4e. Subcortical dementias - Begrippen omgekeerd Flashcards
What does this refer to? And useful in which disease?
With an electrode 2 parts of the basal ganglia (Gb & Sn) are stimulated, leading to more dopamine. The electrodes go to the skull and then to a battery on the clavicula.
Deep Brain Stimulation
Parkinson
What does this refer to?
A combination of degeneration of the basal ganglia and asymmetric atrophy of the frontal and parietal lobes.
Symptoms:
- Dementia: there are features of dementia in the beginning of the disease, especially apraxia (=difficulties with making unvoluntary movements)
- Alien hand syndrome: the patient is not aware of the movements of his arm
A Parkinsonian disorder
Corticobasal Degeneration
What does this refer to?
Especially bradykinesia, but also dementia from the fronto-subcortical pattern, and a lot of falling as initial symptom.
Symptom:
1. Vertical …: difficulties in moving the eyes (they can’t look downward anymore)
A Parkinsonian disorder
Progressive Supranuclear Palsy
What does this refer to?
Difficulties with making unvoluntary movements
Apraxia
What does this refer to?
The patient is not aware of the movements of his arm
Alien hand syndrome
What does this refer to?
Difficulties in moving the eyes (they can’t look downward anymore)
Vertical supranuclear palsy
How is Binwanger’s disease also called?
Vascular dementia
What does this refer to?
- Arteriopathy = damage to the blood vessels, leading to infarcts and …
- … Leukoencephalopathy = white matter disease
Caused by:
A mutation on the NOTCH3 gene, which caused dementia and in 40% migraine with aura
Heriditary vascular dementia
CADASIL = Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leucoencephalopathy
What does this refer to?
Caused by: a mutation on the bètaPP gene, leading to really high mortality.
Conseuence of: amyloidosis = amyloid that sticks to the blood vessels in the brain and eventually they will break and there will be a bleeding
HCHWA-D (Katwijkse ziekte) = Hereditary Cerebral Hemorrhage With Amyloidosis – Dutch type
What does this refer to?
There is a change in the cerebrospinal fluid of the brain leading to enlargement of the ventricles and this leads to brain damage for a part reversible (the only dementia that is for a part treatable).
Normal Pressure Hydrocephalus (NPH)
What does this refer to?
Get some fluid out of the back > then the pressure gets lower > you can see whether there is an improvement of the symptoms.
Lumbar punction (in NPH)
What does this refer to?
From the brain to the stomach to get the CSF into the stomach > symptoms may improve.
Shunt (in NPH)
What does this refer to?
This is the most rare disease of the lectures. It’s a prion disease leading to holes in the brain filled with water and eventually spreading around the whole brain leading to a rapid process of dementia and deterioration.
Creutzfeld Jacob’s Disease (CJD)
What does this refer to?
- Fast progressive dementia (duration +- 4/5 months)
- 2 out of 4:
o Myoclonus = movement of the muscles
o Visual or cerebellar disorders = coordination problems
o (Extra)pyramidal signs
o Akinetic mutism = they can hardly speak at the end of the disease - Specific EEG abnormalities (see notes)
Classic CJD
What does this refer to?
- The following 3:
a. Progressive neuropsychiatric symptoms
b. Duration > 6 months
c. Routine care – no other explanation, no positive family history for spongiform encephalopathy - AND 4 out of 5:
a. Early psychiatric symptoms
b. Persistent painful sensibility disorders
c. Ataxia
d. Myoclonus, chorea or dystonia
e. Dementia
Variant CJD
