Dementia with Lewy bodies
Manifest points at the shared real placeholder prisma/seed/manifests/neuro.dementia.core.v1.ts — no dedicated geriatrics.dementia-lewy-body manifest or problem-package folder yet (deferred; tracked in design brief Open gaps). No rxcui on any RegimenDrug — all pharmacologic entries (rivastigmine, donepezil, memantine, carbidopa-levodopa, melatonin, clonazepam, midodrine, fludrocortisone, droxidopa, quetiapine, pimavanserin) are name-only pending RxNav validation (Stage-A); diagnostic/safety/non-pharm/deprescribing/monitoring actions carry non_pharm:true. THE central safety axis is severe neuroleptic (antipsychotic) hypersensitivity — encoded as a Step-1 alert, contraindication rules, two life_threatening severity triggers, and both setting playbooks; ChEI is first-line for cognition AND hallucinations. Sibling engine ids geriatrics.dementia-alzheimer.core.v1 and geriatrics.delirium.core.v1 are referenced by id (both present on disk in this dossier layer); the 1-year rule (DLB vs Parkinson disease dementia, same Lewy spectrum) is handled in overlap_handling pending a dedicated PD/PDD engine id. calc.moca + calc.acb_scale + calc.gds_15 used (no DLB-specific fluctuation/biomarker calculator adapter in clinical-tools-registry yet); DAT-SPECT/MIBG/PSG indicative biomarkers and Bayesian LRs are deferred to the reasoning-depth pass.
Entry points (5)
- symptomRecurrent well-formed visual hallucinations with progressive cognitive decline (McKeith 2017 core)recurrent_visual_hallucinations_with_cognitive_decline
- symptomFluctuating attention/alertness + spontaneous parkinsonism (bradykinesia/rigidity/rest tremor) with dementia (McKeith 2017 core)fluctuating_cognition_with_parkinsonism
- historyDream-enactment / REM sleep behavior disorder (may predate cognition by years) — prodromal/core DLB (McKeith 2017)rem_sleep_behavior_disorder_dream_enactment
- historySevere deterioration / parkinsonism / NMS-like reaction after an antipsychotic — strong DLB pointer (McKeith 2017 supportive)severe_neuroleptic_sensitivity_reaction
- problem_listEstablished PD developing dementia — apply the 1-year rule (DLB vs PDD; same Lewy spectrum)parkinson_disease_with_new_dementia
Required inputs (15)
- agerequireddemographic • used at FRAMEDLB onset typically >60y; early onset prompts atypical/genetic and prion considerations (McKeith 2017)
- cognitive_symptom_timelinerequiredhistory • used at ENTRYInsidious progressive course over months-to-years distinguishes DLB from delirium and rapidly progressive dementia; defines the 1-year rule vs PD (McKeith 2017)
- core_feature_inventoryrequiredhistory • used at CONTEXTFluctuating cognition, recurrent visual hallucinations, RBD, spontaneous parkinsonism — number of core features drives probable vs possible DLB (McKeith 2017)
- informant_historyrequiredhistory • used at CONTEXTInformant required — fluctuations, hallucinations, dream-enactment, and falls are often unreported by the patient (McKeith 2017)
- parkinsonism_onset_relative_to_cognitionrequiredhistory • used at DIFFERENTIALThe 1-year rule: cognitive onset before/within 1y of parkinsonism = DLB; dementia >1y after established PD = PDD (McKeith 2017)
- rem_sleep_behavior_disorderrequiredhistory • used at CONTEXTRBD (dream-enactment, REM-without-atonia on PSG) is a core feature and may precede cognition by years; high specificity for Lewy pathology (McKeith 2017)
- autonomic_dysfunctionrequiredhistory • used at CONTEXTOrthostatic hypotension, constipation, urinary dysfunction, syncope — supportive features and key safety/management targets (McKeith 2017)
- antipsychotic_exposure_and_responserequiredhistory • used at RED_FLAGSSEVERE neuroleptic hypersensitivity — prior antipsychotic exposure/reaction is a major safety datum; future antipsychotics may be fatal (McKeith 2017)
- current_medsrequiredmedication • used at CONTEXTAnticholinergics/dopamine agonists/amantadine/benzodiazepines worsen cognition, hallucinations, falls — deprescribing target; antipsychotic exposure is a red flag (AGS Beers 2023)
