>>Neurodegenerative diseases; age-dependent progressive deseases, caused by degeneration of the central
nervous system → breakdown of neurons in the peoples brains,
All neurodegenerative disorders are (patho-)physiologically (at the level of the neuroanotomy and the way
neurons communicate with each other) characterized by the following aspects
•Affect “specific subsets of neurons”
•without clear explanation and could be either inherited or acquired →
•“Progress relentlessly”
•Are often age-related, increasing in frequency with advancing age (if it is inherited it may occur at a younger
age)
•Are often accompanied by microscopic signs of four stages of disorder:
•Neuronal pathology (some kind of inflammation that is affecting neurons)
•Neuronal cell death (dissapaearance of the neurons)
•Disappearance of neuronal cell bodies
•Glial proliferation (i.e. cells that support neurons in the CNS, eg. Microglia, Schwann cells, etc.)
« ... brief overview of common clinical features of neurodegenerative diseases » :
1. chronic clinical course is relentlessly progressive until death.
2. disorder is not reversible (you can only help) by any known therapy: drug therapy or gene therapy may give
marginal and temporary improvement.
3. phenotypic variability (is changes from patient to another) is commonly seen (i.e. different expression of
same genetic changes)
4. cognitive impairment and dementia are common manifestations in neurodegenerative (the pathology that
is causing our neurons to dye, )disorders but are not seen in all forms. Types can be differentiated by formal
neuropsychological tests.
5. condition appears to be heritable in a small percentage of cases (but it is usually the lower percentages)
6. In the familial form of the disease, the onset occurs up to a decade before onset of the sporadic form of the
disease.
7. several different neurodegenerative diseases may appear together within a family. (there may be a weakness in
the genome)
8. different clinical manifestations are mediated by dysfunction of different anatomicalregions of degeneration.
9. features of more than one neurodegenerative diseases may appear to coexist in one patient (e.g. ALS and
FTLD).
10. advances in genetics of neurodegenerative diseases have demonstrated that diverse clinical phenotypes may
share similar genotypes, and that clinically similar phenotypes may be associated with a wide variety of
genotypes.
,>> niet kennen voor het examen, it shows the catogorization of sorts of dimentia
Different types of neurons that are associated with different disseases
*refer to neurotransmitters that these neurons are susceptible to
These are neurotransmitters; in some cases they exite and in some cases they inhibit through the responses they
release (picture right)
Parkinsons disease have difficulties with motor control bcs it is linked with dopamine (and depression)
(speech&) lanuage charecteristics in neurodegenerative deseases
,Microtubule falls apart >> so there can’t be transport to neurons = disfonction of neurons that needs to
be neurished → creates cell deads → effects the cerebral cortex and hoppocampus in the first stage
→ Prevalence is low in case of younger people and over 85 there is an exponantion increase in terms
of prevalence
→ there can be a genetic factor, but there are also lifestyle and environmental influences
→ agnosia = unawareness
>> There are different ways of diagnosis based on diagnostic criteria
>> For the dsm5 (diagnostic manual for psychiatrists) = accuracy rate of 65 – 96 → there is some
variability
, 3phases of the disease
Early phase → Anomia = is related to memory issues, is the difficulty to name objects
Mnestic issues influence anomia
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