Disease relevance of Paraneoplastic Syndromes, Nervous System

• Paraneoplastic limbic encephalitis (LE) is considered a particular manifestation of paraneoplastic encephalomyelitis (PEM), a remote effect of cancer almost always associated with anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinoma (SCLC) [1].
• Previous study has shown that the anti-Hu antibody titre of serum samples from patients with paraneoplastic encephalomyelitis/paraneoplastic sensory neuronopathy (PEM/PSN) was significantly higher than that from patients with small cell lung cancer without neurological disturbances (non-PEM/PSN) [2].
• A 65-year-old woman had the opsoclonus-myoclonus syndrome associated with carcinoma of the breast; her myoclonia and opsoclonus did not respond to intravenous diazepam or phenytoin [3].
• Conclusions: Paraneoplastic encephalomyelitis should be considered as a possible cause of coma and central alveolar hypoventilation [4].
• Most of the disorders studied were myoclonic disorders, such as progressive myoclonus epilepsy, the opsoclonus-myoclonus syndrome, and essential myoclonus, but other movement disorders, interictal seizure disorders, and different neurological and nonneurological disorders were also included [5].

High impact information on Paraneoplastic Syndromes, Nervous System

• A neuronal antigen (HuD) recognized by the sera of patients with antibody-associated paraneoplastic encephalomyelitis has been isolated by screening a lambda cerebellar expression library [6].
• HuD, a paraneoplastic encephalomyelitis antigen, contains RNA-binding domains and is homologous to Elav and Sex-lethal [6].
• Autoantigen diversity in the opsoclonus-myoclonus syndrome [7].
• High titers of HuAb were initially reported in serum from patients with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/SN) and SCLC [8].
• Cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid in the pediatric opsoclonus-myoclonus syndrome [9].

Chemical compound and disease context of Paraneoplastic Syndromes, Nervous System

• Sleep disturbance and rage attacks in opsoclonus-myoclonus syndrome: response to trazodone [10].
• Steroid-dependent anti-Hu negative paraneoplastic encephalomyelitis and small cell lung carcinoma [11].
• A 6-year-old female diagnosed with idiopathic opsoclonus-myoclonus syndrome at 22 months of age who failed to respond to treatment with adrenocorticotropic hormone (ACTH), IV gammaglobulin (IVIG), and azathioprine is presented [12].
• Opsoclonus-myoclonus syndrome: gabapentin as a new therapeutic proposal [13].
• Chlormethiazole in the management of the opsoclonus-myoclonus syndrome [3].

Anatomical context of Paraneoplastic Syndromes, Nervous System

• B- and T-cell markers in opsoclonus-myoclonus syndrome: immunophenotyping of CSF lymphocytes [14].

Gene context of Paraneoplastic Syndromes, Nervous System

• Since anti-Hu RRM domain antibodies are associated with an anti-tumor effect and paraneoplastic encephalomyelitis, we tested sera from Hu syndrome patients with the RRMs of DEF-3(g16/NY-LU-12) and LUCA15 [15].
• The expression of mRNA for the neuronal antigen HuD (Elavl4) associated with paraneoplastic encephalomyelitis and sensory neuronopathy was evaluated in the developing and adult rat nervous system [16].
• The serum of all patients with anti-Hu-associated paraneoplastic encephalomyelitis and sensory neuronopathy react with HuD and Hel-N1, but the reactivity with HuC is unknown [17].
• We studied the nervous systems and tumors of five patients with anti-Hu-positive paraneoplastic encephalomyelitis/sensory neuronopathy (PEM/PSN) to determine if the autoantibody found in the serum and CSF was also present in those tissues [18].
• Using immunohistochemistry, we studied the IgG subclass distribution of the anti-Hu antibody in serum, nervous system, and tumor of patients with anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuropathy (PEM/PSN) [19].

References