Anti-SOX1 antibodies in patients with paraneoplastic and non-paraneoplastic neuropathy.
Summary of "Anti-SOX1 antibodies in patients with paraneoplastic and non-paraneoplastic neuropathy."
Anti-SOX1 antibodies have been described to be positive in patients with paraneoplastic Lambert-Eaton myasthenic syndrome and, in a lower amount, in patients with anti-Hu positive paraneoplastic neurological syndromes, and with SCLC alone, respectively. We found 5/32 patients with paraneoplastic neuropathy and, surprisingly, 4/22 patients with neuropathy of unknown origin positive for anti-SOX1 antibodies, whereas no patient with inflammatory neuropathy and no healthy controls showed any reactivity (p=0.007). All patients with neuropathy of unknown origin where followed up for four years without diagnosis of a tumour so far. Anti-SOX1 antibodies are associated with paraneoplastic neuropathies and may define another group of non-paraneoplastic, immune-mediated neuropathies.
Dept. of Neurology, Justus-Liebig-University, Am Steg 14, 35385 Giessen, Germany.
This article was published in the following journal.
Name: Journal of neuroimmunology
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20701982
- DOI: http://dx.doi.org/10.1016/j.jneuroim.2010.07.005
Medical and Biotech [MESH] Definitions
Hu Paraneoplastic Encephalomyelitis Antigens
A family of RNA-binding proteins that are homologues of ELAV protein, Drosophila. They were initially identified in humans as the targets of autoantibodies in patients with PARANEOPLASTIC ENCEPHALOMYELITIS. They are thought to regulate GENE EXPRESSION at the post-transcriptional level.
A paraneoplastic syndrome marked by degeneration of neurons in the medial temporal lobe. Clinical features include behavioral changes, HALLUCINATIONS, loss of short term memory, anosmia, AGEUSIA, and DEMENTIA. Circulating anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinomas are frequently associated with this condition. (From Pathologe 1997 Sep;18(5):406-10; J Int Neuropsychol Soc 1996 Sep;2(5):460-6; Brain 1997 Jun;120(Pt 6):923-8)
Paraneoplastic Cerebellar Degeneration
Cerebellar degeneration associated with a remote neoplasm. Clinical manifestations include progressive limb and GAIT ATAXIA; DYSARTHRIA; and NYSTAGMUS, PATHOLOGIC. The histologic type of the associated neoplasm is usually carcinoma or lymphoma. Pathologically the cerebellar cortex and subcortical nuclei demonstrate diffuse degenerative changes. Anti-Purkinje cell antibodies (anti-Yo) are found in the serum of approximately 50% of affected individuals. (Adams et al., Principles of Neurology, 6th ed, p686)
A diffuse or multifocal peripheral neuropathy related to the remote effects of a neoplasm, most often carcinoma or lymphoma. Pathologically, there are inflammatory changes in peripheral nerves. The most common clinical presentation is a symmetric distal mixed sensorimotor polyneuropathy. (Adams et al., Principles of Neurology, 6th ed, p1334)
In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products.
Stich O, Klages E, Bischler P, Jarius S, Rasiah C, Voltz R, Rauer S. SOX1 antibodies in sera from patients with paraneoplastic neurological syndromes. Acta Neurol Scand: DOI: 10.1111/j.1600-0404.2011....
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