Table 4.
Summary of 17 studies on the effects of various immunotherapies in patients with anti-GAD65Ab-associated CA.
| Age/Gender |
Delay
Subtype |
GAD Abs
MRI |
Induction / Maintenance Therapy |
Outcome Estimation
Follow up Period (Months) |
Levels of GAD Abs | |||
|---|---|---|---|---|---|---|---|---|
| Ishida et al. (1998) | ||||||||
| 66/F | 7 months. Chronic | 77,000 (U/ml). Atrophy | oral PSL / oral PSL, plasma exchange | Ataxias: slight recovery Subsequent progression of ataxia. Death 4 years after first therapy. Low response | Decreased | |||
| Abele et al. (1999) | ||||||||
| 68/F | 18 years Chronic | >1,000 (U/l) Atrophy | 1) IVIg 2) IVIg / none | ICARS: 59→50 ICACRS: 50→48 Low response (for 3 months) | No change | |||
| Takenoshita et al. (2001) | ||||||||
| 72/F | 2 years Chronic | 95,500(U/ml) ND | IVIg / none | Ataxias: unchanged No change (for 3 months) |
No change | |||
| Kono et al. (2001) | ||||||||
| 66/F | 10 months Chronic | > 50,000(U/ml) Normal | plasmapheresis+IVIg /none | Ataxias: unchanged No change (follow up ?) |
Decreased | |||
| Rüegg et al. (2002) | ||||||||
| 62/F | 1 year Chronic | 321 (U/l) Mild atrophy | IVIg / none | Gait ataxia: unchanged No change (for 1 month) |
ND | |||
| Matsumoto et al. (2002) | ||||||||
| 63/F | 1 month Subacute | 10,400(U/ml) Normal | 1) plasmapheresis 2) IVIg / none | Gait with aid→gait without aid (relapsed after 3 weeks) Prominent gait ataxia: unchanged No change (for 1 month) |
Decreased | |||
| Lauria et al. (2003) | ||||||||
| 66/F | 5 months Chronic | 531,000 (U/l) Mild atrophy | [First therapy] mPSL+IVIg / oral PSL [Second therapy] mPSL / oral PSL+CP | ICARS: 60→10 Low response (relapsed after 12 months) ICARS: 36→8 High response (for 6 months) |
Decreased Decreased |
|||
| Birand et al. (2006) | ||||||||
| 38/F | 33 months Chronic? | 6,472 (U/ml) Atrophy | mPSL / none | ICARS: 61→51 Low response (18 months) |
||||
| McFarland et al. (2006) | ||||||||
| 70/M | 9 months Chronic | 10,018 (U/ml) Normal | 1) mPSL 2) plasma exchange 3) mPSL / azathioprine |
ICARS: 78→20 (relapse after 2 months) ICARS: 29→30 ICARS: 30→6 High response (for 12 months from azathioprine medication) |
Decreased Decreased Decreased | |||
| Kim et al. (2006) | ||||||||
| 40/F +Stiff person syndrome |
4 months Chronic | 92,680(U/ml) ND | 1) mPSL 2) mPSL / oral PSL | ICARS: 31→20 (relapse after 4 months) ICARS: 23→11 High response (for 5 month) |
Decreased Decreased | |||
| Rakocevic et al. (2006) | ||||||||
| 30/M | 1 year Chronic | 2,413(nmol/L) Normal | IVIg / none | Ataxias: unchanged No change (follow up ?) |
ND | |||
| Vulliemoz et al. (2007) | ||||||||
| 58/M +Epilepsy |
2 months Subacute | 1/8000 Normal | mPSL / oral PSL, azathioprine |
Gait with aid→gait without aid High response (for 8 months) |
ND | |||
| Age/Gender | Delay Subtype |
GAD Abs MRI |
Induction / Maintenance Therapy |
Outcome Estimation Follow up Period (Months) |
Levels of GAD Abs | |||
| Chang et al. (2007) | ||||||||
| 56/F +Cognition failure |
3 months Subacute | 1,752 (nmol/l) Normal | mPSL / oral PSL, plasma exchange + plasma exchange + mPSL (monthly for 9 months) + mycophenolate mofefill /none | Gait with aid→gait without aid High response (for 15 months) | Decreased | |||
| Bonnan et al. (2008) | ||||||||
| 38/F | 12 months Chronic | >30,000 (U/ml) ND | mPSL × 6+plasma exchange / none | ICARS: 60→52 Low response (36 months) |
Fluctuated | |||
| 45/F | 12 months Chronic | 9,565(U/ml) ND | mPS L × 6 / azathioprine+ periodic IVIg |
ICARS: 8→12 Progression (31 months) |
Fluctuated | |||
| 75/F | 10 months Chronic | 302/(U/ml) ND | mPSL × 6 / none | ICARS: 16→14 No change (12 months) |
Fluctuated | |||
| Georgieva and Parton (2014) | ||||||||
| 45/M +epilepsy |
7 years Chronic | Strongly positive Mild atrophy | [First therapy] IVIg / azathioprine [Second therapy] IVIg / azathioprine [Third therapy] plasma exchange / azathioprine [Fourth therapy] IVIg / azathioprine [Fifth therapy] plasma exchange / azathioprin |
ICARS: 8→4.5 Low response (relapsed after 10 weeks) ICARS: 7.5→5.5 Low response (relapsed after 10 weeks) ICARS 15→6 Low response (relapsed after 12 weeks) ICARS: 12.5→10 Low response (relapsed after 8 weeks) ICARS 12→8 Low response (relapsed after 8 weeks) |
ND | |||
| Planche et al. (2014) | ||||||||
| 72/F | 6 months Subacute | >250 (U/ml) Normal | 1) IVIg×2 2) rituximab / none |
Ataxias: progressive ICARS: 22→10 High response (for 14 months) |
Increased Decreased | |||
| 73/F | 3 years Chronic? | >221 (U/ml) Normal | 1) IVIg×2 2) rituximab / IVIg×3 |
Ataxias: slight recovery ICARS: 22→21 Low response (for 17 months) |
ND No change |
|||
| 65/M | 3 years Chronic? | >1,000 (U/ml) Atrophy | 1) IVIg 2) IVIg+rituximab+CP / none |
Ataxias: progressive ICARS: 35→43 Progressed (for 16 months) |
No change | |||
| Kuchling et al. (2014) | ||||||||
| 74/F | 6 months Subacute | > 2,000 IU/ml Normal | immunoabsorption+ rituximab / none | Gait with aid→gait without aid High response (for 1 month) |
ND | |||
| 76/F | 6 years Subacute | > 2,000 IU/ml Normal | immunoabsorption+ rituximab / none | Prominent gait ataxia: unchanged No change (for 1 month) | ND | |||
Summary of data of 27 clinical trials that included 22 patients from 17 studies.
Abbreviations: SPS: Stiff person syndrome, mPSL; intravenous methylprednisolone, oral PSL; oral prednisolone, IVIg; intravenous immunoglobulins, CP: Cyclophosphamide, ND: Not described.