Background: Paraneoplastic neurological syndromes (PNS) are most commonly recognized in adults with cancer and can often be identified by the presence of serum antibodies to neuronal proteins that are also expressed by the associated tumor. In the pediatric age little emphasis is given to the possibility that neurological involvement is paraneoplastic and there have been few studies investigating the presence of anti-neuronal antibodies. Objective: To run a pilot study on the spectrum of PNS and presence of antibodies to neural antigens in children with malignancies. Methods: 23 children with hematological or other cancers were examined for neurological manifestations and for antibodies to the neuronal antigens that are frequently detected in adult patients with PNS. Results: Nine of the children had neurological symptoms, but in five these could be explained by tumor invasion or chemo toxicity. Antibodies to a Tr-like cerebellar antigen, to voltage-gated potassium channels and to glutamic acid decarboxylase were found in one child each. Results were compared with age- and sex-matched control groups including: (a) 30 sera from healthy children; (b) 25 sera from neurologically non-oncological children; and (c) 20 sera positive for each of the antibodies. Conclusion: These results suggest that PNS, though surprisingly not so uncommon in children, may be associated with immune responses to distinct neuronal antigens. Further work is needed to determine whether screening for new antibodies to neuronal antigens could be a useful aid in the diagnosis and prognosis of neurological syndromes in children.

A pilot study on neurological manifestations and antibodies against neuronal antigens in children with haematological and other cancers

POLIZZI A;RUGGIERI, MARTINO;PULVIRENTI, ALFREDO;
2013-01-01

Abstract

Background: Paraneoplastic neurological syndromes (PNS) are most commonly recognized in adults with cancer and can often be identified by the presence of serum antibodies to neuronal proteins that are also expressed by the associated tumor. In the pediatric age little emphasis is given to the possibility that neurological involvement is paraneoplastic and there have been few studies investigating the presence of anti-neuronal antibodies. Objective: To run a pilot study on the spectrum of PNS and presence of antibodies to neural antigens in children with malignancies. Methods: 23 children with hematological or other cancers were examined for neurological manifestations and for antibodies to the neuronal antigens that are frequently detected in adult patients with PNS. Results: Nine of the children had neurological symptoms, but in five these could be explained by tumor invasion or chemo toxicity. Antibodies to a Tr-like cerebellar antigen, to voltage-gated potassium channels and to glutamic acid decarboxylase were found in one child each. Results were compared with age- and sex-matched control groups including: (a) 30 sera from healthy children; (b) 25 sera from neurologically non-oncological children; and (c) 20 sera positive for each of the antibodies. Conclusion: These results suggest that PNS, though surprisingly not so uncommon in children, may be associated with immune responses to distinct neuronal antigens. Further work is needed to determine whether screening for new antibodies to neuronal antigens could be a useful aid in the diagnosis and prognosis of neurological syndromes in children.
2013
nervous system; immune-mediated; antibodies
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11769/20796
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