BMB 2 - Dementia; Movement Disorders

Question 1

The left hippocampus is more responsible for which aspects of memory?

Answer 1

Language / verbal memory

Question 2

The right hippocampus is more responsible for which aspects of memory?

Answer 2

Visuospatial memory

Question 3

Which brain structure is largely responsible for linguistic and verbal memory?

Answer 3

The left hippocampus

Question 4

Which brain structure is largely responsible for visuospatial memory?

Answer 4

The right hippocampus

Question 5

Which brain structure is largely responsible for emotional memory?

Answer 5

The amygdala

Question 6

Which brain structure is largely responsible for procedural memory?

Answer 6

The basal ganglia and cerebellum

Question 7

Name two memory-processing structures connected by the fornix.

Answer 7

The hippocampus and the mamillary bodies

Question 8

Which neural loop involves the hippocampus and is largely responsible for episodic, declarative memory?

Answer 8

The Papez circuit

Question 9

Which neural loop involves the amygdala and is largely responsible for emotional memory?

Answer 9

The Yakolev circuit

Question 10

What are the major structures of one hemisphere of the Papez neural loop?

Answer 10

The cingulate gyrus, parahippocampal gyrus, hippocampus, fornix, and mamillary body

Question 11

What are the major structures of one hemisphere of the Yakolev neural loop?

Answer 11

Tha amygdala, thalamus, and prefrontal/temporal cortices

(the hypothalamus and brainstem also have input)

Question 12

What is the most important risk factor for Parkinson’s disease?

Answer 12

Age

Question 13

You tap on a patient’s forhead between their eyes (on their glabella); they blink slowly and late.

What is this called?

Answer 13

The Myerson (or glabella) sign

(indicates Parkinson’s)

Question 14

What percentage of Parkinson’s disease patients have depression?

Answer 14

50%

Question 15

Name some of the S/Sy of Parkinson’s disease.

Answer 15

Gait disturbance;

resting tremor;

depression;

Myerson (glabella) sign;

bradykinesia;

cogwheel rigidity;

cognitive impairment;

pin-wheel tremor;

postural instability;

ANS dysfunction

Question 16

What is the major etiology of Parkinson’s disease?

Answer 16

Degeneration of the substantia nigra pars compacta

Question 17

What is the classic triad of Parkinson’s disease?

Answer 17

Resting tremor

Bradykinesia

Rigidity

Question 18

Parkinson’s disease has close associations with which form of neuropathology and dementia?

Answer 18

Lewy body

Question 19

Are Lewy bodies present in Parkinson’s disease?

Answer 19

Yes, in the substantia nigra pars compacta

Question 20

Where within the cell are Parkinsonian Lewy bodies found?

Answer 20

As intranuclear deposits

Question 21

Parkinsonian symptoms with early-onset dementia is likely _______________.

Parkinsonian symptoms with late-onset dementia is likely _______________.

Answer 21

Parkinsonian symptoms with early-onset dementia is likely Lewy body dementia.

Parkinsonian symptoms with late-onset dementia is likely Parkinson’s disease.

Question 22

What are some of the general pharmacological treatment options for Parkinson’s disease?

Answer 22

Levodopa

Dopamine agonists

Amantadine

MAO inhibitors (monoamine oxidase)

CMOT inhibitors (catechol-o-methyl transferase)

(also therapy and deep-brain stimulation)

Question 23

What is the general pharmacological treatment for Lewy body dementia?

Answer 23

Cholinesterase inhibitors

Question 24

Alzheimer’s disease most severely damages which portions of the brain?

Answer 24

The hippocampus;

the posterior cortical areas

Question 25

Alzheimer's disease is associated with a loss of which neurotransmitter from which nucleus?

Answer 25

Acetylcholine; the basal nucleus of Meynert

Question 26

Alzheimer's disease is associated with what neuropathology?

Answer 26

**Tau** neurofibrillary tangles and **beta**-**amyloid** plaques

Question 27

What is the most important risk factor for Alzheimer's disease?

Answer 27

Age

Question 28

What are the genetics of Alzheimer's disease?

Answer 28

***APoE4*** on chromosome **19** increases risk | (***APoE2*** is protective)

Question 29

Early-onset Alzheimer's disease has an onset before what age?

Answer 29

**65 years** | (usually in one's 40s or 50s)

Question 30

What are the genetics of early-onset, familial Alzheimer's disease?

Answer 30

APP, PSEN1, PSEN2

Question 31

What is the mode of inheritance of familial (early-onset) Alzheimer's disease?

Answer 31

Autosomal dominant

Question 32

Most cases of Alzheimer's disease are ____________ (sporadic/familial).

Answer 32

Most cases of Alzheimer's disease are **_sporadic_** (\>95%).

Question 33

**True/False**. Most cases of both Parkinson's disease and also Alzheimer's disease are sporadic and not likely to be inherited.

Answer 33

True.

Question 34

What is the most common form of dementia? And second?

Answer 34

**Alzheimer's disease** (40%) **Vascular dementia** (20%)

Question 35

**True/False**. Vascular dementias can result due to both large-vessel multiinfarcts or chronic small-vessel disease.

Answer 35

True.

Question 36

Describe some of the features of vascular dementia.

Answer 36

Symptoms of Alzheimer's disease + chronic microvascular white matter changes

Question 37

Describe some of the features of frontotemporal dementia (Pick's disease).

Answer 37

Associated with **chromosome** **17** abnormalities **Pick** **bodies** in frontal and temporal lobes **Loss** of **inhibition** **Progressive** **aphasia**

Question 38

Describe some of the features of Lewy body dementia.

