Table 1.
Clinical features of the patients with anti-CRMP2 antibodies.
| Parameters | Patient 1 | Patient 2 |
|---|---|---|
| Gender | Female | Female |
| Age (year) | 38 | 42 |
| Symptoms | Dizziness, fever, slurred speech, visual rotation, unstable walking, opsoclonus–myoclonus | Headache, nausea, and vomiting, left chest pain, right arm fatigue, numbness in both legs |
| CSF | WBC: 38 cells/μl (90% monocytes), glucose: 3.69 mmol/l a , protein: 0.43 g/l | WBC: 240 cells/μl (90% monocytes), glucose: 2.64 mmol/l, protein: 0.80 g/l |
| Autoimmune Abs | Negative: | Negative: |
| AE Abs b (CSF and serum), anti-CRMP5 Ab (serum), anti-Homer3 Ab (serum), thyroid-related Abs c (serum), remaining serum autoimmune Abs d | AE Abs (CSF and serum), anti-AQP4 Ab (CSF and serum), anti-MOG Ab (CSF and serum), anti-GFAP Ab (CSF), thyroid related Abs (serum), remaining serum autoimmune Abs e | |
| Positive: N/A | Positive: | |
| Anti-histone Ab (serum) | ||
| TBA | Serum (+), CSF (+) | Serum (+), CSF (-) |
| Infection tests | Serum anti-M.P. IgM Ab (+), CSF NGS: M.P. (+) | T-spot (-), TB DNA (-), X-Pert (-), AFB (-) |
| MRI | Not remarkable, mild white matter abnormalities | Multiple abnormal signals in white matter in bilateral cerebral hemisphere and brainstem, long-segment spinal cord lesions from medulla to C6 segment |
| Tumor | Tumor antigens/biomarkers f (-) | N/A |
| Diagnosis | Encephalitis | Encephalomyelitis |
| Immunotherapy | IVIG, MP | MP |
| Prognosis (3 months mRS) | 2 | 0 |
Abs, antibodies; AE, autoimmune encephalitis; AFB, acid-fast bacillus test; AQP4, aquaporin 4; CSF, cerebrospinal fluid; CRMP5, collapsin response mediator protein 5; GFAP, glial fibrillary acidic protein; Homer 3, homer scaffold protein 3; IVIG, intravenous immunoglobin; MOG, myelin oligodendrocyte glycoprotein; M.P., Mycoplasma pneumoniae; MP, methylprednisolone; MRI, magnetic resonance imaging; mRS, modified Rankin Scale; N/A, not applicable; NGS, next-generation sequencing; TB DNA, tubercle bacillus DNA; TBA, tissue-based assay (rat brain sections); WBC, white blood cell.
Normal range: 2.8–4.0 mmol/l.
Autoimmune encephalitis antibodies: anti-N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein 2 (Caspr2), gamma-aminobutyric acid receptors B (GABABR), alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptors (AMPAR), dipeptidyl-peptidase-like protein-6 (DPPX), delta/notch-like epidermal growth factor-related receptor (DNER), dopamine-2 receptor (D2R), metabotropic glutamate receptor 5 (mGluR5), glutamate decarboxylase 65 kDa isoform (GAD65), IgLON family member 5 (IgLON5), and glycine receptor alpha 1 (GlyRα1) antibodies.
Thyroid-related antibodies: anti-thyrotropin receptor, thyroid peroxidase, and thyroglobulin antibodies.
Autoantibodies tested for patient 1 serum: anti-Hu (anti-neuronal nuclear antibody type 1, ANNA1), Ri (ANNA2), PNMA family member 2 (Ma2), ANNA3, SRY-box transcription factor 1 (SOX1), double-strand DNA (dsDNA), Smith (Sm), U1 small nuclear RNP (U1-RNP), Zic family member 4 (Zic4), Yo (Purkinje cell cytoplasmic antibody type 1, PCA1), amphiphysin, CRMP5, PCA2, recoverin, Titin, and Tr (DNER) antibodies.
Autoantibodies tested for patient 2 serum: anti-dsDNA, Jo-1 (histidyl-tRNA synthetase, HARS), Sjögren’s syndrome-related antigen A (SSA/Ro52), SSB, Sm, U1-RNP, proliferating cell nuclear antigen, M2 type of antimitochondrial antibodies, centromere protein B, polymyositis scleroderma, Scl-70, and nucleosome AnuA antibodies.
Tumor antigens/biomarkers: carbohydrate antigen (CA)-125, CA-199, CA-242, CA-724, cytokeratin-19 fragment (CYFRA21-1), squamous cell carcinoma antigen, carcinoembryonic antigen, and neuron-specific enolase.