{
  "id": 16388,
  "label": "limbic encephalitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015588",
  "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."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 6,
  "parents": [
    {
      "id": 19673,
      "label": "encephalitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6880,
        7209,
        20092
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9588",
          "ICD10CM:A85",
          "ICD9:323.0",
          "ICD9:323.8",
          "ICD9:323.9",
          "MEDGEN:4027",
          "MESH:D004660",
          "MedDRA:10014581",
          "NCIT:C26760",
          "Orphanet:97275",
          "SCTID:45170000",
          "UMLS:C0014038"
        ],
        "synonyms": [
          "brain inflammation"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "An acute inflammatory process affecting the brain parenchyma. Causes include viral infections and less frequently bacterial infections, toxins, and immune-mediated processes."
      },
      "child_count": 21,
      "reference_id": "MONDO:0019956"
    }
  ],
  "children": [
    {
      "id": 16389,
      "label": "paraneoplastic limbic encephalitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16388,
        18348
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 2,
      "reference_id": "MONDO:0015589"
    },
    {
      "id": 16392,
      "label": "posttransplant acute limbic encephalitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16388,
        24615
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015595"
    },
    {
      "id": 16393,
      "label": "non-herpetic acute limbic encephalitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16388
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025067",
          "MEDGEN:1646654",
          "Orphanet:163924",
          "SCTID:764998005",
          "UMLS:C4707262"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Non-herpetic acute limbic encephalitis is a rare neuroinflammatory/neuroautoimmune disease characterized by an acute (or subacute) onset of disturbance of consciousness (occasionally presenting as convulsions) and high fever, associated with cerebral lesions (on magnetic resonance imaging) that are restricted to the limbic system (particularly the hippocampi and amygdalae), in the absence of viral, bacterial, fungal, paraneoplastic and other disorders."
      },
      "child_count": 0,
      "reference_id": "MONDO:0015596"
    },
    {
      "id": 17521,
      "label": "limbic encephalitis with caspr2 antibodies",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16388
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025088",
          "MEDGEN:1634906",
          "Orphanet:276402",
          "SCTID:763793004",
          "UMLS:C4706582"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Limbic encephalitis with caspr2 antibodies is a rare neuroimmunological disorder characterized by the onset of cognitive deficits, psychiatric disturbances (e.g. personality changes), seizures, peripheral nerve hyperexcitability, dysautonomia, neuropathic pain, insomnia and weight loss, in association with detection of caspr2 antibodies in serum or cerebrospinal fluid, with or without underlying malignancies. Other features reported include blepharoclonus, myoclonic status epilepticus, and dyskinesia."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017179"
    },
    {
      "id": 18178,
      "label": "limbic encephalitis with DPP6 antibodies",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16388
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025125",
          "MEDGEN:1664407",
          "Orphanet:329341",
          "UMLS:C4750833"
        ],
        "synonyms": [
          "limbic encephalitis with DPPX antibodies",
          "limbic encephalitis with dipeptidyl-peptidase 6 antibodies"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018003"
    },
    {
      "id": 25135,
      "label": "autoimmune limbic encephalitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16388,
        20055
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0022492",
          "MEDGEN:419645",
          "Orphanet:623615",
          "UMLS:C2930824",
          "icd11.foundation:1254443511"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A rare autoimmune encephalitis involving the mesial temporal lobes and clinically characterized by subacute onset (i. e. rapid progression of less than three months) of short-term memory deficits, seizures or psychiatric symptoms, such as behavioral changes, anxiety, depression, and psychosis. Further diagnostic criteria are bilateral abnormalities restricted to the mesial temporal lobes in brain MRI, cerebrospinal fluid pleocytosis and/or epileptic or slow-wave activity involving the temporal lobes in EEG, and reasonable exclusion of alternative causes. Paraneoplastic or non-paraneoplastic antibodies against neuronal antigens may be found in serum and/or cerebrospinal fluid."
      },
      "child_count": 0,
      "reference_id": "MONDO:0850097"
    }
  ],
  "roots": [
    {
      "id": 19673,
      "label": "encephalitis"
    }
  ]
}