ABSTRACT
BACKGROUND: Opsoclonus-myoclonus syndrome is a rare clinical condition that has been associated with neuroblastoma. There are few reported examples of ANNA-1/anti-Hu antibodies in children with neuroblastoma and opsoclonus-myoclonus, all in children aged less than three years of age. METHODS: We describe the new onset of focal seizures without alteration of consciousness and opsoclonus-myoclonus in an 11-year-old girl with ANNA-1/anti-Hu positivity and a paraspinal ganglioneuroblastoma. A systematic review of the literature of children with ANNA-1/anti-Hu positivity and malignancy was also performed. RESULTS: Fourteen patients were identified, eight of whom had opsoclonus-myoclonus. Although epilepsia partialis continua has been described in association with several neuronal autoantibodies, association with ANNA-1/anti-Hu has not been reported. CONCLUSIONS: We describe epilepsia partialis continua in a child with ANNA-1/anti-Hu antibodies and neuroblastoma. Testing for antineuronal antibodies should be considered in children presenting with either opsoclonus-myoclonus or epilepsia partialis continua.
Subject(s)
Antibodies, Neoplasm , Autoantibodies , ELAV Proteins , Epilepsia Partialis Continua/diagnostic imaging , Opsoclonus-Myoclonus Syndrome/diagnostic imaging , Spinal Neoplasms/diagnostic imaging , Antibodies, Neoplasm/blood , Antibodies, Neoplasm/cerebrospinal fluid , Autoantibodies/blood , Autoantibodies/cerebrospinal fluid , Child , ELAV Proteins/blood , ELAV Proteins/cerebrospinal fluid , Epilepsia Partialis Continua/blood , Epilepsia Partialis Continua/cerebrospinal fluid , Female , Humans , Opsoclonus-Myoclonus Syndrome/blood , Opsoclonus-Myoclonus Syndrome/cerebrospinal fluid , Spinal Neoplasms/blood , Spinal Neoplasms/cerebrospinal fluidSubject(s)
ELAV Proteins , Intestinal Pseudo-Obstruction/immunology , Limbic Encephalitis/immunology , Neuroblastoma/immunology , Opsoclonus-Myoclonus Syndrome/immunology , Autoantibodies/blood , Autoantibodies/cerebrospinal fluid , ELAV Proteins/blood , ELAV Proteins/cerebrospinal fluid , Female , Humans , Intestinal Pseudo-Obstruction/blood , Intestinal Pseudo-Obstruction/cerebrospinal fluid , Intestinal Pseudo-Obstruction/complications , Limbic Encephalitis/blood , Limbic Encephalitis/cerebrospinal fluid , Limbic Encephalitis/complications , Microtubule-Associated Proteins/blood , Mutation , N-Myc Proto-Oncogene Protein/genetics , Neuroblastoma/blood , Neuroblastoma/cerebrospinal fluid , Neuroblastoma/complications , Opsoclonus-Myoclonus Syndrome/blood , Opsoclonus-Myoclonus Syndrome/cerebrospinal fluid , Opsoclonus-Myoclonus Syndrome/complications , Young AdultSubject(s)
Brain Stem/immunology , Brain Stem/pathology , ELAV Proteins/blood , ELAV Proteins/cerebrospinal fluid , Ganglioneuroblastoma/immunology , Paraneoplastic Syndromes, Nervous System/diagnosis , Female , Ganglioneuroblastoma/diagnostic imaging , Humans , Paraneoplastic Syndromes, Nervous System/diagnostic imaging , Paraneoplastic Syndromes, Nervous System/pathology , Tomography, X-Ray Computed , Young AdultSubject(s)
Cerebrospinal Fluid/cytology , ELAV Proteins/cerebrospinal fluid , Paraneoplastic Syndromes, Nervous System/cerebrospinal fluid , Paraneoplastic Syndromes, Nervous System/immunology , T-Lymphocytes/immunology , Aged , Autoantibodies/analysis , Autoantibodies/cerebrospinal fluid , Biomarkers/analysis , Biomarkers/cerebrospinal fluid , ELAV Proteins/analysis , ELAV-Like Protein 4 , Female , Humans , Immunoassay/methods , Immunoglobulin G/analysis , Immunoglobulin G/cerebrospinal fluid , Immunophenotyping , Male , Middle Aged , Paraneoplastic Syndromes, Nervous System/physiopathology , Predictive Value of Tests , Reproducibility of ResultsABSTRACT
The PNS Euronet group criteria have classified paraneoplastic peripheral neuropathies as definite or possible according as to whether the neuropathy is a classical paraneoplastic disorder, the presence of onconeural antibodies, the delay between tumor and neuropathy, and improvement of the neuropathy with tumor treatment. Denny Brown's subacute sensory neuronopathy and neuropathies with anti-Hu or anti-CV2 antibodies are definite paraneoplastic disorders. Possible paraneoplastic neuropathies are heterogeneous. This classification is useful for the diagnostic of these disorders and the selection of patients in whom a search for cancer is warranted.
