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anti-Hu antibody (type-1 ANNA)

Etiology: 1) associated malignancies a) small cell carcinoma (most common) b) neuroblastoma c) breast cancer d) ovarian cancer e) testicular cancer f) Hodgkin's lymphoma 2) associated neurologic disorders a) sensory & autonomic neuropathies b) limbic encephalopathy c) cerebellar degeneration d) myelopathy e) motor neuropathy Epidemiology: - older persons Pathology: 1) primarily affects dorsal root ganglion cells a) lymphocytic infiltration b) Nageotte nodules 2) degeneration of peripheral & central sensory axons fasciculus gracilis severely affected 3) autoantibody directed towards 35-40 kD nuclear RNA-binding proteins 4) neuronal nuclear antigens a) HuC (ELAV-3) b) HuR (ELAV-1) b) Hel-N1 (ELAV-2) Clinical manifestations: 1) paresthesias or dysesthesias generally start distally & move proximally 2) early sensory change may involve face 3) position & vibration most severely affected 4) sensory ataxia 5) pseudoathetosis 6) early, widespread absence of reflexes 7) neuropathy may precede development of carcinoma for > 3 years, more frequently by a few months 8) neuropathy often progresses in a subacute fashion 9) limbic encephalitis in 10-20% of patients Laboratory: - anti Hu autoantibodies in serum & CSF Management: 1) treatment of underlying neoplasm usually does not effect the neuropathy (exceptions have been noted) 2) response to immunosuppression is generally poor

Related

ELAV-like protein 1; Hu-antigen R; HuR (ELAVL1, HUR) ELAV-like protein 2; Hu-antigen B; HuB; ELAV-like neuronal protein 1; nervous system-specific RNA-binding protein Hel-N1 (ELAVL2, HUB) ELAV-like protein 3; Hu-antigen C; HuC; paraneoplastic cerebellar degeneration-associated antigen; paraneoplastic limbic encephalitis antigen 21 (ELAVL3, HUC, PLE21) paraneoplastic sensory neuropathy

General

paraneoplastic autoantibody antineuronal nuclear antibody (ANNA)

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 651
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 625
  3. Stuve O & Zamvil SS. In: Medical Immunology 10th edition (2001), Section 3, Clinical Immunology. 38. Neurologic diseases
  4. Masdeu JC and Rodriguez-Oroz MC, Abnormalities of Posture and Movement, In: Geriatric Medicine, An Evidence-based approach 4th ed 2003
  5. Mini Panel of 2 tests: Neuronal Nuclear Antibody IgG, Anti-Hu . Neuronal Nuclear Antibody IgG, Anti-Ri Laboratory Test Directory ARUP: 51088
  6. Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012