Dsm-5: Neurocognitive Disorders

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Characteristics of Neurocognitive Disorders (NCDs)

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- Primary clinical deficit: cognitive function, and that are acquired rather than developmental

- Impaired cognition has not been present since birth or very early life. Thus represents a decline from a previously attained level of functioning.

- Unique in that syndromes' underlying pathology and etiology can potentially be determined.

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Cognitive Domains

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1. Complex attention (sustained attention, divided attention, selective attention, processing speed)

2. Executive function (planning, decision making, working memory, responding to feedback/error correction, overriding habits/inhibition, mental flexibility)

3. Learning and memory (immediate memory, recent memory [including free recall, cued recall, and recognition memory], very-long-term memory [semantic; autobiographical], implicit learning)

4. Language (expressive language [including naming, word finding, fluency, and grammar, and syntax] and receptive language)

5. Perceptual-motor (includes abilities subsumed under the terms visual perception, visuoconstructional, perceptual-motor, praxis, and gnosis)

6. Social cognition (recognition of emotions, theory of mind)

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Delirium

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A. Disturbance in attention (i.e., reduced ability to direct, focus, sustain, and shift attention) and awareness (reduced orientation to the environment).

B. Develops over a short period of time (usually hours to a few days), represents a change from baseline attention and awareness, and tends to fluctuate in severity during the course of a day.

C. An additional disturbance in cognition (e.g., memory deficit, disorientation, language, visuospatial ability, or perception).

D. Criteria A and C not better explained by another preexisting, established, or evolving neurocognitive disorder and do not occur in the context of a severely reduced level of arousal, such as coma.

E. There is evidence from the history, physical examination, or laboratory findings that the disturbance is a direct physiological consequence of another medical condition, substance intoxication or withdrawal (i.e., due to a drug of abuse or to a medication), or exposure to a toxin, or is due to multiple etiologies

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Delirium (Specifiers)

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- Substance intoxication delirium

- Substance withdrawal delirium

- Medication-induced delirium

- Delirium due to another medical condition/multiple etiologies

- Acute; Persistent

- Hyperactive; Hypoactive; Mixed level of activity

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Delirium (Essential Feature)

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A disturbance of attention or awareness that is accompanied by a change in baseline cognition that cannot be better explained by a preexisting or evolving neurocognitive disorder (NCD)

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Major Neurocognitive Disorder

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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) 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 independence in everyday activities (i.e., at a minimum, requiring assistance with complex instrumental activities of daily living such as paying bills or managing medications).

The cognitive deficits do not occur exclusively in the context of a delirium.

The cognitive deficits are not better explained by another mental disorder (e.g., major depressive disorder, schizophrenia).

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Major/Minor NCD (Specifiers)

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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 etiologies; Unspecified

With/Without behavioral disturbance

Severity (Mild to Severe): based on difficulties with activity of daily living (i.e., Severe: fully dependent) <-- Severity just for Major NCD

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Mild Neurocognitive Disorder

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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) 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 capacity for independence in everyday activities (i.e., complex instrumental activities of daily living such as paying bills or managing medications are preserved, but greater effort, compensatory strategies, or accommodation may be required).

The cognitive deficits do not occur exclusively in the context of a delirium.

The cognitive deficits are not better explained by another mental disorder (e.g., major depressive disorder, schizophrenia).

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Major/Mild NCDs (Diagnostic Features)

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Core feature: acquired cognitive decline in one or more cognitive domains (Criterion A) based on both 1) a concern about cognition on the part of the individual, a knowledgeable informant, or the clinician, and 2) performance on an objective assessment that falls below the expected level or that has been observed to decline over time.

Neuropsychological testing, educational attainment, and cultural background, is part of the standard evaluation of NCDs and is particularly critical in the evaluation of mild NCD. For major NCD, performance is typically 2 or more standard deviations below appropriate norms (3rd percentile or below). For mild NCD, performance typically lies in the 1–2 standard deviation range (between the 3rd and 16th percentiles).

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Major/Mild NCDs due to Alzheimer's Disease

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The criteria are met for major or mild neurocognitive disorder.

There is insidious onset and gradual progression of impairment in one or more cognitive domains (for major neurocognitive disorder, at least two domains must be impaired).

Criteria are met for either probable or possible Alzheimer’s disease as follows:

For major neurocognitive disorder:

Probable Alzheimer’s disease is diagnosed if either of the following is present; otherwise, possible Alzheimer’s disease should be diagnosed.

Evidence of a causative Alzheimer’s disease genetic mutation from family history or genetic testing.

All three of the following are present:

Clear evidence of decline in memory and learning and at least one other cognitive domain (based on detailed history or serial neuropsychological testing).

Steadily progressive, gradual decline in cognition, without extended plateaus.

No evidence of mixed etiology (i.e., absence of other neurodegenerative or cerebrovascular disease, or another neurological, mental, or systemic disease or condition likely contributing to cognitive decline).

