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Dementia is an umbrella

term for several


diseases affecting
memory, other
cognitive abilities and
behaviour that interfere
significantly with a
person’s ability to
maintain their activities
of daily living. 

Dementia is a syndrome in which there is


deterioration in memory, thinking, behaviour and
the ability to perform everyday activities.
DSM-V DSM-IV ICD 10
The neurocognitive disorders (NCDs) Cognitive Disorders Organic Disorder

Delirium=a confused state caused by Delirium Delirium


illness

The major or mild NCD Dementia Dementia


subtypes

the DSM-IV category of "Amnestic Amnestic Disorder Amnestic Disorder


Disorder,“ and Other Cognitive and Other
now is diagnosed as major NCD due to Disorders Cognitive
another medical condition Disorders
and for which the term dementia would
not be used.

NCD due to another medical condition;


(e.g., 340 [G35] multiple sclerosis)
They result from primary or secondary abnormalities of the central
nervous system
The neurocognitive disorders (NCDs)

Degenerative disorders: Parkinson Disease - Huntington Disease

Frontal lobe Dementia Pick disease - Creutzfeldt-Jakob


Disease
Epilepsy

Multifocal Disruptions-
multiple sclerosis

Brain Attacks=Stroke

Infection =HIV/AIDS---/ Pediatric Autoimmune Lyme Disease


Neuropsychiatric
Disorders(PANDAS)/

Traumatic brain injury


Neurocognitive disorders
Alzheimer ‘s disease is the most common cause of dementia
Vascular dementia is the second cause of dementia and results
from ischemic events and hemorrhages in the brain ,
Hypertension is a known risk factor for VD
AIDS dementia complex {ADC} (is caused by HIV infection of brain)
Parkinson's disease is a brain
disease best known for the
trembling it causes

It is an incurable,
chronic disease and
gradually affects the
muscles and mental
capacity
(PD) - chronic, progressive degenerative disease
• Slow and selective loss of Substantia Nigra
dopaminergic neurons
Clinical features : -PD affects movement by creating
tremors (shaking or trembling, sometimes limited
to a certain part of the body such as arms or legs),
rigidity, and other problems with balance
(postural instability ) and motor skills,
bradykinesia,
-Autonomic dysfunction –orthostatic hypotension ,
constipation and bladder dysfunction, sexual
dysfunction
-Neuropsychiatric disturbances : depression,
dementia, psychosis
Parkinson's Disease (PD) is a degenerative disease of the basal ganglia
that results in a progressive loss of dopamine (DA)

 Muscle rigidity
 Difficulty initiation movement
 Resting tremor
 Weakness
 Gait disturbances
 Masklike face
 Apathy/reduced motivation
 Depression (30-50%)/anxiety (40%)
 Dementia (late period)=25-40% of patients with PD ,
profound memory loss, confusion, disruption in
functioning
 Nightmares
 Hallucinations (visual)/ or delusions (often paranoid)
PD is a synucleinopathy. Fibrils made of insoluble polymers of
alpha synuclein are deposited in the neuronal body, forming
round lamellated eosinophilic cytoplasmic inclusions,
the Lewy bodies (LBs). Alpha synuclein is also deposited in
neuronal processes (Lewy neurites), and in astrocytes and
oligodendroglial cells.
LBs cause neuronal degeneration and death
Normal SN-zona compacta SN in PD-same location
Lewy body
Misfolded alpha-synuclein proteins are converted
into pathological oligomers and higher order
aggregates that form fibrils and deposit into
Lewy bodies and Lewy neurites in affected
neurons of the PD brain.
About 20-60% of people with Parkinson's Disease
develop dementia. Dementia due to Parkinson's Disease
causes difficulty retrieving(recover) memories, depression,
and problems making decisions and carrying out daily
tasks
• Muhammad Ali at the age
of 64
• He has shown signs of
parkinsonism
since the age of 38
LEWY BODY DEMENTIA- LBD
• Parkinson's disease with dementia
(PDD) would be the diagnosis when
dementia onset is more than a year
after the onset of Parkinson's.
• LBD is diagnosed when cognitive
symptoms begin at the same time or
within a year of Parkinson symptoms
People with Lewy Body dementia may also have problems
with their short term memory, word finding difficulties,
difficulty sustaining a line of thought and problems
locating objects in space. They may also experience
symptoms of anxiety and depression.

 It appears to affect men and women alike.


 As yet it is impossible to identify risk factors for developing
the disease.
 rare ‘familial’ cases of Lewy Body disease have been
described.
 there is no cure for Lewy Body disease
 The depression which accompanies this disease for
example will usually respond to antidepressant therapy
Huntington's Disease (HD )
• HD is a (genetic (inherited) hereditary
neurodegenerative disorder characterized by an
excess of undesired movement and lack of muscle
tone
• Children of parents with HD have a 50% chance of
developing the disease themselves

HD is inherited in an
autosomal dominant
 fashion.

