Dementia: Core Definition, Types, and Treatments

Understanding Dementia

Nicholas J. Schor, MD FAPA

www.olneypsychiatry.com

Learning Objectives:

Understand the core definition of Dementia

Understand the various types of Dementia and

what differentiates them from one another

Understand the difference between Dementia and

other conditions (Mild Cognitive Impairment,

Delirium, Pseudodementia)

Understand treatments and prognosis for

Dementia

Dementia—defined

A chronic or persistent disorder of the mental

processes caused by brain disease or injury

and marked by memory disorders, personality

changes, and impaired reasoning…

…which impairs one or more activities of daily

living to the degree that it impacts safety

and/or quality of life.

Generalizations about Dementia



Dementias are primarily COGNITIVE disorders



Acquired and represent decline (i.e. not developmental)



Underlying brain pathology



Major Neurocognitive Disorder: Significant Cognitive Decline, interferes with

independence, not due to delirium, not due to other mental disorder



Minor Neurocognitive Disorder (Mild Cognitive Impairment): moderate

cognitive decline, does NOT interfere with independence, not due to

delirium,not due to other mental disorder



Minor: 1–2 standard deviation range on testing



Major: more than 2 standard deviations on testing



Do not determine based upon percentages on tests alone

Generalizations about Dementia



Memory problems usually

evident early



Memory impairment alone is

not enough to make the

diagnosis…

Cognitive domains specified

DSM-5

:



Complex attention



Executive function



Learning & memory



Language



Perceptual-motor



Social cognition

DSM-IV

:



Memory impairment



Aphasia



Apraxia



Agnosia



Executive dysfunction

Dementia- defined



Memory problems AND at least one additional

cognitive deficit:



  Complex attention 

(maintain information in one’s mind for a short

          time and to manipulate that information)



• 

Executive function 

(

the mental processes that enable us to plan, focus

         attention, remember instructions, and juggle multiple tasks successfully)



• 

Learning & memory 

(short term



long term)



• 

Language 

(word finding difficulties



 aphasia)



• 

Perceptual-motor

(apraxia

-

impaired ability to use known objects,

         agnosia

-trouble recognizing or identifying things despite intact sensations )



• 

Social cognition 

(inappropriate behaviors, less social awareness)

Associated Features



Spatial disorientation



Poor insight and judgment means…they get

themselves in trouble by overestimating

their abilities and underestimating risks



Perceptual Abnormalities:



Delusions- especially persecution



Hallucinations- especially visual

More associated features



Personality Changes:



Disinhibition



Neglect of personal

hygiene



Apathy and withdrawal



Distillation/caricature

What causes Dementia?

ANTERIOR

CLASSIFICATION BASED ON SITE

 POSTERIOR

SUB-CORTICAL

CORTICAL

# HIGHER CORTICAL

ABNORMALTIES

# DYSPHASIA,

AGNOSIA,

APRAXIA

# Eg: ALZHEIMER’S

        DISEASE

# FRONTAL PREMOTOR

CORTEX.

#BEHAVIOURAL

CHANGES,

LOSS OF INHIBITION,

ANTI-SOCIAL

BEHAVIOUR,

FACILE &

IRRESPONSIBLE

# Eg: NORMAL

PRESSURE

HYDROCPHALUS,

HUNTINGTON’S

CHOREA, METABOLICAL

DISEASE

# PARIETAL &

TEMPORAL LOBE’S.

# DISTURBANCE OF

COGNITIVE FUNCTION

(MEMORY &

LANGUAGE)

WITHOUT MARKED

CHANGE IN

BEHAVIOUR.

# Eg: ALZHEIMER’S

        DISEASE

# APATHETIC

#FORGETFUL AND

SLOW POOR ABILITY

TO USE KNOWLEDGE

# ASSOCIATED WITH

OTHER NEUROLOGICAL

SIGNS & MOVEMENT

DISORDERS.

# Eg: PARKINSON’S

DISEASE,

AIDS DEMENTIA

COMPLEX.

