Table 2.
Treatment and outcome in patients with SLONM (novel patients P1-10 and literature data)
| Ref. | Treatment | Outcome |
|---|---|---|
| P 1 a | steroids + NIV | R: improvement of limb and respiratory strength |
| P 2 | NA | NA |
| P 3 | analgetics + physiotherapy | NA |
| P 4 a | steroids + plasmapheresis | NR, cardiac decompensation |
| cyclophosphamide + rituximab | R: stabilisation of weakness | |
| P 5 | none | NA |
| P 6 | NIV | NA |
| P 7 | IVIg + steroids | R: increased muscle strength, CK remained increased |
| P 8 a | auto-PBSCT + melphalan + dexamethasone + lenalidomide | R: increased muscle strength |
| P 9 a | auto-PBSCT + steroids | NA |
| P 10 | none | NA |
| [24] (P 11) | none | NA |
| [9] (P 12) | none | NA |
| [54] a | auto-PBSCT + melphalan | R: paraprotein in serum + rods disappeared, increased muscle strength |
| [12] a | steroids | NR: died within 1y |
| [12] a | steroids | NR: died within 1y |
| [12] | steroids | NR |
| [12] | steroids | NR: stable |
| [12] a | steroids | NR: died within 1y |
| [12] | steroids | NR: stable 14y after onset |
| [12] | steroids + IVIg + methothrexate | NR: stable 6y after onset |
| [12] a | steroids + cyclophosphamide + IVIg | NR: stable 4,5y after onset |
| [12] | none | NA |
| [12] | none | NA |
| [12] | none | NA |
| [12] | none | NA |
| [12] | none | NA |
| [12] | none | NA |
| [11] a | plasmapheresis | NR |
| steroids + azathioprine | R: increased muscle strength, myalgia disappeared, serum paraprotein level decreased; weakness relapsed after discontinuation; stable under long-term medication | |
| [49] | steroids + azathioprine + mycophenolate mofetil | NR: stable for 2y |
| rituximab + cyclophosphamide | R: increased muscle strength, followed by stabilization | |
| [51] a | auto-PBSCT + melphalan | R for 4 m, followed by further loss of muscle strength |
| bortezomib + dexamethasone | R: muscle strength improved | |
| auto-PBSCT + melphalan | R: for 1y, followed by further loss of muscle strength | |
| bortezomib + cyclophosphamide + dexamethasone | R: clinical improvement, paraprotein in blood disappeared | |
| [10] a | plasmapheresis | PR: increased bulbar and neck strength, other muscles NR |
| steroids + cyclophosphamide | PR: increased muscle strength + vital capacity, paraprotein level in blood decreased, patient still disabled | |
| [38] | steroids + azathioprine | NR |
| [8] | steroids | NR |
| [52] a | riluzole | NR |
| IVIg | NR | |
| [26] a | steroids + mycophenolate mofetil | PR: increased muscle strength in legs, neck extensor weakness progressed |
| IVIg, steroids + mycophenolate mofetil | R: slow modest increase of muscle strength for 6 months | |
| [57] | posterior cervical orthodesis | R: improved head drop, minimal rotatory restriction |
| [13] a | steroids + cyclophosphamide + IVIg + rituximab | NR: died 21 m after onset |
| [6] | NIV | condition stabilized, orthopnea disappeared, weakness remained stable |
| [31] | peridural infiltration | R: improvement of lumbar cruralgia, stable for 3y |
| [5] | steroids | NR |
| [58] a | IVIg + steroids | NR |
| auto-PBSCT + melphalan | R: increased muscle strength over 1y, further decrease in muscle strength after 2y | |
| [25] a | IVIg | R: increased muscle strength over 2y with almost complete resolution, stable after 3y therapy |
| [25] a | IVIg + steroids + mycophenolate mofetil | R: increased muscle strength after 1y, but significant weakness remained |
| [27] a | lenalidomide + dexamethasone | R: significantly increased muscle strength over 3 m, continuing for 4y |
| [48] a | auto-PBSCT + melphalan | R: increased muscle strength over 1y, monoclonal protein level in blood undetectable |
| [3] | steroids | NR |
| [21] | steroids + azathioprine | NR |
| [50] | IVIg + steroids | R: increased muscle strength |
| [55] a | steroids | NR |
| auto-PBSCT + melphalan | R: increased muscle strength over 15 m | |
| [1] | steroids | NR |
| [1] a | steroids | NR |
| [4] | steroids | NR |
| [59] a | steroids + azathioprine | PR: myalgia disappeared, but weakness progressed |
| [60] | steroids | R: increased muscle strength, relapse after discontinuation of therapy |
| [61] | steroids + azathioprine | NR |
| [29] | steroids + IVIg | NR: skin rash disappeared, weakness progressed |
| [62] a | steroids + IVIg | NR |
| [63] | NIV | R: respiratory weakness resolved |
NIV noninvasive ventilation, NA not available, R responsive, PR partially responsive, NR not responsive, IVIg intravenous immunoglobulins, CK creatine kinase, auto-PBSCT autologous peripheral blood stem cell therapy, y years, m months
a Patients with monoclonal gammopathy