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.