Table 1. Summary of clinical and pathological findings of Japanese patients with NMO and MS.
| Autopsy | Age (yrs) | Sex |
Disease
duration (yrs) |
|
Clinically estimated
sites of lesions |
Pathologically determined
sites of lesions |
Cause of death | Neurological symptom possibly associated with death |
|---|---|---|---|---|---|---|---|---|
| NMO-1 | 44 | F | 3.8 | 1.6 | O2, S6 | O, S, Bs, Cr, Cl | Pneumonia | Quadriplegia |
| NMO-2 | 44 | F | 1.8 | 2.8 | O2, Bs3, S2 | O, S, Bs, Cr | Pneumonia | Bulbar palsy |
| NMO-3 | 48 | F | 0.5 | 4.0 | O2, Bs1, S1 | O, S, Bs, Cr | Aspiration pneumonia | Quadriplegia |
| NMO-4 | 32 | M | 6.3 | 1.1 | O1, Bs2, S7 | O, S, Bs, Cl, Cr | Bronchial pneumonia | Quadriplegia, Bulbar palsy |
| NMO-5 | 28 | F | 4.7 | 0.6 | O3, Bs2, S2 | O, S, Bs, Cl, Cr | Bronchial pneumonia | Paraplegia, Bulbar palsy |
| NMO-6 | 35 | F | 7.0 | 1.4 | O4, Bs4, S4 | O, S, Bs, Cr | Respiratory failure | Paraplegia, Bulbar palsy |
| NMO-7 | 37 | F | 10.8 | 1.5 | O9, Bs2, S14 | O, S, Bs, Cl, Cr | Respiratory failure | Quadriplegia, Bulbar palsy |
| NMO-8 | 47 | F | 8.3 | 0.1 | O2, Bs1, S2 | O, S, Bs, Cr | Bronchial pneumonia | Bulbar palsy |
| NMO-9 | 54 | F | 4.0 | 1.3 | O1, S6 | O, S | Respiratory failure | Bulbar palsy |
| NMO-10* | 37 | F | 17.0 | 1.1 | O8, Bs1, S9, Cr3 | Bs, S, Cr |
|
Quadriplegia |
| NMOSD | 88 | M | 0.4 | 0.0 | S1 | O, S | Pneumonia | Paraplegia |
| MS-1 | 12 | F | 5.0 | 2.2 | O6, Bs1, S2, Cr3 | O, Bs, S, Cr, Cl | Respiratory failure | Quadriplegia, Bulbar palsy |
| MS-2 | 35 | M | 3.1 | 1.0 | O1, Bs2, S2, Cr2 | O, Bs, S, Cr | Aspiration pneumonia | Quadriplegia |
| MS-3 | 52 | F | 1.3 | 1.5 | Bs1, Cr3 | Bs, Cr | Bronchial pneumonia | Quadriplegia, Bulbar palsy |
| MS-4 | 45 | F | 0.7 | 1.4 | Bs2, S2 | O, S, Bs | Pneumonia | Paraplegia |
| MS-5 | 39 | M | 21.0 | 0.5 | O1, Bsx, Clx, Crx | O, Bs, Cl, Cr | Unknown | Bulbar palsy |
| MS-6 | 29 | F | 0.3 | 4.0 | Bs1, Cr3 | S, Bs, Cr | Respiratory failure | Quadriplegia |
F = female, M = male, MS = multiple sclerosis, NMO = neuromyelitis optica [27,29], NMOSD = neuromyelitis optica spectrum disorder [28].
Lesion sites in clinical exacerbations: O = optic nerve, S = spinal cord, Bs = brainstem, Cl = cerebellum, Cr = cerebrum. Numbers indicate exacerbations in each lesion site (for example, O2 represents two episodes of optic neuritis). x indicates unspecified numbers. Asterisk indicates an NMO case with anti-AQP4 antibody seropositivity.