Dementia Flashcards
What are some of the symptoms of dementia in newly diagnosed (MCI) patients? (2)
What are some hallmarks to look for?
- Poor orientation to time or place
- Impaired recent memory
**Look for mood changes
Alzheimer’s disease (AD):
- Definition
- Disease Characteristics (2)
- Risk factors (3)
Degenerative brain disorder leading to progressive loss of memory and other cognitive functions
- Changes in personality, bx, judgment
- Loss of ADLs
Risks: Advanced age, family hx, Downs’ Syndrome
After age 65 what happens to the prevalence of alzheimer’s disease?
Doubles with every decade; 50% patients over 85 have alzheimer’s
What is the third leading cause of death in the US?
What is the #1 cause of dementia?
Alzheimer’s disease
Describe the general progression of Alzheimer’s disease:
How long is the average course of disease?
Which symptoms show up first?
- Average course of disease = 1 decade
- Memory impairment–> cognitive impairment +/- changes in mood and bx
What are the diagnostic criteria for alzheimer’s disease? (5)
- Memory impairment
- Aphasia, Apraxia, or Agnosia (1 of 3)
- Altered Executive function
- Decline in social/ occupational function
- Gradual onset cognitive decline
What should you look for in the physical exam to dx alzheimer’s?
Subtle signs of neglect and parkinsonian features
What will you seen in the MMSE of a patient with alzheimers?
Decreased recall and reasoning (proverbs) abilities
What 4 things are evaluated by the mini mental status exam?
- Performance of simple tasks
- Abstract thought
- Addition and subtraction
- Communication
Which neurotransmitter is decreased in patients with alzheimer’s dementia?
In which parts of the brain is it decreased?
Acetylcholine: connects these structures to basal forebrain (degenerates w low Ach)
- Hippocampus
- Cerebral cortex
- Amygdala
What are the characteristic senile plaques seen in Alzheimer’s made of?
Where are they located?
Degenerative presynaptic endings +/- astrocytes and microglia (AB peptide)
Located in the neurophil of the cerebral cortex
What are the characteristics neurofibrillary tangles seen in alzheimer’s dementia?
Intracytoplasmic proliferation of twisted filaments located in the pyramidal cells of the hippocampus and cerebral cortex
**Hyperphosphorylated tau
Where is the earliest brain lesion seen in alzheimer’s disease?
What is seen on fMRI?
Physiologic lesion in the hippocampus
Decreased oxidative metabolism seen in fMRI
List three treatment options for patients with Alzheimer’s:
- Cholinesterase inhibitors
(mild to moderate disease) - Antipsychotics/ Antidepressants
(For bx symptoms) - Estrogen replacement therapy
Which AchEi has less neuropsych sx and why?
Rivastigmine; can via patch form for sustained release
How does food affect tacrine metabolism (AchEi)
Decreases bioavailability 30-40%
How does food affect donepezil (AchEi) metabolism?
NOT AT ALL
How does food affect rivastigmine metabolism?
Slows rate and increases absorption 30%
How is galantine metabolism affected by food?
Slows rate but not extent of absorption
Memantine/ Namenda:
What is the MOA?
What does it treat?
Binds NMDA receptors (ANTAgonist)
Given in does of 5-20mg per day to treat Alzheimers
What vitamin is given as a treatment for alzheimers?
Vitamin E (1000U BID)
What are two important preventative measures against developing alzheimers?
Smoking cessation and aerobic exercise
5 Symptoms typically inconsistent with Alzheimer’s Dementia:
1- Sudden onset 2- Focal neurologic findings 3- Seizures (early in the course) 4- Abnormal gait/coordination 5- Preservation of memory function
7 Signs Atypical of Alzheimer’s Disease:
1- Dominant non-memory sx
2- Prominent behavioral, personality, psychotic Sx
3- Early parkinsonism
4- Early bulbar/ brainstem signs
5- Unexplained motor or reflex asymmetries
6- Unexplained (early) UMN or LMN signs
What is the clinical triad for Hydrocephalic Dementia (Normal Pressure Hydrocephalus)?
Which symptoms improve first with spinal tap?
- Dementia
- Gait Apraxia (magnetic gait)
- Urinary incontinence
Gait–> dementia–> incontinence
How is Normal pressure hydrocephalus diagnosed? (2)
- Enlarged ventricles on imaging
- Spinal tap is diagnostic and therapeutic
List the 6 Frontotemporal Dementias?
- Pick’s Disease
- Dementia lacking distinctive histopathologic features
- Progressive subcortical gliosis
- Primary Progressive Aphasia
- ALS Dementia/ MND Dementia
- Disinhibition- Dementia- Parkinsonism-Amyotrophy
What are the diagnostic criteria for Frontotemporal Dementia? (4)
- Language impairment (echolalia, stereotypy, progressive reduced speech)
- Frontal deficits (Decrease drive, interests; increased mental rigidity, social impropriety)
- Change in Affect
- Frontotemporal changes on imaging
What characteristics are preserved in frontotemporal dementia? (2)
- Praxis and visuospatial skills
- Memory
Pick’s Disease:
- Age and population
- 4 symptoms of disease
- Imaging and Path
- 60+ yof
Symptoms:
- Aphasia
- Dementia (spares praxis + visuospatial fxn)
- Personality changes
- Frontal Release signs
Imaging: Frontotemporal atrophy sparing 1/3 superior temporal gyrus; intracytoplasmic PICK BODIES , ballooned neurons
Where in the brain are pick bodies found? (3)
- Cortex
- Basal ganglia
- Brainstem
Which drugs shouldn’t be given to patients with Lewy Body Dementia?