Answer 38

**Corticobasilar** degeneration **Cognitive** and **motor** symptoms Mild **memory** **loss**, **executive functioning problems**, **magnetic** **apraxia**

Question 39

What part of the brain routes input from the cerebellum and basal ganglia to the cortex for control of motor movement?

Answer 39

The **ventral** **lateral** thalamus

Question 40

What part of the brain is targeted by deep-brain stimulation (e.g. for Parkinson's disease or essential tremor)?

Answer 40

The **ventral** **lateral** thalamus

Question 41

Describe the typical etiology and presentation of Huntington's disease.

Answer 41

**CAG** repeats on chromosome **4** --\> Accumulation of abnormal **huntingtin** protein --\> Degeneration of the **caudate** --\> Loss of motor inhibition by basal ganglia --\> **Chorea** / **Hemiballismus** / **Depression** / **Dementia**

Question 42

The motor S/Sy of Huntington's disease largely result due to deposition of the ___________ protein in the medium _________ neurons of the caudate nucleus.

Answer 42

The motor S/Sy of Huntington's disease largely result due to deposition of the **_huntingtin_** protein in the medium **_spiny_** neurons of the caudate nucleus.

Question 43

What is the mode of inheritance of Huntington's disease?

Answer 43

Autosomal dominant

Question 44

Name the relevant, respective symptomatic treatment(s) for each of the following aspects of Huntington's disease: **Chorea** --- **Depression**​ --- **Psychosis**​ --- **Cognition**​ ---

Answer 44

Name the relevant, respective symptomatic treatment(s) for each of the following aspects of Huntington's disease: **Chorea** --- _VMAT2 inhibitors_ *(e.g. tetrabenazine) (decreasing dopamine)* **Depression**​ --- _SSRIs_ **Psychosis**​ --- _Antipsychotics_ **Cognition**​ --- _No known treatment_

Question 45

What are some of the general symptomatic treatments for dementia?

Answer 45

_Acetylcholinesterase inhibitors_: **donepezil**, **galantamine**, and **rivastigmine** _N-methyl-D-aspartate antagonists_: **memantine** _**Diet**, **exercise**, **sleep**_

Question 46

Alzheimer's disease alone is responsible for \_\_\_\_\_% of cases of dementia. Vascular dementia alone is responsible for \_\_\_\_\_% of cases of dementia. Frontal and mixed dementias (e.g. Pick's, Lewy body, progessive aphasia, etc.) are responsible for \_\_\_\_\_% of cases of dementia. Other types (e.g. Parkinson's, TBIs, toxins, infections, metabolic issues, etc.) are responsible for \_\_\_\_\_% of cases of dementia.

Answer 46

Alzheimer's disease alone is responsible for **_40_** % of cases of dementia. Vascular dementia alone is responsible for **_20_** % of cases of dementia. Frontal and mixed dementias (e.g. Pick's, Lewy body, progessive aphasia, etc.) are responsible for **_25_** % of cases of dementia. Other types (e.g. Parkinson's, TBIs, toxins, infections, metabolic issues, etc.) are responsible for **_15_** % of cases of dementia.

Question 47

What is a tic?

Answer 47

A premonitory urge to move

Question 48

What is the definition of chronic tic disorder?

Answer 48

_Either_ ≥ 1 motor _or_ ≥ 1 verbal tics that began before age 18 and are present for ≥ 1 year

Question 49

Define Tourette's syndrome.

Answer 49

A tic disorder with onset of symptoms **before** **18** years of age; **≥ 2 motors tics and ≥ 1 vocal/phonetic tics** throughout the overall course of the disease

Question 50

What are the major comorbidities associated with Tourette's syndrome?

Answer 50

**OCD** and **ADHD**

Question 51

What are the phonetic tics often seen in Tourette's syndrome?

Answer 51

**Throat clearing, coughing, grunting, anything that makes sounds** **Coprolalia**: shouting of obscenities *(only 5-15% of patients)*

Question 52

What are the motor tics often seen in Tourette's syndrome?

Answer 52

**Copropraxia**: obscene movements

Question 53

Name some of the treatments available for reducing the burden of tic disorders generally (and Tourette's syndrome specifically).

Answer 53

Agents which **deplete or block dopamine** **Clonazepam** **Botulinum** toxin injections

Question 54

Describe essential tremor.

Answer 54

A **familial**, **bilateral** **movement** **disorder**

Question 55

Does alcohol help or exacerbate the involuntary motor movement of essential tremor?

Answer 55

Help

Question 56

Name some of the treatments used for managing essential tremor.

Answer 56

**Topiramate** (first-line) **Propranolol** (_not_ for the elderly) **Primidone** **Deep brain stimulation**

Question 57

How does cerebellar ataxia typically present?

Answer 57

Slow, progressive worsening of symptoms over the years involving **speech**, **swallowing**, **coordination**, **gait**, or **ataxia**

Question 58

How is cerebellar ataxia managed?

Answer 58

**_Only_ by treating underlying causes** (e.g. repleting vitamin deficiency, giving steroids for inflammatory conditions, treating underlying cancers, etc.)

Question 59

What is generically meant by the term 'dystonia?'

Answer 59

Sustained, abnormal muscle contractions (e.g. as an adverse reaction of antipsychotic use)

Question 60

Name some of the adverse motor effects of antipsychotic use.

Answer 60

Dystonia Akathisia Tardive dyskinesia

Question 61

How is generalized dystonia treated? How about acute focal dystonia? How about dystonia due to antipsychotic use?

Answer 61

Baclofen Botulinum toxin IV anticholinergics

Question 62

How are tardive syndromes (i.e. dystonia, akathisia, dyskinesia) treated?

Answer 62

_Removal of the offending agent_ (usually an antipsychotic); tetrabenazine; symptomatic management