Lecture Subcortical Dementia

Question 1

What are symptoms/ the clinical diagnoses of PD?

Answer 1

Bradykinedia AND at least tremor during rest or rigidity

Question 2

What are the charactaristics in the brain that determine the definite diagnosis of PD post-mortem

Answer 2

Depigmentation of the substantia nigra and lewy body in the brainstem

Question 3

PD has six stages, where does it start?

Answer 3

Starts in the lowe brain stem then develops further into the brain to eventually cortical areas

Question 4

In Huntingtons, the indirect pathway becomes less/ more active leading to over-/ understimulation of the thalamus.

In Parkinson’s, the indirect pathway becomes less/ more active leading to over-/ understimulation of the thalamus

Answer 4

In Huntingtons, the indirect pathway becomes less active (too little inhibition) leading to overstimulation of the thalamus.

In Parkinson’s, the indirect pathway becomes more active (too much inhibition) leading to understimulation of the thalamus.

Question 5

What are the 3 clinical subtypes of PD?

Answer 5

• Tremor-dominant: Mild disease progression
• Akinetic-rigid: More severe cognitive impairment
• Postural instability and gait difficulty: Cognitive
  impairment and severe disease progression

Question 6

What are the most pronounced cognitive problems in patients with PD?

Answer 6

• Executive functioning (e.g. bradyphrenia = sloweness
  of thought)
• Memory: Retrieval inefficiencies (free recall), relative intact
  recognition
• Micrographia = small cramped handwriting

Question 7

What is the difference between PD Dementia and Lewy Body Dementia?

Answer 7

Dementia in PD has insidious onset and slow progression. Lewy Body dementia and motor problems progress in the same year.

Question 8

What is vascualr parkinsonism?

Answer 8

Vascualar dementia caused by damage in motor areas of the brain

Question 9

What are 3 treatment forms for PD?

Answer 9

1. Levodopa
2. Psychological interventions
3. Deep Brain Stimulation (Globus pallidus and subthalamic nucleus are stimulated -> More dopamine -> Improves symptoms)

Question 10

Which parkinsonian disorders resemble PD but start in other parts of the brain?

Answer 10

Corticobasal degeneration (CBD) with symptoms: alien hand and dementia apraxia

Progressive supranucleus palsy (PSP) with symptoms: eye movement disorders and dementia fronto-subcortical pattern

Question 11

If one of the parents had HD how great is the chance of inheriting the disease?

Answer 11

50% (autosomal dominant)

Question 12

What are the two different types of movement disturbances patient’s with HD can have?

Answer 12

Hypokinectic: hardly any movement
Hyperkinectic uncontrollable movement

Question 13

What are some typical motor problems in patient’s with HD?

Answer 13

Chorea: excessive unwanted movements
Bradykinesia: Slowing of wanted movements

Question 14

On cognitive aspect what is one of the first symptoms in patient’s with HD?

Answer 14

Problems in executive functioning: attention, visual, inflexibility, disinhibition

Question 15

What are characteristics of the brain we see in MRI in the early stages of HD?

Answer 15

Atrophy of the basal ganglia, esp. striatum

Question 16

What are characteristics of the brain we see in vascular dementia?

Answer 16

White matter hyperintensities (hard to differentiate from MS) & microbleeds leading to change in blood flow

Question 17

What is the difference between mild vascular cognitive disorder and major vascular cognitieve disorder

Answer 17

Mild has no or minor interference. Major = vascular dementia

Question 18

What are large vessel disease and small vessel disease?

Answer 18

Large vessel disease: Strategic infarct or multi-infarcts (multi-infarct dementia)
Small vessel disease: Lacunar infarcts, white matter lesions, microhemorrhages

Question 19

What are the most pronounced cognitive deficits in vascualr dementia?

Answer 19

EF, fatigue & language

Question 20

CADASIL and HCHWA-D are two types of hereditary vascular dementias. What happends in the brain?

Answer 20

CADASIL: Artereophaty = damage/ abnormal blood vessels. Leads to white matter disease.
HCHWA-D: Amyloid sticks to blood vessels in the brain, they break leeds to bleeding.

Question 21

What do little blackholes on a MRI indicate?

Answer 21

Microbleeds

Question 22

What happends in the brain in normal pressure hydrocephalus?

Answer 22

Change in CSF leads to enlargement of ventricles leading to brain damage (crowding of the brain tissue)

Question 23

Where does Creutzfeld Jacob’s Disease (CJD/ Mad cows disease) leads to?

