Table 2.
Response to different immunotherapies in the main series of SPS, cerebellar ataxia, and limbic encephalitis associated with GAD Ab reported in literature.
| Number of Patients | Treatment Schedule | Outcome after Treatment | Treatment-Related Complications | Mortality during FU | Reference | |
|---|---|---|---|---|---|---|
| IVIg Placebo controlled study |
16 | 2 g/kg, divided into two daily doses, every month for 3 months | 11 out of 14 patients (79%) improve with regard to muscle rigidity, spasms, and functional ability to walk | None | 12.5% | Dalakas et al., 2005 [33,23] |
| High-dose CS | 2 | Oral or intravenous steroids | - Distal stiffness: 1/5 slight improvement and 1/5 worsening - Axial stiffness: ½ slight improvement |
NA | NA | Barker et al., 1998 [109] |
| High-dose CS | 2 | Prednisone 100 mg/d, and decrease | 1. improvement: no symptoms at 10 d 2. improvement |
1. insomnia, increased anxiety, de- pressed mood 2. hypokalemia, cushingoid features |
0% | Piccolo et al., 1988 [126] |
| PE | 1 | No details | Stable: mRS 4 | NA | NA | McKeon et al., 2012 [45] |
| PE | 3 | No details | Stable | NA | NA | Barker et al., 1998 [109] |
| PE | 1 | No details | Marked improvement | NA | NA | Brashear et al., 1991 [127] |
| Rituximab Double blind placebo-controlled study | 14 | 2 biweekly infusions of 1g each | Primary outcome (change in stiffness scores at 6 months): non-significant effect | Some infusion-related reactions | NA | Dalakas et al., 2017 [34] |
| Rituximab | 1 | - 1000 mg at 0 and 14 day | Partial Improvement on scores: -stiff: 4/6 to 1 - sensitivity: 5/7 to 4 |
NA | 0% | Sevy et al., 2012 [128] |
| Rituximab | 1 | 1000 mg at 0 and 7 day | Improvement: mRS 4 to 1 | NA | 0% | Bacorro et al. 2010 [129] |
| IVIg | 1 | 2 g/kg over 5 days every month for 3 months | Partial improvement (ICARS 65 to 37) |
NA | NA | Pedroso et al., 2011 [130] |
| IVIg | 1 | IVIg every month for 2 months | Partial improvement (ICARS 59 to 48) |
NA | 0% | Abele et al., 1999 [131] |
| IVIg | 3 | 0.4 g/kg/day for 5 days, followed by two cycles of single monthly doses 1g/kg | Partial improvement for 1/3 No improvement for 2/3 |
NA | NA | Aguiar et al., 2017 [112] |
| High-dose CS | 1 | MP 1000 mg/ day for 5 d | Improvement: ICARS 60 to 36 | NA | 0% | Lauria et al., 2003 [132] |
| High-dose CS | 1 | MP 1000 mg/day for 5 d | Improvement: ICARS 38 to 22 at the beginning and ICARS 7 at 3 months | NA | 0% | Virgilio et al., 2009 [133] |
| PE + Rituximab | 2 | 7–10 cycles of plasmapheresis + 1000 mg rituximab IV | 1. High response during 1 month 2. No change |
NA | 0% | Kuchling et al., 2014 [134] |
| High-dose CS | 1 | MP 500 mg/d, 6 days | High improvement | NA | 0% | Marchiori et al., 2001 [135] |
| PE | 1 | Two cycles of 7 PE + 500 mg MPx3/AZA+ oral corticosteroids | Important improvement the first month: decreasing of seizures, low response after | NA | NA | Mazzi et al., 2008 [136] |
| High-dose CS | 11 | median total dose 19 g (3–30 g) | 45% of response | 55%: -Cushing syndrome in three patients, hyperglycaemia, sleep disorders, nervousness and psychosis |
NA | Malter et al., 2015 [137] |
| IVIg | 5 | Dose: range 3–4 g for a median of 3 months | 20% (1 patient) with seizure response | 0% | NA | Malter et al., 2015 [137] |
| PE | 8 | 1 (or 2) sequence of 16 sessions in median (range: 11–26) | 13% (1 patient) of response | 0% | NA | Malter et al., 2015 [137] |