Table 3

Paraclinical features of onconeuronal and surface antibody-associated syndromes

      Investigation
Antibody and syndrome
CSF inflammatory (pleocytosis, OCBs)Intrathecal synthesis?CSF antibodies detectedMRI abnormal acuteTemporal lobe high signalMultifocal/otherCerebellar atrophyEEG abnormalPredominant pattern nerve conduction studies/EMG
Onconeuronal
Hu (ANNA1) LE
Hu (ANNA1) BE
Hu (ANNA1) PCDLateLate
Hu (ANNA1) neuropathySN, SMN, A, (D)
Yo (PCA1) PCDLateSN, SMN, PRN, A
Ri (ANNA2)
Ma (PNMA1/2)(Ma2)(Ma2)
Ma2/Ta (PNMA2)
Amphiphysin**CMU activity in SPS
Amphiphysin neuropathySN, SMN, PRN, A
Zic4****
KELCH11
mGLuR1Late
mGLuR5
Tr/DNER PCD*Late
CRMP5/CV2 mixedIf LE*SN, SMN, PRN, A
CRMP5/CV2 choreaBGSlowing*
CRMP5/CV2 neuropathySN, SMN, PRN, A
VGCC LEMS↓ CMAPs
VGCC PCD˄LEMS in ~40%
Surface neuronal
NMDAR-Ab-E
GABAA
GABAB
AMPAR+
CASPR2–Morvan’sNeuromyotonia
CASPR2–LE+Myokimia
  • Paraclinical features of onconeuronal and surface antibody-associated syndromes compiled from references cited in this review—key: Embedded Imagenot delineated, Embedded Imageusually absent, Embedded Imagesometimes (up to ~30%), Embedded Imagequite frequent (~30% to 60%), Embedded Imagefrequent (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.