Lecture 4: Subcortical dementia Flashcards
What is the triad of symptoms in PD?
Bradykinesia (or akinesia), tremor, rigidity
Name some non-motor symptoms of PD.
- Olfactory dysfunction (early)
- Changes in personality and mood
- Excessive daytime sleepiness & REM sleep behavior disorder
- Autonomic dysfunction
- Psychotic symptoms
Explain the ‘dog figure’ in normal people vs. PD patients.
Normal: the thalamus is a station that serves as a filter for the cortex. It has an excitatory effect on the (motor) cortex, but it is inhibited by the globus pallidus and substantia nigra, so it’s never too active (dog on the leech).
PD: loss of dopamine > too much inhibition of thalamus > loss of excitation to the cortex > lack of motor activity (dog is doing nothing).
Which 3 clinical subtypes of PD do exist? Explain.
- Tremor-dominant: you can see their tremors really easy on the outside, but they have a mild disease progression.
- Akinetic-rigid: they hardly move (more severe cognitive impairment)
- Postural instability & gait difficulty: falling a lot, more severe form (more cognitive impairment and faster progression)
Which 3 (cognitive) domains are most pronounced in PD?
EF, memory (intact recognition vs. in AD), micrographia
Which 3 types of treatment can be offered to patients with PD?
- Medication: L-dopa is a dopamine precursor that replaces the diminished dopamine > especially motor functioning can be improved. It can give some side-effects (hallucinations, too much involuntary movements)
- Psychological: e.g. PEPP (support is very important)
- Deep Brain Stimulation (DBS): with an electrode 2 parts of the basal ganglia (Gb & Sn) are stimulated, leading to more dopamine. The electrode goes to the skull and then to a battery on the clavicula. You can’t have a lot of cognitive impairments for this surgery. The patient is awake during surgery. The patient still gets Levodopa besides DBS.
What are Parkinsonian disorders? Name them and explain.
They resemble PD but start in other parts of the brain and are more rare.
Corticobasal Degeneration (CBD): a combination of degeneration of the basal ganglia and asymmetric atrophy of the frontal and parietal lobes.
- Dementia: in the beginning of the disease, especially apraxia (difficulties with making voluntary movements in a certain order)
- Alien hand syndrome: the patient is not aware of the movements of his arm.
Progressive Supranuclear Palsy (PSP): especially bradykinesia, but also dementia from the fronto-subcortical pattern, and a lot of falling as initial symptom.
- Vertical supranuclear palsy: difficulties in moving the eyes (they can’t look down anymore)
What are the technical details of HD?
In a normal person there are 17-29 CAG repeats on the gene encoding for “huntingtin”, in HD patients there are 38+ repeats > it fabrics the wrong protein > it clumps in the basal ganglia > neural loss.
What are 2 motor symptoms present in HD?
Chorea and bradykinesia (or akinesia)
What kind of atrophy do you see in the early stages of HD? And in later stages?
Early stages: atrophy of the basal ganglia
Later stages: global atrophy
Explain the ‘dog figure’ in patients with HD.
In PD there is too much inhibition of the thalamus, in HD there is hardly any inhibition leading to too much excitation of the motor cortex here (the dog runs away)
What is one of the first cognitive symptoms in HD?
EF (attention, inflexibility, disinhibition)
Some kinds of HD subtypes perform better on the cognitive assessment than others. Which ‘subtypes’ are there and which one performs better?
Hyperkinetic patients (most of the patients) perform significantly better on the cognitive assessment than hypokinetic patients (hardly any movements).
Which form of vascular dementia has a slower onset?
Binwanger’s disease
Which domains are mostly affected in VD?
- Neglect (on the right side)
- EF
- Language/speech
- Cognitive slowing
Which 3 neurological symptoms may be present in VD?
Motor slowing, dysartria, hemiparesis
Which 2 heriditary vascular dementia do exist? Explain them.
CADASIL = cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy
It’s caused by the NOTCH3 gene, which causes dementia and in 40% migraine with aura. It leads to:
- Arteriopathy: damage to the blood vessels, leading to infarcts and …
- … Leukoencephalopathy: white matter disease
HCHWA-D (Katwijkse ziekte) = hereditary cerebral hemorrhage with amyloidosis - dutch type
It’s caused by a mutation on the bètaPP gene, leading to a really high mortality. It’s a consequence of:
- Amyloidosis: amyloid that sticks to the blood vessels in the brain and eventually causing a bleeding
What happens in NPH?
There is a change in the cerebrospinal fluid of the brain leading to enlargement of the ventricles and this leads to brain damage that is for a part reversible. It’s called idopathic because we don’t know the exact cause.
What are 2 distinct symptoms of NPH?
- Urinary incontinence
- Gait disturbances
- Walking apraxia (difficulties with starting walking)
- Magnetic walk (feet are stuck to the ground like magnetics)
What sentence lets you remember the symptoms present in NPH?
Wet, wacky, and wobbly
Which 2 types of treatment can be offered to patients with NPH?
- Lumbar punction: to get some fluid out of the back > then the pressure gets lower > improvement of symptoms
- Shunt: from the brain to the stomach to get the cerebrospinal fluid into the stomach
Especially the gait improves, and also the sphincter control (=the urinary continence is gone). But on cognition there is hardly any effect in most of the people
What happens in CJD?
It’s a prion disease leading to holes in the brain filled with water, eventually spreading around the whole brain, leading to a rapid process of dementia and deterioration.
Name 4 characteristics of sporadic/classic CJD.
Explain the last one. (3x)
- Fast progressive dementia (duration 4/5 months)
- Specific EEG abnormalities
- Cortical ribboning
- (Extra)pyramidal signs (variety of movement disorders)
- Myoclonus (brief, involuntary twitching of a muscle)
- Visual or cerebellar disorders (coordination problems)
- Akinetic mutism (they can hardly speak at the end of the disease)
Name 5 characteristics of variant CJD.
- Especially neuropsychiatric symptoms in the beginning
- Duration > 6 months
- Ataxia (coordination problems)
- Myoclonus/chorea/dystonia
- Persistent painful sensibility disorders
