Table 1.
Patients with MG as inaugural disease followed by ALS.
| Patients | Nationality | MG classification | Localization of MG symptoms | Abnormal fatigability | Neostigmine test or response to therapy | AChR-ab (nmol/l) | RNS CMAP decrement (%) | CT scan of mediastinum | Associated immune-mediated disease |
|---|---|---|---|---|---|---|---|---|---|
| 1 (20) | Indian | Ocular MG | Ocular | + | + | 8.04 (N < 0.04) | 19–33 | Normal | No |
| 2 (21) | Japanese | Generalized MG | Ocular, bulbar, UL, LL | + | + | 3.8 | >10 | Thymectomy | No |
| 3 (22) | French | Generalized MG | Ocular, head drop | + | + | 40 (N < 0.5) | >10 | Normal | No |
| 4 (22) | French | Generalized MG | Bulbar | NA | + | 8.4 (N < 0.5) | >10 | Thymectomy | No |
| 5 (22) | French | Generalized MG | Bulbar | + | + | >8 (N < 0.5) | NA | NA | Basedow’s disease |
| 6—case 2 | Chinese | Ocular MG | Ocular | + | + | 39.06 (N < 0.4) | 5 | Normal | No |
| 7 (4) | Italian | Generalized MG | Ocular, bulbar, LL | + | + | Positive | 27 | Normal | No |
| 8 (4) | Italian | Ocular MG | Ocular | NA | + | Positive | 21 | Thymoma | No |
| 9 (4) | Italian | Ocular MG | Ocular | + | + | Negative | 12 | Normal | No |
| 10 (4) | Italian | Ocular MG | Ocular | + | + | Negative | ND | Normal | Hyperthyroidism |
| 11 (4) | Italian | Generalized MG | Ocular, bulbar, UL, LL | + | + | Negative | 20 | Normal | No |
| 12 (25) | Israel | Generalized MG | Ocular, bulbar, limbs | + | + | Positive | NA | Normal | Thyroiditis, ANA(+) |
| No. patients | Interval between two conditions | ALS classification | Site of onset of ALS | Regions of EMG neurogenic pattern | MG relapsed while ALS onset | Immune-modulating therapy after ALS onset | Effects on myasthenia/ALS symptoms | Riluzole | Prognosis (worsened or ALS survival) |
| 1 (20) | 38 years | Definite | UL + LL | UL/LL/bulbar/paraspinal | Yes | IVIG | Transient effect | No | Worsened |
| 2 (21) | 41 years | Probable | Bulbar | UL/LL/bulbar | No | Prednisolone, tacrolimus | −/no effect | Yes | Died/3 years |
| 3 (22) | 11 months | Probable | UL proximal | UL/LL/bulbar | No | IVIG, plasmapheresis | −/no effect | No | Died/26 months |
| 4 (22) | 319 months | Probable-lab supported | UL proximal | UL/LL | No | IVIG, corticosteroids | −/partial improvement during the first 6 months | Yes | Died/2 years |
| 5 (22) | 8 months | Probable-lab supported | LL distal | UL/LL/bulbar | No | IVIG, corticosteroids | −/no effect | Yes | Died/6 years |
| 6—case 2 | 5 years | Probable | LL proximal | UL/LL/bulbar/paraspinal | Yes | IVIG | Complete resolution/no effect | No | Died/28 months |
| 7 (4) | 3 months | Probable | LL | NA | No | NA | NA | NA | Died/6 months |
| 8 (4) | 2 years | Probable | UL + LL distal | NA | No | NA | NA | NA | Died/12 months |
| 9 (4) | 1 years | Definite | Bulbar | NA | No | NA | NA | NA | Respiratory failure after 12 months |
| 10 (4) | 22 years | Probable | Bulbar | NA | No | NA | NA | NA | Respiratory failure after 12 months |
| 11 (4) | 6 months | Definite | Bulbar | NA | No | NA | NA | NA | Alive after 12 months |
| 12 (25) | 2 years | Definite ALS–FTD | Limbs | UL/LL | No | Prednisolone, azathioprine, MSC | Both improved | No | Transient improved |
M, male; F, female; UL, upper limb; LL, lower limb; NA, not applied; CMAP, compound muscle action potential; CT, computed tomography; EMG, electromyogram; IVIG, intravenous immunoglobulin; ALS–FTD, amyotrophic lateral sclerosis–frontotemporal dementia; MSC, mesenchymal stem cells; ND, not done; MG, myasthenia gravis; AChR-ab, acetylcholine receptor antibodies; RNS, repetitive nerve stimulation.