Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes

Curr Oncol Rep. 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y.


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.

Keywords: Anti-AMPA receptor (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) encephalitis; Anti-CV2/CRMP5 antibodies; Anti-Caspr2 encephalitis (contactin-associated protein-like 2); Anti-DNER (Delta/Notch-like epidermal growth factor-related receptor) antibodies; Anti-GABAbR (γ-aminobutyric acid receptor-B) encephalitis; Anti-Hu antibodies; Anti-Ma2 antibodies; Anti-glycine receptor (GlyR) antibodies; Anti-leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis; Anti-mGLUR1 (metabotropic glutamate receptor 1); Anti-mGluR5 (metabotropic glutamate receptor 5); Autoimmune encephalitis; Limbic encephalitis; Morvan syndrome; N-methyl-D-aspartate receptor (NMDAR) encephalitis; Onconeuronal antibodies; Ophelia syndrome; Opsoclonus-myoclonus syndrome; Paraneoplastic cerebellar degeneration; Paraneoplastic encephalomyelitis; Paraneoplastic neurological syndromes; Progressive encephalomyelitis with rigidity and myoclonus; Stiff person syndrome.

Publication types

  • Review

MeSH terms

  • Antibodies / blood
  • Central Nervous System Diseases / blood*
  • Central Nervous System Diseases / complications
  • Central Nervous System Diseases / pathology
  • Central Nervous System Diseases / therapy
  • Early Detection of Cancer
  • Encephalitis / blood*
  • Encephalitis / complications
  • Encephalitis / pathology
  • Encephalitis / therapy
  • Hashimoto Disease / blood*
  • Hashimoto Disease / complications
  • Hashimoto Disease / pathology
  • Hashimoto Disease / therapy
  • Humans
  • Neurons / metabolism
  • Neurons / pathology
  • Paraneoplastic Syndromes, Nervous System / blood*
  • Paraneoplastic Syndromes, Nervous System / complications
  • Paraneoplastic Syndromes, Nervous System / pathology
  • Paraneoplastic Syndromes, Nervous System / therapy
  • Synapses / metabolism
  • Synapses / pathology


  • Antibodies

Supplementary concepts

  • Hashimoto's encephalitis