Table 1.
Clinical and Immunological Features of Patients With and Without PNS With GAD Antibodies
| Characteristic | PNS (n = 15) |
Non-PNS (n = 106) |
P Value |
|---|---|---|---|
| Age, mean (range), y | 60 (29–80) | 48 (5–79) | .03 |
| Female, No. (%) | 6 (40) | 88/102 (87) | <.001 |
| Diabetes mellitus at onset, No./total No. (%) | 2/13 (15) | 31/74 (42) | .12 |
| Other organ-specific autoimmune disorder, No./total No. (%) | 4/13 (31) | 43/75 (57) | .13 |
| Clinical syndrome | |||
| Encephalitis (LE, n = 5)a | 6 (26)b | 17 | <.001 |
| Cerebellar ataxia (PCD, n = 1)a | 4 (9)b | 39 | |
| Stiff-person syndrome | 2 (6)b | 32 | |
| Opsoclonus-myoclonus syndromea | 1 (100)b | 0 | |
| PEMa | 1 (100)b | 0 | |
| Otherc | 1 (100)b | 0 | |
| Isolated epilepsy | 0 (0)b | 18 | |
| GAD65-ab titer, median (IQR) | |||
| Serum, ×105 U/mL | 10.5 (1.2–31.9) | 5.9 (2.9–13.2) | .79 |
| CSF, ×103 U/mL | 3.5 (1.2–57.1) | 7.5 (1.7–17.2) | .86 |
| GAD67-ab, No./total No. (%) | |||
| Serum | 8/13 (62) | 93/106 (88) | .03 |
| CSF | 4/7 (57) | 61/61 (100) | <.001 |
| Neuronal cell-surface antibodies, No./total No. (%) | 8/15 (53)d | 12/106 (11)e | <.001 |
Abbreviations: CSF, cerebrospinal fluid; GAD, glutamic acid decarboxylase; IQR, interquartile range; LE, limbic encephalitis; PCD, paraneoplastic cerebellar degeneration; PEM, paraneoplastic encephalomyelitis; PNS, paraneoplastic neurological syndromes.
a
Classic PNS.
b
Percentage of patients in whom the syndrome was paraneoplastic.
c
Axial rigidity, vertigo, and dysautonomia (see the Results section).
d
γ-Aminobutyric acid bR antibody (n = 3), γ-aminobutyric acid aR antibody (n = 2), glycine receptor antibody (n = 1), and unknown antigen (n = 2).
e
γ-Aminobutyric acid aR antibody (n = 6) and glycine receptor antibody (n = 6).