the central nervous system (CNS), delirium often involves perceptual disturbances, abnormal psychomotor activity, ad sleep cycle impairment. The condition develops over a short period of time (usually hours to a few days)and tends to fluctuate in severity during the course of a day. Cognition is also impaired, which can include memory problems, disorientation, language difficulties, or impairment of visuospatial ability and perception. The symptoms are not better explained by another preexisting or evolving neurocognitive disorder. Major causes of delirium include CNS disease (e.g., epilepsy), systemic disease (e.g., cardiac failure), and either intoxication or withdrawal from pharmacological or toxic agents.

 Substance intoxication delirium may be caused by alcohol, cannabis, phencyclidine, opioids, inhalants, sedatives, hypnotics, anxiolytic drugs, amphetamines, cocaine, or other substances.

A diagnosis of “major neurocognitive disorder”, also known as dementia, is given when there is evidence of significant cognitive decline from a previous level of performance in one or more cognitive domains: complex attention, executive function, learning and memory, language, perceptual-motor, or social cognition. The diagnosis is based on:

• Concern of the individual, a knowledgeable informant, or the clinician that there has been a significant decline in cognitive function; and
• A substantial impairment in cognitive performance, preferably documented by standardized neuropsychological testing, or in its absence, another quantified clinical assessment.

The cognitive deficits interfere with the individual’s independence in everyday activities, at a minimum, requiring assistance with activities of daily living such as paying bills or managing medications. The cognitive impairment does not occur exclusively in the context of delirium and is not better explained by another mental disorder (e.g., major depressive disorder, schizophrenia).

Major neurocognitive disorder may be due to:

• Alzheimer’s disease
• Frontotemporal lobar degeneration
• Lewy body disease
• Vascular disease
• Traumatic brain injury
• Substance/medication use
• HIV infection
• Prion Disease
• Parkinson’s disease
• Huntington’s disease
• Another medical condition
• Multiple medical conditions
• An unspecified cause

Symptoms of major neurocognitive disorder may occur in any of the following cognitive domains.

Complex Attention
This category applies to attention and processing speed. Examples:

• Has increased difficulty in environments with multiple stimuli (TV, radio, conversation)
• Is easily distracted by competing events in the environment.
• Has difficulty holding new information in mind, such as recalling phone numbers or addressed just given, or reporting what was just said.
• Is unable to perform mental calculations
• All thinking takes longer than usual, and components to be processed must be simplified to one or a few.

Executive Function
This category applies to decision making, working memory, responding to feedback/error correction, overriding habits/inhibition, mental flexibility. Examples:

• Abandons complex projects.
• Needs to focus on one task at a time.
• Needs to rely on others to plan activities of daily living or to make decisions.

Learning and Memory
This category applies to immediate and recent memory (including free recall, cued recall, and recognition memory), very long-term memory, and implicit learning. Examples:

• Repeats self in conversation, often within the same conversation.
• Cannot keep track of short list of items when shopping, or of plans for the day.
• Requires frequent reminders to orient to the task at hand.

Language
This category applies to using language, including naming, word finding, fluency, grammar and syntax, and comprehension. Examples:

• Has significant difficulties with speaking or comprehension.
• Often uses general use terms such as “that thing” and “you know what I mean” and prefers general pronouns rather than names.
• With severe impairment, many of recall names of closer friends and family.
• Idiosyncratic word usage, grammatical errors, and short utterances occur.
• Stereotype of speech occurs, echolalia (repetition of words and noises) and automatic speech typically precede mutism.

Perceptual-Motor
This category applies to visual perception and recognition, hand-eye coordination, and carrying out learned movements. Examples:

• Has significant difficulties with previously familiar activities (using tools, driving motor vehicle), navigating in familiar environments.
• Is often more confused at dusk, when shadows and lowering levels of light change perceptions.

