{
  "id": 16389,
  "label": "paraneoplastic limbic encephalitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015589",
  "properties": {
    "xrefs": [
      "GARD:0025065",
      "ICD9:323.81",
      "NCIT:C4350",
      "Orphanet:163895",
      "SCTID:445014002",
      "icd11.foundation:1147819644"
    ],
    "synonyms": [
      "limbic encephalitis"
    ],
    "categories": [
      {
        "ref": "MONDO:0002254",
        "name": "syndromic disease"
      },
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "A rare disorder characterized by degenerative changes in the limbic area of the brain. Causes include infections and autoimmune conditions; it may also manifest as a paraneoplastic syndrome, most often caused by small cell lung carcinoma. Signs and symptoms include behavioral changes, hallucinations and dementia."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 1,
  "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": 18348,
      "label": "paraneoplastic neurologic syndrome",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6799,
        20314
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007326",
          "ICD9:331.89",
          "MEDGEN:155656",
          "MedDRA:10072106",
          "Orphanet:36388",
          "SCTID:192877007",
          "UMLS:C0751911"
        ],
        "synonyms": [
          "PCD",
          "PNS",
          "nervous system paraneoplastic syndrome",
          "paraneoplastic syndrome of nervous system",
          "paraneoplastic cerebellar degeneration"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A paraneoplastic syndrome that involves the nervous system."
      },
      "child_count": 14,
      "reference_id": "MONDO:0018215"
    }
  ],
  "children": [
    {
      "id": 16390,
      "label": "classic paraneoplastic limbic encephalitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16389
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025066",
          "Orphanet:163898"
        ],
        "synonyms": [
          "classic paraneoplastic limbic encephalitis, with or without intracellular antigens"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Classic paraneoplastic limbic encephalitis is a rare neuroimmunological disorder characterized by the sudden onset of seizures, progressive memory impairment (which may develop into dementia) and psychiatric manifestations (e.g. depression, personality changes, loss of social inhibition) associated with cancer (most commonly small-cell carcinoma of the lung) in the absence of tumor cell invasion of the nervous system. Other reported features include ataxia, dystonia, paresthesia, tremors, paranoid ideation, and hallucinations. The presence of antibodies that act on neuronal antigens (such as anti-Hu, anti-Ma2, anti-amphiphysin) are typically observed."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015590"
    }
  ],
  "roots": [
    {
      "id": 16388,
      "label": "limbic encephalitis"
    },
    {
      "id": 18348,
      "label": "paraneoplastic neurologic syndrome"
    }
  ]
}