{
  "id": 16392,
  "label": "posttransplant acute limbic encephalitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015595",
  "properties": {
    "xrefs": [
      "GARD:0020051",
      "MEDGEN:1657779",
      "Orphanet:163921",
      "UMLS:C4750744"
    ],
    "synonyms": [
      "PALE",
      "pale"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Posttransplant acute limbic encephalitis is a rare, acquired, non-paraneoplastic limbic encephalitis disorder, that develops in the setting of treatment-related immunosuppression, typically after allogeneic hemapoietic stem cell transplantation, characterized by onset of confusion, headache, anterograde amnesia, seizures and/or loss of consciousness 2-6 weeks following transplantation. Bilateral, non-enhancing T2 hyperintensities in limbic structures are observed on magnetic resonance imaging. Mild cerebrospinal fluid pleocytosis and syndrome of inappropriate antidiuretic hormone secretion may also be associated."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 16388,
      "label": "limbic encephalitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19673
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080741",
          "GARD:0008742",
          "ICD9:323.9",
          "MEDGEN:137895",
          "MESH:D020363",
          "Orphanet:163892",
          "SCTID:230192003",
          "UMLS:C0338430"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A group of autoimmune conditions characterized by inflammation of the limbic system and other parts of the brain.The cardinal sign of limbic encephalitis is a severe impairment of short-term memory; however,symptoms may also include confusion, psychiatric symptoms, and seizures.The symptomstypically develop over a few weeks or months, but they may evolve over a few days. Limbic encephalitis is often associated with an underlying neoplasm (paraneoplastic limbic encephalitis); however some cases never have a neoplasm identified (non-paraneoplastic limbic encephalitis). Delayed diagnosis is common, but improvements are being made to assist in early detection. Various tests including imaging studies (MRI, PET) laboratory tests (CSF analysis), and tests that measure the electrical activity of the brain (EEG) may be utilized to confirm a diagnosis. Treatment includes removal of the neoplasm (if identified) and immunotherapy."
      },
      "child_count": 6,
      "reference_id": "MONDO:0015588"
    },
    {
      "id": 24615,
      "label": "disease related to transplantation",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        29383
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:745174",
          "Orphanet:306644",
          "UMLS:C1998172"
        ],
        "definition": "Disease that occurs as a consequence of immunosuppression in a recipient of a solid organ or bone marrow transplant, or as a consequence of the transplantation."
      },
      "child_count": 5,
      "reference_id": "MONDO:0700220"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 16388,
      "label": "limbic encephalitis"
    },
    {
      "id": 24615,
      "label": "disease related to transplantation"
    }
  ]
}