Neurocognitive Disorders Flashcards
What is Dementia/Major Neurocognitive Disorder?
Functional brain syndrome resulting from a broad category of brain diseases or brain injury that result in a significant decline in multiple areas of cognitive functioning and the ability to perform routine ADLs from a previous baseline. Impairments are permanent.
Onset is often insidious
What cognitive/behavioral functions are typically affected by dementia?
—Recent episodic (short term) memory —Receptive/expressive language —Semantic memory (factual knowledge) —Executive functions —Motivation —Behavioral regulation
What are the Clinical Diagnostic requirements for Dementia/Major Neurocognitive Disorder?
—Poor memory + at least 1 other cognitive complaint
—Evidence of impairments on formal testing
—Impairments in routine daily activities like cooking/meal prep, bill paying/financial management, meds management, self care etc
***if only memory is impaired its Amnestic disorder
What is Mild Cognitive Impairment?
A transitional stage between normal aging and dementia. Functional brain syndrome in which the onset and progression of cognitive deficits is beyond that expected for an individuals age and level of education, but progress of cognitive deficits is NOT severe enough to impair daily functioning
What are the 4 subtypes of Mild Cognitive Impairment?
—Amnestic/Nonamnestic
—Single Domain/Multiple Domain
What % of individuals with Mild Cognitive Impairment convert to dementia each year?
13%
What are the clinical requirements for a diagnosis of mild cognitive impairment?
—>1 cognitive complaints
—Evidence of impairment on formal testing
—Daily functioning relatively intact
How do you assess daily functioning?
Functional Activities Questionnaire FAQ.
—Completed by informant and rates examinees ability to perform activities in 10 functional categories on a 0-3 scale
0=normal
1= has difficulty but does by self
2= requires assistance to complete activity
3=Dependent/unable to perform activity even with assistance
Scores summed 0-30
Clinical cutoff =9
—indicative of functional impairment and possible cognitive impairment
Major vs Mild NCD is distinguished based on level of impairment in what 6 cognitive domains?
—Attention —Executive functions —New learning and memory —Language —perceptual motor abilities —Social cognition
What labs should you get when evaluating for NCDs?
—Routine imaging (CT and MRI)
—BCB
—Serology (B12, HIV, Syphilis)
—Liver and thyroid function tests
Optional:
—Functional imaging (fMRI, PET)
—EEG
What is delirium?
Characterized by disturbances of
—Ability to direct, focus, maintain, and shift attention
—Awareness of the immediate environment
—Memory, orientation, and language
Results from direct physiologyical consequences of —Medical conditions —substance intoxication or withdrawal —exposure to a toxin —Multiple conditions/exposures
Develops over a comparatively short amount of time (hours to days)
Can be superimposed on a major or mild NCD
Common in hospitals and the elderly and clears/improves once underlying condition resolves
What is the most common NCD type?
Neurocognitive disorder due to Alzheimer’s disease
Accounts for 50-70% of neurocognitive disorders
What are the risk factors for NCD d/t Alzheimer’s?
—Hx of head injury —Low education —Female —First degree relative with Alzheimer’s —APOE E4 Gene (codes for apoliprotein E production)
What is the Disease course of NCD d/t Alzheimer’s like?
Insidious onset
—initial complaints of poor recent episodic memory
—Forgetfulness and confusion may be more apparent in unfamiliar environments
—Apathy/withdrawal may be present early
—As progresses, other cognitive functions, instrumental ADLs, and insight are affected
—Motor skills and basic sensory functions are NOT affected
What is the average survival length of NCD d/t Alzheimers?
8 years from diagnosis at 65
3 years from diagnosis at age 90
What neuroanatomical changes do you see in NCD d/t Alzheimer’s ?
Cortical and subcortical atrophy —Temporal lobes —Hippocampi —Temporal parietal association cortex —Frontal lobes
Dilation/Enlargement of ventricles
—Hydrocephalus ex vacuo
Sultan enlargement
What neuro chemistry components do you see in NCD d/t Alzheimers?
Depletion of Acetylcholine.
Apolipoprotein E4 (APOE4) —Genetic risk for earlier onset —E4 single copy= 1.75x risk —E4 two copies=8x risk
Amyloid Plaques
—Comprised of beta amyloid 42 protein that collects into plaques and causes inflammation and neuronal death
Neurofibrillary tangles
—Comprised of tau proteins in neurons that are atypically folded and result in neuronal death
—NFTs also seen in cases of TBI and FTLD
What is Major/Mild Frontotemporal NCD?
Characterized by progressive loss of neurons in frontal and temporal lobes
—NFTs may be present
—Amyloid plaques are rare
—Insidious onset
The peak age of onset is 55-65 and accounts for 20-25% of NCD cases under age 65
Two subtypes
—Behavioral variant
—Language variant
What is the survival length post onset of Major/Mild Frontotemporal NCD?
Survival = 6-11 years post onset
What is the Behavioral Variant of Major/Mild Frontotemporal NCD?
