Pick Dementia

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 Objectives:

 What Is Pick's Disease?

 Causes
 Symptoms
 Pick's Disease vs. Alzheimer's Disease
 Diagnosis
 Treatment
 Creutzfeldt-Jakob disease (CJD)
 About CJD
 Clinical and Pathologic Characteristics
 Occurrence and Transmission
 Treatment
 What Is Pick's Disease?
 Pick's disease is a kind of dementia similar to Alzheimer's but far less common. It affects parts of the
brain that control emotions, behavior, personality, and language. It's also a type of disorder known as
frontotemporal dementia (FTD) or frontotemporal lobar degeneration (FTLD).

 Your brain uses a transport system to help move around the nutrients it needs. This system is made
of proteins that like railroad tracks guiding trains guide nutrients where they need to go. The proteins
that keep the tracks straight are called tau proteins.

 When you have Pick's disease, the tau proteins don't work the way they should. You may also have
more of them in your brain than other people.

 These abnormal clumps of tau proteins are called Pick bodies. Pick bodies "derail" your transport
system. The track is no longer straight, and nutrients in the brain can't get where they need to go.
This causes brain damage that can't be reversed.
 Causes, ETIOLOGY:

Up to 25% of people with Pick's disease received a gene that causes it

from a parent. Experts aren't sure why it happens in other cases.

Pick disease is a taupathy, with accumulation of abnormal

tau protein in the brain.
Tau protein is a highly soluble microtubule-associated
protein (MAPT) and promotes microtubule polymerization
and stabilization. Tau protein in the brain is heterogeneous,
due to alternative splice forms and post-translational
modifications.
Accumulation of abnormal protein leads to progressive
neuronal dysfunction and loss

Genetic associations
3-repeat Pick disease has been shown to be associated with
mutations in MAPT on exons and introns 9 and 10. Three
missense mutations in exon 9, one mutation in intron 9 ,one
deletion mutation in exon 10 and one mutation in intron 10
and exon 12 have been reported.
 Symptoms:
Pick bodies typically form in the frontal and temporal lobes
of the brain. These sections control your behavior,
personality, and speech. Symptoms usually show up in
those areas.

You may:
o Act aggressively toward others
o Be uninterested in everyday activities
o Be very aware of everything you do all
the time
o Feel irritable or agitated
o Have drastic and quick mood swings
o Have trouble feeling warmth,
sympathy, or concern for others
o Have trouble with unplanned activities
o Make rash decisions
o Repeat actions over and over
o Say and do inappropriate things

Some people become hungry all the time, and

some develop an unhealthy "sweet tooth" and
eat much more sugar than they should.
 Pick's Disease vs. Alzheimer's Disease
Pick's disease has many of the same causes and symptoms that Alzheimer's does. But there are key differences.

Unlike people with Alzheimer's disease, people with Pick's disease:

o Are diagnosed earlier in life

o Don't have hallucinations or delusions
o Don't tend to get lost in familiar places
o Have a harder time making sense of their words or the words
of others
o Have behavior problems early on (behavior problems usually
come late in Alzheimer's)
o Don't have as many memory loss problems
 Diagnosis:
To find out if you have Pick's disease, your doctor will ask about your
symptoms and go over your medical history. Then you'll have special tests
that check your memory, behavior, language, and other mental functions.
These are usually pencil and paper tests. You'll answer questions in writing
and may be asked to draw certain objects.

The doctor may also recommend a blood test

that looks at your DNA to see if you have the
gene that causes Pick's disease.

To get a better picture of what's happening in your brain, your doctor

may order imaging tests, such as:

Magnetic resonance imaging (MRI): Powerful magnets and radio

waves are used to make detailed images of your brain.
Single-photon emission computed tomography (SPECT) or a positron
emission tomography (PET) scan:
A radioactive substance and a special camera create 3-dimensional
pictures that show what areas of your brain are more or less active.