Inheritance is independent
of gender, and the
phenotype does not skip
generations
Huntington's Disease (HD ) is characterized by an excess
of undesired movement and lack of muscle tone
• HD affects cognition, emotion, and movement. It can
start as early as 4 years of age or as late as 85 years of
age, but most commonly affects people in their late 30s
or early 40s
• As the disorder progresses, movements become
uncontrolled, resulting in purposeless, rapid motions,
such as flexing and extending the fingers, raising and
lowering the shoulders or grimacing
Reported rates of behavioral symptoms in HD
• Irritability 38–73% (episodic aggressive behaviour)
• Apathy 34–76% (little desire to do anything)
• Anxiety 34–61%
• Mood swings and bouts of Depressed 33–69%
• Obsessive and compulsive 10–52%of clients
• Psychotic 3–11%
Huntington's Disease (HD ) is characterized by an
excess of undesired movement and lack of muscle
tone

Area of the brain most


damaged in early Huntington's
disease—striatum
(shown in purple)

A microscope image of a neuron with inclusion (stained orange)


Infection- HIV/AIDS - AIDS dementia complex (ADC)-
occurs in approximately 15% to 25% of people with
AIDS

HIV/AIDS is a disorder of the immune system caused by


a virus that can also destroy brain tissue
Dementia due to HIV/AIDS is characterized by
forgetfulness, slowness and difficulties with
concentration and problem solving
It can also include apathy (a lack of energy, interest,
and/or emotion) and psychotic symptoms, such as
delusions (fixed, false beliefs) and hallucinations (hearing,
seeing, touching, tasting, or smelling things that are not truly
present).
Because HIV/AIDS affects people of all ages, dementia
due to HIV/AIDS can occur in children as well as adults
Infection-Pediatric Autoimmune
Neuropsychiatric Disorders-PANDAS
–are a neurological complication associated with
streptococcal infections in some children

The antibodies produced to fight the streptococci


bacteria can trigger an autoimmune reaction

This reaction is most frequently directed against cells in


the heart and joints(rheumatic fever), but in 20-30 of
cases it react with basal ganglia, casing antibody
mediated inflammation , leading to CNS dysfunction

antibodies to streptococci attack the basal ganglion


cells of the brain,
Lyme Disease-Болезнь Лайма
tick-borne encephalitis клещевой энцефалит
Lyme disease, also known as Lyme borreliosis, is
an infectious disease caused by bacteria of
the Borrelia type
The most common sign of infection is an expanding
area of redness, known as erythema migrans, that
Adult deer tick, begins at the site of a bite about a week after it has
 Ixodes scapularis.
occurred. The rash is typically neither itchy nor painful
early symptoms include fever, headache, and feeling tired. If untreated, symptoms may
include loss of the ability to move one or both sides of the face, joint pains, severe
headaches with neck stiffness, or heart palpitations, Month to years later, repeated
episodes of joint pain and swelling may occur. Occasionally, people develop shooting pains or
tingling in their arms and legs.
About 10 to 20% of people despite appropriate treatment also
develop joint pains, have problems with memory, and feel tired
much of the time-reason= diffuse inflammatory process in the
basal ganglia and cerebral cortex
Dementia due to head trauma can Head Trauma
result from a single major head
injury (car accident) or from a The degree and
repeated series of head injuries,
[such those sustained by football nature of cognitive
players or boxers (sometimes impairment due to
called dementia pugilistica)] head trauma
depends on the
People with this type of location and severity
dementia often experience
amnesia, of the brain injury.
persistent memory loss, This kind of dementia is
most common among
irritability, young men who engage
problems sustaining attention, in highly risky behaviors
such as riding a
depression, motorcycle without a
apathy, helmet and driving while
intoxicated
and other personality changes
This is a clinical term to describe patients with personality
(frontal lobe) or language (temporal lobe) changes
Most commonly occurring in people who are
between 50 - 60 years old FRONTOTEMPORAL DEMENTIA=
life span -2-10 years after diagnosis
Pick's Disease &
Creutzfeldt-Jakob Disease (CJD)

Pick's Disease is the most common


of the fronto-temporal
dementias,
cell loss in the frontal lobes is
apparent
It may be sporadic or familial, and
an as yet unidentified mutation
on a gene on Chromosome 17 is
believed to be the cause of some
cases
Frontal lobe dementia- Pick's Disease is characterized by

