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The understanding of the manifestations, mechanisms, and management of autoimmune encephalitis has expanded dramatically in recent decades. Immune-mediated encephalitides are comparable in incidence and prevalence to infectious etiologies, and are associated with significant morbidity, especially when there is a delay in recognition and treatment. As such, clinicians from many specialties must develop a functional understanding of these disorders. Herein we provide an overview of the autoimmune and paraneoplastic encephalitides, including those associated with either intracellular or cell surface/synaptic neuronal autoantibodies. After briefly reviewing the current understanding of the pathobiology of autoimmune encephalitis, we combine a neuroanatomical approach with specific antibody syndromes to provide the reader with a clinically relevant review of these disorders. The clinical manifestations, diagnosis, and management of autoimmune encephalitis are reviewed, with an emphasis on clinical relevance. We also introduce updates in the field, including autoimmune encephalitis associated with novel cancer immunotherapies, infectious triggers of autoimmune encephalitis, and autoimmune encephalitis with demyelinating overlap syndromes. [ABSTRACT FROM AUTHOR]

ABSTRACT Introduction: Patients with Purkinje cell cytoplasmic autoantibody type 1 (PCA-1-IgG, a.k.a. anti-Yo) classically present with a paraneoplastic syndrome characterized by subacute onset cerebellar ataxia with rapid progression. Recently, a broader spectrum of neurological presentations has been recognized, which include peripheral neuropathy, but detailed case descriptions are lacking. Methods: A patient presented with a painful progressive multifocal sensorimotor polyneuropathy including face pain. Whole body positron emission tomography (PET), exploratory abdominal laparotomy and nerve biopsy were combined with serial neurological examinations and neuro-immunological testing to diagnose and determine treatment. Results: PCA-1 autoimmunity was identified. Although a PET scan failed to detect a tumor, the patient underwent an exploratory laparotomy, given the strong association of PCA-1-IgG autoantibodies with underlying malignancy. The patient was diagnosed with an ovarian lymphoepithelial cancer that was subsequently treated with chemotherapy. Conclusions: PCA-1-IgG autoantibodies can occur in the setting of peripheral neuropathy and predict type of cancer. Multifocal neuropathy with face pain and treatment-resistance is a specific clinical pattern associated with PCA-1-IgG autoantibodies and ovarian cancer. Muscle Nerve 48:827-831, 2013 [ABSTRACT FROM AUTHOR]