You may also have a lumbar puncture. Your doctor will use a long needle to take a small amount of fluid from an area near your spine for screening. In rare
cases, your doctor might want to take a small amount of your brain tissue to test. This is called a biopsy.
 Treatment:
 There's no cure for Pick's disease, and medications can't
slow it down. It can progress slowly, but usually it steadily
gets worse over time. Some people live as long as 10 years
with the disease.

 Your doctor can recommend treatment to help you

deal with many of your symptoms. They may suggest
behavioral therapy to help control any dangerous
behavior and antidepressants to help with agitation or
aggression.
 About Creutzfeldt-Jakob disease:
 Classic CJD is a human prion disease. It is a neurodegenerative disorder with
characteristic clinical and diagnostic features. This disease is rapidly
progressive and always fatal. Infection with this disease leads to death usually
within 1 year of onset of illness.

 Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal

neurodegenerative disorder believed to be caused by an abnormal isoform of a
cellular glycoprotein known as the prion protein. CJD occurs worldwide and the
estimated annual incidence in many countries, including the United States, has
been reported to be about one case per million population.

 The vast majority of CJD patients usually die within 1 year of illness onset. CJD
is classified as a transmissible spongiform encephalopathy (TSE) along with
other prion diseases that occur in humans and animals. In about 85% of
patients, CJD occurs as a sporadic disease with no recognizable pattern of
transmission. A smaller proportion of patients (5 to 15%) develop CJD because
of inherited mutations of the prion protein gene. These inherited forms include
Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.
 Clinical and Pathologic Characteristics:
Clinical and Pathologic Characteristics Distinguishing Classic CJD from Variant CJD

Characteristicv Classic CJD Variant CJD

Median age at death 68 years
Median duration of illness 4-5 months
Clinical signs and symptoms Dementia; early neurologic
signs
28 years
13-14 months
Prominent psychiatric/behavioral
symptoms; painful dysesthesias;
delayed neurologic signs

Periodic sharp waves on Often present Often absent

electroencephalogram
“Pulvinar sign” on MRI* Not reported Present in >75% of cases
Presence of “florid plaques” on Rare or absent Present in large numbers
neuropathology
Immunohistochemical analysis of brain Variable accumulation Marked accumulation of protease-
tissue resistance prion protein

Presence of agent in lymphoid tissue Not readily detected Readily detected

Increased glycoform ratio on Not reported Marked accumulation of
immunoblot analysis of protease- protease-resistance prion
resistance prion protein protein
 Occurrence and Transmission:

 Classic CJD has been recognized since the early 1920s. The most common form of classic
CJD is believed to occur sporadically, caused by the spontaneous transformation of
normal prion proteins into abnormal prions. This sporadic disease occurs worldwide,
including the United States, at a rate of roughly 1 to 1.5 cases per 1 million population
per year, although rates of up to two cases per million are not unusual. The risk of CJD
increases with age; the 1979-2018 average annual rate in the United States was 3.6
cases per million in persons 50 years of age or older.

 Whereas the majority of cases of CJD (about 85%) occur as sporadic disease, a smaller
proportion of patients (5-15%) develop CJD because of inherited mutations of the prion
protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome
and fatal familial insomnia.
 Diagnosis And
Treatment

o Diagnosis of CJD typically entails spinal tap,

electroencephalography, and other procedures to
assess neurological function in order to rule out
conditions that might produce similar symptoms.
Diagnosis is confirmed through brain biopsy, in
which a small section of tissue is removed from
the brain and examined in a laboratory.
o Scientists are developing tests capable of
detecting prions in cerebrospinal fluid and blood.
Such tests could enable early diagnosis and
improve prion screening for blood transfusions.
o There is no known cure for CJD, nor can the
progression of the disease be delayed by
medication or surgery. Hence, treatment is
supportive, being aimed primarily at minimizing
pain and discomfort
Thank you for the
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