NCD--Substance Induced, + d/t HIV, Prion, Parkinsons, Huntingtons, Other

Question 1

what are the DSM criteria for substance/medication induced Major or Mild NCD

Answer 1

A–> criteria are met for a major or mild NCD

B–> neurocognitive impairments do not occur exclusively during the course of a delirium and persist beyond the usual duration of intoxication and acute withdrawal

C–> involved substance or medication and duration and extent of use are capable of producing the neurocognitive impairment

D–> temporal course of the neurocognitive deficits is consistent with the timing of substance or medication use and abstinence (i.e deficits remain stable or improve after a period of abstinence)

E–> neurocognitive disorder not attributable to another medical condition and is not better explained by another disorder

Question 2

what types of alcohol relayed NCDs are there

Answer 2

major NCD: nonamnestic-confabulatory type

major NCD: amnestic-confabulatory type

mild NCD

Question 3

what are the 4 types of substances listed in the DSM for substance/medication induced Major or Mild NCD

Answer 3

alcohol

inhalant

sedative/hypnotic/anxiolytic

other or unknown

Question 4

what is a specifier for substance/medication induced Major or Mild NCD

Answer 4

persistent–> neurocognitive impairment continues to be significant after an extended period of abstinence

Question 5

what neurocognitive impairment is seen most predominantly in NCDs due to sedative/anxiolytic/hypnotic drugs/meds

Answer 5

greater disturbances in MEMORY than in other cognitive functions

Question 6

NCD due to alcohol frequently manifests with a combination of what impairments

Answer 6

impairments in EXECUTIVE FUNCTIONING and MEMORY and LEARNING domains

Question 7

what are the features of alcohol-induced amnestic confabulatory NCD (korsakoffs)

Answer 7

prominent amnesia (severe difficulty learning new information with rapid forgetting)

tendency to confabulate

*may co occur with signs of thiamine encephalopathy (wernicke’s) with associated features such as nystagmus and ataxia

Question 8

what ocular abnormality is associated with wernicke’s encephalopathy

Answer 8

lateral gaze paralysis (ophthalmoplegia)

Question 9

what are the more common neurocognitive symptoms related to methamphetamine use? what kind of overall NCD profile is seen in methamphetamine use

Answer 9

difficulties with learning and memory

difficulties with executive function

*most common neurocognitive profile approximates that seen in vascular NCD

Question 10

what ocular abnormalities can be associated with methamphetamine use

Answer 10

evidence of vascular injury–> i.e focal weakness, unilateral incoordination, asymmetrical reflexes)

Question 11

what is the rate of NCD of intermediate duration in those with hx alcohol abuse

Answer 11

30-40% in the first 2 months of abstinence

–> mild NCD may persist especially in those who do not achieve stable abstinence before until after age 50

Question 12

is major NCD due to alcohol abuse common

Answer 12

no–> MAJOR NCD is rare, may result from concomitant nutritional deficits as in alcohol-induced amnestic confabulatory NCD

Question 13

what is seen on MRI of individuals with chronic alcohol abuse

Answer 13

cortical thinning

white matter loss

enlargement of sulci and ventricles

*it is possible to observe NCDs without neuroimaging correlates however

*many of these changes reverse after period of of succesful abstinence

Question 14

those with sub/med induced NCD may have deficits in what areas that are beyond that seen in many other NCDs

Answer 14

reduced cognitive capacity

difficulty concentrating

Question 15

what are the criteria for major/mild NCD due to HIV infection

Answer 15

A–The criteria are met for major or mild neurocognitive disorder.
B–There is documented infection with human immunodeficiency virus (HIV).
C–The neurocognitive disorder is not better explained by non-HIV conditions, including secondary brain diseases such as progressive multifocal leukoencephalopathy or cryptococcal meningitis.
D–The neurocognitive disorder is not attributable to another medical condition and is not better explained by a mental disorder.

Question 16

what pattern of NCD is seen in major/mild NCD due to HIV infection

Answer 16

“subcortical pattern”

–> prominently impaired EXECUTIVE FUNCTION, slowing of processing speed, problems with more demanding attentional tasks, difficulty in learning new information

–> in major NCD due to HIV, SLOWING may be prominent

Question 17

major/mild NCD due to HIV infection show relatively preserved function in what cognitive areas

Answer 17

recall of learned information is relatively preserved

language difficulties are uncommon

Question 18

what % of those infected with HIV have at least mild neurocognitive disturbance

Answer 18

1/3 to 1/2

*may not meet full criteria for NCD

estimated 25% meet criteria for mild NCD
estimated fewer than 5% meet criteria for major NCD

Question 19

how might major/mild NCD due to HIV infection present in developing countries in which perinatal HIV transmission is common

Answer 19

may present as neurodevelopmental delay

Question 20

what are the criteria for major/mild NCD due to Prion disease

Answer 20

A–The criteria are met for major or mild neurocognitive disorder.
B–There is insidious onset, and rapid progression of impairment is common.
C–There are motor features of prion disease, such as myoclonus or ataxia, or biomarker evidence.
D–The neurocognitive disorder is not attributable to another medical condition and is not better explained by another mental disorder.

