Major/Mild Neurocog D/O with Lewy Bodies Flashcards
criterion A for M/M NCD with Lewy Bodies
the criteria are met for M/M NCD
criterion B for M/M NCD with Lewy Bodies
disorder has an INSIDIOUS onset and GRADUAL progression
criterion C for M/M NCD with Lewy Bodies
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
what are the core diagnostic features of M/M NCD with Lewy Bodies listed in the DSM criteria
- FLUCTUATING cognition with PRONOUNCED variations in attention and alertness
- RECURRENT visual hallucinations that are WELL FORMED and detailed
- spontaneous features of PARKINSONISM, with onset SUBSEQUENT to the development of the cognitive decline
what are the suggestive diagnostic features of M/M NCD with Lewy Bodies listed in the DSM criteria
- meets criteria for REM sleep behaviour disorder
2. severe neuroleptic sensitivity
what are the hallmarks of M/M NCD with Lewy Bodies
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
what are the early cognitive changes noticed in M/M NCD with Lewy Bodies and how does this differ from alzheimers?
lewy body–> early changes in attention and executive dysfunction
alzheimers–> early changes in learning and memory
what is the pattern of symptoms observed in M/M NCD with Lewy Bodies
symptoms fluctuate in a pattern that can resemble a delirium, but no adequate underlying cause can be found
for how long, by convention, are cognitive symptoms of M/M NCD with Lewy Bodies usually seen before motor symptoms (i.e spontaneous parkinsonism)
about 1 year
what % of those with M/M NCD with Lewy Bodies have severe sensitivity to neuroleptics?
up to 50%
therefore should use these meds with extreme caution in managing psychiatric manifestations
what are some physical symptoms that may support a diagnosis of M/M NCD with Lewy Bodies
patients frequently experience:
- repeated falls
- syncope
- transient episodes of unexplained loss of consciousness
autonomic dysfunction, including orthostatic hypotension and urinary incontinence may be seen
what psychiatric manifestations are common in M/M NCD with Lewy Bodies
auditory and other non visual hallucinations
systematized delusions
delusional misidentification
depression
what is the population prevalence estimate for M/M NCD with Lewy Bodies
0.1% to 5% of the general elderly population
what is the prevalence estimate of M/M NCD with Lewy Bodies amongst all dementia cases
1.7% to 30.5%
in brain biopsies after death, in what % of specimens are the pathological lesions known as lewy bodies found
20-35% of all cases of dementia
what is the male:female ration in M/M NCD with Lewy Bodies
1.5:1
what might you find on investigation of prior history in someone eventually diagnosed with M/M NCD with Lewy Bodies
there is often a prodromal history of confusional episodes (delirium) of acute onset, often precipitated by illness or surgery
what is the major distinguishing feature between M/M NCD with Lewy Bodies and Parkinsons disease?
the order in which the cognitive and motor symptoms emerge
where are lewy bodies primarily found in LBD? in Parkinsons?
lewy bodies are primarily CORTICAL in location in LBD
lewy bodies are primarily found in the BASAL GANGLIA in Parkinsons
what is the natural course of M/M NCD with Lewy Bodies
cognitive manifestations present first, then motor–> progression may be characterized by occasional plateaus but eventually progresses through severe dementia to death
what is the average duration of survival with M/M NCD with Lewy Bodies
5-7 years
when do most people affected with LBD start showing symptoms
between 6th-9th decade of life
most cases have onset in mid-70s
what are risk factors for M/M NCD with Lewy Bodies
familial aggregation may occur, and several risk genes have been IDed, but in most cases, there is NO family history
what is the underlying neurodegenerative disease/pathology in M/M NCD with Lewy Bodies
primarily a SYNUCLEINOPATHY due to ALPHA-SYNUCLEIN misfolding and aggregation
what feature, seen on SPECT or PET scans, may suggest M/M NCD with Lewy Bodies
low striatal dopamine transporter uptake
what other features on SPECT/PET scan may be helpful in diagnosing M/M NCD with Lewy Bodies
reduced striatal dopamine transporter uptake
generalized low uptake on SPECT/PET perfusion scan with reduced occipital activity
what finding on EEG might suggest M/M NCD with Lewy Bodies
prominent slow wave activity with temporal lobe transient waves
what finding on CT/MRI might suggest M/M NCD with Lewy Bodies
relative preservation of medial temporal structures
what finding on MIBG myocardial scintigraphy might suggest M/M NCD with Lewy Bodies
abnormal (low uptake) MIBG scintigraphy suggesting sympathetic denervation
how does functional decline compared between those with LBD and those with other NCDs
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
what is the main ddx for M/M NCD with Lewy Bodies
Parkinson’s disease
if someone develops symptoms of parkinsons, but then also develops symptoms of cognitive decline within 1 year of diagnosis, what is the final diagnosis?
M/M NCD with Lewy Bodies (not parkinsons)
1 year has to have elapsed for the diagnosis to stay parkinsons
what other disorders frequently co occur with M/M NCD with Lewy Bodies
alzheimer’s disease
cerebrovascular pathology
in what % of alzheimers cases is there a concomitant synuclein pathology
60% of cases (if amygdala-restricted cases are included)
