Major/Mild Neurocog D/O with Lewy Bodies

Question 1

criterion A for M/M NCD with Lewy Bodies

Answer 1

the criteria are met for M/M NCD

Question 2

criterion B for M/M NCD with Lewy Bodies

Answer 2

disorder has an INSIDIOUS onset and GRADUAL progression

Question 3

criterion C for M/M NCD with Lewy Bodies

Answer 3

the disorder meets a combination of core diagnostic features and suggestive diagnostic features for either probable or possible NCD with Lewy Bodies

for probable M/M NCD with Lew Bodies, the individual has to have TWO core features, or one suggestive feature with one or more core features

for possible M/M NCD with Lew Bodies, the individual has to have only ONE core feature, or one or more suggestive features

also, the disturbance is not better explained by cerebrovascular disease, another neurdegenerative disease, the effects of a substance or another mental, neurological or systemic disorder

Question 4

what are the core diagnostic features of M/M NCD with Lewy Bodies listed in the DSM criteria

Answer 4

1. FLUCTUATING cognition with PRONOUNCED variations in attention and alertness
2. RECURRENT visual hallucinations that are WELL FORMED and detailed
3. spontaneous features of PARKINSONISM, with onset SUBSEQUENT to the development of the cognitive decline

Question 5

what are the suggestive diagnostic features of M/M NCD with Lewy Bodies listed in the DSM criteria

Answer 5

1. meets criteria for REM sleep behaviour disorder

2. severe neuroleptic sensitivity

Question 6

what are the hallmarks of M/M NCD with Lewy Bodies

Answer 6

progressive cognitive impairment (with early changes in ATTENTION and EXECUTIVE FUNCTION rather than learning and memory)

recurrent complex visual hallucinations

concurrent symptoms of REM sleep behaviour disorder (can be a very early manifestation)

hallucinations in other sensory modalities

depression

delusions

spontaneous parkinsonism

Question 7

what are the early cognitive changes noticed in M/M NCD with Lewy Bodies and how does this differ from alzheimers?

Answer 7

lewy body–> early changes in attention and executive dysfunction

alzheimers–> early changes in learning and memory

Question 8

what is the pattern of symptoms observed in M/M NCD with Lewy Bodies

Answer 8

symptoms fluctuate in a pattern that can resemble a delirium, but no adequate underlying cause can be found

Question 9

for how long, by convention, are cognitive symptoms of M/M NCD with Lewy Bodies usually seen before motor symptoms (i.e spontaneous parkinsonism)

Answer 9

about 1 year

Question 10

what % of those with M/M NCD with Lewy Bodies have severe sensitivity to neuroleptics?

Answer 10

up to 50%

therefore should use these meds with extreme caution in managing psychiatric manifestations

Question 11

what are some physical symptoms that may support a diagnosis of M/M NCD with Lewy Bodies

Answer 11

patients frequently experience:

• repeated falls
• syncope
• transient episodes of unexplained loss of consciousness

autonomic dysfunction, including orthostatic hypotension and urinary incontinence may be seen

Question 12

what psychiatric manifestations are common in M/M NCD with Lewy Bodies

Answer 12

auditory and other non visual hallucinations

systematized delusions

delusional misidentification

depression

Question 13

what is the population prevalence estimate for M/M NCD with Lewy Bodies

Answer 13

0.1% to 5% of the general elderly population

Question 14

what is the prevalence estimate of M/M NCD with Lewy Bodies amongst all dementia cases

Answer 14

1.7% to 30.5%

Question 15

in brain biopsies after death, in what % of specimens are the pathological lesions known as lewy bodies found

Answer 15

20-35% of all cases of dementia

Question 16

what is the male:female ration in M/M NCD with Lewy Bodies

Answer 16

1.5:1

Question 17

what might you find on investigation of prior history in someone eventually diagnosed with M/M NCD with Lewy Bodies

Answer 17

there is often a prodromal history of confusional episodes (delirium) of acute onset, often precipitated by illness or surgery

Question 18

what is the major distinguishing feature between M/M NCD with Lewy Bodies and Parkinsons disease?

Answer 18

the order in which the cognitive and motor symptoms emerge

Question 19

where are lewy bodies primarily found in LBD? in Parkinsons?

Answer 19

lewy bodies are primarily CORTICAL in location in LBD

lewy bodies are primarily found in the BASAL GANGLIA in Parkinsons

Question 20

what is the natural course of M/M NCD with Lewy Bodies

Answer 20

cognitive manifestations present first, then motor–> progression may be characterized by occasional plateaus but eventually progresses through severe dementia to death

Question 21

what is the average duration of survival with M/M NCD with Lewy Bodies

Answer 21

5-7 years

Question 22

when do most people affected with LBD start showing symptoms

Answer 22

between 6th-9th decade of life

most cases have onset in mid-70s

Question 23

what are risk factors for M/M NCD with Lewy Bodies

Answer 23

familial aggregation may occur, and several risk genes have been IDed, but in most cases, there is NO family history

Question 24

what is the underlying neurodegenerative disease/pathology in M/M NCD with Lewy Bodies

Answer 24

primarily a SYNUCLEINOPATHY due to ALPHA-SYNUCLEIN misfolding and aggregation

Question 25

what feature, seen on SPECT or PET scans, may suggest M/M NCD with Lewy Bodies

Answer 25

low striatal dopamine transporter uptake

Question 26

what other features on SPECT/PET scan may be helpful in diagnosing M/M NCD with Lewy Bodies

Answer 26

reduced striatal dopamine transporter uptake

generalized low uptake on SPECT/PET perfusion scan with reduced occipital activity

Question 27

what finding on EEG might suggest M/M NCD with Lewy Bodies

Answer 27

prominent slow wave activity with temporal lobe transient waves

Question 28

what finding on CT/MRI might suggest M/M NCD with Lewy Bodies

Answer 28

relative preservation of medial temporal structures

Question 29

what finding on MIBG myocardial scintigraphy might suggest M/M NCD with Lewy Bodies

Answer 29

abnormal (low uptake) MIBG scintigraphy suggesting sympathetic denervation

Question 30

how does functional decline compared between those with LBD and those with other NCDs

Answer 30

individuals with LBD are more functionally impaired than would be expected for their cognitive deficits when contrasted with individuals with other neurodegenerative diseases (i.e alzheimers)

• this is largely a result of MOTOR and AUTONOMIC impairments which cause problems with toileting, transferring, and eating
• sleep disorders and prominent psychiatric symptoms may also add to functional difficulties

Question 31

what is the main ddx for M/M NCD with Lewy Bodies

Answer 31

Parkinson’s disease

Question 32

if someone develops symptoms of parkinsons, but then also develops symptoms of cognitive decline within 1 year of diagnosis, what is the final diagnosis?

Answer 32

M/M NCD with Lewy Bodies (not parkinsons)

1 year has to have elapsed for the diagnosis to stay parkinsons

Question 33

what other disorders frequently co occur with M/M NCD with Lewy Bodies

Answer 33

alzheimer’s disease

cerebrovascular pathology

Question 34

in what % of alzheimers cases is there a concomitant synuclein pathology

Answer 34

60% of cases (if amygdala-restricted cases are included)