Behavioral Neurology

Question 1

Aphemia

Answer 1

Apraxia of speech characterized by complete articulatory failure in the presence of preserved writing, comprehension and oropharyngeal function.
Lesion in the left inferior frontal gyrus (Broca’s area)

Question 2

Broca’s aphasia

Answer 2

Expressive aphasia. Nonfluent speech; can’t name or repeat. Written language also affected. Comprehension intact. Due to a lesion in the posterior part of the dominant inferior frontal gyrus

Question 3

Wernicke’s Aphasia

Answer 3

Receptive aphasia. Comprehension impaired. Speech is fluent but nonsensical. Lesion in posterior portion of dominant superior temporal gyrus.

Question 4

Conduction aphasia

Answer 4

Impaired repetition. Speech is fluent but not perfect and patients can comprehend spoken word. Naming an writing are affected. Lesion in the arcuate fasciculus (fibers connecting Broca’s and Wernicke’s areas).

Question 5

Global aphasia

Answer 5

Expressive and receptive aphasia due to a lesion in the dominant perisylvian area, involving Broca’s and Wernicke’s areas. Patients have nonfluent speech, poor comprehension, poor repetition and naming.

Question 6

Transcortical motor aphasia

Answer 6

Expressive aphasia but can repeat (unlike Broca’s aphasia). Due to a lesion in the dominant supplementary motor areas, the connections between the SMA and Broca’s area or above or anterior to Broca’s area. May be due to stroke involving watershed territory between ACA and MCA.

Question 7

Transcortical sensory aphasia

Answer 7

Receptive aphasia but can repeat. Lesion at the junction of the temporal, parietal and occipital lobes.

Question 8

Transcortical Mixed Aphasia

Answer 8

Nonfluent speech and poor comprehension with repetition

Question 9

Alexia without agraphia

Answer 9

aka pure word blindness. Lesion of the medial region of the dominant occipital lobe or medial and inferior occipitotemporal regions and splenium of the corpus callosum. Can also occur with lesion of periventricular white matter around the occipital horn of the lateral ventricle in the dominant hemisphere

Question 10

Alexia with agraphia

Answer 10

Lesion of the dominant angular gyrus

Question 11

Balint’s Syndrome

Answer 11

Oculomotor apraxia, optic ataxia, simultagnosia and issues with depth perception.
Due to bilateral parietal-occipital damage

Question 12

Auditory Verbal agnosia/pure word deafness

Answer 12

Can hear sounds but can’t understand words or repeat. Can read and write.
Localization: deep dominant superior temporal lesion or bilateral lesions in the midportion of the superior temporal gyrus.

Question 13

Alien Limb Syndrome

Answer 13

Patient may not recognize their own limb or may feel can’t control it. May have involuntary movement of limb.
Due to lesion of corpus callosum, ACA infarct, basal ganglionic degeneration.

Question 14

Anton’s Syndrome

Answer 14

Cortical blindness that patient is not aware that they have.
Lesion in bilateral occipital lobes.

Question 15

Capgras Syndrome

Answer 15

Patients have delusion that people around them have been replaced by imposters.

Question 16

Charles Bonnet Syndrome

Answer 16

Patients with vision loss see things in the space where vision was lost. Complex visual hallucinations, usually colored patterns, images of people, animals , plants or inanimate objects. Patients are typically elderly with normal cognition and know they are having hallucination.

Question 17

Ganser Syndrome

Answer 17

Patients give approximate but wrong answers.

Question 18

Gerstmann’s Syndrome

Answer 18

Finger agnosia, agraphia, acalculia, right-left confusion..
Due to a lesion of the angular gyrus of the dominant hemisphere. Brodmans’ area 39 (parietal lobe)
Common cause is infarction of the inferior division of the MCA

Question 19

Kluver-Bucy Syndrome

Answer 19

Patients hyperoral, hypersexual and placid.

Lesion in bilateral anterioral temporal lobe.

Question 20

Wernicke Encephalopathy

Answer 20

Confusion, ataxia, nystagmus.

Due to thiamine deficiency. Alcoholics with thiamine deficiency and get glucose before thiamine may have this triggered.

