1. Non-Alzheimer’s Dementias
Scott Morgan MSIII
December 2012

2. DSM-IV Definition of Dementia
Major impairment of memory and learning as well as one of:
Impairment handling complex tasks
Impairment in reasoning ability
Impairment spatial ability and orientation
Impaired language
Insidious and progressive onset
Symptoms interfere with relationships, work performance, and social activities

3. Recognizing Dementia Memory loss rarely reported by patient
Spouse or family member usually reports
Family reported memory loss is a better predictor
Commonly thought to just be from aging
Insidious onset often causes a delay in reporting memory loss
Must be distinguished from delirium and depression

4. Mental Status Tests

5. Parkinson Disease Dementia
Prevalence of dementia in Parkinson Disease (PD) patients is 41%
Mild cognitive impairment is common in PD
Does not always meet criteria for dementia
Genetic factors associated with Parkinson Disease Dementia (PDD)
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

6. Early Clinical Features of PDD
Executive dysfunction with deficiencies in:
Set shifting
Attention
Planning
Visuospatial impairment
Facial recognition problems
Impaired tone contrast
Verbal memory
Difficulty remembering words
Unable to explain their problems

7. Late Clinical Features of PDD
Psychosis
Visual hallucinations
Delusions
Spousal infidelity paranoia
Mood disorders
Depression
Anxiety
Apathy
Sleep disturbances
Sleep fragmentation
Nightmares
REM sleep behavior disorder
Patient has normal dreams without the muscle atonia

8. Treatment of PDD Cholinesterase inhibitors For psychosis
Have a benefit for cognition
No evidence for improvement of neuropsychiatric symptoms
For psychosis
Quetiapine
Clozapine
Avoid anticholinergic medications

9. Dementia with Lewy Bodies
Second most common degenerative dementia
10-22% of all dementia cases
There have been familial cases, but most are sporadic
Multiplications of alpha-synuclein gene are associated with familial DLB
Only known risk factor is increased age
Mean age of onset is 75
Some studies show more likely in males
Still debated and evidence is not strong

10. 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

11. Diagnosing DLB Features of Alzheimer’s Disease and Parkinson Disease
More rapid onset than Alzheimer’s
Can have amyloid plaques similar to Alzheimer’s
Not nearly as numerous as Alzheimer’s
Different from PDD by
Onset of dementia compared to parkinsonian symptoms
Before, during, or very shortly after parkinsonian signs
Can detect brain atrophy with MRI and PET scans
Not necessary for diagnosis but can provide supportive evidence

12. Clinical Features of DLB
Very similar to PDD
Fluctuating cognition and alertness
Patients can “blank out” or lose consciousness
Episodes last from seconds to days
Visual hallucinations
Early sign in DLB compared to late in PDD
Parkinsonism
Gait disorders
Bradykinesia
Limb rigidity

13. Treatment of DLB Treatments are just for symptoms
No treatment has been found to have disease modifying effects
Selegiline has been used as a treatment
Limited evidence for effectiveness

14. Vascular Dementia Aka multi-infarct dementia
Makes up 10-20% of dementia cases
Risk factors
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

15. Pathology of VaD Large artery infarcts
Usually cortical but sometimes subcortical
Small artery or lacunar infarcts
Exclusive subcortical in small penetrating arteries
Affect basal ganglia, caudate, thalamus, internal capsule, cerebellum, and brainstem
Chronic subcortical ischemia
Small arteries in periventricular white matter
Order of vulnerability – neuron, oligodendrocyte, myelinated axon, astrocyte, endothelial cell

16. Diagnosis of VaD Neuroimaging MRI is most sensitive
CT is usually first line scan
Step-wise loss of cognition and memory
Disease progresses with each ischemic event
Each progression can be large or small

17. Clinical Features of VaD
Specific to area affected
Medial frontal
Executive function
Apathy
Left parietal
Aphasia
Apraxia
Agnosia
Right parietal
Hemineglect
Confusion
Agitation
Visuopatial difficulty
Constructional difficulty
Medial temporal
Anterograde amnesia

18. Frontotemporal Dementia
Aka:
Picks Disease
Pick complex
Frontal lobe dementia
Age of onset is late 50s to early 60s
Average age is 58
As the name implies it is a degeneration of the frontal and temporal lobes

19. Pick Bodies Build up of tau proteins in neurons
Cause neurons to swell and appear spherical
Silver staining for diagnosis
Help distinguish from other dementias

20. Clinical Features of FTD
Unique because of personality changes
Aggressiveness
New onset of criminal activity
Socially inappropriate
Sexually inappropriate
Lack of insight
Progressive non-fluent aphasia
Word-finding difficulty and speech errors
Unusual eating patterns
Some patients will binge on alcohol
Incontinence

21. Diagnosis and Treatment of FTD
MRI is most common scan used to diagnose
Very little evidence to support pharmacological treatment
Used to treat symptoms
Acetylcholinesterase inhibitors used to be used for treatment but falling out of favor
SSRIs are used to treat depression if this is a symptom
Neuroleptics can be used but side effects must be weighed against benefits

22. Summary Alzheimer’s dementia is most common dementia
Memory loss is the major hallmark of dementia
Dementias are often difficult to diagnose and treat
Diagnosis is mainly by clinical features rather than using imaging scans
Most treatments are for symptoms not disease modification

23. Citation
Hake, Ann Marie et al. Clinical Features and Diagnosis of Dementia with Lewy Bodies. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12 Hake, Ann Marie et al. Epidemiology, Pathology, and Pathogenesis of Dementia with Lewy Bodies. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12 Randolph, Christopher. Frontotemporal Dementia: Clinical Features and diagnosis. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12 Rodnitzky, Robert. Parkinson Disease Dementia. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12 Shalden, Marie-Florence et al. Evaluation of Cognitive Impairment and Dementia. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12 Wright, Clinton. Etiology, Clinical Manifestations, and Diagnosis of Vascular Dementia. UpToDate.com. Wolters Kluwer Health. October Accessed: 11/26/12