Encephalomyelopathies

Pathogenesis and Pathophysiology. Strong evidence exists that these neurological syndromes result from attacks by humoral and cellular arms of the immune system. Some patients with paraneoplastic encephalomyelitis with small cell lung cancer harbor high titers of IgG antibodies against a set of 35- to 40-kd proteins that are selectively expressed in the nuclei of neurons throughout the human nervous system and in all small cell lung cancers, some neuroblastomas, and medulloblastomas.y y y These antibodies, designated as anti-Hu, are present in higher concentrations in the CSF than in the serum, suggesting that their synthesis likely occurs within the central nervous system. y The antibodies can be detected within the neuronal nuclei of patients with paraneoplastic encephalomyelitis. A correlation between sites of accumulation and those areas most affected by the paraneoplastic syndrome is observed. y Although there is a high correlation between the presence of high titers of anti-Hu antibody and the development of paraneoplastic encephalomyelitis and sensory neuronopathy the exact role of these antibodies in the pathogenesis of this disease is unclear. These proteins are likely to promote neuronal differentiation and maintenance of the

_TABLE 47-10 -- PARANEOPLASTIC SYNDROMES AFFECTING THE NERVOUS SYSTEM_

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