1) ALZHEIMER’S DISEASE (60% OF ALL CASES)

2) CEREBRO VASCULAR DISEASES (20% OF ALL CASES)

MULTI INFARCT (ATHEROSCLEROTIC) DEMENTIA

SUBCOURTICAL VASCULAR DISEASE

BINSWANGER’S DISEASE

3) NEURO DEGENERATIVE-

PICKS 

DISEASE

; HUNTINGTON’S CHOREA; PARKINSON’S DISEASE

4) INFECTIONS-

CREUZFELD-JACKOB’S DISEASE; HIV (AIDS DEMENTIA COMPLEX);

VIRAL ENCEPHALITIS; PROGRESSIVE MULTIFOCAL LEUCOENCEPHALOPATHY

5) NORMAL PRESSURE HYDROCEPHALUS

5) NUTRITIONAL-

WERNICKE KORSAKOFF (THIAMINE DEFICIENCY); B12 DEFICIENCY; FOLATE DEFICIENCY

5) METABOLIC-

HEPATIC DISEASE; THYPOID DISEASE; PARATHYROID DISEASE; CUSHING’S DISEASE

5) CHRONIC INFLAMMATORY-

COLLAGEN VASCULAR DISEASE & VASCULITIS, MULTIPLE SCLEROSIS

5) TRAUMA-

HEAD INJURY; PUNCH-DRUNK SYNDROME

5) TUMOR SOME-

eg: SUBFRONTAL MENINGOMA

CASES ARE TREATABLE; 10 DASH 15% ARE REVERSIBLE.

CLASSIFICATION BASED ON CAUSE

Alzheimer’s

Named after Dr. Alois

Alzheimer - a clinical

psychiatrist and

neuroanatomist

First reported

November 3, 1906

•

Most common type of dementia; accounts for an estimated

60 to 80 percentage

 of cases.

Symptoms:



Difficulty 

remembering names and recent events 

is often an early

clinical symptom.



Apathy

 and 

depression

 are also often early systoms.



Later symptoms include 

impaired judgments, disorientation, confusing,

behaviour changes and difficulty speaking, swallowing and walking.

Brain changes:



Hallmark abnormalties are 

deposits of the protein fragment

beta-amyloid (plaques) and twisted strands of the protein tau (tangles)

as well as evidence 

of nerve cell damage and death in the brain.

ALZHEIMER’S DISEASE

•

Previously known as 

multi-infarct

 or 

post-stroke

 dementia, vascular dementia is the

second

 most 

common

 cause of dementia after Alzheimer’s disease. (20% of all cases)

Eg: 

MUTLI-INFARCT DEMENTIA (ATHEROSCLEROTIC), SUBCORTICAL VD

(BINSWANGER’S DISEASE-SUBCORTICAL LEUCOENCEPHALOPATHY)

Symptoms:



Impaired judgement or ability to plan steps needed to complete a task 

is more

likely to be the initial sysmptom, as opposed to the memory loss often associated with

initial symptoms of Alheimer’s.



Occurs 

because of brain injuries 

such as 

microscopic bleeding and blood vessel

blockage.



The 

location

 of the brain injury determines how the individual’s thinking and physical

functioning are affected

Brain changes:



Brain imaging 

can often detect blood vessel problems implicated in vascular

dementia.

CEREBROVASCULAR DEMENTIA

Binswanger’s Disease



Progressive brain damage caused by arteriosclerosis and

tiny clots in the blood vessels that supply the structures of

the mid-brain (thalamus, basal ganglia).



Formerly called “hardening of the arteries”



Progressive memory loss, urinary incontinence



Psychomotor slowness

- slowed motor control/walking



Apathetic, depressed, withdrawn, executive dysfunction,

decreased spontaneity.



Slowly progresses over 5-10 years

Binswanger’s Disease

DEMENTIA CAN OCCUR AS A SYMPTOM OF MORE WIDESPREAD DEGENERATIVE

DISORDERS

Eg: 

HUNTINGTON’S DISEASE, DIFFUSE LEWY BODY DISEASE, PROGRESSIVE SUPRANUCLEAR

PALSY, MOTOR NEURON DISEASE ETC.

