Dementia and Behavioral Neurology

Question 1

What is used in PET scans to image beta-amyloid plaques in the brain in AD research?

Answer 1

Pittsburgh Compound B

Question 2

What is memantine (Namenda)?

Answer 2

NMDA receptor blocker used in AD

Question 3

Where is ACh produced?

Answer 3

Nucleus Basalis of Meynert

Question 4

Alzheimer’s disease is uncommon in patients less than 60 years old. In such patients, where the disease often runs in the family, what protein is likely to be mutated?

Answer 4

Amyloid precursor

Question 5

Which protein increases AD risk and lowers mean age of onset?

Answer 5

e4 allele of apolipoprotein E

Question 6

Which genetic disease is thought to be a neurodevelopmental model for AD?

Answer 6

Down’s Syndrome

Question 7

How can delirium be best distinguished from Alzheimer’s dementia?

Answer 7

Dementia lacks fluctuations in level of arousal

Question 8

Types of medications that can cause dementia symptoms:

Answer 8

anticholinergics, benzos, opiates

Question 9

What are Lewy Bodies composed of?

Answer 9

alpha-synuclein; seen namely in Parkinson’s disease

Question 10

A highly suggestive sign of diffuse lewy body dementia (other than motor/gait abnormalities):

Answer 10

fluctuating cognition with variations in attention and alertness

Question 11

LBD patients are hypersensitive to which medications?

Answer 11

Antipsychotics

Question 12

Charles-Bonnet Syndrome

Answer 12

Patients are mentally healthy, often with significant visual loss. Hallucinations of small animals and people. Understand that they are not real and are typically not bothered by them.

Question 13

An unusual feature of some patients with frontotemporal degeneration may be:

Answer 13

The development of new artistic abilities

Question 14

3 patterns of behavior in frontotemporal degeneration (Pick’s disease):

Answer 14

Disinhibition; Apathy; Language Disturbance.

Occurs at younger age (< 65) and has more rapid progression, memory is often relatively unaffected.

Question 15

What is Ropinirole (Requip)?

Answer 15

DA agonist used in PD. Can increase impulsivity (i.e. gambling, hypersexual behavior).

Question 16

Which vitamin abnormality is responsible for Wernicke-Korsakoff syndrome?

Answer 16

thiamine/B1 deficiency

Question 17

What is Ganser’s syndrome?

Answer 17

Prison psychosis or “the syndrome of approximate answers.”

Question 18

Typically presents in a “step-wise” fashion and patients have focal neurological deficits.

Answer 18

Vascular dementia

Question 19

After VP shunt there is risk for what? Why?

Answer 19

SDH; due to the reduction of intracranial pressure that pulls the brain away from the meninges and tears veins.

Question 20

Mneumonic for NPH

Answer 20

Wobbly, weird, and wet. Magnetic gait, dementia, and urinary urgency/frequency.

Question 21

Transient Global Amnesia

Answer 21

Complete and reversible anterograde and retrograde memory loss lasting up to 24 hours. Thought to be migraine phenomemnon in context of emotional event or sexual activity.

Question 22

Creutzfelt-Jacob Disease

Answer 22

Prion disease characterized by rapid onset change in mental status AND myoclonus provoked by a startle.

Question 23

Kluver-Bucy Syndrome

Answer 23

Caused by damage to bilateral amygdalae. Hypersexuality, diminished fear response/docile, overeat/PICA, difficulty recognizing familiar faces/objects.

Question 24

Where is the lesion in Wernicke’s/Receptive aphasia?

Answer 24

Left superior temporal gyrus

Question 25

Where is the lesion in Broca’s/Expressive aphasia?

Answer 25

Left inferior frontal gyrus

Question 26

Where is the lesion in patients with conduction aphasia?

Answer 26

Arcuate fasciculus

Question 27

Abnormal Prosody lesion is where?

Answer 27

Right inferior frontal gyrus, giving monotonicity to speech.

Question 28

Where is the lesion in pure word deafness?

Answer 28

Bilateral damage to posterior superior temporal lobes (auditory cortex), cannot comprehend the meaning of speech.

Question 29

Where is the lesion in akinetic mutism (can speak under extreme duress)?

Answer 29

bilateral cingulate gyrus

Question 30

What supplies Broca’s and Wernicke’s areas?

Answer 30

The MCA

Question 31

What is dysarthria?

Answer 31

A speech problem caused by trouble with the muscles of articulation.

Question 32

What is dysphonia?

Answer 32

A problem of vocal cords and patients sound hoarse or breathy when they speak.

Question 33

What is anomic aphasia?

Answer 33

Trouble naming objects

Question 34

What is Gerstmann’s Syndrome?

Answer 34

Lesion to the dominant angular gyrus resulting in agraphia, acalculia, finger agnosia, left/right confusion.

Question 35

What is apraxia?

Answer 35

A disorder of skilled movement not caused by weakness, akinesia, deafferentation, abnormal tone/posture, movement disorders, intellectual deterioration, uncooperativeness.

Question 36

What is prosopagnosia and where is the lesion?

Answer 36

Inability to recognize familiar faces; Fusiform gyrus (FFA)

Question 37

Alexia without agraphia

Answer 37

Left occipital lobe, involving the splenium of the corpus callosum. Visual information reaches only the right occipital lobe, but cannot be accessed by the language areas of the brain. Therefore, patients cannot read. Since the language areas are otherwise fine, people can write.

Question 38

What is Anton’s syndrome?

Answer 38

Denial of blindness due to damage in bilateral occipital lobes.

Question 39

What is Bálint’s syndrome and how is it characterized?

Answer 39

Due to damage to both posterior parietal lobes:

1. Optic ataxia: incoordination of hand and eye movement.
2. Oculomotor apraxia: the inability to voluntarily guide eye movements.
3. Simultanagnosia: the inability to perceive more than one object at a time in one’s visual field.

Question 40

What is posterior reversible encephalopathy syndrome?

Answer 40

Acute onset of confusion and cortical blindness, usually due to sudden, drastic increases in BP.

Question 41

What is Kleine-Levin syndrome?

Answer 41

A rare sleep disorder characterized by persistent episodic hypersomnia and cognitive or mood changes. Many patients also experience hyperphagia, hypersexuality and other symptoms.

Question 42

What is pseudobulbar palsy?

Answer 42

Seen in diseases such as MS, ALS, AD, laughing/tears for no reason, no explanation/emotion. Treated with dextromethorphan/quinidine.

Question 43

Corticobasal degeneration results in what?

Answer 43

Alien hand syndrome. Classiaclly described as a consequence of epilepsy surgery and CC severing.