Transcript Non-Alzheimer’s Dementias

Non-Alzheimer’s
Dementias
Scott Morgan MSIII
December 2012
S
DSM-IV Definition of Dementia
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Major impairment of memory and learning as well as one
of:
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Impairment handling complex tasks
Impairment in reasoning ability
Impairment spatial ability and orientation
Impaired language
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Insidious and progressive onset
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Symptoms interfere with relationships, work performance,
and social activities
Recognizing Dementia
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Memory loss rarely reported by patient
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Spouse or family member usually reports
Family reported memory loss is a better predictor
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Commonly thought to just be from aging
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Insidious onset often causes a delay in reporting memory
loss
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Must be distinguished from delirium and depression
Mental Status Tests
Parkinson Disease Dementia
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Prevalence of dementia in Parkinson Disease (PD) patients
is 41%
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Mild cognitive impairment is common in PD
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Does not always meet criteria for dementia
Genetic factors associated with Parkinson Disease
Dementia (PDD)
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Multiplications of alpha-synuclein gene
Microtubule-associated protein tau (MAPT) H1/H2 and
MAPT H1/H1 genes
Epsilon 2 and epsilon 4 alleles of the apolipoprotein gene
Early Clinical Features of PDD
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Executive dysfunction with deficiencies in:
• Set shifting
• Attention
• Planning
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Visuospatial impairment
• Facial recognition problems
• Impaired tone contrast
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Verbal memory
• Difficulty remembering words
• Unable to explain their problems
Late Clinical Features of PDD
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Psychosis
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Mood disorders
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Visual hallucinations
Delusions
• Spousal infidelity paranoia
Depression
Anxiety
Apathy
Sleep disturbances
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Sleep fragmentation
Nightmares
REM sleep behavior disorder
• Patient has normal dreams without the muscle atonia
Treatment of PDD
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Cholinesterase inhibitors
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For psychosis
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Have a benefit for cognition
No evidence for improvement of neuropsychiatric symptoms
Quetiapine
Clozapine
Avoid anticholinergic medications
Dementia with Lewy Bodies
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Second most common degenerative dementia
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There have been familial cases, but most are sporadic
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Multiplications of alpha-synuclein gene are associated with
familial DLB
Only known risk factor is increased age
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10-22% of all dementia cases
Mean age of onset is 75
Some studies show more likely in males
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Still debated and evidence is not strong
Lewy Body
•Round eosinophilic intranuclear
inclusion
•Found in nuclei of neurons of
substantia nigra
•Aggregations of alpha-synuclein
•Only detectable by postmortem
biopsy
•Do not show up on any scans
Diagnosing DLB
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Features of Alzheimer’s Disease and Parkinson Disease
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More rapid onset than Alzheimer’s
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Can have amyloid plaques similar to Alzheimer’s
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Different from PDD by
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Not nearly as numerous as Alzheimer’s
Onset of dementia compared to parkinsonian symptoms
Before, during, or very shortly after parkinsonian signs
Can detect brain atrophy with MRI and PET scans
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Not necessary for diagnosis but can provide supportive
evidence
Clinical Features of DLB
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Very similar to PDD
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Fluctuating cognition and alertness
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Visual hallucinations
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Patients can “blank out” or lose consciousness
Episodes last from seconds to days
Early sign in DLB compared to late in PDD
Parkinsonism
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Gait disorders
Bradykinesia
Limb rigidity
Treatment of DLB
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Treatments are just for symptoms
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No treatment has been found to have disease modifying
effects
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Selegiline has been used as a treatment
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Limited evidence for effectiveness
Vascular Dementia
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Aka multi-infarct dementia
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Makes up 10-20% of dementia cases
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Risk factors
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Very weak evidence to show shared risk factors with
cerebrovascular disease
Some evidence points to metabolic syndrome as a risk factor
Advanced age is a risk factor for dementia after a stroke or
subclinical infarct
Pathology of VaD
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Large artery infarcts
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Small artery or lacunar infarcts
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Usually cortical but sometimes subcortical
Exclusive subcortical in small penetrating arteries
Affect basal ganglia, caudate, thalamus, internal capsule,
cerebellum, and brainstem
Chronic subcortical ischemia
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Small arteries in periventricular white matter
Order of vulnerability – neuron, oligodendrocyte, myelinated
axon, astrocyte, endothelial cell
Diagnosis of VaD
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Neuroimaging
• MRI is most sensitive
• CT is usually first line scan
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Step-wise loss of cognition and
memory
• Disease progresses with each
ischemic event
• Each progression can be large or
small
Clinical Features of VaD
Specific to area affected
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Medial frontal
• Executive function
• Apathy
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Left parietal
• Aphasia
• Apraxia
• Agnosia
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Right parietal
• Hemineglect
• Confusion
• Agitation
• Visuopatial difficulty
• Constructional difficulty
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Medial temporal
• Anterograde amnesia
Frontotemporal Dementia
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Aka:
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Age of onset is late 50s to early 60s
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Picks Disease
Pick complex
Frontal lobe dementia
Average age is 58
As the name implies it is a degeneration of the frontal and
temporal lobes
Pick Bodies
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Build up of tau proteins
in neurons
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Cause neurons to swell
and appear spherical
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Silver staining for
diagnosis
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Help distinguish from
other dementias
Clinical Features of FTD
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Unique because of personality changes
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Aggressiveness
New onset of criminal activity
Socially inappropriate
Sexually inappropriate
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Lack of insight
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Progressive non-fluent aphasia
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Unusual eating patterns
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Word-finding difficulty and speech errors
Some patients will binge on alcohol
Incontinence
Diagnosis and Treatment of FTD
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MRI is most common scan used to diagnose
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Very little evidence to support pharmacological treatment
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Used to treat symptoms
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Acetylcholinesterase inhibitors used to be used for treatment
but falling out of favor
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SSRIs are used to treat depression if this is a symptom
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Neuroleptics can be used but side effects must be weighed
against benefits
Summary
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Alzheimer’s dementia is most common dementia
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Memory loss is the major hallmark of dementia
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Dementias are often difficult to diagnose and treat
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Diagnosis is mainly by clinical features rather than using
imaging scans
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Most treatments are for symptoms not disease modification
Citation
Hake, Ann Marie et al. Clinical Features and Diagnosis of Dementia with Lewy
Bodies. UpToDate.com. Wolters Kluwer Health. October 2012. Accessed: 11/26/12
Hake, Ann Marie et al. Epidemiology, Pathology, and Pathogenesis of Dementia with
Lewy Bodies. UpToDate.com. Wolters Kluwer Health. October 2012. Accessed:
11/26/12
Randolph, Christopher. Frontotemporal Dementia: Clinical Features and diagnosis.
UpToDate.com. Wolters Kluwer Health. October 2012. Accessed: 11/26/12
Rodnitzky, Robert. Parkinson Disease Dementia. UpToDate.com. Wolters Kluwer
Health. October 2012. Accessed: 11/26/12
Shalden, Marie-Florence et al. Evaluation of Cognitive Impairment and Dementia.
UpToDate.com. Wolters Kluwer Health. October 2012. Accessed: 11/26/12
Wright, Clinton. Etiology, Clinical Manifestations, and Diagnosis of Vascular
Dementia. UpToDate.com. Wolters Kluwer Health. October 2012. Accessed: 11/26/12