Question 21

Anosodiaphoria

Answer 21

lack of concern about one’s impairment, specifically used in association with indifference to paralysis.

Question 22

Anosognosia

Answer 22

denial/lack of awareness of one’s impairment; often seen with right parietal lesions.

Question 23

Susac’s Syndrome

Answer 23

Microangiopathy involving the brain, retina, and cochlea that usually present in young women.
Susac’s syndrome is associated with a triad:
1. BRAO
2. Encephalopathy
3. Sensorineural hearing loss

Brain MRI may resemble MS. There are multiple areas of high T2 involving white and gray matter. Corpus callosum often involved

Question 24

Whipple’s Disease

Answer 24

Due to gram positive bacillus (Tropheryma whippelii).
Dx Triad: dementia, supranuclear gaze palsy, myoclonus
Pathognomonic: oculomasticatory and skeletal myorhythmias
May also have convergence-divergence nystagmus

Question 25

akinetic mutism

Answer 25

seen with medial frontal lesions, specifically of the anterior cingulate gyrus

Question 26

asomatognosia

Answer 26

a category of neglect in which a patient denies ownership of a limb contralateral to a lesion of the supramarginal gyrus of the parietal lobe (usually nondominant)

Question 27

Alzheimer Genetics

Answer 27

Presenilin 1-chr 14 (aggressive AD) Presenilin 2-chr 1 Apolipoprotein E4 on chr 19: modifies risk--3x higher Amyloid Precursor Protein (APP)--chro 21

Question 28

Types of Memory--Duration

Answer 28

Immediate--amount of info. someone can keep in conscious awareness w/o active memorization. Test with Forward Digit Span. Working--test by manipulation of info retained in immediate memory Recent--ability to register and recall specific items after a delay of minutes to hours--usually impaired first in AD and tested by three-word recall Remote--historical life events and long-known info.

Question 29

Types of Memory--Content

Answer 29

Declarative--facts and events, aka explicit - -Episodic-specific events and contexts (most impaired early in AD) - -Semantic--facts such as vocabulary and concepts Nondeclarative--- aka implicit; memory of skills and other acquired behaviors Procedural

Question 30

FDG-PET in Dementias

Answer 30

AD-bilateral parietotemporal hypometabolism FTD-fontal and anterior temporal Huntingtons-head of caudate Diffuse Lewy Body--occipital PD-no hypometabolism PSP--global metabolic reduction in regions including the anterior cingulate, basal ganglia (esp. caudate and putamen), thalamus and upper brainstem

Question 31

Orbitofrontal lesions

Answer 31

OCD traits, disinhibition, emotional lability, hypersexuality, anxiety, depression, impulsiveness, antisocial behavior

Question 32

Familial Frontotemporal dementia genetics

Answer 32

17q21 | chromosomes 3 and 9 also

Question 33

3 variants of FTD

Answer 33

Behavioral Variant (most common): personality change, abulia, apathy, social withdrawal, social disinhibition, impulsivity, lack of insight, poor personal hygiene, stereotyped or ritual behaviors, hyperphagia, suddenly new artistic abilities or hobbies, emotional blunting, loss of empathy, mental rigidity, distractibility, impersistence, perseverative behavior and impaired organizational skills Progressive nonfluent aphasia: characterized in early stages by anomia, word-finding difficulty, impaired object naming, effortful speech with preserved comprehension. Behavior and social interaction remain unaffected until late in the disease at which point the patient becomes globally aphasic. Semantic dementia/progressive fluent aphasia: progressive speech disturbance with normal fluency but impaired comprehension, anomia, and semantic paraphasias. May clinically resemble a transcortical sensory aphasia

Question 34

Lesion Dorsomedial nucleus of the thalamus

Answer 34

Abulia, anterograde amnesia, social disinhibition and motivation loss.

