{
  "id": 19307,
  "label": "idiopathic hemiconvulsion-hemiplegia syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019485",
  "properties": {
    "xrefs": [
      "GARD:0019085",
      "MEDGEN:639806",
      "NANDO:1200596",
      "Orphanet:86908",
      "SCTID:230407006",
      "UMLS:C0549118"
    ],
    "synonyms": [
      "HHE syndrome",
      "IHHS",
      "hemiconvulsion-hemiplegia-epilepsy syndrome"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "A rare acute encephalopathy with inflammation-mediated status epilepticus characterized by infancy-onset of refractory unilateral, mainly clonic status epilepticus during or shortly after a febrile episode without evidence of central nervous system infection, followed by permanent or transient hemiplegia with a minimum duration of one week. The majority of children develop pharmaco-resistant epilepsy a few months later. Brain imaging shows edematous swelling of the affected hemisphere at the time of the initial status, followed by hemiatrophy that does not correlate with any vascular territory."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 19724,
      "label": "infantile epilepsy syndrome",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16436
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019436",
          "Orphanet:98258"
        ],
        "synonyms": [
          "epilepsy syndrome of infancy",
          "infantile epilepsy syndrome",
          "infantile onset epilepsy syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "An epilepsy syndrome that occurs between 28 days to one year of life."
      },
      "child_count": 9,
      "reference_id": "MONDO:0020071"
    },
    {
      "id": 24405,
      "label": "idiopathic disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        29381
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:548250",
          "UMLS:C0277553"
        ],
        "synonyms": [
          "idiopathic disorder"
        ],
        "definition": "A disease or disorder for which the cause is of uncertain or unknown."
      },
      "child_count": 79,
      "reference_id": "MONDO:0700007"
    },
    {
      "id": 25084,
      "label": "childhood-onset epilepsy syndrome with developmental and/or epileptic encephalopathy",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19725
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0027303"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A childhood-onset epilepsy syndrome where the onset of the condition includes manifestations of cognitive, neurological, or psychiatric impairment, stagnation, or regression, due directly to the underlying etiology. In contrast, an epileptic encephalopathy (EE) is present when the encephalopathy is caused by the epileptic activity. The term developmental and epileptic encephalopathy (DEE) is used when both factors contribute to the patient’s condition."
      },
      "child_count": 5,
      "reference_id": "MONDO:0800500"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 19724,
      "label": "infantile epilepsy syndrome"
    },
    {
      "id": 24405,
      "label": "idiopathic disease"
    },
    {
      "id": 25084,
      "label": "childhood-onset epilepsy syndrome with developmental and/or epileptic encephalopathy"
    }
  ]
}