Table 2 Treatment and outcome in patients with SLONM (novel patients P1-10 and literature data)

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