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| Can hidden cancer trigger an immune response which destroys the nervous
system?
Could an early onset lung cancer be one cause of bulbar onset ALS?
CNS spinal fluid can be tested for antibodies.
Paraneoplasia and autoimmunologic injury of the nervous system: the
anti-Hu syndrome.
Rosenblum MK.
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New
York, NY 10021.
In recent years, antineuronal autoantibodies of varying antigenic
specificity have come to be associated with a number of paraneoplastic
neurologic disorders. Anti-Hu is a polyclonal complement-fixing IgG
directed against a 35 to 40 kilodalton protein concentrated in the
nuclei of neurons throughout the central and peripheral neuraxes. Its
elaboration at high titer in serum and cerebrospinal fluid is
invariably associated with a neurologic syndrome characterized chiefly
by subacutely evolving sensory neuropathy and an array of central
disturbances that include bulbar and cerebellar dysfunction, limbic
encephalitis and motor neuron disease. The manufacture of anti-Hu IgG
is triggered in a great majority of cases by underlying small cell
carcinomas of pulmonary origin, typically limited in stage and
otherwise silent, that aberrantly express the native neuronal antigen
or an antigenically indistinguishable epitope. Both neoplastic and
diseased neural tissues contain lymphocytes of B and T lineage
specifically cognizant of the Hu antigen as well as concentrated
anti-Hu IgG bound to tumor cells and neurons, respectively. These
observations suggest that an immune response serving initially to limit
the growth and spread of its inciting neoplasm comes subsequently to be
misdirected against the nervous system of the host, resulting in
autoimmunologically-mediated neurologic injury. Clinical,
neuropathologic and immunologic data derived from a series of 71
sero-confirmed cases of the anti-Hu-associated paraneoplastic sensory
neuronopathy/encephalomyelitis complex are reviewed.
Publication Types:
* Review
* Review, Tutorial
PMID: 8293180 [PubMed - indexed for MEDLINE]
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