Social Cognition
This category applies to recognition of emotions, social behavior and sensitivity toward others. Examples:

• Behavior is clearly out of acceptable social range.
• Shows insensitivity to social standards of modesty in dress, or of political, religious, or sexual topics of conversation.
• Focuses exclusively on a topic despite group’s disinterest or direct feedback.
• Makes decisions without regard to safety (e.g., inappropriate clothing for weather or social setting).

There are a number of psychiatric and neurological changes that can occur with major neurocognitive disorder (dementia). These include:

• Changes in personality. Preexisting personality traits may be accentuated during the development of dementia. Patients with dementia may become introverted, have paranoid delusions, or be generally hostile to family members and caretakers. Patients with frontal and temporal involvement are likely to have marked personality changes and may be irritable and explosive.
• Hallucinations and delusions. An estimated 20 to 30 percent of patients with dementia (primarily those with Alzheimer’s type) have hallucinations, and 30 to 40 percent have delusions, primarily of a paranoid nature. Physical aggression is common in demented patients who also have psychotic symptoms.
• Mood disorders. Depression and anxiety affect 40 to 50 percent of patients with dementia, although the full syndrome of depressive disorder may be present in only 10 to 20 percent. Patients with dementia also may exhibit pathological laughter or crying with no apparent provocation.
• Neurological Issues. Seizures occur in approximately 10 percent of patients with Alzheimer’s type of dementia and 20 percent of patients with vascular dementia. Patients with vascular dementia may experience headaches, dizziness, faintness, weakness, and sleep disturbances.
• Sundowner syndrome. This condition is characterized by drowsiness, confusion, impaired balance and coordination, and accidental falls.

“Mild neurocognitive disorder” is the term for individuals who fall between the cognitive changes of aging and early dementia.

A diagnosis of mild neurocognitive disorder is given when there is evidence of modest cognitive decline from a previous level of performance in one or more cognitive domains: complex attention, executive function, learning and memory, language, perceptual-motor, or social cognition. The diagnosis is based on:

• Concern of the individual, a knowledgeable informant, or the clinician that there has been a mild decline in cognitive function; and
• A modest impairment in cognitive performance, preferably documented by standardized neuropsychological testing, or in its absence, another quantified clinical assessment.

The cognitive deficits do not interfere with the individual’s independence in everyday activities, such as paying bills or managing medications, but greater effort, compensatory strategies, or accommodation may be required. The cognitive impairment does not occur exclusively in the context of delirium and is not better explained by another mental disorder (e.g., major depressive disorder, schizophrenia). Some minor impairment in memory may accompany normal aging.

Mild neurocognitive disorder may be due to:

• Alzheimer’s disease
• Frontotemporal lobar degeneration
• Lewy body disease
• Vascular disease
• Traumatic brain injury
• Substance/medication use
• HIV infection
• Prion Disease
• Parkinson’s disease
• Huntington’s disease
• Another medical condition
• Multiple medical conditions
• An unspecified cause

Symptoms of mild neurocognitive disorder may occur in any of the following cognitive domains.

Complex Attention
This category applies to attention and processing speed. Examples:

• Normal tasks take longer than previously.
• Begins to find errors in routine tasks; finds work needs more double-checking than previously.
• Thinking is easier when not competing with other things (radio, TV, other conversations, cell phone, driving).

Executive Function
This category applies to decision making, working memory, responding to feedback/error correction, overriding habits/inhibition, mental flexibility. Examples:

• Increase effort is required to complete multistage projects.
• Has increased difficulty multitasking or difficulty resuming a task interrupted by a visitor or phone call.
• May complain of increased fatigue from the extra effort required to organize, plan, and make decisions.
• May report that large social gatherings are more taxing or less enjoyable due to increased effort required to follow shifting conversations.

Learning and Memory
This category applies to immediate and recent memory (including free recall, cued recall, and recognition memory), very long-term memory, and implicit learning. Examples:

• Has difficulty recalling recent eventsand relies increasingly on list-making or a calendar.
• Needs occasional reminders or re-reading to keep track of characters in a movie or novel.
• Occasionally may repeat self over a few weeks to the same person.
• Loses track of whether bills have already been paid.

