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Multiple Sclerosis & Neuroimmunology

Paraneoplastic and Non-Paraneoplastic Neurological Syndromes Associated with Neuronal Auto Antibodies

Antibodies against intracellular antigens (Classical onconeuronal antibodies)
Antibody Clinical/anatomical syndrome Associated tumor % of idiopathic cases
Hu (ANNA1) Limbic encephalitis, ataxia, neuropathy SCLC (small cell lung cancer) 2%
Ri (ANNA2) Ataxia, involuntary movements Breast, SCLC 4%
Yo (PCA1) Ataxia Ovary, breast 2%
CV2 (CRMP5) Limbic encephalitis, involuntary movements, visual changes SCLC, thymoma 4%
Ma2 Involuntary movements and parkinsonism Testicular seminoma 4%
Tr/DNER Ataxia Hodgkin's lymphoma 10%
Amphiphysin Stiff person syndrome Breast, SCLC 5%
GAD65 Stiff person syndrome, limbic encephalitis, ataxia Rarely thymoma 75%
Antibodies against neuromuscular and ganglionic synaptic receptors
Antibody Clinical/anatomical syndrome Associated tumor % of idiopathic cases
VGCC Lambert-Eaton myasthenic syndrome, ataxia, neuropathy SCLC 79%
ACHR-G Autonomic dysfunction idiopathic Majority are idiopathic
Antibodies against surface antigens
Antibody Clinical/anatomical syndrome Associated tumor % of idiopathic cases
NMDA Limbic encephalitis, Psychosis, speech dysfunction, involuntary movements, coma, seizures, autonomic dysfunction Ovarian teratoma or rarely testicular 41% (especially children)
AMPA Limbic encephalitis SCLC, breast, thymoma 30%
GABA-A Seizures, stiff person syndrome Thymoma 95%
GABA-B Limbic encephalitis SCLC 50%
Glycine R Stiff person syndrome, involuntary movements idiopathic 97%
mGluR1/anti Homer-3 Ataxia Hodgkin's lymphoma Mostly paraneoplastic
mGluR5 Limbic encephalitis Hodgkin's lymphoma Mostly paraneoplastic
VGKC (CASPR2) Limbic encephalitis, neuromyotonia Rare: thymoma, SCLC 70%
VGKC (LGI1) Limbic encephalitis, seizures Rare: thymoma, SCLC 70%
DPPX Involuntary movements, dysautonomia Rare: Lymphoma 90%
D2 Involuntary movements idiopathic 100%