Classification of Dementia

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    C H A I R P E R S O N : D R . K R I S H N A M U R T H YP R E S E N T E R : D R . P R I M A D SOUZA

    CLASSIFICATION OF

    DEMENTIA

    03.08.2011

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    Dementia is defined as a progressive impairment of

    cognitive functions occurring in clear consciousness

    Derived from the Latin word dementatus, meaning outof one's mind

    Prevalence: moderate to severe dementia

    5% in the general population >65 yrs

    20-40% in the general population >85 yrs

    Male to female ratio is 0.6 to 0.8

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    Alzheimer's disease: 50% - 60%

    Vascular dementia: 15%

    Mixed vascular and Alzheimer's dementia:15%

    Fronto-temporal dementias: 5%

    Other dementias: 10% (Lewy body dementia, Pick's

    disease, NPH, alcoholic dementia, infectious dementia)

    Reversible dementias: 5%

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    Better understanding of the disorder, uniformity

    Underlying pathology, severity

    Investigation and management

    Availability and timely application of effective treatment

    Prognosis and planning..

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    Decline in memory- registration, storage and retrieval of

    new information

    Impairment of thinking, reasoning capacity and

    reduction in flow of ideas

    Impair personal activities of daily living

    Evident for at least 6 months

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    F00 Dementia in Alzheimers disease

    F00.0 with early onset

    F00.1 with late onset

    F00.2 atypical or mixed type

    F00.9 unspecified

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    F01 Vascular dementia

    F01.0 Vascular dementia of acute onset

    F01.1 Multi- infarct dementia

    F01.2 Subcortical vascular dementia

    F01.3 Mixed cortical and sub cortical

    F01.8 Other vascular dementia

    F01.9 Unspecified

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    F02 Dementia in other diseases classified elsewhere

    F02.0 Dementia in Picks disease

    F02.1 Dementia in Creutzfeldt-Jacob disease

    F02.2 Dementia in Huntingtons disease

    F02.3 Dementia in Parkinsons disease

    F02.4 Dementia in HIV disease

    F02.8 Dementia in other diseases classified elsewhere

    F03 Unspecified dementia

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    Multiple cognitive deficits including memory impairement

    Plus atleast one of: aphasia, apraxia, agnosia or disturbance

    in executive functioning

    Sufficiently severe to cause impairement in occupational or

    social functioning

    Decline from previously higher level of

    functioning

    Exclude delirium

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    294.1x Dementia of Alzheimers type

    294.10 without behavioral disturbance

    294.11 with behavioral disturbance

    Specify subtype:

    With early onset: onset is at 65yrs or below

    With late onset: onset after age 65 yrs

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    290.4x Vascular dementia

    290.41 with delirium

    290.42 with delusions

    290.43 with depressed mood

    290.40 uncomplicated

    Specify: with behavioral disturbances

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    Dementia due to General medical conditions:

    .10 without behavioral disturbance

    .11 with behavioral disturbance

    294.1x Dementia due to HIV Disease

    294.1x Dementia due to Head trauma

    294.1x Dementia due to Parkinsons disease

    294.1x Dementia due to Huntingtons disease

    294.1x Dementia due to Picks disease

    294.1x Dementia due to CJD

    294.1x Dementia due to other GMC

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    Dementia due to substance use

    Dementia due to multiple etiologies

    294.8 Dementia NOS

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    Cortical

    Sub cortical

    Mixed

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    SUBCORTICAL

    No aphasia

    Impaired recall> recognition Calculation preserved late

    Executive functiondisproportionately affected

    Cognitive processing slowed

    early Personality apathetic

    Mood depressed

    Speech dysarthric

    Posture bowed Coordination impaired

    Motor speed slowed

    Chorea, tremor, tics,dystonia

    CORTICAL

    Aphasia early

    Recall = recognition Involved early

    Consistent with otherinvolvement

    Normal till late in disease

    Unconcerned

    Euthymic

    Articulate until late

    Upright

    Normal until late

    Normal

    absent

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    Primary:

    Diseases, injuries & insults that affect the brain directly or

    with prediliction

    Alzheimers, Picks, dementia with Lewy bodies, Parkinsons

    Secondary:

    Systemic diseases/ disorders that attack the brain as one of

    multiple organs or systems of the body involved

    HIV dementia, nutritional deficiency, metabolic

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    Degenerative dementias:

    Alzheimer's disease

    Frontotemporal dementias (e.g., Pick's disease)

    Parkinson's disease

    Lewy body dementia

    Miscellaneous:

    Huntington's disease

    Wilson's disease

    Metachromatic leukodystrophy

    Neuroacanthocytosis

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    Cardiovascular:

    Infarction (single, multiple or lacunar)

    Binswanger's disease (subcortical arteriosclerotic encephalopathy)

    Hemodynamic insufficiency

    Physiologic:

    Normal pressure hydrocephalus

    Metabolic:

    Vitamin deficiencies (e.g., vitamin B12, folate)

    Endocrinopathies (e.g., hypothyroidism)

    Chronic metabolic disturbances (e.g., uremia)

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    Tumor:

    Primary or metastatic: e.g., meningioma or metastatic breast or

    lung cancer

    Traumatic: Dementia pugilistica

    posttraumatic dementia

    Subdural hematoma

    Infections:

    Prion diseases: e.g., Creutzfeldt-Jakob disease

    Acquired immune deficiency syndrome

    Syphilis

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    Demyelinating diseases:

    Multiple sclerosis

    Drugs and toxins:

    Alcohol Heavy metals

    Irradiation

    Pseudodementia due to medication (e.g.anticholinergics)

    Carbon monoxide

    Psychiatric:

    Pseudodementia of depression

    Cognitive decline in late-life schizophrenia

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    Mild dementia:

