5 Parkinson´s, Lewy bodies, Huntington´s Flashcards
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Most common forms of dementia associated with movement disorders
- Parkinson’s Disease Dementia (PDD)
- Dementia with Lewy Bodies (DLB)
- Corticobasal Degeneration (CBD)
- Progressive Sapranuclear Palsy (PSP)
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The neuropathological features of these diseases divide them into two categories
- PDD + DLB -> Synucleinopathies
- CBD + PSP -> Tauopathies
The distinction is made easier between the two categories than within the categories, especially within the category of PDD and DLB.
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Epidemiology
PDD=
The prevalence of dementia in Parkinson’s Disease is 8-93 %. Risk factors are age, low education, socioeconomic status and a family history of Parkinson’s disease.
DLB= This is the second most common cause of dementia.
CBD=
Dementia and neurobehavioral abnormalities are now accepted as a common presenting problem (previously thought to be rare in CBD).
PSP= Only known risk factor is age.
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Clinical and neurological presentation
DBL and PDD
DBL and PDD=
The essential feature for differentiating between PDD and DLB is the time of onset of the motor signs:
- PDD=
Behavioural and cognitive symptoms are seen more than 12 months after the manifestation of motor symptoms. - DLB=
Behavioural symptoms occur before the motor signs or within the first 12 months after the manifestation of motor signs.
For the diagnosis of PDD, the prior diagnosis of PD is required. PD becomes most often
symptomatic during the 6th decade of life.
The most common cognitive complaint for PDD and DLB is memory
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Clinical and neurological presentation
CBD
CBD=
- Cognitive changes are often accompanied with visual distortions and hallucinations
- The onset is also usually around the 6th decade of life.
- Meantime of death after the diagnosis: within 7 years
- The typical initial complaints of DCB are: clumsiness, stiffness or jerkiness of an arm or sometimes a leg
- Sometimes the ‘alien-hand’ syndrome is seen: the individual senses a lack of ownership over one of his hands.
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Clinical and neurological presentation
PSP
PSP=
- Typically occurs after 60, with particularly high incidence rates after 80
- The survival rates vary across 5-10 years after the diagnosis
- PSP shows a gradual, progressive beginning, at 40 or later
- The earliest symptoms are imbalance (evident in falls), rigidity, impoverished reflexes and dysarthia.
- When present, the cognitive changes are usually visual impairments and concentration and executive problems
- PSP could have a parkinsonian subtype
- This PSP parkinsonian subtype has a longer duration (+/- 12 years), a less severe progression and shows less prototypical cognitive impairments.
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Neuropathology
PDD and DLB=
In both PDD and DLB: there is a presence of aggregates of alpha-sunuclei, in the form of Lewy Bodies (LB) or Lewy Neurites (LN). In DLB a substantial number of patients also have amyloid plaques, which are seen less in PDD. PD has been involve significant dysfunction of the dopaminergic and cholinergic system.
CBD=
In CBD there are seen ballooned neurons, predominantly in the frontotemportal cortex. Also, taucontaining neuronal inclusions are seen in the cortex and stadium. The pattern shows an asymmetric atrophy.
PSP=
PSP compromises the entire Substantia Nigra (unlike PD) and dopaminergic depletion is comparable in caudate and putamen. Tangles are found in the brain stem, basal ganglia, dentate and the nucleus basalis of Meynert.
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Structural and functional neuroimaging findings
PDD=
In PDD there exists an association between dementia and neurocortical medial temporal and amygdala atrophy.
DLB= In DLB, greater temporal, parietal and occipital atrophy than in PDD.
CDB= Cortical atrophy is seen in CDB, especially in the frontoparietal part.
PSP=
In PSP the atrophy is seen in the midbrain, frontal atrophy is linked to behavioural problems and executive dysfunction.
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Neuropsychological hallmarks
Attention and working memory
PD=
Span task = preserved, but as the disease progresses, the impairments will show up.During the early manifestations of PD, working memory deficits will already be present, which also will keep progressing.
DLB= Greater impairments on the Stroop Task than PDD.
CBD= Progressing impairments in the digit span task and Stroop task.
PSP= Deficits in visual attention.
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Neuropsychological hallmarks
Executive functions
Executive functions=
Planning is slowed and inaccurate in PDD. Both PDD and DLB show a poor performance on the card sorting task.
CBD= Executive dysfunctions are common, but less compromised than in FTD.
PSP=
Early in the process of PSP executive dysfunctions are seen, leading to problems in planning, problem solving and cognitive flexibility. The progression in these deficits is rapid. The ‘applause sign’ (preservation from clapping to command) is seen in 3⁄4 of patients and distinguishes PSP from FTD.
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Neuropsychological hallmarks
Language
PDD and DLB=
Are similarly impaired in verbal fluency.
CBD=
Progressive aphasia is common, especially non-fluent aphasia. Fluent aphasia is rare in CBD. Also phonological problems.
PSP= Hypophonia and dysarthia occur early in the process and are common
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Neuropsychological hallmarks
Learning and memory
Learning and memory=
PDD shows retrieval deficits, PDD and DLB show both similar severe memory deficits. The differences are made on qualitative aspects:
- DLB= Poorer recall, more rapid forgetting. - PDD= Preservative errors. - CBD= Both encoding and retrieval deficits.
PSP=
Episodic deficits, but these are less impaired compared to PDD, DLB and AD. Free recall is also impaired. Recognition is normal
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Neuropsychological hallmarks
Visuospatial and spatial functions
Severe deficits in both PDD and DLB on numerous visuospatial skills and construction tasks (for instance: drawing a clock)
CDB=
Poor constructive drawing
PSP=
Impairment is voluntary vertical eye movements. Early in PSP there are seen subtle abnormalities in clock drawing.
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Neuropsychological hallmarks
Neuropsychiatric features
Depression is common in PDD. (approximately 50% of the patients), but is often undertreated. Anxiety disorders show a comparable prevalence in PDD and DLB. Psychosis is more common in DLB than in PDD.
CBD= Depression is most seen in CBD patients, also, but less: apathy, irritability and agitation.
PSP=
Apathy is the most common neuropsychiatric feature of PSP (almost in 90% of patients), depression is seen less or almost never.
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Other movement disorders with dementia
- Huntington’s Disease;
- Sydenham’s Chorea;
- Wilson’s Disease;
- Cerebrovascular Disease
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Neuropsychological assessment
- Review of the medical record
- Clinical interview
- Screening instruments
- Assessment of neuropsychiatric symptoms
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Review of the medical record
Assign special attention to age, age of onset (of motor and cognitive symptoms), side of onset (of motor symptoms), nature of parkinsonian symptoms, motor fluctuations, visual problems, hallucinations, timing of medications, pathological sleepiness, attention fluctuations and comorbid conditions
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Clinical interview
Verify information from the medical record. Ask about the family history of dementias or movement disorders. PDD patients are accurate reporters of their disabilities (even when they show cognitive deficits), but are worse at reporting memory impairments. PDD and DLB patients may complain about memory deficits, which may actually represent other than memory deficits. Prepare the patient for evaluation, and let them report when they don’t feel good
