{
  "id": 19547,
  "label": "primary progressive aphasia",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019806",
  "properties": {
    "xrefs": [
      "DOID:0081388",
      "EFO:0009053",
      "GARD:0008541",
      "MEDGEN:79466",
      "MESH:D018888",
      "NCIT:C85024",
      "Orphanet:95432",
      "UMLS:C0282513"
    ],
    "synonyms": [
      "Mesulam syndrome",
      "PPA",
      "primary progressive aphasia syndrome"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 7208,
      "label": "neurodegenerative disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4657
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:1289",
          "EFO:0005772",
          "ICD9:349.89",
          "MEDGEN:17999",
          "MESH:D019636",
          "NCIT:C4802",
          "SCTID:80690008",
          "UMLS:C0027746"
        ],
        "synonyms": [
          "degenerative disease",
          "brain degeneration",
          "central nervous system degenerative disorder",
          "central nervous system neurodegenerative disorder",
          "degenerative disorder of central nervous system",
          "cerebral degeneration disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A disorder of the central nervous system characterized by gradual and progressive loss of neural tissue and neurologic function."
      },
      "child_count": 22,
      "reference_id": "MONDO:0005559"
    }
  ],
  "children": [
    {
      "id": 12923,
      "label": "GRN-related frontotemporal lobar degeneration with Tdp43 inclusions",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17600,
        19547
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060672",
          "GARD:0010004",
          "MEDGEN:375285",
          "OMIM:607485",
          "UMLS:C1843792"
        ],
        "synonyms": [
          "FTLD-TDP, GRN-related",
          "aphasia, primary progressive",
          "dementia, hereditary dysphasic disinhibition",
          "frontotemporal dementia with TDP43 inclusions, GRN-related",
          "frontotemporal dementia, ubiquitin-positive",
          "frontotemporal lobar degeneration with TDP43 inclusions, GRN-related",
          "frontotemporal lobar degeneration with ubiquitin-positive inclusions"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A frontotemporal dementia characterized by variable phenotypic expression typically including social, behavioral, or language deterioration, rather than memory or motor deficits and the presence of TARDBP-positive inclusions that has material basis in mutation in the GRN gene on chromosome 17q21.31."
      },
      "child_count": 2,
      "reference_id": "MONDO:0011842"
    },
    {
      "id": 17115,
      "label": "logopenic progressive aphasia",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19547
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081389",
          "GARD:0010791",
          "MEDGEN:907332",
          "Orphanet:250831",
          "SCTID:716380002",
          "UMLS:C4274665"
        ],
        "synonyms": [
          "LPA",
          "Logopenic primary progressive aphasia",
          "Logopenic variant PPA"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Logopenic progressive aphasia (lv-PPA) is a form of primary progressive aphasia (PPA), characterized by impaired single-word retrieval and naming and impaired repetition with spared single-word comprehension and object knowledge."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016644"
    }
  ],
  "roots": [
    {
      "id": 7208,
      "label": "neurodegenerative disease"
    }
  ]
}