Question 21

what type of onset and progression would you expect for major/mild NCD due to Prion disease

Answer 21

insidious onset, rapid progression of impairment (i.e progression to major NCD over as little as 6 months)

Question 22

what are some motor features of prion disease

Answer 22

myoclonus

ataxia

Question 23

what is the most common spongiform encephalopathy/prion disease

Answer 23

sporadic creutzfeldt-jakob disease (CJD)

Question 24

how do those with CJD typically present

Answer 24

neurocognitive deficits

ataxia

abnormal movements–> myoclonus, chorea, dystonia

startle reflex is common

Question 25

what are the characteristic biomarker features of prion disease

Answer 25

recognized lesions on MRI with DWI or FLAIR

tau or 14-3-3 protein in CSF

characteristic TRIPHASIC waves on EEG

(family history or genetic testing for rare familial forms)

Question 26

what would be seen on EEG in prion diseases

Answer 26

characteristic triphasic waves

Question 27

what proteins would be seen in CSF in prion diseases

Answer 27

tau or 14-3-3 protein

Question 28

what is the annual incidence of sporadic CJD

Answer 28

1-2 per 1 million people

Question 29

what are prodromal symptoms of prion disease

Answer 29

fatigue, anxiety, problems with appetite or sleeping, difficulties with concentration

Question 30

what is the most sensitive diagnostic test for prion diseases currently

Answer 30

MRI with DWI–> see multifocal gray matter hyper-intensities in subcortical and cortical regions

Question 31

what is the expected onset and progression of major/mild NCD due to parkinsons disease

Answer 31

insidious onset

GRADULE progression of impairment

Question 32

list features that are frequently present in the context of major/mild NCD due to parkinsons disease

Answer 32

apathy

depressed mood

anxious mood

hallucinations

delusions

personality changes

REM sleep behaviour disorder

excessive daytime sleepiness

Question 33

what is the prevalence of parkinsons disease in people older than 85

Answer 33

3%

Question 34

what % of those with parkinsons disease will develop a major NCD sometime in the course of their illness

Answer 34

75%

Question 35

what are risk factors for development of parkinsons disease

Answer 35

exposure to herbicides and pesticides

RFs for development of NCD in parkinsons = older age at disease onset and increasing duration of disease

Question 36

what type of neuroimaging may be helpful to distinguish lewy body vs non-lewy body dementias

Answer 36

dopatmine transporter scans i.e DaT scans or structural neuroimaging scans

Question 37

how do you distinguish between the two lewy body dementias (parkinsons and dementia with lewy bodies)

Answer 37

onset and timing–> for parkinsons, motor and other symptoms must have been present for about a year before onset of cognitive symptoms

for dementia with lewy bodies, cognitive symptoms begin at the same time or shortly before motor symptoms

Question 38

what are the early cognitive changes seen in huntingtons disease

Answer 38

executive function (rather than learning and memory)

often precede the emergence of the typical motor abnormalities of huntingtons disease

Question 39

what are the typical motor abnormalities of huntingtons disease

Answer 39

bradykinesia and chorea

Question 40

what is the genetic abnormality responsible for huntingtons disease

Answer 40

CAG trinucleotide repeat expansion in the HTT gene on chromosome 4

*fully penetrant, autosomal dominant (repeat length of 36 or more is invariably associated with huntingtons disease)

Question 41

what psychiatric symptoms may be associated with huntingtons disease

Answer 41

depression

irritability

anxiety

obsessive-compulsive symptoms

apathy

psychosis–more rare

Question 42

what is the average age at diagnosis of huntingtons disease

Answer 42

40

Question 43

what is the median survival after motor symptom diagnosis of huntingtons disease

Answer 43

about 15 years

*psychiatric and cognitive symptoms of huntingtons disease can predate motor symptoms by as much as 15 years

Question 44

what neuroimaging changes are seen in those with huntingtons disease

Answer 44

volume loss in basal ganglia (particularly caudate nucleus and putamen)