{
  "vercy": "1.0-draft",
  "publication": {
    "status": "research-draft",
    "adjudicationStatus": "unreviewed",
    "publishableCanonical": false,
    "generatedAt": "2026-09-13T04:06:08Z",
    "providers": [
      "Claude"
    ],
    "breadth": "written by Claude from model knowledge without web access - no source was read, every claim is a lead to verify",
    "pass": 2,
    "wave": 3,
    "engine": "claude",
    "enrichment": {
      "pass": 3,
      "engine": "claude",
      "producedAt": "2026-10-06T10:00:00Z",
      "ingestedAt": "2026-10-06T07:10:50+00:00",
      "fields": [
        "agent_conduct",
        "ethics",
        "owners"
      ]
    },
    "gate": {
      "passed": true,
      "version": "1.0",
      "at": "2026-10-06T19:52:01+00:00",
      "evidence": [
        "completeness 1.00",
        "sense: Wikidata description",
        "2 live sources",
        "review by codex: pass"
      ]
    }
  },
  "metaModel": {
    "id": "THING-Q649602",
    "registryId": "vr.tr.ohtahara-syndrome",
    "name": "Ohtahara syndrome",
    "version": "0.2.0-wave.3",
    "entryKind": "thing",
    "family": "Thing Registry",
    "domain": [
      "XCT.STA"
    ],
    "status": "research-draft"
  },
  "canonicalUrl": "https://ver.cy/models/thing/q649602/",
  "model": {
    "registry_id": "vr.tr.ohtahara-syndrome",
    "name": "Ohtahara syndrome",
    "purpose": "Let an agent explain Ohtahara syndrome in general terms from paediatric neurology sources, describe causes, diagnosis and management, relay the current ILAE classification and genetic subtypes, and route families to specialists and support organisations.",
    "definition": "A rare and severe epileptic encephalopathy beginning in the first months of life, characterised by tonic spasms and a burst-suppression pattern on EEG, caused by structural brain abnormalities or by mutations in genes such as STXBP1, KCNQ2, SCN2A and others catalogued as early infantile epileptic encephalopathies numbered by gene, with poor prognosis for development and frequent evolution to West syndrome; Ohtahara syndrome is now grouped by the ILAE within early infantile developmental and epileptic encephalopathy and is managed by paediatric neurologists.",
    "what_it_is_for": "Not applicable; a condition.",
    "affordances": [
      "explain the condition",
      "relay causes and classification",
      "relay management",
      "route families"
    ],
    "distinguishing_features": [
      "Neonatal onset",
      "Burst-suppression EEG",
      "Tonic spasms",
      "Severe prognosis"
    ],
    "appearance": "Not a visible object; infant seizures with a burst-suppression EEG.",
    "visual_identification": [
      "Early infantile epileptic encephalopathy with burst suppression",
      "Structural and genetic forms; early infantile epileptic encephalopathy types numbered by gene",
      "West syndrome begins later with hypsarrhythmia; benign neonatal seizures have good prognosis"
    ],
    "physical_properties": [
      {
        "quantity": "onset",
        "typical_range": "first 3",
        "unit": "months",
        "note": "often first weeks"
      },
      {
        "quantity": "incidence",
        "typical_range": "about 1 in 100000",
        "unit": "births",
        "note": "estimates"
      },
      {
        "quantity": "ILAE reclassification",
        "typical_range": "2022",
        "unit": "year",
        "note": "as EIDEE"
      }
    ],
    "families_and_kinds": [
      "structural Ohtahara syndrome from brain malformations",
      "genetic forms by gene such as STXBP1, KCNQ2, SCN2A, CDKL5 and others catalogued as early infantile epileptic encephalopathy numbers",
      "metabolic causes",
      "evolution to West syndrome and Lennox-Gastaut syndrome"
    ],
    "related_models": [
      {
        "relation": "is a kind of",
        "target": "infantile epileptic encephalopathy",
        "why": "in registry terms"
      },
      {
        "relation": "is a kind of",
        "target": "neonatal period electroclinical syndrome",
        "why": "in registry terms"
      },
      {
        "relation": "is classified by",
        "target": "International League Against Epilepsy",
        "why": "within EIDEE"
      },
      {
        "relation": "often evolves into",
        "target": "West syndrome",
        "why": "in infancy"
      }
    ],
    "identifiers": [
      {
        "scheme": "ICD-11",
        "value_or_pattern": "8A62.0",
        "note": "developmental and epileptic encephalopathies related"
      },
      {
        "scheme": "Orphanet",
        "value_or_pattern": "ORPHA:1934",
        "note": "Ohtahara syndrome"
      },
      {
        "scheme": "OMIM",
        "value_or_pattern": "308350 and others",
        "note": "EIEE subtypes"
      }
    ],
    "standards_and_regulation": [
      "ILAE classification of epilepsy syndromes",
      "Paediatric neurology guidelines on infantile epilepsies",
      "Rare disease frameworks and genetic testing guidance"
    ],
    "failure_modes_and_hazards": [
      "Agents giving personal medical advice",
      "Confusing with other infantile epilepsies",
      "Stale classification",
      "Overgeneralising prognosis across genetic subtypes"
    ],
    "in_scope": [],
    "out_of_scope": [],
    "characteristics": [],
    "agent_conduct": {
      "may": [
        "Explain Ohtahara syndrome and its genetic and structural causes from medical sources.",
        "Point families to epilepsy and rare disease support organisations.",
        "Distinguish it from other infantile epilepsies."
      ],
      "must_not": [
        "Give a prognosis or treatment plan for a specific child.",
        "Generalise outcomes across genetic subtypes.",
        "Promote unproven treatments.",
        "Share or speculate about a named child's diagnosis."