Antipsychotics–May worsen sx
What are the diagnostic criteria for Lewy Body Dementia? (3 core features, 5 accessory features)
Core Features (2/3 probable):
- Fluctuating cognition: attention/ alertness
- Well formed visual hallucinations
- Parkinsonism
+/- falling, syncope, LOC, neuroleptic sensitivity, delusions/ other hallucinations
What are Lewy bodies?
Eosinophilic cytoplasmic inclusions within substantial nigra (Seen in Lewy body Dementia)
Progressive Supranuclear Palsy
(Steel Richardson Olsezewski Syndrome):
- Age and population
- Presentation (4 features)
- 40+ yo men
Presentation
- Supranuclear gaze deficits
- Parkinsonism
- Pseudobulbar palsy, dysphagia, dysarthria
- Echolalia, Palilalia
What is seen on imaging with Progressive Supranuclear Palsy (2 areas damaged)?
Pathology?
Which parts of the brain are spared?
Imaging:
Atrophic pons + midbrain
Path:
- NF TANGLES* in midbrain
- *Spared cortex and cerebellum
Which supra nuclear gaze deficits manifest first in PSP?
Down gaze–> up gaze–> horizontal
Apraxic eyelid opening evident
Cortical-Basal Ganglionic Degneration:
- Age, population
- Presentation (3)
- 70+ M=F
- Presents asymmetrically, unilaterally
- Extrapyramidal rigidity
- ***Alien Hand Syndrome; agosnia of limbs and upper extremities
- Supranuclear palsy
What is seen on imaging in Cortical-Basal Ganglionic Degeneration?
Path?
Imaging: Asymmetric parietal lobe atrophy
Path: Degenerating cortex + substantia nigra, WITHOUT PICK BODIES OR, TANGLES
Which NT is deficient in Huntington’s Disease?
2
GABA and Ach in striatum
What are the three treatments for Huntington’s?
- Haldol
- Tetrabenazine
- Klonopin
Wilson’s Disease: Inheritance pattern, Chromosome, age of onset?
AR, Chromosome 13
Onset 11-25 years
How does wilson’s disease present clinically? (4)
Labs (2)?
- Subcortical dementia w psychosis
- Dysphagia, dysarthria
- Rigidity, tremor
- KF rings
- Labs: ^ Cu, Low Ceruloplasmin
Which area of the brain is atrophic in Wilson’s disease?
Describe the path findings:
- What type of cells?
- What type of degeneration and where specifically?
Brick red, atrophic Basal ganglia
- Opalski cells (Astrocytes)
- spongy degeneration/ cavation of putamen
Treatment for wilson’s disease?
Penicillamine, Pyridoxine, Zinc Acetate, liver transplant
What are the diagnostic criteria for AIDS dementia
2 of the following cognitive declines for at least 1 month:
- Attention/ concentration
- Info processing
- Abstract reasoning
- Visuospatial skill
- Memory/ learning
- Speech/ language
Progressive Multifocal Leukoencephalopathy:
- Viral etiology?
- Who gets this?
- How does it present?(3)
- Describe the Path?
- Papovirus (JC Virus, SV- 40 Virus)
- Immunosuppressed
Presents with dementia, visual loss, Progressive hemiparesis
Path: occipital demyelination
What are the treatments for PML?
- Vistidide
- Cytarabine
- HAART
Which Major Human Slow Virus is Sporadic?
What do you see on FLAIR MRI?
Path Changes?
CJD; patients die within 12 mos
- Bilateral high signal in anterior basal ganglia on MRI
- Spongiform changes in brain
What are the 5 types of vascular dementia?
- Multi infarct (large vessel)
- Strategic single infarct (PCA, ACA, Thalamic, BF)
- Small Vessel with Multiple Lacunes (Binswanger)
- Hypoperfusion
- Hemorrhagic
What is the most common cause of dementia?
Small vessel changes
What is seen on MRI that indicates small vessel ischemic change?
Hypodense areas around vessels
What are the diagnostic criteria for Binswanger’s Type Vascular dementia?
Dementia on clinical exam + 2 of the following :
- HTN or vascular disease
- Cerebrovascular disease
- Subcortical brain dysfunction (gait, rigidity, neurogenic bladder)
Will see bilateral subcortical leukokariosis (Attenuated white matter) on CT/ MRI
What does “CADASIL” stand for?
How does the disease present and on what chromosome is the gene?
When in lifedoes the disease present? (4)
Cerebral Autosomal Dominant Arteriopathy w/ Subcortical Infarcts + Leucoencephalopathy
Disease on Chromosome 19
Presents 20-40 yoa with recurrent stroke, dementia, classical MIGRAINE, depression/ mania
What should be done in a nutritional workup for dementia? (5)
- B12, B1
- Vit D
- TSH, Free T4
- RPR
- Paraneopalstic panel
Dementia with NF tangles in midbrain?
PSP
