{
  "id": 16334,
  "label": "infantile bilateral striatal necrosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015518",
  "properties": {
    "xrefs": [
      "GARD:0005040",
      "MEDGEN:167090",
      "NANDO:2100242",
      "NANDO:2200888",
      "Orphanet:1576",
      "SCTID:718174008",
      "UMLS:C0795996",
      "icd11.foundation:1947032348"
    ],
    "synonyms": [
      "IBSN",
      "infantile bilateral striatal necrosis",
      "infantile striatonigral degeneration",
      "infantile striatonigral necrosis",
      "SNDI",
      "striatal degeneration familial",
      "striatonigral degeneration infantile"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Several syndromes of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. IBSN can be familial or sporadic."
  },
  "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": 11268,
      "label": "familial infantile bilateral striatal necrosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4370,
        5100,
        16334
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0017141",
          "MEDGEN:1672478",
          "OMIM:271930",
          "Orphanet:225154",
          "UMLS:C4087174",
          "icd11.foundation:1873983370"
        ],
        "synonyms": [
          "familial IBSN",
          "familial infantile striatonigral degeneration",
          "familial infantile striatonigral necrosis",
          "hereditary infantile bilateral striatal necrosis",
          "FBSN",
          "SNDI",
          "bilateral striatal Necrosis, infantile",
          "familial bilateral striatal necrosis",
          "infantile bilateral striatal necrosis",
          "striatal degeneration, familial",
          "striatonigral degeneration, infantile"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "The familial form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis."
      },
      "child_count": 3,
      "reference_id": "MONDO:0010080"
    },
    {
      "id": 16922,
      "label": "sporadic infantile bilateral striatal necrosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16334
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020549",
          "MEDGEN:1672494",
          "Orphanet:225147",
          "UMLS:C4087175",
          "icd11.foundation:1174703901"
        ],
        "synonyms": [
          "ABSN",
          "acute bilateral striatal necrosis",
          "sporadic IBSN",
          "sporadic infantile striatonigral degeneration",
          "sporadic infantile striatonigral necrosis"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Sporadic infantile bilateral necrosis is the sporadic form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016394"
    }
  ],
  "roots": [
    {
      "id": 7208,
      "label": "neurodegenerative disease"
    }
  ]
}