Neurodegenerative disorders

Question 1

hypothyroidism, vitamin B12 deficiency, neurosyphilis, normal pressure hydrocephalus
are all what?

Answer 1

Reversible causes of Dementia

Question 2

Loss of dopaminergic neurons (depigmentation)

of substantia nigra pars compacta

Answer 2

Parkinson

Question 3

Lewy bodies are found in what disease?

Answer 3

Parkinson

Lewy body dementia

Question 4

MPTP, a contaminant in illegal drugs, is metabolized to MPP+, which is toxic and can cause

Answer 4

Parkinson

damage to Substania Nigra

Question 5

Atrophy of caudate and putamen with ex vacuo ventriculomegaly.

Answer 5

Huntington’s

Question 6

Tremor (pill-rolling tremor at rest)

Rigidity (cogwheel)

Akinesia (or bradykinesia)

Postural instability

Shuffling gait

Small handwriting (micrographia)

Answer 6

Parkinson

Question 7

Autosomal dominant trinucleotide (CAG)n repeat expansion

Answer 7

Huntington’s

Question 8

gene on chromosome 4

Neurodegenerative disease

Answer 8

HTT gene

Huntington’s

Question 9

chorea, athetosis, aggression, depression, dementia (sometimes initially mistaken for substance abuse).

Answer 9

Huntington’s

Question 10

Anticipation results from Tri nucleotide expansion in what codon and gene

seen in what disease?

Answer 10

Huntington’s

CAG –> HTT gene

Question 11

Neuronal death via NMDA-R binding and glutamate excitotoxicity.

Answer 11

Huntington’s

Question 12

High dopamine
Low GABA & ACh
in brain

Answer 12

Huntington’s

Question 13

Most common cause of dementia in elderly

Answer 13

Alzheimer’s

Question 14

Down syndrome patients haveincreased risk because APP is located on chromosome 21

Answer 14

Alzheimer’s

Question 15

Associated with the following altered proteins:

ApoE-2:______ risk of sporadic form

ApoE-4:______ risk of sporadic form

Answer 15

Alzheimer’s

2: decreased
4: increased

Question 16

APP, presenilin-1, presenilin 2: familial forms (10%) with risk of ________

Answer 16

earlier onset Alzheimer’s

Question 17

Wide spread cortical atrophy, especially hippocampus

Narrowing of gyri and widening of sulci

Answer 17

Alzheimer’s

Question 18

_______ are composed of extra cellular β amyloid core; found in gray matter

Amyloid β (Aβ) synthesized by cleaving amyloid precursor protein (APP)

In what disease?

Answer 18

Senile (Alzheimer) plaques
(looks like black dot aggregates)

Alzheimer’s

Question 19

Intracellular, hyper phosphorylated tau protein are insoluble cytoskeletal elements called?

Number of these correlates with degree of which dementia?

Answer 19

Neurofibrillary Tangles in Alzheimer’s
(Poppy’s Troll hair)

*Tangles consist Tau protein, which forms part of the microtubule.

Question 20

intracellular eosinophilic proteinaceous rods in hippocampus are called? In what’s disease?

Answer 20

Hirano bodies

Alzheimer’s

Question 21

Early changes in personality and behavior
(gambling, shopping, hypersexual, aggressive, etc.),

or aphasia (primary progressive aphasia).

May have associated movement disorders

Answer 21

Fronto-temporal lobe dementia (Pick’s disease)

due to Fronto-temporal lobe degeneration

Question 22

Round inclusions of hyper phosphorylated tau are called what and seen in what dementia?

Answer 22

Pick bodies  (Brownish/black spots on histo)
seen in Fronto-temporal lobe dementia (Pick's disease)

Question 23

Aggregates of ubiquitinated TDP-43

a DNA/mRNA binding protein

Answer 23

Fronto-temporal lobe dementia (Pick’s disease)

look brown

Question 24

Visual hallucinations

Answer 24

Lewy Body Dementia

Question 25

dementia with fluctuating cognition/alertness

Answer 25

Lewy Body Dementia

Question 26

REM sleep behavior disorder, and parkinsonism

Answer 26

Lewy Body Dementia

Question 27

Called _______ dementia if cognitive and motor symptom on set <1 year apart

Answer 27

Lewy body dementia *otherwise considered dementia 2° to Parkinson disease

Question 28

Intracellular Lewy bodies located primarily in the ____

Answer 28

cortex

Question 29

Result of multiple arterial infarcts and/or chronic ischemia.

Answer 29

Vascular Dementia

Question 30

Step-wise decline in cognitive ability with late-onset memory impairment

Answer 30

Vascular Dementia

Question 31

2nd most common cause of dementia in elderly

Answer 31

Vascular Dementia

Question 32

MRI or CT shows multiple cortical and/or subcortical infarcts.

Answer 32

Vascular Dementia

Question 33

Rapidly progressive (weeks to months) dementia with myoclonus (“startle myoclonus”) seizures and/or ataxia

Answer 33

Creutzfeldt -Jakob disease

Question 34

Commonly see periodic sharp waves on EEG

Answer 34

Creutzfeldt -Jakob disease

Question 35

Increased 14-3-3 protein in CSF

Answer 35

Creutzfeldt -Jakob disease

Question 36

Spongiform cortex (vacuolization without inflammation).

Answer 36

Creutzfeldt -Jakob disease

Question 37

Prions PrPc alpha sheets are converted to | PrPsc β pleated sheet resistant to ______.

