Table 1.
Neurological Findings & Immunotherapy Responses
| Neurological disorder | Total patients (N=236) | Classified according to highest VGCC-P/Q or VGCC-N Ab value | Both VGCC- P/Q and VGCC-N detected (N=21) | Immunotherapy response | ||||
|---|---|---|---|---|---|---|---|---|
| High (N=7) | Medium (N=79) | Low (N=150) | No response (N=38) | Modest response (N=22) | Robust Response (N=9) | |||
| Peripheral neuropathy* | 61 | 2 | 20 | 39 | 9 | 6 | 4 | 2 |
| None | 49 | 0 | 16 | 33 | 0 | 1 | 0 | 1 |
| Cognitive | 41 | 3 | 9 | 29 | 5 | 8 | 0 | 3 |
| Miscellaneous | 37 | 0 | 14 | 23 | 1 | 3 | 0 | 0 |
| Dysautonomia | 19 | 0 | 8 | 11 | 0 | 3 | 2 | 0 |
| Parkinsonism | 11 | 0 | 4 | 7 | 1 | 2 | 0 | 0 |
| Demyelinating disease | 11 | 0 | 5 | 6 | 2 | 1 | 1 | 1 |
| Anterior horn cell disorders | 11 | 0 | 2 | 9 | 1 | 3 | 0 | 0 |
| Radiculopathy | 10 | 0 | 7 | 3 | 2 | 2 | 0 | 0 |
| Seizures | 10 | 0 | 3 | 7 | 0 | 1 | 0 | 1 |
| Neuromuscular junction defect | 10 | 2 | 5 | 3 | 0 | 0 | 8 | 0 |
| Cerebellar ataxia (non-demyelinating) | 9 | 2 | 2 | 5 | 2 | 3 | 2 | 0 |
| Myopathy | 7 | 0 | 5 | 2 | 0 | 1 | 3 | 0 |
| Brachial plexopathy | 4 | 0 | 1 | 3 | 0 | 0 | 1 | 1 |
| Myelopathy (non-demyelinating) | 4 | 0 | 4 | 0 | 0 | 2 | 0 | 0 |
| Dystonia | 3 | 0 | 0 | 3 | 0 | 0 | 0 | 0 |
| Chorea | 1 | 0 | 0 | 1 | 0 | 1 | 0 | 0 |
| Sensorineural hearing loss | 1 | 0 | 0 | 1 | 0 | 1 | 0 | 0 |
| Stiff person syndrome | 1 | 0 | 1 | 0 | 0 | 0 | 1 | 0 |
EMG available in 55: length-dependent and primarily axonal sensorimotor (33), length-dependent mixed axonal and demyelinating (4), brachial plexopathy (4), sensorimotor demyelinating (2), sensory neuronopathy (2), severe, diffuse motor-predominant axonal polyradiculoneuropathy (1), multiple mononeuropathies (1), multiple subacute cranial neuropathies (1), multiple chronic cranial neuropathies (1), mild axonal neuropathy with fasciculations (1), severe chronic diffuse patchy polyradiculoneuropathy (1), sensorimotor polyradiculoneuropathy with demyelinating features (1), patchy primarily axonal polyradiculoneuropathy (1), and normal findings (2). Three patients were diagnosed with small fiber neuropathy only (normal EMG, 2; on clinical grounds alone, 1). Biopsied sural nerve tissue (2 patients) did not reveal inflammation.
Abbreviations: VGCC, voltage-gated calcium channel.