SOX-1¶

Sry-like high mobility group box 1¶

Other names¶

Anti-glial nuclear antibody

Description¶

Anti-SOX-1 is an antibody directed against an intracellular antigen highly expressed in the cerebellar cortex which is associated with Lambert-Eaton myasthenic syndrome, paraneoplastic cerebellar degeneration and less frequently limbic encephalitis, typically associated with small cell lung cancer.

SOX-1 was first identified as the antigen recognized by anti-glial nuclear antibodies (AGNA).

Mechanism of action¶

Onconeuronal antibodies usually target intracellular antigens that are expressed in both neuronal tissues and cancer cells. They are generally not directly pathogenic.

Tissue injury tends to arise from a parallel cytotoxic T-cell response, which results in irreversible neuronal damage and cell death, corresponding to guarded responses to current immunosuppressive approaches.

Associated clinical features¶

• Lambert-Eaton Myasthenic Syndrome
• Cerebellar degeneration
• Limbic encephalitis
• Peripheral neuropathy

Associated neoplasia¶

More strongly associated with SCLC than with a particular neurologic presentation. SOX-1 antibodies have also been identified in rare cases of sensory neuropathy following immune checkpoint inhibition for cancer.

Laboratory method¶

Notes of performance characteristics¶

Commercial line-blot assays may fail to identify 25% of the cases; cell-based assays may be more sensitive.³

Grade¶

Next steps¶