Chapter 8 Pt 4

Question 1

what are the CORE clinical features of lewy body dementia

Answer 1

progressive cognitive decline
WAXING and WANING of cognition, especially in the areas of ATTENTION and ALERTNESS
VISUAL HALLUINATIONS- usually VIVID, COLORFUL, WELL-FORMED IMAGES of ANIMALS or PEOPLE
Development of EXTRAPYRAMIDAL SIGNS at least 1 year AFTER COGNITIVE DECLINE becomes evident

Question 2

what are SUGGESTIVE features of lewy body dementia

Answer 2

REM SLEEP BEHAVIOR DISORDER

ANTIPSYCHOTIC SENSITIVITY

Question 3

how is lewy body dementia diagnosed

Answer 3

definitely- autopys

POSSIBLE NCD with lewy body: only one core feature or ≥ 1 suggestive feature

PROBABLY NCD with LEwy bodies: ≥2 core features OR 1 core feature and ≥ suggestive feature

Question 4

how is lewy body dementia treated

Answer 4

CHOLINESTERASE INHIBITORS for cognitive ad behvioral symoms

QUETIPINE and CLOZAPINE for psychotic symptoms (lowest effective dose for shortest time possible)

Levodopa-Carbidoa for parkinsonism (not as effective as in idiopathic Parkinsonism, may exacerbate REM sleep disorder)

melatonin and/or clonazepam for REM sleep behavior disorder

Question 5

what is frontotemporal degeneration

Answer 5

a diverse group of clinical and pathological disorders that typically present between the ages of 45-65

Question 6

what are the clinical manifestations of frontotemporal degeneration

Answer 6

cognitive defects in ATTENTION, ABSTRACTION, PLANNING, and PROBLEM SOLVING

Question 7

what are the clinical manifestations of the behavioral variant of frontotemporal degeneration

Answer 7

DISINHIBITED verbal, physical, or sexual behavior
overeating or oral exploration of inanimate objects
lack of emotional warmth, empathy, or sympathy
apathy or inertia
perseveration, repetitive speech, rituals, or obsessions
decline in SOCIAL COGNITION and/or EXECUTIVE ABILITIES

Question 8

what are the clinical manifestations of the language variant frontotemporal degeneration

Answer 8

difficulties with SPEECH and COMPREHENSION

Question 9

what is relatively spared in frontotemporal degeneration

Answer 9

learning/memory and perceptual-motor function

Question 10

what is seen patholgocially in frontotemoral degeneration

Answer 10

marked atrophy of frontal and temporal lobes

Question 11

how is frontotemporal degeneration diagnosed

Answer 11

can’t be done till AUTOPSY

FTD is PROBABLE if frontotemporal atrophy is evident on structural imaging or hyperactivity is visualized on functional imaging in context of the characteristic clinical signs

Question 12

how is frontotemporal degeneration treated

Answer 12

symptom-focused
serotonergic mediations (SSRIs, trazodone) may help reduce disinhibition, anxiety, impulsivity, repetitive behaviors, and eating disorders

Question 13

what is the most common infectious agent known to cause cognitive impairment

Answer 13

HIV

25% of people with HIV meet mild NCD criteria and <5% meet major NCD criteria

Question 14

what are tis factors for HIV dementia

Answer 14

severe immunosuppression
high viral loads in CSF
advanced HIV infection

Question 15

what are the clinical manifestations of HIV dementia

Answer 15

variable presentation depending on the parts of the brain affected
decline may be observed in executive functioning, attention, working memory, psychomotor activity
psychiatric and neuromotor symptoms may be present

Question 16

how is HIV dementia diagnosed

Answer 16

mild or major NCD attributable to confirmed HIV infection

Question 17

what is treatment for HIV dementia

Answer 17

HAART improves cognition in some patinets

psychostimulants target fatigue, apathy, and psychomotor retardation

Question 18

what is Huntington’s Disease

Answer 18

CAG repeats in HUNTINGTIN (HTT) protein on chromosome 4

autosomal DOMINANT

Question 19

what are the clinical manifestations of Huntington;s

Answer 19

MOTOR, COGNITIVE, and PSYCHIATRIC symptoms
average age of onset is 40
cognitive decline and behavioral changes can precede onset of motor signs by up to 15 years
EXECUTIVE FUCNTION is the primary cognitive domain affected

Question 20

what psychiatric manifestations are seen in Huntingtons

Answer 20

depression
apathy
irritability
obsessions
impulsivity
INCREASED RATE OF SUICIDE

Question 21

what movement disorders are seen in Huntington’s

Answer 21

CHOREA (jerky, dance-like movements)

BRADYKINESIA

Question 22

How is Huntignton’s diagnosed

Answer 22

extrapyramidal movement disorder in an individual with either a FAMILY HISTORY of HD or GENETIC TESTING that confirms an increased number of CAG repairs in the HTT gene
mild or major NCD due to HD may be diagnosed prior to onset of motor signs if an individual is determined to be at risk based on family history or genetic testing

Question 23

how is Huntington’s disease treated

Answer 23

symptom-directed therapy

TETRABENAZINE or ATYPICAL (second generation0 ANTIPSYCHOTIC