Language
This category applies to using language, including naming, word finding, fluency, grammar and syntax, and comprehension. Examples:

• Has noticeable word-finding difficulty.
• May substitute general for specific terms.
• May avoid use of specific names of acquaintances.
• Grammatic errors involve subtle omission or incorrect use of articles, preposition, auxiliary verbs, etc.

Perceptual-Motor
This category applies to visual perception and recognition, hand-eye coordination, and carrying out learned movements. Examples:

• May need to rely more on maps or others for directions.
• Uses notes and follows others to get to a new place.
• May find self lost or turned around when not concentrating on task.
• Is less precise in parking.
• Needs to expend greater effort for spatial tasks such as carpentry, assembly, sewing, or knitting.

Social Cognition
This category applies to recognition of emotions, social behavior and sensitivity toward others. Examples:

• Has subtle changes in behavior or attitude, often described as a change in personality, such as less ability to recognize social cues or read facial expressions.
• May demonstrated decreased empathy, increased extraversion or introversion, decreased inhibition, or subtle or episodic apathy or restlessness.

Some studies have indicated that as many as 40 percent of Alzheimer’s patients have a family history of Alzheimer’s type of dementia; therefore, genetic factors are presumed to play a part in the development of the disorder, at least in some cases. Alzheimer’s type dementia has shown linkage to chromosomes 1, 14, and 21.

“Pseudodementia” occurs in depressed people who complain of impaired memory but, in fact, have a depressive disorder. When the depression is treated, the cognitive defects disappear.

Pseudodementia typically involves three cognitive components: memory issues, deficits in executive functioning, and deficits in speech and language. Specific cognitive symptoms might include trouble recalling words or remembering things in general, decreased attention and concentration, difficulty completing tasks or making decisions, decreased speed and fluency of speech, and impaired processing speed.

Most types of dementia cannot be cured, but there are ways to manage symptoms and to slow down the progression of dementia.

Pharmacotherapy
Doneprezil (Aricept), rivastigmine (Excelon), and galantamine (Reminyl) are cholinesterase inhibitors used to treat mild to moderate cognitive impairment in Alzheimer’s disease. Memantine (Namenda)protects neurons from excessive amounts of glutamate, which may be neurotoxic. The drug is sometimes combined with donepezil. It has been known to improve dementia.

Other drugs being tested for cognitive-enhancing activity include general cerebral metabolic enhancers, calcium channel inhibitors, and serotonergic agents. Some studies have shown that selegiline (Elderpryl), a selective type B monoamine oxidase inhibitor, may slow the advance of dementia.

Estrogen replacement therapy may reduce the risk of cognitive decline in postmenopausal women; however, more studies are needed to confirm this effect. Several epidemiological studies indicate a reduced risk of dementia in persons taking antihypertensive medications, cholesterol-lowering drugs, antioxidants, and anti-inflammatory drugs. However, no randomized controlled trials verify these data.

Psychosocial Therapies
Patients facing a progression of dementia often benefit from supportive and educational psychotherapy in which the nature and course of their illness are clearly explained. They may also benefit from assistance in grieving and accepting the extent of their disability.

Psychotherapy and counseling for family members of patients with dementia may also be of great value. A common problem that develops among caregivers involves their self-sacrifice in caring for a patient. A gradually developing resentment is often suppressed because of the feelings of guiltit produces. Therapists can help caregivers understand the complex mixture of feelings associated with seeing a loved one decline and can provide caregivers permission to express these feelings.

Preventive Lifestyle Factors
Exercise is reported to delay the onset of cognitive deterioration in general. Other factors associated with a reduced risk of cognitive impairment and dementia later in life include high education and socioeconomic status in early life, high work complexity, rich social network, social engagement, mentally stimulating activity, nonsmoking, and living with a partner during mid-life. Following a healthy diet, such as adherence to a Mediterranean diet, and intake of antioxidants and polyunsaturated fatty acids are also associated with a decreased dementia risk.

Risk factors for dementia include high blood pressure, obesity, high cholesterol, diabetes, and smoking.