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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Epidemiology

The California Encephalitis Project was established in 1998 to identify the etiologic agents and to study epidemiology and clinical characteristics of encephalitis. In 2009, they reported ten cases of NMDA receptor antibodies and concluded that unlike classic paraneoplastic encephalitis, anti-NMDA receptor encephalitis affects younger patients [13]. Since, an increasing number of cases have been reported to the California Encephalitis Project, making NMDA receptor antibodies a significant cause of encephalitis among young patients. Between 2007 and 2011, 761 cases of encephalitis of uncertain etiology in individuals aged <30 years were reported to the California Encephalitis Project. Of these, 32 patients were tested positive for anti- NMDAR encephalitis; however, viral encephalitis was diagnosed in only 42 patients [14]. Although anti-NMDAR encephalitis was initially thought to affect young women, often with teratomas, it can affect men and children, with or without any identifiable tumor [15]. Overall, 75% of patients with anti-NMDAR encephalitis can significantly recover when diagnosed promptly [10].

Among paraneoplastic syndrome, Lambert-Eaton myasthenic syndrome, which affects approximately 3% of patients with small-cell lung cancer, and myasthenia gravis, which affects 15% of patients with thymoma, are common [16]. Up to 9% of patients with small-cell lung cancer have at least one form of paraneoplastic syndrome (commonly Lambert-Eaton myasthenic syndrome, sensory neuronopathy, or limbic encephalitis) [16]. y-Aminobutyric acid (GABA-B) receptor antibodies are also responsible for paraneoplastic limbic encephalitis in patients with small-cell lung cancer [17].

 
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