Anti Hu Antibody
From Anvita Health Wiki
Contents |
Pathology
- associated malignancies
- small cell carcinoma (most common)
- neuroblastoma
- associated neurologic disorders
- primarily affects dorsal root ganglion cells
- lymphocytic infiltration
- Nageotte nodules
- degeneration of peripheral & central sensory axons fasciculus gracilis severely affected
- autoantibody directed towards 35-40 kD nuclear RNA-binding proteins
- neuronal nuclear antigens
Clinical-manifestations
- paresthesias or dysesthesias generally start distally & move proximally
- early sensory change may involve face
- position & vibration most severely affected
- sensory ataxia
- pseudoathetosis
- early, widespread absence of reflexes
- neuropathy may precede development of carcinoma for > 3 years, more frequently by a few months
- neuropathy often progresses in a subacute fashion
Laboratory
- anti Hu autoantibodies in serum & CSF
Management
- treatment of underlying neoplasm usually does not effect the neuropathy (exceptions have been noted)
- response to immunosuppression is generally poor
More General Terms
Additional Terms
- 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)
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 651
- Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 625
- Stuve O & Zamvil SS. In: Medical Immunology 10th edition (2001), Section 3, Clinical Immunology. 38. Neurologic diseases
- Masdeu JC and Rodriguez-Oroz MC, Abnormalities of Posture and Movement, In: Geriatric Medicine, An Evidence-based approach 4th ed 2003