Question 35

Lesion of anterior nucleus of the thalamus

Answer 35

dysfunction of limbic relay and memory formation

Question 36

Lesions causing akinetic mutism

Answer 36

Patient generally has preserved awareness with open eyes but remains immobile, mute and does not respond to commands - bilateral globus pallidus interna - bilateral ACA infarcts to the frontal lobes - lesions to the medial frontal lobes

Question 37

silver-staining, spherical aggregations of tau proteins in neurons

Answer 37

Pick bodies of Pick's disease

Question 38

globose neurofibrillary tangles in subcortical nuclei and tufted astrocytes

Answer 38

PSP

Question 39

Memantine

Answer 39

low-to-moderate affinity noncompetitive NMDA receptor antagonist that inhibits glutamate stimulation and thus theoretically limits overactivation and toxicity to remaining cholinergic neurons.

Question 40

Acetylcholinesterase inhibitors used in tx of AD

Answer 40

Donepezil: pure acetylcholinesterase inhibitor Rivastigmine: combined acetylcholinesterase and butyrylcholinesterase antagonist-->both result in limiting break down of acetylcholine. Galantamine: combined acetylcholinesterase inhibitor and allosteric nicotinic modulator

Question 41

Pathology of Alzhemier's disease

Answer 41

Amyloid plaques intraneuronal neurofibrillary tangles granulovacuolar degeneration: neuronal intracytoplasmic granule containing vacuoles amyloid angiopathy: amyloid deposition in walls of small and medium sized arteries Hirano bodies: cytoplasmic inclusions composed mainly of actin and actin-associated proteins

Question 42

familial CJD

Answer 42

AD inheritance | prion protein gene (PRNP) coding for PrP located on chromosome 20p.

Question 43

Prosopagnosia

Answer 43

inability to recognize faces | localize to bilateral lesion of the temporo-occipital regions (bilateral fusiform gyri}--bilateral PCA infarct or AD

Question 44

Foix-Chavany-Marie Syndrome aka anterior opercular syndrome

Answer 44

severe dysarthria, bilateral voluntary paralysis of lower CN's with preserved involuntary and emotional innervation. Assoc. w/ bilateral anterior opercular syndrome

Question 45

Ideational apraxia

Answer 45

impairment in the sequence of motions needed to carry out a specific movement (told to pour water into cup and drink but "drinks" before pouring water) seen in patients with bifrontal or biparietal dysfunction, as occurs in neurodegenerative disorders

Question 46

ideomotor apraxia

Answer 46

use of a body part as an object during pantomime, as well as unusual movements and postures lesion in dominant parietal cortex, in or around the area of the superior marginal and angular gyrus.

Question 47

conduction apraxia

Answer 47

impairment in imitation of movement | localization not well-defined

Question 48

conceptual apraxia

Answer 48

misconception of the function of objects in the environment--using fork to eat soup seen in diffuse neurodegenerative processes, lesions in nondominant hemisphere

Question 49

disassociation apraxia

Answer 49

inability to execute a movement on command but normal ability to imitate occurs in L hand in L hemisphere language dominant patients with L MCA infarct

Question 50

dressing apraxia

Answer 50

localizes to right parietal lobe; often occurs with neglect syndrome

Question 51

lesions to dorso-lateral prefrontal cortex

Answer 51

personality changes, lack of ability to plan or to sequence actions or tasks DLPFC also involved in verbal and design fluency, organizational skills, problem-solving, abstract thinking, shifting sets/topics and creative reasoning

Question 52

lesions to cingulate cortex

Answer 52

akinetic mutism, apathy and abulia, anterograde and retrograde amnesia and confabulation

Question 53

Tests of Executive function

Answer 53

``` Continuous Performance Task Controlled Oral Word Association Digit vigilance test Figural fluency test Halstead category test Paced Auditory Serial Addition test Wisconsin card sorting task--patient arranges cards based on specific concept Symptol digit modalities test ```

Question 54

Trail making tests

Answer 54

Part A-simple processing speed and attention | Part B--patient connects consecutive numbers and letters--set shifting and working memory in addition to Part A stuff.

Question 55

Ideomotor apraxia

Answer 55

impaired control of skilled movements--incorrect posture when using tool but know what tool should be used for using handle of hammer instead of head

Question 56

Ideational apraxia

Answer 56

inability to correctly order a series of movements to perform an action, making multistep actions confusing