Paraclinical features of onconeuronal and surface antibody-associated syndromes
| Investigation Antibody and syndrome | CSF inflammatory (pleocytosis, OCBs) | Intrathecal synthesis? | CSF antibodies detected | MRI abnormal acute | Temporal lobe high signal | Multifocal/other | Cerebellar atrophy | EEG abnormal | Predominant pattern nerve conduction studies/EMG |
| Onconeuronal | |||||||||
| Hu (ANNA1) LE | |||||||||
| Hu (ANNA1) BE | |||||||||
| Hu (ANNA1) PCD | Late | Late | |||||||
| Hu (ANNA1) neuropathy | SN, SMN, A, (D) | ||||||||
| Yo (PCA1) PCD | Late | SN, SMN, PRN, A | |||||||
| Ri (ANNA2) | |||||||||
| Ma (PNMA1/2) | (Ma2) | (Ma2) | |||||||
| Ma2/Ta (PNMA2) | |||||||||
| Amphiphysin | * | * | CMU activity in SPS | ||||||
| Amphiphysin neuropathy | SN, SMN, PRN, A | ||||||||
| Zic4 | * | * | * | * | |||||
| KELCH11 | |||||||||
| mGLuR1 | Late | ||||||||
| mGLuR5 | |||||||||
| Tr/DNER PCD | * | Late | |||||||
| CRMP5/CV2 mixed | If LE* | SN, SMN, PRN, A | |||||||
| CRMP5/CV2 chorea | BG | Slowing* | |||||||
| CRMP5/CV2 neuropathy | SN, SMN, PRN, A | ||||||||
| VGCC LEMS | ↓ CMAPs | ||||||||
| VGCC PCD | ˄ | LEMS in ~40% | |||||||
| Surface neuronal | |||||||||
| NMDAR-Ab-E | |||||||||
| GABAA | |||||||||
| GABAB | |||||||||
| AMPAR | + | ||||||||
| CASPR2–Morvan’s | Neuromyotonia | ||||||||
| CASPR2–LE | + | Myokimia | |||||||
Paraclinical features of onconeuronal and surface antibody-associated syndromes compiled from references cited in this review—key:
not delineated, usually absent, sometimes (up to ~30%), quite frequent (~30% to 60%), frequent (more than ~60%).+Tested on a subset of patients.
˄Isolated case reports of VGCC and LE.
*Based on small reports (typically ~5 or fewer).
A, axonal; AMPAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; ANNA, antineuronal nuclear antibody; BE, brainstem encephalitis; CASPR2, contactin-associated protein-like 2; CMAP, compound muscle action potential; CMU, continuous motor unit; CRMP5, collapsin response-mediator protein-5; CSF, cerebrospinal fluid; D, demyelinating; DNER, delta and notch-like epidermal growth factor-related receptor; EEG, electroencephalogram; GABAA/B, γ-amino butyric acid (alpha/beta subunit); GI, gastrointestinal; IT, immunotherapy; KELCH11, Kelch-like protein 11; LE, limbic encephalitis; LEMS, Lambert-Eaton myasthenic syndrome; mGluR1/5, metabotropic glutamate receptor 1/5; Morvan’s, Morvan’s syndrome; NMDAR, N-methyl-D-aspartate receptor; NMDAR-Ab-E, NMDAR-antibody encephalitis; OCB, oligoclonal band; PCA, Purkinje cell cytoplasmic antibody; PCD, paraneoplastic cerebellar degeneration; PNMA1/2, paraneoplastic antigen Ma1/2; PRN, polyradicular neuropathy; SMN, sensory motor neuropathy; SN, sensory neuropathy; SPS, stiff-person syndrome; VGCC, voltage-gated calcium channel.
