ALS Regional Onset and Spread: Network-Level Staging Model

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ALS Regional Onset and Spread: Network-Level Staging Model

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experiment Created: 2026-04-02T10:01:41 By: crosslink-v2 Quality: 67% ✓ SciDEX ID: experiment-exp-wiki-experiments-als-regi

🧫 Experiment Protocol Clinicalproposed

SUMMARY

# ALS Regional Onset and Spread: Network-Level Staging Model ## Background and Rationale Amyotrophic lateral sclerosis (ALS) exhibits remarkably stereotyped patterns of regional onset and systematic spread through interconnected motor networks, yet the mechanisms governing this selective vulnerability and predictable progression remain poorly understood. Unlike other neurodegenerative diseases that show more diffuse pathology, ALS demonstrates clear anatomical hierarchies of involvement, with co

METHODOLOGY NOTES

**Phase 1: Multi-Center Patient Recruitment and Clinical Staging (Months 1-12)** • Recruit 500 ALS patients across 10 centers with confirmed diagnosis per El Escorial criteria • Stratify by onset phenotype: limb-onset (n=300), bulbar-onset (n=150), respiratory-onset (n=50) • Collect detailed clinical history including symptom onset timeline and progression mapping • Perform standardized assessments: ALSFRS-R, King's staging system, MiToS staging • Document family history and collect genetic samples for C9orf72, SOD1, TARDBP, FUS screening **Phase 2: Longitudinal Neuroimaging Acquisition (Months 3-24)** • Acquire 3T MRI at baseline, 6, 12, 18 months: T1-weighted, DTI (64 directions), resting-state fMRI • Perform cortical thickness analysis using FreeSurfer pipeline • Generate structural connectivity matrices using probabilistic tractography (FSL PROBTRACKX2) • Calculate functional connectivity networks using CONN toolbox with 264-node parcellation • Quantify corticospinal tract integri

▸Metadatasource: {'type': 'manual', 'source_name': 'wiki'

source	{'type': 'manual', 'source_name': 'wiki', 'extracted_by': 'backfill_v1', 'extraction_date': '2026-04-16T01:00:16.905126Z'}
summary	# ALS Regional Onset and Spread: Network-Level Staging Model ## Background and Rationale Amyotrophic lateral sclerosis (ALS) exhibits remarkably stereotyped patterns of regional onset and systematic s
entities	{'genes': ['HNRNPA2B1/SETX/TARDBP'], 'diseases': ['ALS']}
model_system	human
_schema_version	1
experiment_type	clinical
primary_outcome	Validate ALS Regional Onset and Spread: Network-Level Staging Model
methodology_notes	Phase 1: Multi-Center Patient Recruitment and Clinical Staging (Months 1-12) • Recruit 500 ALS patients across 10 centers with confirmed diagnosis per El Escorial criteria • Stratify by onset phen
replication_status	single_study
extraction_metadata	{'backfill_at': '2026-04-16T01:00:16.905132', 'needs_review': True, 'extraction_notes': 'Backfilled from wiki source (no PMID available)', 'extraction_confidence': 0.4}

📊 Evidence Profile Foundational

Evidence Balance

+0%

Certainty

100%

Debates

0

Incoming

673

Outgoing

457

0 supporting 0 contradicting 0 neutral

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ALS Regional Onset and Spread: Network-Level Staging Model

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