Characterized by insidious onset behavioral and personality changes and lack of insight.
—Memory and othe cognitive functions are initially preserved
Core behavioral features are: —Behavioral disinhibition —Apathy or inertia —Loss of sympathy or empathy —Perseverative, stereotyped, compulsive/ritualistic behavior —Food cravings or hyper-orality
What is the Language Variant of Major/Mild Frontotemporal NCD?
Prominent decline in some aspect of language functioning
—Expressive language/speech production
—Word finding problems
—Object naming
—Grammar
—Comprehension of words, objects, concepts
Memory is typically noticeably less affected than language
Visuoperceptual abilities relatively unaffected
Two subtypes:
—Primary progressive aphasia
—Semantic Dementia
What is Lewy Body Dementia? (NCD with Lewy Bodies)
Atypical Parkinsonism/Parkinson’s plus disorder —Progressive supranuclear palsy —Corticobasal degeneration —Multiple system atrophy —Olivopontocerebellar atrophy
It shares features of Parkinson’s Disease and Alzheimers Disease and is often misdiagnosed as Parkinson’s.
—Parkinson (motor) symptoms begin well after cognitive and psychiatric symptoms
—Dementia in 50-80% of PD cases
—PD onset to Dementia is 10 years
What are the clinical features of NCD with Lewy Bodies?
Cognitive Decline
—Insidious but progressive
—Complex attention/executive functions affected early
—Visuospatial functions noticeably affected
—Recent memory affected late
—Language largely NOT affected
Visual Hallucinations: —Complex and organized —Lower visual fields —Non distressing —Recognized as hallucinations —Med sensitivity:: Levodopa may worsen hallucinations
They have fluctuations in arousal and cognitive functioning
Other clinical features: —Depression and anxiety —REM sleep behavior disorder —Autonomic dysfunction —Med Sensitivity
What are the Parkinsonian symptoms of NCD with Lewy Bodies?
—Shuffling gait/decreased arm swing while walking
—Decreased facial expression (Masked facies)
—Rigidity and stiffness of movement
—Cogwheeling
—Tremor less frequent than PD
**Antipsychotics may worsen Parkinsonian symptoms
What are Lewy Bodies and what are the other Neuro characteristics of NCD with Lewy Bodies?
Atypical protein deposits
—Present throughout brain
—Similar to Lewy Bodies found in substantial nigra in PD
Diffuse cerebral atrophy
Motor symptoms due to loss of dopaminergic neurons (PD)
Cognitive sx due to loss of cholinergic neurons (AD)
What is Vascular NCD?
Caused by problems in the brains vascular supply. Previously called multi infarct dementia. Emerges over a 5-10 year period.
It’s the 2nd most common form of NCD in the elderly
What are risk factors for Vascular NCD?
Hypertension (80%) Hyperlipidemia Diabetes Coronary artery disease Untreated obstructive sleep apnea Obesity
What does vascular NCD often co occur with?
Alzheimer’s disease
What causes Vascular NCD?
—Multiple small CVAs
—Single strategic infarcts
—Diffuse small vessel cerebral vascular disease
—Cerebral Amyloid Angiopathy (accumulations of beta amyloid plaques along walls of cerebral arteries and the breakdown/rupture of vessel walls)
What are the Cognitive and Behavioral changes seen in vascular NCD?
Cognitive changes:
—Specific functions affected depend upon locations of CVAs
—Possible lateralized presentation
—Psychomotor slowing/impaired attention/executive dysfunction
Behavioral changes:
—Apathy
—Disinhibition
—Emotional lability/Pseudobulbar affect
What is CTE?
A progressive neurodegenerative disease that shares features with Alzheimers and Frontotemporal lobe degeneration (FTLD) and is believed to be caused by repeated brain trauma-mTBI and subconcussive blows.
Ends in a dementia syndrome.
What are the symptoms of CTE?
Early Sx:
—Memory and executive dysfunction
—Changes in mood and emotional regulation
—Behavioral changes/impulse control problems
Symptom onset months/years after concussions/blows to the head and worsen progressively
What is CTE Incidence and predictors?
Boxing= 17% Football= 5-25%
Predictors: —Increased exposure —Age at retirement —Duration of career —Number of bouts —Poor performance —Difficult to knockout —“Brawlers” —APOE 4 Gene
What are the brain anatomical features seen in CTE?
—Ventricular dilation —Cavum septum pellucidum —Medial temporal atrophy —Shrinkage of mammillary bodies —Pallor of the substantia nigra
What Histological features are seen in CTE?
Tau protein positive neurofibrillary tangles in the neocortex
—Predominant in frontal lobe and medial temporal lobe
—Concentrated around penetrating vessels
Neuropil threads in the cortex
—Filaments of abnormally organized phosphorylated tau and ubiquitin proteins
Diffuse amyloid plaques throughout neocortex +/- neuritic plaques
Sparing of the hippocampus