 Drastic personality changes,


 Deterioration of social skills,
 Emotional blunting
 Memory problems
 Apathia or highly agitation /euphoria
 Jealous delusion
 Repeat the words of the other person
 Poor insight
 Patient cannot participate or focus on
answering questions
FTD Behavioral Variant-Examples of behavior changes: (1) Dorsolateral
Prefrontal: -Reduced problem-solving and reasoning (2) Orbitofrontal Prefrontal:
-Being impulsive or losing “filter” -Acting out in socially inappropriate ways -
Participating in risky behavior -Sexually risky behavior, gambling, theft -Becoming
fixated on topics (3) Medial Prefrontal: -Losing interest and motivation
Pick's disease, also known as
Frontotemporal Dementia
The characteristic histological feature of
this disease—a protein tangle that
appears as a large body in
neuronal tissue—is named a Pick
body
Brain MRI in Pick's disease

PiD -the presence of large,


dark-staining
aggregates of
proteins in neurological
tissue,
ballooned cells, which are
known as Pick cells

Ballooned neuron pick cell 


CJD occurs worldwide at a rate of about 1 case per
million population per year
 Personality changes
deutscher Neurologe  Anxiety
 Depression
 Memory loss
 Impaired thinking
 Blurred vision
 Insomnia
Hans-Gerhard  Difficulty speaking
Creutzfeldt
Alfons Maria Jakob.  Difficulty
swallowing
 Sudden, jerky
movements
variant (vCJD):
is caused by the consumption of food contaminated
with prions

• kuru was possibly transmitted through cannibalism in Papua


New Guinea when family members would eat the body of a
dead relative as a sign of mourning
• In the 1950s, cannibalism was banned in Papua New Guinea.
• In the late 20th century, however, kuru reached epidemic
proportions in certain Papua New Guinean communities,
therefore suggesting that vCJD may also have a
similar incubation period of 20 to 50 years.
• while mortuary cannibalism was banned in Papua New
Guinea in the 1950s, that does not necessarily mean that the
practice ended. 15 years later the practice continued
Frontal lobe dementia- CJD –a
rare Fatal brain CJD is characterized by
disorder that causes problems with attention,
inevitable death concentration, appetite,
within 2 to 12 month vision, and coordination,
memory problems
• The disorder usually
affects adults between 40
Mental deterioration
and 60 years of age, but
occurs very rapidly-
can occur at any age
hallucination ( seeing
• CJD is the human thing upside down),
equivalent of bovine ataxia, hyperreflexia,
spongiform delirium, dementia
encephalopathy or mad
cow disease,“
Frontal lobe dementia- Creutzfeldt-Jakob Disease (CJD)

There are 2 form of CJD 1) classic –arise spontaneously be


genetically transmitted
from 5-15% of CJD cases have a genetic component;
many people have no family history of the disorder
2) variant –is caused by eating diseased meat or cattle products
or receiving infected blood products
the disease is often transmitted through infected medical
equipment in procedures such as transplantation of the cornea
of the eye.
This newly discovered pathogen, "slow viruses" is called a prion
(proteinaceous infectious particle)
Prions transform normal protein molecules into infections one,
eventually eating spongy holes in the brain , that destroy the
person’s ability to function
Creutzfeldt–Jakob disease
 (/krɔɪtsfɛlt jɑ koʊb/ 
tonsil biopsy in variant CJD. Prion Protein
immunostaining

Spongiform change in CJD


Dementia due to Wernicke-
Wernicke-Korsakoff Syndrome Korsakoff Syndrome is
Wernicke-Korsakoff characterized by :
Syndrome results from a confusion,
deficiency in thiamine apathy (a lack of interest,
(Vitamin B1) and is often and/or emotion),
the result of chronic,
an inability to engage in
severe alcoholism
meaningful conversation
This syndrome can also result
( impaired ability to discuss
from general malnutrition,
severe eating disorders, or
current events or one's
the effects of outlook on life),
chemotherapy (often used hallucinations (sensing things
in cancer treatment). that are not truly present),
and severe memory
impairment
Multi-infarct dementia, also known as vascular
dementia, is the second most common form of
dementia after Alzheimer disease(AD) in older
adults

The term refers to a group of syndromes caused


by different mechanisms all resulting in
vascular lesions in the brain.

Early detection and accurate diagnosis are


important, as vascular dementia is at least
partially preventable
77% of stroke are thrombic or embolic , leading to ischemia of brain tissue
23% is hemorrhagic
Brain Attack-stroke-major psychiatric symptoms
cause of cerebrovascular dementia Of
Stroke include:
 cognitive
impairment
apathy
disordered emotions
(emotional
lability=pathological
laughing and
crying(execsive to the
situation)),
 mood disorders-
depresion 40—50%
Depressed people have greater platelet activation
Secretion of 5-HT by platelets produces aggregation or
clumping of blood cells, which makes people with
depression more susceptible to atherosclerosis, thrombosis
and vasoconstriction

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