{
  "id": 16033,
  "label": "progressive non-fluent aphasia",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015059",
  "properties": {
    "xrefs": [
      "DOID:0081390",
      "GARD:0010793",
      "MEDGEN:148373",
      "MESH:D057178",
      "MedDRA:10029542",
      "NCIT:C85025",
      "Orphanet:100070",
      "SCTID:716281000",
      "UMLS:C0751706"
    ],
    "synonyms": [
      "Agramatic variant of PPA",
      "Agramatic variant of primary progressive aphasia",
      "Primary Progressive Nonfluent aphasia",
      "non-fluent variant PPA",
      "non-fluent primary progressive aphasia"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Progressive non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD), characterized by agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech (AOS). Language comprehension is relatively preserved."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 1,
  "parents": [
    {
      "id": 7073,
      "label": "movement disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6799
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:480",
          "EFO:0004280",
          "ICD9:333.90",
          "ICD9:333.99",
          "MEDGEN:10113",
          "MESH:D009069",
          "NCIT:C116757",
          "SCTID:60342002",
          "UMLS:C0026650"
        ],
        "synonyms": [
          "movement disease",
          "movement disorder",
          "movement disorders"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Neurological conditions resulting in abnormal voluntary or involuntary movement, which may impact the speed, fluency, quality and ease of movement."
      },
      "child_count": 54,
      "reference_id": "MONDO:0005395"
    },
    {
      "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"
    }
  ],
  "children": [
    {
      "id": 11992,
      "label": "semantic dementia",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16033,
        17505
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0051060",
          "DOID:0081391",
          "GARD:0010792",
          "MEDGEN:83268",
          "NANDO:1200550",
          "OMIM:600274",
          "Orphanet:100069",
          "UMLS:C0338462"
        ],
        "synonyms": [
          "dementia, frontotemporal",
          "dementia, frontotemporal, with or without parkinsonism",
          "semantic primary progressive aphasia",
          "semantic variant PPA",
          "FTD",
          "Ftdp17",
          "Ftld with Tau inclusions",
          "Pallidopontonigral Degeneration",
          "Pick Complex",
          "Wilhelmsen-Lynch disease",
          "dementia, frontotemporal, with Parkinsonism",
          "disinhibition-dementia-Parkinsonism-amyotrophy Complex",
          "frontotemporal dementia",
          "frontotemporal dementia with Parkinsonism",
          "frontotemporal lobar Degeneration with Tau inclusions",
          "frontotemporal lobe dementia",
          "multiple system tauopathy with presenile dementia"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Semantic dementia (SD) is a form of frontotemporal dementia (FTD), characterized by the progressive, amodal and profound loss of semantic knowledge (combination of visual associative agnosia, anomia, surface dyslexia or dysgraphia and disrupted comprehension of word meaning) and behavioral abnormalities, attributable to the degeneration of the anterior temporal lobes."
      },
      "child_count": 2,
      "reference_id": "MONDO:0010857"
    }
  ],
  "roots": [
    {
      "id": 7073,
      "label": "movement disorder"
    },
    {
      "id": 12923,
      "label": "GRN-related frontotemporal lobar degeneration with Tdp43 inclusions"
    }
  ]
}