Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes
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The disorders of the central nervous system associated with cancer by remote immune-mediated mechanisms are a heterogeneous group. These disorders encompass the classic paraneoplastic disorders and the recently recognized autoimmune encephalitis associated with antibodies against neuronal cell surface or synaptic proteins that occur with or without cancer association. In the last decade, the new surge of interest in neuronal diseases associated with anti-neuronal antibodies led to the rapid discovery of new forms of disease that have different manifestations and were not previously suspected to be immune mediated. The recognition of these syndromes is important because it may lead to early detection of an underlying malignancy and prompt initiation of treatment, improving chances for a better outcome.
KeywordsLimbic encephalitis Onconeuronal antibodies Paraneoplastic neurological syndromes Paraneoplastic encephalomyelitis Paraneoplastic cerebellar degeneration Autoimmune encephalitis Stiff person syndrome Progressive encephalomyelitis with rigidity and myoclonus Opsoclonus-myoclonus syndrome Morvan syndrome Ophelia syndrome N-methyl-d-aspartate receptor (NMDAR) encephalitis Anti-AMPA receptor (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) encephalitis Anti-GABAbR (γ-aminobutyric acid receptor-B) encephalitis Anti-leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis Anti-Caspr2 encephalitis (contactin-associated protein-like 2) Anti-glycine receptor (GlyR) antibodies Anti-mGluR5 (metabotropic glutamate receptor 5) Anti-mGLUR1 (metabotropic glutamate receptor 1) Anti-Hu antibodies Anti-Ma2 antibodies Anti-DNER (Delta/Notch-like epidermal growth factor-related receptor) antibodies Anti-CV2/CRMP5 antibodies
Compliance with Ethical Standards
Conflict of Interest
Ronnyson Susano Grativvol declares that he has no conflict of interest.
Wagner Cid Palmeira Cavalcante declares that he has no conflict of interest.
Luiz Henrique Martins Castro declares that he has no conflict of interest.
Ricardo Nitrini has received research funding from FAPESP, has served on advisory boards for Janssen-Cilag and Brazilian Nutricia, has received travel funding and reimbursement from Novartis, serves as an editor for Dementia & Neuropsychologia, serves on the editorial board for Alzheimer’s Disease & Associated Disorders and International Journal of Alzheimer’s Disease, and has served as a guest speaker for Novartis and Danone (Nutricia).
Mateus Mistieri Simabukuro declares that he has no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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