Answer 23

Disease leads to holes in the brain filled with water spreading to the whole brain leading to a rapid process of deterioration.

Question 24

There are two types of Creutzfeld Jacob’s Disease; CJD Classic and Variant CJD what are the main differences between them?

Answer 24

Classic = progressive dementia, with specific EEG signs
Variant = progressive neuropsychiatric, no positive family history, you see no sign on EEG

Question 25

What classical symptoms are absent in subcortical dementia?

Answer 25

Aphasia, apraxia, and agnosia

Question 26

How do subcortical dementias differ from cortical dementias?

Answer 26

In subcortical dementias, cognitive impairments involve the fundamental functions. These include arousal, attention, processing speed, motivation, and emotionality (crucial for survival)

In contrast, in cortical dementias, the specific functions affected include language abilities, reasoning and problem solving, learning, and praxis, (carry out behavior and are the most highly evolved of human activity)

Question 27

What is the extrapyramidal motor system and how does it differ from the pyramidal motor system?

Answer 27

The extrapyramidal motor system includes the basal ganglia (caudate, putamen, and globus pallidus), subthalamic nucleus, substantia nigra, and their interconnections to each other and to thalamic nuclei. It modulates movement and maintains muscle tone and posture.

In contrast, the pyramidal motor system consists of upper and lower motor neurons that guide purposeful and voluntary movement.

Question 28

What are the two types of movement disorders that can be conceptualized in relation to the basal ganglia?

Answer 28

Movement disorders can be conceptualized as having either excessive abnormal involuntary movements (dyskinesia) or halting initiation and slowed execution of directed movement (akinesia or bradykinesia).

Question 29

What are the common symptoms of Parkinson’s disease?

Answer 29

Tremors, bradykinesia, akinesic difficulty initiating movement and muscular rigidity

Question 30

What are the risk factors for Parkinson’s disease?

Answer 30

Genetic and environmental factors, with the majority of cases having unknown etiology.

Smoking has been identified as a reverse risk factor, as smokers are less likely to develop Parkinson’s disease than nonsmokers. Coffee drinking has also been linked to a lower risk of the disease, particularly in men.

Question 31

What are the subtypes of Parkinson’s disease?

Answer 31

The subtypes of Parkinson’s disease include early disease onset, tremor dominant, non-tremor dominant, and rapid disease progression with dementia

Question 32

Which neuropsychiatric disorder is very common in PD?

Answer 32

Depression

Question 33

What are the three main symptom of Huntington’s disease?

Answer 33

Motor disturbance, cognitive impairment, and psychiatric disorders.

Question 34

What is the cause of Huntington’s disease?

Answer 34

Huntington’s disease is caused by an excessive number of trinucleotide CAG repeats in the HD gene that encodes the protein huntingtin located on chromosome 4.

Question 35

How is clinical diagnosis of Huntington’s disease typically made?

Answer 35

Clinical diagnosis of Huntington’s disease is typically made by determination of an otherwise unexplainable and characteristic extrapyramidal movement disorder with appropriate family history.

Question 36

Is Huntington’s disease an autosomal dominant disease?

Answer 36

Yes, Huntington’s disease is an autosomal dominant disease, meaning that if a person carries the HD gene, there is a 50% chance that their offspring will also have the disease if they live long enough.

Question 37

What brain structures are typically affected in Huntington’s disease?

Answer 37

Mainly the caudate nucleus and putamen in the corpus striatum

Question 38

Is the severity of symptoms and the speed of progression correlated with the age of onset in Huntington’s disease?

Answer 38

Yes, the earlier the onset of Huntington’s disease, the more severe the symptoms and the faster its progression.

Question 39

What is the Unified Huntington Disease Rating Scale used for?

Answer 39

The Unified Huntington Disease Rating Scale was developed to facilitate disease characterization for research purposes.

Question 40

Can psychiatric disturbance or dementia precede the appearance of obvious motor responses in Huntington’s disease?

Answer 40

Yes, in more than half of cases, psychiatric disturbance or dementia precedes the appearance of obvious motor responses in Huntington’s disease.

Question 41

What are the key features of memory deficits in Huntington patients?

Answer 41

Defective retrieval, which appears most prominently on free recall trials and visual memory deficits

Question 42

What is the most common psychiatric disorder in Huntington patients?

Answer 42

Depression