{
  "id": 16922,
  "label": "sporadic infantile bilateral striatal necrosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0016394",
  "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."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 16334,
      "label": "infantile bilateral striatal necrosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7208
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 2,
      "reference_id": "MONDO:0015518"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 16334,
      "label": "infantile bilateral striatal necrosis"
    }
  ]
}