- falls_and_syncope_historyrequiredhistory • used at CONTEXTRepeated falls/syncope (parkinsonism + orthostatic hypotension) — supportive feature driving a falls/autonomic program (McKeith 2017)
- b12requiredlab • used at INITIAL_WORKUPB12 deficiency is a reversible/contributing cause — mandatory exclusion before attributing to DLB (NICE NG97)
- tshrequiredlab • used at INITIAL_WORKUPHypothyroidism is a reversible/contributing cause — mandatory exclusion (NICE NG97)
- structural_brain_mrirequiredimaging • used at INITIAL_WORKUPMRI (or CT) excludes NPH/subdural/tumor/strategic infarct; relative medial-temporal preservation supports DLB over AD (McKeith 2017)
- dlb_indicative_biomarker_statushistory • used at BRANCHING_WORKUPReduced DAT-SPECT/PET striatal uptake, abnormal MIBG cardiac scintigraphy, PSG-confirmed REM-without-atonia are indicative biomarkers raising diagnostic certainty (McKeith 2017)
- caregiver_and_capacityrequiredhistory • used at CONTEXTCaregiver availability/burden + decision-making capacity drive neuroleptic-avoidance education, ACP, driving, and disposition (NICE NG97)
12-phase flow (12)
- 1FRAMEConfirm scope: chronic insidious progressive cognitive decline in an older adult with Lewy-spectrum features; not acute delirium and not rapidly progressive (<1-2y → CJD) dementia (McKeith 2017)inputs: age, cognitive_symptom_timelineadvance: Chronic progressive cognitive complaint with ≥1 Lewy-spectrum pointer established; acute/rapid course excluded or pivoted (McKeith 2017)
- 2ENTRYCapture trigger: recurrent visual hallucinations with decline, fluctuating cognition + parkinsonism, dream-enactment/RBD, a severe antipsychotic reaction, or PD developing dementia (McKeith 2017)inputs: cognitive_symptom_timelineadvance: Entry trigger documented with onset and tempo (McKeith 2017)
- 3CONTEXTInformant history; full core-feature inventory (fluctuation, visual hallucinations, RBD, parkinsonism); autonomic review (orthostasis/constipation/urinary), falls/syncope, hyposmia, hypersomnia, depression/apathy; medication review (anticholinergic/dopaminergic/sedative burden, prior antipsychotic exposure); caregiver availability + capacity (McKeith 2017; NICE NG97)inputs: core_feature_inventory, informant_history, rem_sleep_behavior_disorder, autonomic_dysfunction, current_meds, falls_and_syncope_history, caregiver_and_capacityactions: workup.cga, workup.beers_screen, workup.fallsadvance: Informant-corroborated core/supportive-feature profile and contributory-factor context established (McKeith 2017)
- 4RED_FLAGSSEVERE NEUROLEPTIC HYPERSENSITIVITY — flag any antipsychotic exposure/consideration (irreversible parkinsonism, NMS-like, autonomic collapse, rapid decline, death); superimposed delirium (common, low threshold, AVOID antipsychotic); severe orthostatic syncope/falls; rapid progression (<1-2y → CJD) (McKeith 2017)inputs: antipsychotic_exposure_and_response, cognitive_symptom_timelineactions: workup.deliriumadvance: Neuroleptic-avoidance alert raised; no red flag, or red flag pivots to delirium/rapid-dementia workup (McKeith 2017)
- 5INITIAL_WORKUPObjective cognitive testing (MoCA — sensitive to fluctuating attention/visuospatial deficit); functional assessment; anticholinergic burden (ACB); depression screen (GDS-15); reversible-cause labs B12/TSH + CMP/CBC; structural MRI (or CT) to exclude NPH/subdural/tumor/strategic infarct and assess relative medial-temporal preservation (McKeith 2017; NICE NG97)inputs: b12, tsh, structural_brain_mri, core_feature_inventoryactions: workup.dementia, calc.moca, calc.acb_scale, calc.gds_15, panel.metabolic, panel.cmp, panel.cbc, panel.tshadvance: Cognitive/functional profile quantified; reversible/contributing causes excluded or treated; structural imaging reviewed (McKeith 2017)
- 6BRANCHING_WORKUPIndicative biomarkers to raise diagnostic certainty: DAT-SPECT/PET (reduced striatal dopamine transporter uptake), I-123 MIBG myocardial scintigraphy (reduced cardiac uptake), PSG-confirmed REM-sleep-without-atonia; supportive 18F-FDG-PET occipital hypometabolism/cingulate-island sign; targeted rapid-dementia workup if course is rapid (CJD: MRI DWI, CSF RT-QuIC, EEG) (McKeith 2017)inputs: dlb_indicative_biomarker_statusadvance: Indicative-biomarker status resolved (probable vs possible DLB) or rapid-dementia workup launched (McKeith 2017)
