{
  "id": 16385,
  "label": "febrile infection-related epilepsy syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015584",
  "properties": {
    "xrefs": [
      "GARD:0011005",
      "MEDGEN:1381987",
      "NANDO:1200600",
      "NANDO:2100249",
      "NANDO:2200903",
      "NORD:1889",
      "Orphanet:163703",
      "SCTID:725413002",
      "UMLS:C4049262",
      "icd11.foundation:1316435973"
    ],
    "synonyms": [
      "AERRPS",
      "DESC syndrome",
      "FIRES",
      "Febrile Infection-Related Epilepsy Syndrome (FIRES)",
      "acute encephalitis with refractory repetitive partial seizures",
      "acute non-herpetic encephalitis with severe refractory status epilepticus",
      "devastating epileptic encephalopathy in school-aged children",
      "fever-induced refractory epileptic encephalopathy in school-aged children",
      "idiopathic catastrophic epileptic encephalopathy",
      "severe refractory status epilepticus owing to presumed encephalitis",
      "status epilepticus owing to presumed encephalitis"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "A rare, potentially fatal, epileptic encephalopathy characterized by explosive-onset of recurrent multifocal and bilateral tonic-clonic seizures following an unspecific febrile illness. The syndrome develops without a clear acute structural, toxic or metabolic cause, in a patient without previous epilepsy. FIRES is a subgroup of new-onset refractory status epilepticus (NORSE), and requires a preceding febrile infection as a mandatory feature."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "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": 25084,
      "label": "childhood-onset epilepsy syndrome with developmental and/or epileptic encephalopathy"
    }
  ]
}