    Consistent forgetfulness that is more marked for recent events

    Inability to function effectively in interests and more complex

    activities (work, community, home, social activities)

    Maintained social judgment

    May require prompting to perform activities of daily living he or

    she is able to complete independently these tasks

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    Moderate dementia:

    Short-term memory is poor

    Long-term memory may be only slightly affected

    Impaired social judgment

    Cannot perform independently outside of the home

    Activities in the home are usually limited to simple chores Interests are severely curtailed

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    Severe dementia:

    Severe memory loss-Severe deficits in long-term short-term

    memory

    Disorientation usually to time and place

    Inability to independently function inside or outside of the

    home,

    Requirement of help with activities of daily living (toileting,

    bathing, and eating)

    Possible incontinence

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    Profound dementia:

    Patient is unintelligible & unable to follow simple

    commands

    Incontinent

    Unable to ambulate or to accomplish purposeful tasks

    May be bedbound, unresponsive, have swallowing

    difficulties and contractures.

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    Reversible/ Treatable dementias:

    5% of dementias

    Causes:

    Metabolic: hypothyroidism, hypoparathyroidism

    Normal pressure hydrocephalus

    Nutritional deficiencies: vitamin B12 or folate deficiencies

    Tumor or other space-occupying lesion-chronic SDH Infection: syphilis, AIDS

    Drugs: any drug with anticholinergic activity

    Emotional: depression

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    Progressive /irreversible dementia:

    Alzheimer disease

    Vascular dementia

    Dementia with lewy bodies

    Fronto-temporal dementia

    Huntingtons disease

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    Senile dementia:

    onset after age 65 yrs

    Eg: Alzheimers disease

    Presenile dementia:

    onset before age 65 yrs

    Eg: Picks, Huntingtons, HIV dementia

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    1. Diseases in which dementia is associated with clinical

    and laboratory signs of othermedical disease

    HIV infection

    Endocrine: hypothyroidism, Cushings

    nutritional deficiencies

    CNS infections

    Chronic drug/ heavy metal exposure

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    2. Diseases in which dementia is associated with other

    neurologic signs but not with other obvious medical disease

    A. Invariably associated with other neurologic signs

    Huntingtons disease

    Multiple sclerosis

    Parkinsons disease

    CJD, myoclonic epilepsy

    B. Often associated with other neurologic signs Cerebral infarct, tumors, trauma

    Lewy body disease

    NPH

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    3. Diseases in which dementia is the only evidence of

    neurologic disease or medical disease

    A. Alzheimer's

    B. Picks

    C. Some cases of AIDS

    D. Fronto-temporal and frontal lobe dementias

    E. Degenerative disease of unspecified type

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    National Institute of Neurological Communicative

    Disease and Stroke and the Alzheimer's Disease and

    Related Disorders Association

    Definite: based on histological evidence

    Probable: based on clinical criteria

    Possible: atypical features or coexisting disorders such as

    Parkinsons disease

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    PSEUDODEMENTIA

    Onset well demarcated

    History short

    Rapidly progressive

    H/o previous psychiatricdifficulty or recent crisis

    C/o cognitive function

    Affective change present

    Behavior unaffected

    No nocturnal exacerbation

    Examination: I dont know,little effort spent

    Inconsistent memory loss

    Specific memory gaps

    DEMENTIA

    Indistinct

    Long history

    Initially unnoticed

    Uncommon

    Little c/o cognitive loss Apathetic, shallow

    emotions

    Compatible with memoryloss

    Nocturnal accentuation Tries items, struggles with

    tasks

    Recent>remote, consistent

    No specific memory gaps

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    Memory impairement accompanied by abnormal memory

    test scores

    Normal cognitive function

    Preserved activities of daily living

    No social or occupational impairement

    1020% progress to dementia yearly

    Risk factors- degree of cognitive impairement

    Evaluation similar to dementia

    Treatment controversial

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    Neurocognitive Disorders

    Minor Neurocognitive Disorder (MCI), with the necessary

    neurocognitive impairment in only one domain

    Major Neurocognitive Disorder or Dementia-involve at

    least two domains.

    Memory impairment would not be necessary fordiagnosing either of these conditions.

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    Subclassified according to etiologye.g.,

    Alzheimer disease,

    vascular neurocognitive disorder,

    Frontotemporal degeneration,

    Lewy Body disease,

    Mixed (specify which ones),

    Not otherwise specified

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    Specifiers to better define the clinical condition

    Course (transient, remitting, persistent but stable, persistent

    and worsening, persistent with fluctuations)

    Age of onset (

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    4th century AD- Oribasius- a disease of cerebral atrophy thatcaused loss of intellectual capacity and weakness ofmovement

    Esquirol: acute, chronic, and senile

    Senile dementia was defined as a cerebral affectioncharacterized by a weakening of the sensibility,understanding and will

    1845- Wilhelm Griesinger - senile dementia as a disease of thecerebral arteries

    1907-Alois Alzheimer was the first to identify specifichistopathological changes associated with progressivedegenerative dementia

    Aphasia, apraxia, agnosia, and the histopathological findingof neurofibrillary tangles and milar foci

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    CORTICAL SUBCORTICAL

    Aphasia early

    Recall = recognition Involved early

    Consistent with otherinvolvement

    Normal till late in disease

    Unconcerned

    Euthymic

    Articulate until late

    Upright Normal until late

    Normal

    absent

    No aphasia

    Impaired recall> recognition Calculation preserved late

    Executive functiondisproportionately affected

    Cognitive processing slowed

    early Personality apathetic

    Mood depressed

    Speech dysarthric

    Posture bowed

    Coordination impaired

    Motor speed slowed

    Chorea, tremor, tics,dystonia