Table 2.
Clinical features of patients treated for paraneoplastic neurological disorder
|
CSF†
|
Initial
|
6 months
|
|||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Arm-Pt. | Age Sex | Cancer | Syndrome | Duration (mo) | Protein (mg/dl) | Cells | Antibody | Ab titer‡ | RS | Barthel | ChemoTx | Ab titer‡ | RS | Barthel | Outcome | Cancer Status | Last f/u (mo) |
| A-1 | 60/M | None1 | PLE Gastroparesis | 6 | 61 | 2 | ANNA-1 | 3,840 | 2 | 95 | CYT 20 mos | 960 | 1 | 100 | Improved | CR | 41, alive |
| A-2 | 36/M | Thymoma | PLE | 9.5 | 180 | 1 | None | — | 2 | 85 | CYT 9 mos | — | 1 | 100 | Improved | CR | 29, alive |
| A-3 | 55/F | Ovarian | PCD | 0.5 | ND | ND | PCA-1 | 1,920 | 3 | 95 | CYT 30 mos | 240 | 2 | 100 | Improved | CR | 38, alive |
| A-4 | 78/F | SCLC | PSN | 4.5 | ND | ND | ANNA-1 | 15,360 | 2 | 90 | CYT 2 2 mos | 7,680 | 2 | 100 | Improved | CR | 36, alive |
| A-5 | 71/F | None | Ataxia, optic neuropathy | 3.5 | 105 | 2 | CRMP-5 | 960 | 3 | 85 | CYT 8 mos | 960 | 3 | 95 | Improved | — | 31, alive |
| A-6 | 55/F | Ovarian | PCD | 0.5 | 56 | 50 | PCA-1 | 3,840 | 3 | 55 | CYT 2 3 mos | 3,840 | 3 | 95 | Improved | CR | 30, dead |
| A-7 | 72/F | Ovarian | PCD | 1 | 83 | 7 | PCA-1 | 122,880 | 3 | 80 | CYT a,b 4 mos | ND | 4 | 55 | Worse | Active | 24, alive |
| A-8 | 74/F | Fallopian | PCD | 1 | 60 | 7 | PCA-1 | 7,680 | 4 | 40 | a,b 2 mos | 15,360 | 5 | 15 | Worse | CR | 25, alive |
| A-9 | 53/F | Ovarian | Motor neuropathy | 8.5 | 97 | 57 | PCA-1 | 3,840 | 4 | 25 | CYT 4 1 mo | 7,680 | 5 | 10 | Worse | CR | 14, dead |
| A-10 | 73/M | TCC | Ataxia, encephalitis | 3 | 186 | 44 | None | — | 4 | 25 | None | ND | 6 | 0 | Worse | — | 0, dead |
| B-1 | 63/M | SCLC | PSN | 3.5 | 135 | 22 | ANNA-1 | 7,680 | 3 | 85 | Etoposide Carboplatin | 3,840 | 2 | 90 | Improved | CR | 25, alive |
| B-2 | 57/F | Breast | PCD | 5.5 | 49 | 1 | None | — | 4 | 45 | Doxorubicin CYT | — | 3 | 80 | Improved | CR | 27, alive |
| B-3 | 46/F | Peritoneal | PCD | 1 | ND | ND | PCA-1 | 122,880 | 4 | 25 | Paclitaxel Carboplatin | 1,920 | 3 | 65 | Improved | CR | 34, alive |
| B-4 | 39/F | Ovarian | PCD | 7.5 | ND | ND | PCA-1 | 15,360 | 4 | 45 | Doxorubicin5 | ND | 4 | 45 | Stable | Active | 23, alive |
| B-5 | 60/F | Ovarian | PCD | 1.5 | 84 | 12 | PCA-1 | 122,880 | 4 | 65 | Paclitaxel Carboplatin | 61,440 | 4 | 35 | Worse | CR | 29, alive |
| B-6 | 56/F | Ovarian | PCD | 5 | 75 | 21 | PCA-1 | 15,360 | 4 | 40 | Paclitaxel Carboplatin | 3,840 | 4 | 20 | Worse | CR | 30, alive |
| B-7 | 65/F | Endometrial | PCD | 1 | ND | ND | PCA-1 | 61,440 | 3 | 95 | Doxorubicin CYT | 7,680 | 4 | 70 | Worse | CR | 25, alive |
| B-8 | 71/M | SCLC | PLE, PSN | 1.5 | ND | ND | ANNA-1 | 61,440 | 4 | 75 | Etoposide Carboplatin | ND | 6 | 0 | Worse | — | 2, dead |
| B-9 | 66/F | Ovarian | PCD | 5.5 | 64 | 0 | PCA-1 | 960 | 4 | 60 | Paclitaxel Carboplatin | ND | 6 | 0 | Worse | — | 2, dead |
| B-10 | 57/F | SCLC | Encephalitis | 2.5 | 79 | 1 | ANNA-1 | 7,680 | 4 | 30 | None | ND | 6 | 0 | Worse | — | 1, dead |
Abbreviations used are as follows: Ab, antibody; ANNA-1, antineuronal nuclear antibody, type 1; ChemoTx, chemotherapy; CR, cancer in remission; CRMP-5, collapsin-response mediator protein antibody; CSF, cerebrospinal fluid; CYT, cyclophosphamide; f/u, follow-up; ND, not done; PCA-1, Purkinje cell antibody, type 1; PCD, paraneoplastic cerebellar degeneration; PLE, paraneoplastic limbic encephalitis; PSN, paraneoplastic sensory neuropathy; RS, Rankin score; SCLC, small-cell lung carcinoma; TCC, transitional cell carcinoma.
CSF analysis. Normal values: protein 0-45 mg/dl, cells (nucleated cells per microliter) <5.
Antibody titers are the reciprocal of the highest dilution yielding a positive result by immunofluorescence assay.
Non-small-cell lung carcinoma diagnosed after completion of study, 32 months after symptom onset.
CYT discontinued because of persistent leukopenia despite dose reduction.
Subsequently continued on oral mycophenolate mofetil without improvement.
Oral CYT discontinued because of intractable nausea; continued with monthly intravenous CYT treatments.
Patient elected to discontinue chemotherapy after 1 cycle. Lost to follow-up after 6-month assessment.