Clinical Dementia

Last Minute Review

Dementia — Last Minute Review

A last-minute review of high-yield facts — dense tables and one-liners for RITE/Board prep. Not a substitute for the full notes.
Major Dementias — Head-to-Head Comparison
Feature AD DLB FTD (bvFTD) VaD
Age Onset >65 (sporadic); 30–60 (familial) >50 45–65 >60
Presenting Symptom Episodic memory loss (encoding deficit) Visual hallucinations, fluctuating cognition, RBD Personality change, disinhibition, apathy Stepwise cognitive decline, executive dysfunction
Memory Early, prominent (encoding failure — poor recognition) Less impaired early; retrieval deficit (recognition better) Relatively preserved early Retrieval deficit; recognition > recall
Behavior Apathy, depression; late agitation/sundowning Depression, apathy, anxiety, Capgras delusion Disinhibition, compulsions, hyperorality, loss of empathy Apathy, depression, pseudobulbar affect
Hallucinations Late, if present Recurrent, well-formed visual (core feature per McKeith 2017 criteria; core features = fluctuating cognition, visual hallucinations, RBD, parkinsonism) Rare Rare
Motor Features Normal until late Spontaneous parkinsonism (symmetric); RBD; falls Parkinsonism late; may overlap CBS/PSP/MND Focal signs, gait disorder, pseudobulbar palsy
EEG Generalized slowing (nonspecific) Fluctuating generalized slowing; posterior slow waves Usually normal early Focal slowing (if infarct)
MRI Hippocampal/medial temporal atrophy; posterior parietal Relative hippocampal preservation vs AD; generalized atrophy Frontal + anterior temporal atrophy (often asymmetric) White matter lesions, lacunes, strategic infarcts
PET/SPECT FDG: temporoparietal hypometabolism; amyloid PET +; tau PET + FDG: occipital hypometabolism; DAT scan ↓ uptake FDG: frontal/anterior temporal hypometabolism Patchy hypometabolism matching vascular territory
CSF Aβ42 ↓, p-tau ↑, t-tau ↑ May have low Aβ42 (co-AD pathology); α-synuclein SAA emerging Neurofilament light ↑; no specific marker No specific fluid biomarker
Pathology Aβ42 plaques + hyperphosphorylated tau NFTs (3R+4R) α-synuclein Lewy bodies (neocortical + limbic distribution drives DLB cognition; brainstem-predominant = PD) Tau (3R or 4R) or TDP-43 Vascular injury ± mixed AD pathology
Genetics PSEN1 (14), APP (21), PSEN2 (1); APOE ε4 risk GBA, SNCA (rare); APOE ε4 risk factor C9orf72, MAPT, GRN NOTCH3 (CADASIL); HTRA1 (CARASIL)
Treatment Lecanemab and donanemab (anti-amyloid mAbs); AChE inhibitors; memantine Rivastigmine; avoid typical antipsychotics; melatonin for RBD SSRIs (trazodone); no disease-modifying therapy Vascular risk factor management; AChE inhibitors (modest)
💎 Board Pearl
The encoding deficit in AD (poor recognition AND recall) distinguishes it from the retrieval deficit in DLB/VaD/subcortical dementias (recognition preserved, recall impaired). This is a classic board differentiator.
FTD Variants
Variant Core Features Language Behavior Atrophy Pattern Pathology Genetics
bvFTD Early personality change, disinhibition, apathy, loss of empathy, hyperorality, executive dysfunction Echolalia, perseveration (late) Prominent from onset: disinhibition, compulsions, social inappropriateness Frontal + anterior temporal (often asymmetric) Tau (3R or 4R) or TDP-43 C9orf72 (TDP-43); MAPT (tau); GRN (TDP-43)
nfvPPA (nonfluent/agrammatic) Effortful, halting, agrammatic speech; apraxia of speech Nonfluent; grammar errors; comprehension preserved for single words Intact early Left posterior frontal / insular (perisylvian) Tau (4R — CBD/PSP-type) Usually sporadic; occasional MAPT
svPPA (semantic) Loss of word meaning; surface dyslexia; prosopagnosia (right-sided) Fluent but empty speech; anomia; lost single-word comprehension Behavioral changes late (overlap bvFTD) Left anterior temporal “knife-edge” atrophy TDP-43 (Type C) Rarely familial
lvPPA (logopenic) Word-finding pauses; phonemic paraphasias; impaired sentence repetition Slow, hesitant; intact grammar; intact single-word comprehension Intact early Left posterior temporal / inferior parietal AD pathology (Aβ + tau) in most cases AD genetics (APOE ε4 risk)
FTD-ALS/MND bvFTD + upper and lower motor neuron signs; poor prognosis (2–3 yr) Variable bvFTD-type behavioral changes Frontal atrophy + motor cortex involvement TDP-43 C9orf72 (most common genetic link FTD–ALS)
Abbreviations: bvFTD = behavioral variant frontotemporal dementia; nfvPPA = nonfluent/agrammatic variant primary progressive aphasia; svPPA = semantic variant PPA; lvPPA = logopenic variant PPA; FTD-ALS/MND = FTD with amyotrophic lateral sclerosis/motor neuron disease; CBS = corticobasal syndrome; PSP = progressive supranuclear palsy
💎 Board Pearl
lvPPA is the “language phenotype of AD” — most cases have underlying amyloid + tau pathology, unlike nfvPPA (tau) and svPPA (TDP-43). Memory aid: svPPA = TDP, nfvPPA = tau, lvPPA = AD. Criteria: bvFTD per Rascovsky 2011 criteria (≥3 of 6 behavioral features for possible bvFTD); PPA classified per Gorno-Tempini 2011 classification. Tau isoforms: Pick disease = 3R; CBD / PSP = 4R; AD = mixed 3R + 4R.
Alzheimer Disease — Genetics & Biomarkers

