Behavioral Neurology

Question 1

Synucleinopathies

Answer 1

PD, MSA, DLB

Question 2

Alpha-synuclein aggregate in cytoplasmin of neurons - found in what disorder?

Answer 2

= Lewy bodies

DLB

Question 3

Tau-positive astrocytes - found in what disorder?

Answer 3

= “tufted astrocytes”

PSP

Question 4

PSP findings on MRI

Answer 4

midbrain atrophy

“Hummingbird sign” / “Mickey Mouse sign”

Question 5

Clinical findings in PSP

Answer 5

parkinsonism, falls, supranuclear gaze palsy

Question 6

Clinical findings in MSA

Answer 6

parkinsonism, ataxia, autonomic dysfunction

Question 7

MSA findings on MRI

Answer 7

atrophy of cerebellum, pons, inferior olives

“hot cross bun sign” / “putaminal rim sign”

Question 8

Clinical findings in CBD

Answer 8

parkinsonism, apraxia, aphasia, alien hand syndrome

Question 9

Ballooned neurons - found in what disorder?

Answer 9

CBD, Pick’s

Question 10

Clinical findings in DLB

Answer 10

parkinsonism, dementia (cognitive fluctuations), autonomic dysfunction, visual hallucinations

Question 11

Neurofibrillary tangles - found in what disorder?

Answer 11

AD

Question 12

Tauopathies

Answer 12

AD, PSP, FTD, CBD, CTE, Parkinson-Dementia Complex of Guam

Question 13

Define “aphemia”

Where is the lesion that leads to this?

Answer 13

Problem with speech production (ability to write is preserved)

Small lesion in Broca’s area

*ability to write sets aphemia apart from aphasias

Question 14

Where is the lesion that causes Broca’s aphasia?

Answer 14

Posterior part of the dominant inferior frontal gyrus

Brodmann’s areas 44 and 45

Question 15

Where is the lesion that causes Wernicke’s aphasia?

Answer 15

Posterior part of the dominant superior temporal gyrus

Brodmann’s area 22

Question 16

What clinical abnormality is seen in conduction aphasia?

Where is the lesion that leads to this?

Answer 16

Impaired repetition (naming, writing)
(speech is fluent, patients can comprehend the spoken word)

Arcuate fasciculus (fibers connecting Broca’s and Wernicke’s areas)

Question 17

Where is the lesion that causes:
Transcortical Motor Aphasia
Transcortical Sensory Aphasia

Answer 17

Dominant supplementary motor area (SMA)
*watershed territory ACA-MCA

Dominant junction of temporal, parietal, and occipital lobes
*watershed territory MCA-PCA

Question 18

What distinguishes transcortical aphasias from other aphasias?

Answer 18

Preserved repetition

Transcortical Motor Aphasia (expressive, like Broca’s)
Transcortical Sensory Aphasia (receptive, like Wernicke’s)

Question 19

Match the genetic defect with the chromosome:
Amyloid precursor protein (APP)
ApoE
Presenilin 1
Presenilin 2
Superoxide dismutase (SOD1)

1
14
19
21

Answer 19

APP - 21
ApoE - 19
Presenilin 1 - 14
Presenilin 2 - 1
SOD1 - 21

Question 20

Abundant Hirano bodies - found in what disorder?

Answer 20

AD

Hirano bodies = eosinophilic inclusions found in cytoplasm of hippocampal neurons

Question 21

Loss of neurons in which area of the brain cause decreased acetylcholine and choline acetyltransferase seen in AD?

Answer 21

Nucleus basalis of Meynert

Question 22

CBD findings on MRI

Answer 22

Asymmetric parietal lobe atrophy