Symptoms:



People with 

dementia with LEWY bodies 

often have 

memory loss and thinking

problems

 common in Alzheimer’s, but are more likely than people with Alzheimer’s

to have initial or early symptoms such as 

sleep disturbances, well-formed visual

hallucinations, and muscle rigidity or other parkinsonian movement features.

Brain changes:



Lewy bodies 

are

 abnormal aggregations

 (or clumps) of the 

protein Alpha-synuclein.

When they develop in the 

brain cortex

, dementia can result. Alpha–synuclein also

aggregates in the brains of people with Parkinson’s disease, but the aggregate



may appear in a pattern that is different from dementia with Lewy bodies.

OTHER CAUSES

Lewy Body Dementia



Similar issues as Alzheimer’s PLUS:



Motor Dysfunction/Parkinsonism

(rigidity, tremor, shuffling gait, falls)



Sensitivity to antipsychotic medications



Autonomic dysfunction

-impairment in regulation of temperature, BP,

eliminatory functions, sexual dysfunction



Hallucinations earlier in the disease



REM Sleep Behavior Disorder 

(severe form of sleepwalking)



Greater fluctuation between good/bad days

-can relate to wide variation

in attention

REM Sleep Behavior Disorder



 https://www.youtube.com/watch?v=rFXYRQ9xPUA

Treatment of Lewy Body Dementia



Similar to treatment of Alzheimer’s-

cholinesterase inhibitors/memantine



Avoidance of antipsychotics

(when possible)



+/- use of Parkinson’s medications



Huntington’s disease is a progressive brain disorder caused a 

single defective

gene on chromosome 4.

Symptoms:



Include Huntington’s chorea, dysarthria, dysphasia, facial twitching, chameleon or trombone tongue a

severe decline in thinking and reasoning skills, and irritability, depression and other mood changes.

Brain changes:



The gene defect causes 

abnormalities in a brain protein

 that, overtime, lead to 

worsening

symptoms.

HUNTINGTON’S DISEASE

MIXED DEMENTIA



In mixed dementia abnormalities link to 

more than one type of dementia occur simultaneously

 in the

brain.

Brain changes:



Characterized by the hallmark abnormalities of more than one type of dementia – most 

commonly,

Alzheimer’s and vascular dementia, 

but also 

other types,

 such as 

dementia with Lewy bodies.

Eg: 

PICK’S DISEASE, HUNTINGTON’S CHOREA, PARKINSON’S DISEASE

NEURO GENERATIVE DEMENTIA:

As Parkinson’s disease progresses, it often results in a progressive dementia similar to

dementia with Lewy bodies or Alzheimer’s.

Symptoms:



Problems with 

movement

 or a common symptom early in the disease. If dementia

develops, symptoms are often similar to dementia with Louis bodies.



RESTING TREMOR, AKINETIC RIGIDITY, BEN POSTURE, HYPOMIMIA

Brain changes:



Alpha-synuclein clumps 

are likely to begin in an area deep in the brain called the

substantia nigra

. These clumps I thought to 

cause degeneration of the nerve cells

that produce dopamine.

Parkinson’s disease

EG: 

CREUTZFELD-JACOB DISEASE, HIV INFECTION (AIDS-DEMENTIA COMPLEX), VIRAL ENCEPHALITIS,

PROGRESSIVE MULTIFOCAL LEUCOENCEPHALOPATHY

DEMENTIA CAUSED BY INFECTIONS



CJD is the most common human form of a group of 

rare, fatal brain disorders

 affecting

people and certain other mammals. Variant CJD 

(“mad cow disease”) 

occurs in cattle,

and has been transmitted to people under certain circumstances.

Symptoms:



Rapidly 

fatal

 disorder that impairs memory and coordination and causes behavior changes.

Brain changes:



Results from 

misfolded prion protein 

that causes a 

“domino effect” 

in which

prion protein throughout the brain misfolds and thus malfunctions.

