Table 1 Clinical presentation of patients with anti-AMPAR encephalitis
Case Number | Age (years)/sex | Onset to Diagnosis (weeks) | Onset mode | Initial symptoms | Other symptoms presented during disease course | MRI (interval since disease onset) | EEG | CSF | AMPAR antibodies (sample type and titer) | Other antibodies | Tumor state |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 68/F | 57 | Subacute | Psychiatric disturbances | Confusion, amnesia, ataxia, dysarthria, urinary incontinence | Increased signal in right basal ganglia (10 days) | Diffuse low amplitude beta wave activity | Normal WBC, 55 mg/dL protein | CSF 1:32; blood 1:32 | Blood Hu (+) | Not Found |
2 | 52/M | 32 | Acute | Amnesia | Confusion, psychiatric disturbances | Increased signal in bilateral frontal subcortex (7 months) | NA | Normal WBC, 84 mg/dL protein | CSF (−); blood 1:10 | (−) | Lung cancer by contrast-enhanced CT |
3 | 51/F | 20 | Chronic | Amnesia | Sleep disorders, dizziness, right leg numbness and paresis | Increased signal in left medial frontoparietal lobe, right cingulate cortex, and bilateral cerebellar hemispheres (10 months) | NA | Normal WBC, 66 mg/dL protein | CSF 1:10; blood 1:32 | (−) | Not Found |
4 | 76/F | 3 | Acute | Psychiatric disturbances and amnesia | – | Normal (1 week) | Normal | WBC 15/μL, normal protein | CSF (−); blood 1:10 | (−) | Small cell lung carcinoma by pathology |
5 | 64/F | 11 | Chronic | Psychiatric disturbances and amnesia | Confusion | NA | NA | NA | CSF (−); blood 1:100 | NA | Thymoma by pathology |
6 | 50/F | NA | Subacute | Psychiatric disturbances and amnesia | Confusion, intermittent fever | Normal (2 weeks) | Normal | Normal WBC, normal protein | CSF (+); blood (+), both titers unknown | NA | Malignant thymoma (B3) by pathology |
7 | 59/F | 12 | Subacute | Amnesia | Confusion, altered level of consciousness, psychiatric disturbances, involuntary movement, dizziness, right face and perioral numbness | Increased signal in left frontal lobe, left parietal lobe and right temporal lobe (2 months) | Diffuse abnormal (low amplitude and decreased slow wave activities) | Normal WBC, 58 mg/dL protein | CSF 1:100; blood 1:100 | NA | Malignant thymoma (B3) by pathology |
8 | 63/M | 5 | Chronic | Amnesia, psychiatric disturbances and ataxia | Confusion, sleep disorders, bilateral deafness, dysphagia | Increased signal in medial temporal lobes (9 days) | Slightly increased theta activity | Normal WBC, 86 mg/dL protein | CSF 1:10; blood 1:100 | (−) | Not Found |
9 | 51/F | 3 | Acute | psychiatric disturbances | Confusion, fever, apathy, dysarthria, dysphagia, arrythmia, difficulty in defecation and urination, central hypoventilation | Diffuse increased signal in bilateral cortex and subcortex (1 week) | NA | Normal WBC, normal protein | CSF (+); blood (+), both titers unknown | Blood Hu (+) | Possible thymoma by CT |