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OTHER NEUROCOGNITIVE DISORDER
 

Prediction: Chronic, Progressive

      Occupational-Economic:
  • Progressive cognitive deterioration (memory, learning, executive functioning, language)
  • Irrationality (delusions, hallucinations)
      Social:
  • Eventually needs total nursing care
  • Negative emotion (anxiety, depression)
  • Antagonism (hostility)
  • Disinhibition (agitation, irresponsibility, impulsivity, dangerous risk taking)
      Medical:
    Denial of illness; genetic mutation on genetic testing

SYNOPSIS

Dementia In Other Diseases Classified Elsewhere F02 - ICD10 Description, World Health Organization
Cases of dementia due, or presumed to be due, to causes other than Alzheimer disease or cerebrovascular disease. Onset may be at any time in life, though rarely in old age.

    F02.0 Dementia in Pick disease

    A progressive dementia, commencing in middle age, characterized by early, slowly progressing changes of character and social deterioration, followed by impairment of intellect, memory, and language functions, with apathy, euphoria and, occasionally, extrapyramidal phenomena.

    F02.1 Dementia in Creutzfeldt-Jakob disease

    A progressive dementia with extensive neurological signs, due to specific neuropathological changes that are presumed to be caused by a transmissible agent. Onset is usually in middle or later life, but may be at any adult age. The course is subacute, leading to death within one to two years.

    F02.2 Dementia in Huntington disease

    A dementia occurring as part of a widespread degeneration of the brain. The disorder is transmitted by a single autosomal dominant gene. Symptoms typically emerge in the third and fourth decade. Progression is slow, leading to death usually within 10 to 15 years.

    F02.3 Dementia in Parkinson disease

    A dementia developing in the course of established Parkinson disease. No particular distinguishing clinical features have yet been demonstrated.

    F02.4 Dementia in human immunodeficiency virus [HIV] disease

    Dementia developing in the course of HIV disease, in the absence of a concurrent illness or condition other than HIV infection that could explain the clinical features.
Major or Mild Frontotemporal Neurocognitive Disorder - Diagnostic Criteria, American Psychiatric Association

An individual diagnosed with major or mild frontotemporal neurocognitive disorder needs to meet all of the following criteria:

  • The criteria are met for major or mild neurocognitive disorder:

    • Major Neurocognitive Disorder

    • 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).

    • Mild Neurocognitive Disorder

    • 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).

  • The disturbance has insidious onset and gradual progression.

  • Either:

    • Behavioral variant:

      • Three or more of the following behavioral symptoms:

        • Behavioral disinhibition

        • Apathy or inertia

        • Loss of sympathy or empathy

        • Perseverative, stereotyped or compulsive/ritualistic behavior

        • Hyperorality and dietary changes

      • Prominent decline in social cognition and/or executive abilities

    • Language variant:

      • Prominent decline in language ability, in the form of speech production, word finding, object naming, grammar, or word comprehension.

  • Relative sparing of learning and memory and perceptual-motor function.

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

  • Probable frontotemporal neurocognitive disorder is diagnosed if either of the following is present; otherwise, possible frontotemporal neurocognitive disorder should be diagnosed:

    • Evidence of a causative frontotemporal neurocognitive disorder genetic mutation from either family history or genetic testing.

    • Evidence of disproportionate frontal and/or temporal lobe involvement from neuroimaging.

    Possible frontotemporal neurocognitive disorder is diagnosed if there is no evidence of a genetic mutation, and neuroimaging has not been performed.
This diagnosis is for cases of dementia due to causes other than Alzheimer disease or cerebrovascular disease. Onset may be at any time in life, though rarely in old age. The more common disorders in this category are dementia in: Pick disease, Creutzieidt-Jakob disease, Huntington disease, Parkinson disease, and human immunodeficiency virus [HIV] disease. Less common disorders in this category are dementia in: cerebral lipidosis, epilepsy, hepatolenticular degeneration, hypercalcaemia, hypothyroidism, intoxications, multiple sclerosis, neurosyphilis, niacin deficiency, polyarteritis nodosa, system lupus erythematosus, trypanosomiasis, uraemia, and vitamin B12 deficiency. Treatment depends on the underlying physical cause of this dementia. Some of these dementias can be reversed with treatment (e.g., hypothyroidism, intoxications, vitamin deficiencies etc.). Some of these dementias lead to death within one or two years (e.g., Creutzfeldt-Jakob disease). Others lead to death within 10 to 15 years (e.g., Huntington disease).

Effective Therapies

Outcome depends on the physical cause of the dementia.

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