Table 1.
Overview of clinical findings of individual patients
| Summary | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | |
|---|---|---|---|---|---|---|---|---|
| Sex (m/f) | 3/4 | Male | Male | Female | Female | Female | Female | Male |
| Age, years (median + range) | 31.7 (21–41) | 24 | 37 | 21 | 41 | 26 | 34 | 39 |
| Infections/ vaccination prior to disease | 5/7 | Skin infection left foot | GI infection, skin efflorescences | Viral infection of respriratory tract | Vaccination | Unclear | Pyelonephritis | Unclear |
| Cranial MRI | No abnormal findings | No abnormal findings | No abnormal findings | No abnormal findings | No abnormal findings | No abnormal findings | No abnormal findings | No abnormal findings |
| Spinal Cord MRI | LETM | LETM: whole spine | LETM: thoracic spine | LETM: Th3-10 | LETM: C2-6 | LETM: Medulla oblongata-C9 | LETM: C2-6 | LETM: Th2-11 |
| Gadolinium-uptake in sMRI | Negative | Negative | Negative | Negative | Negative | Negative | Negative | Negative |
| Optic neuritis | Negative | Negative | Negative | Negative | Negative | Negative | Negative | Negative |
| CSF cells (mean + range) | 196 (49–353)/µl | 136/µl | 325/µl | 264/µl | 179/µl | 49/µl | 353/µl | 67/µl |
| Neutrophil granulocytes in CSF | 2/7 positive | No | No | Yes | No | No | Yes | No |
| CSF protein (mean + range) | 464,7 (64 – 1100) mg/dl | 141 mg/dl | 64 mg/dl | 77 mg/dl | 784 mg/dl | 150 mg/dl | 1100 mg/dl | 937 mg/dl |
| OCB | Negative | Negative | Negative | Negative | Negative | Negative | Negative | Negative |
| CSF Lactate (mmol/l) | 2.5 (mean) | 2.5 | 3.1 | 2.9 | 1.8 | 2.1 | 3.4 | 1.7 |
| QAlb | 13.61 (mean) | 17.65 | 9.25 | 14.52 | 11.36 | 18.49 | 10.58 | 13.39 |
| CSF IgA (mg/l) | 11.57 (mean) | 25.3 | 7.68 | 8.73 | 14.74 | 4.93 | 12.11 | 7.51 |
| CSF IgG (mg/l) | 104 (mean) | 159 | 56 | 72 | 172 | 93 | 72 | 104 |
| CSF IgM (mg/l) | 6.27 (mean) | 18.8 | 1.2 | 15.3 | 1.8 | 2.4 | 1.1 | 3.3 |
| MRZ reaction | Negative | positive for VZV (1.95) | Not tested | Not tested | Positive for VZV (1.8) | Neg | Neg | Positive for measles (2.1) |
| MOG-Ab in serum (range) | 1:32–1:3200 | 1:320 | 1:32 | 1:100 | 1:32 | 1:100 | 1:3200 | 1:100 |
| MOG-AB status in remission | 5/7 negative | Negative | Negative | Not tested | Positive | Negative | Negative | Negative |
| AQP4 antibodies | Negative | Negative | Negative | Negative | Negative | Negative | Negative | Negative |
| Second relapse | 1/7 | No | No | No | Yes | No | No | No |
| Acute therapy | Recovery on IVMPS, IVIG or plasma exchange | Recovery on IVIG | Recovery on plasma exchange | Recovery on plasma exchange | Recovery on plasma exchange | Recovery on plasma exchange | Recovery on plasma exchange | Partial recovery on IVMPS |
| Immunotherapy | Induction with rituximab (six patients) or cyclophosphamide(1 patient) | Rituximab | Rituximab | Rituximab | Rituximab | Rituximab | Induction rituximabdeescalation to azathioprine | Induction of cyclophosphamide deescalation to azathioprine |
| Outcome | Marked recovery in four patients, partial recovery in three patients | Marked recovery | Marked recovery | Marked recovery | Partial recovery | Marked recovery | Partial recovery | Partial recovery |
CSF cerebrospinal fluid, MRI magnetic resonance imaging, IVIG intravenous immunoglobulins, IVMPS intravenous methylprednisolone, LETM longitudinal extensive transverse myelitis, MOG myelin oligodendrocyte glycoprotein, OCB oligoclonal bands, qAlb albumin quotient, AQP4 aquaporin 4, VZV varicella zoster virus