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Anti-N-methyl-D-aspartate receptor encephalitis associated with chronic myelogenous leukemia, causality or
Ying Yu1, Jun-Li Liu2, Dai-Shi Tian3
1Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Background:
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most frequent autoimmune paraneoplastic encephalitis, and is primarily associated with ovarian teratomas. Here, we report the first case of a patient diagnosed with chronic myelogenous leukemia (CML) during the recovery phase of anti-NMDAR encephalitis.
Case Presentation:
The patient was admitted with fever, headache, and seizures. Brain MRI revealed a cerebrospinal fluid (CSF)-containing arachnoid cyst in the left temporal lobe with no other abnormal signals. EEG showed diffuse background slowing in the delta-theta range. The patient tested positive for anti-NMDAR antibodies in both the serum and CSF. One year after the onset of encephalitis, the patient was referred to the Department of Hematology for extreme leukocytosis. Karyotype analysis showed the presence of Philadelphia chromosome t(9;22)(q34;q11). Quantitative reverse transcriptase PCR analysis further identified BCR/ABL1 fusion transcripts; thus, CML was diagnosed.
Conclusions:
To the best of our knowledge, this is the first case of anti-NMDAR encephalitis associated with CML. This report should alert clinicians to consider CML as a malignancy that is possibly associated with limbic encephalitis.
Insights
This study reports the first association between anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and chronic myelogenous leukemia (CML). This finding suggests CML may be a potential underlying malignancy in limbic encephalitis cases.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a common autoimmune neurological disorder, frequently linked to ovarian teratomas.
- This condition involves antibodies attacking NMDARs in the brain, leading to inflammation and neurological symptoms.
Observation:
- A patient presented with symptoms of anti-NMDAR encephalitis, including fever, headache, and seizures.
- Diagnostic tests confirmed anti-NMDAR antibodies in serum and cerebrospinal fluid (CSF).
- A year later, the patient developed extreme leukocytosis, leading to a diagnosis of chronic myelogenous leukemia (CML) with the Philadelphia chromosome and BCR/ABL1 fusion transcripts.
Findings:
- This case represents the first documented instance of anti-NMDAR encephalitis occurring in a patient with chronic myelogenous leukemia (CML).
- The diagnosis of CML was made during the recovery phase of the encephalitis.
Implications:
- This association suggests that CML should be considered as a potential paraneoplastic condition in patients diagnosed with limbic encephalitis.
- Clinicians should maintain a high index of suspicion for underlying malignancies, including CML, in patients with autoimmune encephalitis.
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