Creutzfeld-Jacob

 disease

Kuru

Kuru



A 

transmissible spongiform encephalopathy

 (prion

disease)that causes physiological and neurological effects

which ultimately lead to death. It is characterized by

progressive cerebellar ataxia, or loss of

coordination/control over muscle movements, and

uncontrollable laughter.



Can incubate 5-13 years



Seen in the Fore people of Papua New Guinea



Caused by funerary cannibalism



Last case seen 2005

Kuru



Differential diagnosis: 

Creutzfeldt–Jakob

disease



Causes: 

Transmission of infected prion

proteins



Symptoms: 

Body tremors, random outbursts

of laughter



Prevention: Avoid practices of cannibalism



A condition that leads to 

the loss of intellectual abilities such as memory,

judgment, and abstract thinking. 

It can also cause

 changes in personality.



AIDS Dementia Complex 

(or ADC) is a type of dementia that occurs in advanced

stages of AIDS (acquired immunodeficiency syndrome)

These are some of the first signs and symptoms of ADC:

•

Short attention span

•

Trouble remembering

•

Poor judgment

•

Slow thinking and a longer time needed to do tasks

•

Irritability

•

Unsteady gait, tremor, or trouble staying balanced

•

Poor hand coordination

•

Social withdrawal or depression

In later stages, you may have more server symptoms:

•

Extreme mood swings

•

Psychosis

•

Loss of bladder and bowel control

HIV Infection (AIDS-Dementia complex)

Symptoms:



Symptoms 

include difficulty walking, memory loss 

and

 inability to control urination.

Brain changes:



Caused by the build up of 

fluid in the brain

. Can sometimes be corrected with

surgical installation of a shunt in the brain to drain excess fluid.

Normal Pressure Hydrocephalus

Eg: 

WERNICKE-KORSAKOFF (THIAMINE DEFICIENCY), B12 DEFICIENCY, FOLIC ACID DEFICIENCY

Nutritional



Korsakoff syndrome is a chronic memory disorder caused by severe 

deficiency of the thiamine

(vitamin B-1)

. The most common causes 

alcohol misuse

.

Symptoms:



Memory problems 

may be strikingly severe while other 

thinking and social skills seem relatively unaffected.

Brain changes:



Thiamine helps brain cells produce energy from sugar. When thiamine levels fall too low, 

brain cells

cannot generate enough energy to function properly.

WERNICKE-KORSAKOFF SYNDROME

Magnetic Gait in NPH

https://www.youtube.com/watch?v=vXtYuHqNVII

Wernicke-Korsakoff Syndrome



Confusion



Ataxia



Opthalmoplegia



Nystagmus



Amnesia



Confabulation



Caused by

demyelination of

the mammillary

bodies in the

brain

Eg: 

HEPATIC DISEASES, THYROID DISEASES, PARATHYROID DISEASE, CUSHING’S SYNDROME

By Metabolic disorders

Eg: 

COLLAGEN VASCULAR DISEASE, VASCULITIS, MULTIPLE SCLEROSIS

Chronic inflammatory



A condition seen in 

boxers and alcoholics, 

caused by 

repeated cerebral concussions and

characterized by 

weakness in the lower limbs, unsteadiness of gait, slowness of

muscular movements, hand tremors, hesitancy of speech, and mental dullness.



Boxers encephalopathy

, dementia pugilistica. A syndrome affecting 10-20% of

professional boxers, which is the cumulative result of recurrent brain damage and

progressive communicating hydrocephalus and due to extrapyramidal and cerebellar

lesions that translate into dysarthria, ataxia, tremors, pyramidal lesions — causing

mental deterioration and personality changes – e.g., rage reaction and morbid

jealousy – ‘Othello syndrome’

PUNCH-DRUNK SYNDROME

Eg: 

HEAD INJURY, PUNCH-DRUNK SYNDROME

Trauma

Frontotemporal Dementia (Pick’s Disease)



Pathologically / clinically heterogeneous disorder with focal

degeneration of frontal and/or temporal lobes



 Onset typically late 50’s‐ early 60’s; mean age 58

    Earliest manifestations



 Personality changes / social behavior changes (behavioral

variant)