AD Genetics

Gene Chromosome Inheritance Mechanism
APP 21 Autosomal dominant Amyloid precursor protein; Down syndrome (trisomy 21) → extra APP copy → universal AD neuropathology by age 40; clinical dementia typically emerges in the 50s (mean onset ~55 yr)
PSEN1 14 Autosomal dominant Most common cause of familial early-onset AD; γ-secretase subunit; onset 30–50 yr; most aggressive
PSEN2 1 Autosomal dominant Rarest familial form; γ-secretase subunit; variable onset 40–70 yr
APOE ε4 19 Risk factor (NOT deterministic) Strongest genetic risk factor for sporadic AD; ε4 heterozygote ~3× risk; ε4/ε4 homozygote ~12–15× risk; ε2 is protective
TREM2 6 Risk factor Microglial receptor; variants impair amyloid clearance; ~2–4× risk (similar to single ε4 allele)
Alzheimer amyloid plaque histology
Alzheimer — neuritic amyloid plaques + neurofibrillary tangles.Mikael Häggström, M.D. · CC0 · Wikimedia Commons

AD Biomarkers

Biomarker AD Pattern Interpretation
CSF Aβ42 Decreased Trapped in plaques; Aβ42/40 ratio more accurate than Aβ42 alone
CSF p-tau (181, 217) Increased Specific for AD tau pathology; p-tau 217 emerging as most accurate single fluid biomarker
CSF t-tau Increased Nonspecific marker of neuronal injury (also ↑ in CJD, stroke)
Amyloid PET (florbetapir, florbetaben, flutemetamol) Positive (cortical uptake) Highly sensitive; a negative amyloid PET makes biologically defined AD very unlikely
Tau PET (flortaucipir) Positive (temporal → parietal spread) Correlates with clinical severity; follows Braak staging pattern
FDG-PET Temporoparietal hypometabolism Posterior cingulate involved early; occipital cortex spared (unlike DLB)
MRI Hippocampal / medial temporal atrophy Posterior parietal atrophy in posterior cortical atrophy variant
Plasma p-tau 217 Increased Blood-based biomarker; high accuracy for AD diagnosis; clinical adoption growing
💎 Board Pearl
The AT(N) framework defines AD biologically: A+ (amyloid positive) is required to be on the Alzheimer continuum. A+T+N+ = full AD pathological change. A− = not AD, regardless of T or N status.
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