Cortical dementias
What is Dementia?
It is a syndrome due to disease of the
brain , usually of a chronic or progressive
nature , in which there is disturbance of
multiple higher cortical functions
(memory, thinking, orientation,
comprehension, calculation, learning
capacity, and judgement ).
Consciousness is not clouded.
Deterioration of emotional control, social
behavior and motivation.
Cortical dementia
Cortical Dementia
Cognitive functions such as language, praxis (the ability to
synthesize and sequence motor tasks), and visuospatial functions can
be impaired by diseases that affect the cortex, e.g., stroke, Alzheimer
disease, frontotemporal dementia, and Creutzfeldt-Jacob disease.
Often these diseases affect other aspects of hemispheric function,
such as memory (temporal lobe) and motivation (frontal lobes).
Cortical Vs. Subcortical dementia
The concept of subcortical vs. cortical dementia was introduced in
the 1970s based on the different clinical and neuropsychological
findings of different kinds of dementias. The reason the distinction is
clinically useful is that many of the so-called "reversible" dementias
present as sub-cortical dementias, whereas Alzheimer's dementia
presents as a cortical dementia. Making this differentiation may
therefore help in determining the prognosis.
The classic cortical dementia is dementia of the Alzheimers
type, which primarily involves the medial temporal cortex and
occipito-parietal cortex. Cortical dysfunction presents with
aphasia, memory loss with impaired memory storage, apraxia,
and problems with calculations, abstraction, and judgment.
Sub cortical dementias involve the caudate nucleus and the
thalamus; they can occur in conjunction with depression,
alcohol use, and many medications, as well as with
Huntington's Disease, Parkinson's, and Wilson's disease. Sub
cortical dementias may also occur in association with vascular
disease that affects sub cortical structures. Sub cortical
dementias typically present with cognitive slowing
(bradyphrenia), slowed memory retrieval, apathy, personality
changes, and executive dysfunction. However, language is
relatively preserved in comparison with cortical dementias.
Alzheimers Disease
Guidelines includes:
Presence of dementia.
Insidious onset with slow
deterioration.
Absence of other organic condition
that can induce dementia( e.g.
hydrocephalous, neurosyphilis).
Disturbance in executive functioning
(e.g. planning, reasoning).
Changes Caused by
Alzheimer's
Diminished blood flow
Neurofibrillary Tangles
Neuritic Plaques
Degeneration of hippocampus,
cerebral cortex, hypothalamus, and
brain stem
Normal vs AD Brain
Normal Alzheimers
brain brain
Changes in the Brain weight since the shrinkage due to AD
Comparing Normal section
with AD
CreutzfeldtJakob disease
or CJD is a degenerative neurological disorder (brain disease) that is
incurable and invariably fatal. CJD is at times called a human form of
mad cow disease (bovine spongiform encephalopathy or BSE) even
though classic CJD is not related to BSE; however, given that BSE is
believed to be the cause of variant CreutzfeldtJakob (vCJD) disease in
humans, the two are often confused.
In CJD, the brain tissue develops holes and takes on a sponge-like
texture. This is due to a type of infectious protein called a prion. Prions
are misfolded proteins which replicate by converting their properly folded
counterparts.
You know its CJD when
The first symptom of CJD is rapidly progressive
dementia, leading to memory loss, personality
changes and hallucinations.
This is accompanied by physical problems such
as speech impairment, jerky movements
(myoclonus), balance and coordination
dysfunction (ataxia), changes in gait, rigid
posture, and seizures.
The duration of the disease varies greatly, but
sporadic (non-inherited) CJD can be fatal within
months or even weeks (Johnson, 1998). In some
people, the symptoms can continue for years
Holes appear in staining of
brain.
onge like structural changes in brain with
CJD
Causes
The CJD prion is dangerous because it promotes refolding of native
proteins into the diseased state The number of misfolded protein
molecules will increase exponentially and the process leads to a large
quantity of insoluble protein in affected cells. This mass of misfolded
proteins disrupts cell function and causes cell death. Mutations in the
gene for the prion protein can cause a misfolding of the dominantly
alpha helical regions into beta pleated sheets. This change in
conformation disables the ability of the protein to undergo digestion.
Once the prion is transmitted, the defective proteins invade the brain
and are produced in a self-sustaining feedback loop.
TRANSMISSION:
Through harvest brain products for research work.
Use of Human Growth hormones.
Through Cannibalism.
Use of contaminated unsterilized surgical instruments.
Picks disease
Another important cortical form of Dementia
is Picks disease.
Pick's disease is a rare neurodegenerative
disease that causes progressive
destruction of nerve cells in the brain
A defining characteristic of the disease is build-up of
tau proteins in neurons, accumulating into silver-
staining, spherical aggregations known as "Pick
bodies".
A little history
Pick's disease is named after Arnold Pick, a professor of
psychiatry from the University of Prague who first
discovered and described the disease in 1892 by
examining the brain tissue of several deceased patients
with histories of dementia. As a result, the
characteristic histological feature of this diseasea
protein tangle that appears as a large body in neuronal
tissueis named a Pick body. In 1911, Alois Alzheimer
also noted the complete absence of senile plaques and
neurofilbrillary tangles as well as the presence of Pick
Bodies and occasional ballooned neurons.
Symptoms
The symptoms of Pick's disease include difficulty in
speech and thinking, behavioral changes, impaired
regulation of social conduct (e.g. breaches of
etiquette, tactlessness, disinhibition),
passivity, inertia, over-activity, pacing and wandering.
The changes in personality allow doctors to distinguish
between Pick's disease and Alzheimer's disease.
Pick's disease is one of the causes of the clinical
syndrome of frontotemporal lobar degeneration which
has three subtypes. Pick's disease pathology is
associated more with the frontotemporal dementia and
progressive nonfluent aphasia subtypes than the
semantic dementia subtype.
Picks cell bodies appear in stains/Brain
atrophy signs on Brain (right).
Pathophysiology behind
Picks!
PiD was first recognized as a distinct disease separate from other
neurodegenerative diseases because of the presence of large, dark-staining
aggregates of proteins in neurological tissue as well as the aforementioned
ballooned cells, which are known as Pick cells. Pick bodies are almost universally
present in patients with PiD, but some new cases of atypical Picks disease have
come to light that lack noticeable Pick bodies. A variety of stains can aid in the
visualization of Pick bodies and Pick cells, but immunohistochemical staining using
anti-tau and anti-ubiquitin antibodies have proven the most efficient and specific.
Case history: Pick's Disease
This 59 year old woman had a three year history of a progressive alteration in social behavior which included apathy and occasional disinhibition. Images reveal severe focal shrinkage of temporal and frontal lobes bilaterally.
Normal area Shrinking of fronto-temporal region
anks for the patience. From: Neh