- 7DIFFERENTIALDLB (≥2 core, or 1 core + ≥1 indicative biomarker = probable) vs Alzheimer disease (early prominent amnesia, late hallucinations), vascular dementia (stepwise, infarct burden, early gait/executive), Parkinson disease / PDD (the 1-year rule), delirium (acute fluctuating inattention — but frequently superimposed), and CJD (rapidly progressive) (McKeith 2017)inputs: parkinsonism_onset_relative_to_cognition, core_feature_inventoryadvance: Probable/possible DLB assigned with core-feature + biomarker support; siblings differentiated; 1-year rule applied vs PD/PDD (McKeith 2017)
- 8RISK_STRATIFICATIONDiagnostic certainty (probable vs possible DLB by core-feature/biomarker count); functional stage and MoCA trajectory; SAFETY stratification — neuroleptic-exposure risk, fall/syncope risk (parkinsonism + orthostatic hypotension), anticholinergic cognitive burden (ACB), delirium vulnerability (McKeith 2017; AGS Beers 2023)inputs: core_feature_inventory, autonomic_dysfunctionactions: calc.moca, calc.acb_scale, calc.news2advance: Diagnostic certainty + functional stage assigned; neuroleptic/fall/autonomic/delirium risks stratified (McKeith 2017)
- 9TREATMENTConfirm DLB + raise the NEUROLEPTIC-AVOIDANCE ALERT (card/EHR flag) FIRST; cholinesterase inhibitor (rivastigmine/donepezil) FIRST-LINE for cognition AND hallucinations ± memantine adjunct; cautious LOW-dose levodopa only for disabling parkinsonism (worsens hallucinations/orthostasis — avoid dopamine agonists/anticholinergics/amantadine); RBD — melatonin first-line (limit/avoid clonazepam — sedation/falls); autonomic OH — non-pharm + midodrine/fludrocortisone/droxidopa, manage constipation/urinary; psychosis — treat triggers + non-pharm FIRST, AVOID antipsychotics, only cautious low-dose quetiapine or pimavanserin if truly unavoidable; treat depression (avoid anticholinergic antidepressants)/apathy; deprescribe anticholinergics rigorously; caregiver education + ACP + falls program (McKeith 2017; NICE NG97)inputs: core_feature_inventory, current_meds, autonomic_dysfunction, caregiver_and_capacityadvance: Neuroleptic-avoidance alert active; ChEI started; parkinsonism/RBD/autonomic/psychosis pathways set; anticholinergics deprescribed; caregiver/ACP/falls plan in place (McKeith 2017)
- 10DISPOSITIONPredominantly outpatient with neurology/cognitive-disorders/geriatrics co-management; inpatient only for superimposed delirium, neuroleptic-induced severe sensitivity reaction, severe orthostatic syncope/recurrent injurious falls, or disabling refractory psychosis/caregiver breakdown; long-term-care planning in advanced disease (NICE NG97)inputs: caregiver_and_capacityadvance: Care setting + multidisciplinary referrals (neurology, sleep, autonomic/falls, social work, caregiver support) confirmed (NICE NG97)
- 11MONITORINGCognitive/functional re-assessment (MoCA + ADL/IADL) and fluctuation tracking every 6-12 months; parkinsonism progression and levodopa tolerability (hallucinations/orthostasis); orthostatic vitals at each visit; RBD control; ChEI tolerability (GI, bradycardia/syncope); ACTIVE neuroleptic-exposure surveillance (medication reconciliation every encounter); caregiver burden each visit (McKeith 2017; NICE NG97)inputs: core_feature_inventory, current_meds, autonomic_dysfunctionactions: calc.moca, calc.acb_scaleadvance: Monitoring cadence set; no untreated neuroleptic exposure, intolerable ChEI/levodopa ADR, uncontrolled orthostasis, or caregiver crisis (McKeith 2017)
- 12FOLLOWUPAdvance care planning revisited at each transition; neuroleptic-avoidance card/alert reinforced with patient, caregivers, and all treating clinicians; driving/finances/capacity reviewed; falls/autonomic program maintained; caregiver respite/support; palliative/end-of-life planning and ChEI/levodopa deprescribing decisions in advanced disease (NICE NG97)inputs: caregiver_and_capacityadvance: ACP documented; neuroleptic-avoidance alert disseminated; caregiver support arranged; next cognitive/autonomic review scheduled (NICE NG97)