Answer 37

Creutzfeldt -Jakob disease proteases *(look like purple specks with some white halos)

Question 38

α synuclein (eosinophilic inclusion) +

Answer 38

(Lewy bodies) Lewy body demetia and parkinson's

Question 39

Parkinsonism degeneration of midbrain and frontal subcortical white matter

Answer 39

Progressive supranuclear palsy

Question 40

Gait disturbances, vertical gaze palsy, abnormally phosphorylated Tau protein, loss of executive functions

Answer 40

Progressive supranuclear palsy

Question 41

composed of α synuclein | intracellular eosinophilic inclusions

Answer 41

``` Lewy Bodies (pink smooth dots, may have brown smudges near them) ```

Question 42

may cause amyloid angiopathy and/or intracranial hemorrhage | in what disease?

Answer 42

``` Senile Plaques (Aß core) Alzheimer's ```

Question 43

Giant Cells Infected Oligodendrocytes (viral) Cerebral atrophy Worsening memory

Answer 43

HIV assoc. Dementia

Question 44

AD is caused by a decrease in the acetylcholine level due to a deficiency of ________.  The decline in acetylcholine is most notable in the nucleus basalis of Meynert, which participates in memory and cognition.

Answer 44

choline acetyltransferase

Question 45

As PD progresses, the therapeutic window for levodopa narrows, possibly due to natural or levodopa-induced ____ degeneration.

Answer 45

naigrostriatal

Question 46

Ataxia early in disease Urinary incontinence Dilated ventricles w/o sulcal widening on neuroimaging cognitive dysfunction

Answer 46

Normal Pressure Hydrocephalus Wet Wobbly Wacky Reversible with LP drainage of CSF

Question 47

It is thought to be caused by a virus with a mutated or absent matrix protein that prevents mature (enveloped) virion particles from forming.  However, the virus continues to replicate intracellularly, leading to a persistent, nonproductive infection Accumulation of viral nucleocapsids within neurons and oligodendrocytes results in the formation of intranuclear inclusions and eventually leads to inflammation, demyelination, and gliosis. Patients have high titers of viral antibodies in the serum and cerebrospinal fluid (detectable as oligoclonal bands).  The disease causes various neurologic deficits and dementia, and is relentlessly progressive and fatal.

Answer 47

Subacute sclerosing panencephalitis (SSPE) | Measles

Question 48

In Huntington's, prominent atrophy is seen bilaterally in the caudate nucleus and putamen, leading to the enlargement of the ____ ventricles. 

Answer 48

frontal horns of the lateral ventricles

Question 49

___ agonist medications used to treat Parkinson disease have been associated with the abrupt onset of impulse control disorders,

Answer 49

Dopamine

Question 50

Nigrostriatal degeneration in Parkinson disease reduces activity of the ___ and its projections to the cortex, resulting in bradykinesia and rigidity. 

Answer 50

thalamus

Question 51

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the globus pallidus INTERNUS or ____ nucleus as it promotes thalamo-cortical disinhibition with improved mobility.

Answer 51

subthalamic

Question 52

Multiple ___ infarcts would be expected in vascular dementia.

Answer 52

lacunar

Question 53

Negri bodies are round eosinophilic inclusions seen in the cytoplasm of pyramidal HIPPOCAMPAL neurons and ____ cells in patients infected with the rabies virus.

Answer 53

cerebellar Purkinje

Question 54

MOA: decreases glutamate release

Answer 54

Riluzole | ALS

Question 55

Thin anterior roots Mild atrophy of the precentral gyrus Reccurrent INFECTIONS Loss of neurons in the anterior horn (LMN lesion) Degeneration and atrophy of the lateral corticospinal tracts (UMN lesion) Loss of neurons in the motor nuclei of CN V, IX, X, XII Muscle: denervation atrophy (small angular fibers)

Answer 55

ALS | Amyotrophic Lateral Sclerosis

Question 56

Patients with ALS most often die of

Answer 56

respiratory failure due to inspiratory muscle weakness (causing decreased vital capacity due to weak diaphragm), expiratory muscle weakness (causing a weak cough), bulbar dysfunction (causing dysphagia and chronic aspiration).

Question 57

____ may cause syncope due to enhanced parasympathetic tone that leads to bradycardia and atrioventricular block with reduced cardiac output.

Answer 57

``` Acetylcholinesterase inhibitors (donepezil, rivastigmine) for AD ```

Question 58

a peripheral nervous system autoimmune demyelinating  polyneuropathy that typically causes lower motor neuron manifestations (eg, areflexia, weakness) in a symmetric ascending pattern.

Answer 58

Guillain-Barré syndrome

Question 59

Vitamin B12 and __ deficiency can cause ataxia and loss of propioception (Neurological Symptoms).

Answer 59

E

Question 60

___ is seen in children more often than adults.  It manifests with irritability, loss of developmental milestones (in infants), or learning problems (in older children).  Gastrointestinal symptoms (eg, constipation, nausea, abdominal pain) also occur.

Answer 60

Lead intoxication

Question 61

damages the ventral white commissure, leading to bilateral loss of pain and temperature sensation that is limited to the affected levels (typically the arms and hands)

Answer 61

Syringomyelia | atrophy of intrinsic hand muscles also a feature

Question 62

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the ____ or subthalamic nucleus as it promotes thalamo-cortical disinhibition with improved mobility.

Answer 62

globus pallidus INTERNUS

Question 63

Refractory symptoms of Parkinson disease may benefit from high-frequency deep brain stimulation of the globus pallidus INTERNUS or subthalamic nucleus as it promotes _____ with improved mobility.

Answer 63

thalamo-cortical disinhibition

Question 64

Dopamine agonist medications used to treat Parkinson disease have been associated with the abrupt onset of ____ disorders

Answer 64

impulse control *Pramipexole, Ropinorole, Bromocriptine