For mild neurocognitive disorder:

Probable Alzheimer’s disease is diagnosed if there is evidence of a causative Alzheimer’s disease genetic mutation from either genetic testing or family history.

Possible Alzheimer’s disease is diagnosed if there is no evidence of a causative Alzheimer’s disease genetic mutation from either genetic testing or family history, and all three of the following are present:

Clear evidence of decline in memory and learning.

Steadily progressive, gradual decline in cognition, without extended plateaus.

No evidence of mixed etiology (i.e., absence of other neurodegenerative or cerebrovascular disease, or another neurological or systemic disease or condition likely contributing to cognitive decline).

The disturbance is not better explained by cerebrovascular disease, another neurodegenerative disease, the effects of a substance, or another mental, neurological, or systemic disorder.

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Major/Mild NCDs due to Alzheimer's Disease

(Core Features)

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(Besides Criteria A): an insidious onset and gradual progression of cognitive and behavioral symptoms (Crit. B)

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Major or Mild Vascular Neurocognitive Disorder

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The criteria are met for major or mild neurocognitive disorder.

The clinical features are consistent with a vascular etiology, as suggested by either of the following:

Onset of the cognitive deficits is temporally related to one or more cerebrovascular events.

Evidence for decline is prominent in complex attention (including processing speed) and frontal-executive function.

There is evidence of the presence of cerebrovascular disease from history, physical examination, and/or neuroimaging considered sufficient to account for the neurocognitive deficits.

The symptoms are not better explained by another brain disease or systemic disorder.

Probable vascular neurocognitive disorder is diagnosed if one of the following is present; otherwise possible vascular neurocognitive disorder should be diagnosed:

Clinical criteria are supported by neuroimaging evidence of significant parenchymal injury attributed to cerebrovascular disease (neuroimaging-supported).

The neurocognitive syndrome is temporally related to one or more documented cerebrovascular events.

Both clinical and genetic (e.g., cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) evidence of cerebrovascular disease is present.

Possible vascular neurocognitive disorder is diagnosed if the clinical criteria are met but neuroimaging is not available and the temporal relationship of the neurocognitive syndrome with one or more cerebrovascular events is not established.

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Major or Mild Vascular Neurocognitive Disorder (Diagnostic Features)

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The diagnosis of major or mild vascular neurocognitive disorder (NCD) requires the establishment of an NCD (Criterion A) and the determination that cerebrovascular disease is the dominant if not exclusive pathology that accounts for the cognitive deficits (Criteria B and C). Vascular etiology may range from large vessel stroke to microvascular disease; the presentation is therefore very heterogeneous, stemming from the types of vascular lesions and their extent and location(Román et al. 1993). The lesions may be focal, multifocal, or diffuse and occur in various

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Major or Mild Neurocognitive Disorder Due to Traumatic Brain Injury

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The criteria are met for major or mild neurocognitive disorder.

There is evidence of a traumatic brain injury—that is, an impact to the head or other mechanisms of rapid movement or displacement of the brain within the skull, with one or more of the following:

Loss of consciousness.

Posttraumatic amnesia.

Disorientation and confusion.

Neurological signs (e.g., neuroimaging demonstrating injury; a new onset of seizures; a marked worsening of a preexisting seizure disorder; visual field cuts; anosmia; hemiparesis).

The neurocognitive disorder presents immediately after the occurrence of the traumatic brain injury or immediately after recovery of consciousness and persists past the acute post-injury period.

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Major or Mild Neurocognitive Disorder Due to Traumatic Brain Injury (Diagnostic Features)

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Major or mild NCD due to traumatic brain injury (TBI) is caused by an impact to the head, or other mechanisms of rapid movement or displacement of the brain within the skull, as can happen with blast injuries(Ropper and Gorson 2007). Traumatic brain injury is defined as brain trauma with specific characteristics that include at least one of the following: loss of consciousness, posttraumatic amnesia, disorientation and confusion, or, in more severe cases, neurological signs (e.g., positive neuroimaging, a new onset of seizures or a marked worsening of a preexisting seizure disorder, visual field cuts, anosmia, hemiparesis) (Criterion B).

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Major or Mild Neurocognitive Disorder Due to Traumatic Brain Injury (Associated Features)

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may be accompanied by disturbances in emotional function (e.g., irritability, easy frustration, tension and anxiety, affective lability); personality changes (e.g., disinhibition, apathy, suspiciousness, aggression); physical disturbances (e.g., headache, fatigue, sleep disorders, vertigo or dizziness, tinnitus or hyperacusis, photosensitivity, anosmia, reduced tolerance to psychotropic medications); and, particularly in more severe TBI, neurological symptoms and signs (e.g., seizures, hemiparesis, visual disturbances, cranial nerve deficits) and evidence of orthopedic injuries.