 Language deficits



 Slowly progressive to more global dementia



 Some with motor symptoms



 1/3 with family history

Frontotemporal Dementia

Frontotemporal Dementia

FTD Subtypes



Behavioral variant ( BV )



 Progressive Nonfluent Aphasia ( PNFA )



 Semantic Dementia ( SD ) - progressive fluent aphasia



 Motor Syndromes

                ‐ Motor Neuron Disease ( MND )

                ‐ Corticobasilar Degeneration ( CBD )

                ‐ Progressive Supranuclear Palsy ( PSP )

Frontotemporal Dementia-Behavioral Variant



Most common presentation of FTD



Personality changes

           – Apathy



 withdrawal; decreased spontaneity; abulia

           - Social disinhibition / impulsivity / increased sentimentality /  violent

              aggression



Lack of insight



Lack of concern



 Loss of social awareness

               – Offensive remarks; inappropriate behavior

               – Hygiene; inappropriate elimination

               – Antisocial acts; criminal acts

               – Inappropriate sexual comments

Frontotemporal Dementia



Stereotypical or ritual behaviors

      -preference for same foods, counting, catch-phrase,

        pacing, hoarding



Mental Rigidity-inflexible routines, impaired adaption



 Eating pattern changes ‐ overeat or binge, increased

alcohol use, hyperorality-the need to put things in mouth



Emotional blunting / loss of empathy

     ‐ Self‐ centered

     - Difficulty recognizing others’ emotional expression

     - Inability to see others’ point of view

Frontotemporal Dementia-Behavioral variant



Cognitive function relatively intact early

(MMSE)

      – with progression 



 impaired executive

        function, problem‐solving, attention

       – Memory and visuospatial skills less severe



 Altered speech – aspontaneity, echolalia,

paucity of speech, perseveration, pressured,

speech

Frontotemporal Dementia-Progressive

Nonfluent Aphasia



Anomia is initial manifestation ‐ word‐finding; object naming



Progressively dysfluent speech ‐ simplification; circumlocution;

phonemic paraphasia-using wrong word i.e. ‘drive’ instead of

‘car’



Ultimately, difficulty with comprehension; may become mute



Restricted to expressive language for few years; global dementia

later



Some develop behavioral manifestations or motor neuron

disease



MRI – left perisylvian atrophy

Semantic Dementia



 Initially a progressive speech disturbance



– Impaired comprehension; anomia; semantic parapahasias

     – Normal fluency 



 effortless speech lacking meaning & information

     – Normal repetition



Dyslexia / dysgraphia



Read and write phonetically

Verbal fluency tests – category fluency > letter fluency



 MRI‐ temporal atrophy, L > R

Semantic Dementia



 Episodic memory (autobiographical) is relatively

preserved



Less commonly right temporal lobe deficits –recognizing

faces / voices



 Behavioral problems usually within few years

FTD-Motor Syndromes



Motor Syndromes

   ‐ Motor Neuron Disease (MND)

   ‐ Corticobasal Degeneration (CBD)

   ‐ Progressive Supranuclear Palsy (PSP)

FTD-Motor Syndromes-Motor Neuron Disease



 Precede or follow dementia, usually

behavioral variant



 50 % with MND have / develop dementia



Progressive atrophy / flaccidity /

fasciculation's of muscles



Clinical features of both FTD‐MND usually

within two years



 Course more rapidly progressive



 MRI‐ bifrontal atrophy

FTD-Corticobasilar Degeneration



Asymmetric rigidity / apraxia



 Alien limb syndrome



 Dystonia



 Impaired joint position, 2‐point discrimination,

agraphesthesia



 Mirror movements



 Gait impairment and gaze palsy



Cognitive impairment develops in most



 MRI‐ asymmetric atrophy of frontoparietal, basal

           ganglia and/or cerebral peduncles

FTD-Progressive Supranuclear Palsy



Supranuclear vertical gaze palsy



Axial dystonia (muscles of chest, back, abdomen)



 Bradykinesia



 Rigidity



 Falls



 Most with dementia and overlap with behavioral

    variant FTD



 MRI‐ symmetric atrophy of superior cerebellar

    peduncle and midbrain

Mild Cognitive Impairment

The 

difference

 between 

MCI

 and

dementia

 is that any of the

symptoms that are seen in 

mild

cognitive impairment

 do not cause

any interference with the person's

daily level of activities.

