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Paraneoplastic neurological syndromes.

Francesc Graus¹, Josep Dalmau

¹Service of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d'Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain. fgraus@clinic.ub.es

Current Opinion in Neurology

|October 9, 2012

Summary

Advances in paraneoplastic neurological syndromes (PNS) highlight specific syndromes and the role of surface antigen antibodies. These antibodies aid in management and predict immunotherapy response, expanding diagnostic capabilities for complex neurological cases.

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Area of Science:

Neurology
Immunology
Oncology

Background:

Paraneoplastic neurological syndromes (PNS) can present with subtle symptoms, sometimes obscuring their paraneoplastic origin.
Specific syndromes like anti-N-methyl-D-aspartate receptor encephalitis, often linked to ovarian teratomas in women, exemplify this diagnostic challenge.

Purpose of the Study:

To review recent advancements in paraneoplastic neurological syndromes (PNS).
To focus on specific syndromes and the impact of antibodies targeting surface antigens in their clinical management.

Main Methods:

Literature review of recent advances in PNS.
Analysis of antibody targets and their association with specific neurological syndromes.
Correlation of antibody presence with clinical presentation and treatment response.

Main Results:

Anti-N-methyl-D-aspartate receptor encephalitis, associated with ovarian teratoma, can exhibit an 'extreme delta brush' EEG pattern.
Myelopathy, brainstem encephalitis (Ri antibodies), limbic encephalitis (GABA(B) receptor antibodies), and Morvan's syndrome (CASPR2 antibodies) are linked to specific autoantibodies.
Tr antibodies, targeting DNER, are associated with cerebellar ataxia and Hodgkin's lymphoma.

Conclusions:

The repertoire of antibodies relevant to PNS has expanded to include those against surface antigens.
While not confirming a paraneoplastic origin, these antibodies indicate a favorable prognosis with immunotherapy.