      ],
      "requires_human": [
        "Decisions on treatment, surgery or palliative care for an infant."
      ]
    },
    "ethics": {
      "considerations": [
        "Families face grave decisions and need honest, compassionate information.",
        "Rare disease families are targets for unproven therapies."
      ],
      "affected_parties": [
        "Infants",
        "Parents and siblings",
        "Bereaved families"
      ]
    },
    "owners": {
      "steward": "Nobody owns the condition; paediatric neurology teams answer for care.",
      "master_systems": [
        "Rare disease registries"
      ]
    }
  },
  "sources": [
    {
      "id": "wikidata",
      "title": "Wikidata item Q649602: Ohtahara syndrome",
      "url": "https://www.wikidata.org/wiki/Q649602",
      "url_status": "live",
      "what_it_supports": "identity and sense of the item",
      "checked_by": "wikidata-api"
    },
    {
      "id": "wikipedia-en",
      "title": "Wikipedia: Ohtahara syndrome",
      "url": "https://en.wikipedia.org/wiki/Ohtahara_syndrome",
      "url_status": "live",
      "what_it_supports": "general description of the item",
      "checked_by": "wikidata-api sitelink"
    }
  ],
  "structure": {
    "bundles": [
      {
        "id": "help",
        "name": "Help",
        "description": "Getting help first.",
        "rationale": "Safety.",
        "layers": [
          {
            "id": "seizures",
            "name": "Seizures",
            "description": "Infant seizures.",
            "findings": [
              {
                "id": "seizures-finding",
                "name": "Seizures",
                "description": "Seizures.",
                "questions": [
                  {
                    "text": "What should parents do if an infant has seizures or spasms, per emergency guidance?",
                    "kind": "action"
                  },
                  {
                    "text": "Is the user describing seizures in an infant, which need emergency or urgent care now?",
                    "kind": "boundary"
                  }
                ]
              }
            ]
          },
          {
            "id": "care",
            "name": "Care",
            "description": "Specialist care.",
            "findings": [
              {
                "id": "care-finding",
                "name": "Care",
                "description": "Care.",
                "questions": [
                  {
                    "text": "How is Ohtahara syndrome diagnosed by paediatric neurologists with EEG, imaging and genetic testing, in general terms?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Is the user asking about their own child, which needs the neurology team?",
                    "kind": "boundary"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "understand",
        "name": "Understand",
        "description": "The condition.",
        "rationale": "Clinical.",
        "layers": [
          {
            "id": "definition",
            "name": "Definition",
            "description": "Definition and features.",
            "findings": [
              {
                "id": "definition-finding",
                "name": "Definition",
                "description": "Definition.",
                "questions": [
                  {
                    "text": "What is Ohtahara syndrome, and what are its seizure types and EEG features?",
                    "kind": "definition"
                  },
                  {
                    "text": "Is the question about Ohtahara syndrome, West syndrome or another infantile epilepsy?",
                    "kind": "boundary"
                  }
                ]
              }
            ]
          },
          {
            "id": "causes",
            "name": "Causes",
            "description": "Causes and subtypes.",
            "findings": [
              {
                "id": "causes-finding",
                "name": "Causes",
                "description": "Causes.",
                "questions": [
                  {
                    "text": "What structural, genetic and metabolic causes exist, and how are genetic subtypes numbered?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Which entry fits the specific gene?",
                    "kind": "action"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "manage",
        "name": "Manage",
        "description": "Management and outlook.",
        "rationale": "Clinical.",
        "layers": [
          {
            "id": "treatment",
            "name": "Treatment",
            "description": "Treatment.",
            "findings": [
              {
                "id": "treatment-finding",
                "name": "Treatment",
                "description": "Treatment.",
                "questions": [
                  {
                    "text": "What treatments are used, including anti-seizure medicines, ketogenic diet and surgery for structural causes, per guidelines?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Which references are standard?",
                    "kind": "provenance"
                  }
                ]
              }
            ]
          },
          {
            "id": "outlook",
            "name": "Outlook",
            "description": "Outlook and support.",
            "findings": [
              {
                "id": "outlook-finding",
                "name": "Outlook",
                "description": "Outlook.",
                "questions": [
                  {
                    "text": "What is the prognosis, and what support exists for families?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Which sources are cited?",
                    "kind": "provenance"
                  }
                ]
              }
            ]
          }
        ]
      },
      {
        "id": "context",
        "name": "Context",
        "description": "Classification and research.",
        "rationale": "Context.",
        "layers": [
          {
            "id": "classification",
            "name": "Classification",
            "description": "ILAE classification.",
            "findings": [
              {
                "id": "classification-finding",
                "name": "Classification",
                "description": "Classification.",
                "questions": [
                  {
                    "text": "How did the ILAE reclassify Ohtahara syndrome within early infantile developmental and epileptic encephalopathy?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Which entry fits developmental and epileptic encephalopathy?",
                    "kind": "action"
                  }
                ]
              }
            ]
          },
          {
            "id": "research",
            "name": "Research",
            "description": "Research.",
            "findings": [
              {
                "id": "research-finding",
                "name": "Research",
                "description": "Research.",
                "questions": [
                  {
                    "text": "What research addresses gene-targeted therapies, with findings attributed?",
                    "kind": "provenance"
                  },
                  {
                    "text": "Which entry fits epilepsy research?",
                    "kind": "action"
                  }
                ]
              }
            ]
          }
        ]
      }
    ]
  },
  "openQuestions": [
    "Should the genetic subtypes be separate entries?",
    "How should ILAE and rare disease sources be linked?",
    "The registry entry has merged aliases naming numbered genetic subtypes; should they be split off?"
  ],
  "statistics": {
    "bundles": 4,
    "layers": 8,
    "findings": 8,
    "questions": 16
  }
}