The rate of progression to dementia

among persons with MCI is 7.1% per

year in contrast to the rate of

progression among cognitively normal

persons of 0.2% per year. In typical

clinical settings, where a diagnosis of

MCI is likely to be made later in the

course, the rate of progression to

dementia may be even higher

A manifestation of global cognitive

impairment seen along with core

symptoms of Major Depressive Disorder,

most often in an older individual. Not a

true dementia as the cognitive symptoms

tend to remit once the underlying Major

Depressive Disorder is treated, typically

with antidepressants.

PSEUDODEMENTIA

PSEUDODEMENTIA

Even if depression and

cognitive problems clear,

they may be prodromal for

eventual irreversible

dementia (40% on 3-year

follow-up).

PSEUDODEMENTIA

       Delirium



Disturbance of consciousness (awareness of

the environment) and attention,



PLUS…

PLUS…



Changes in 

cognition

 (ie, “thinking”-

memory, orientation, language, etc)

 OR

 OR



Perceptual disturbances

The Course of Delirium



Evolves rapidly (hours to days)



Usually resolves rapidly as well:



May be self-limited, persist for

weeks, or progress to death



Degree of impairment fluctuates

Disturbance in sleep-wake cycle

Easily distracted by irrelevant stimuli

Changes in activity level

-Restlessness, hyperactivity

-Picking at clothes, getting out of

bed

-OR  hypoactivity (lethargy)

Emotional disturbances- mood

lability, anger, irritability, euphoria,

apathy

Speech or language disturbances

Perceptual abnormalities common

-Illusions, hallucinations, delusions

Neurological deficits/dysfunction

What Are the Causes?



DIRECT: Brain pathology:  head injury, seizures (during and after),

strokes, infections



INDIRECT: Systemic Illness: electrolyte abnormalities, dehydration,

uremia, hepatic encephalopathy, cardiovascular compromise



Sensory deprivation



After surgery (post-operative state)- ie. “ICU Psychosis”



Side effects of medications or toxins or with abused recreational

drugs: “

Substance-Induced Delirium

”

- NMS (Neuroleptic Malignant Syndrome)

- Serotonin Syndrome

Treating Delirium



Considered a Medical Emergency



Supportive care in an ICU setting



Safety- close monitoring



Remove offending agent, treat

underlying cause

Treatment of Dementia

Medications

Few that treat core pathology.

Most medications are used

symptomatically (i.e. sedatives for

sleep disturbance, antipsychotics

for psychosis, etc.)

SSRI antidepressants are first line

for most mood/behavioral

symptoms.

SSRI may slow disease process in

Alzheimers

Environment

SUPERVISION

‘Balanced’ stimulation

Nutrition

Non-pharmacologic treatments

Distract, Deflect, Defer



Start an activity



Play music



Ask the person for help (i.e. folding laundry)



Show them photographs



Games/apps on cell phone



Ask them questions about themselves.



Sometimes it works, sometimes it doesn’t.

Non-pharmacologic treatments

Karate  Vs.  Judo

Non-pharmacologic treatments

Non-pharmacologic treatments

To tell the truth….or not.

Clinicians refer to this as ‘reality testing’.

Evaluate on a case by case basis.

Many family members are uncomfortable lying to

their loved ones, but often it is the kindest, most

loving response to protect them from emotional

distress.

Prognosis

Prognosis



Except for the few reversible dementias, all

dementias are progressive and ultimately fatal.



People become increasingly passive/withdrawn

and ultimately require full care.



More likely to die from indirect causes of

dementia: FALLS, infections, inability to swallow.



Many die of other diseases of aging-

heart/liver/kidney disease, cancer, etc.



Those who do die from dementia ultimately

succumb to intractable seizures secondary to

brain damage.