anti-Hu Paraneoplastic Syndrome Research Articles

Neuroimmunology of OMS and ANNA-1/anti-Hu paraneoplastic syndromes in a child with neuroblastoma.

Paraneoplastic neurologic disorders have been at the frontier of neuroimmunology for decades and remain urgent. Newer tools for the diagnosis and treatment of neuroinflammation include autoantibody screening, lymphocyte subset analysis, oligoclonal bands, and profiling of chemokines/cytokines and brain-related proteins in CSF and blood/serum. Opsoclonus-myoclonus syndrome (OMS), a paraneoplastic disorder without a known diagnostic antibody marker, is associated with remote neuroblastoma and is often relapsing.1 Paraneoplastic encephalomyelitis with type 1 antineuronal nuclear antibodies (ANNA-1) (alias anti-Hu)2 also may occur in children with neuroblastoma3–5 and exhibits intrathecal B- and T-cell inflammation in adults.6 We now provide a detailed neuroimmunologic profile of a child with both relapsing OMS and ANNA-1 paraneoplastic syndromes and neuroblastoma. Acknowledgment: Dr. Pranzatelli is a clinician-scientist; President and Founder of the National Pediatric Neuroinflammation Organization, Inc.; and an Adjoint Professor of Neurology at the University of Colorado School of Medicine. The National Pediatric Myoclonus Center relocated from Springfield, IL (SIU School of Medicine) to Orlando, FL, in 2014 to become the current independent, nonprofit organization. The authors thank Elizbeth D. Tate, MN, ARNP, C-FNP, who participated in clinical care, the patient, her family, and referring and treating physicians.

A Rare Case of Primary Mediastinal Seminoma (PMS) Associated with Anti-Hu Paraneoplastic Syndrome (PS) (P4.238)

A Rare Case of Primary Mediastinal Seminoma (PMS) Associated with Anti-Hu Paraneoplastic Syndrome (PS) (P4.238)

Ondine's Curse: A Case of Anti-Hu Paraneoplastic Syndrome

A 60-year-old patient presented with neurological symptoms which proved to be a paraneoplastic syndrome secondary to a small-cell lung cancer. The investigations and treatment for these syndromes are discussed. The patient improved with high dose steroids and chemotherapy.

Long-lasting remission after rituximab treatment in a case of anti-Hu-associated sensory neuronopathy and gastric pseudoobstruction

Anti-Hu paraneoplastic syndromes are usually associated with an underlying neoplasia, although a few patients who are anti-Hu-positive never develop cancer after long-term follow-up. Tumour therapy remains the mainstay of therapeutic options, and early immune therapy in parallel is advisable. When no tumour is found, immunologically-based therapies are nowadays the only options. Recent studies have shown rituximab associated with the tumour therapy to be effective for some patients with anti-Hu paraneoplastic syndrome. We report a case of a patient with anti-Hu antibodies-associated sensory neuronopathy and gastric pseudo-obstruction without an underlying neoplasia four years after the first manifestation who has achieved sustained improvement for two years after treatment with rituximab. This case report supports the effectiveness of rituximab in these syndromes, even for cases where no underlying neoplasia is demonstrated. Further studies are warranted in order to confirm these preliminary data.

Lung Carcinoma with Anti-Hu Paraneoplastic Syndrome

This 54-year-old smoker presented with weight loss and a burning sensation in her hands and feet. Her pupils were asymmetric and unresponsive to light.

Complex partial status epilepticus associated with anti-Hu paraneoplastic syndrome

Although seizures can be a manifestation of paraneoplastic disorders, there are few descriptions of the association between the anti-Hu paraneoplastic syndrome and epilepsia partialis continua. A new case of refractory complex partial status epilepticus in a patient with a paraneoplastic syndrome associated with a poorly differentiated mediastinal tumor that expressed Hu antigen is described clinically, pathologically, and electrographically. We discuss the presentation of focal seizures in a disease that is characterized by diffuse pathologic involvement of the brain. The progression of EEG, MRI, and clinical findings during the course of the illness is also discussed. To our knowledge, this is the first description of paraneoplastic epilepsia partialis continua associated with diffuse pathologic abnormalities.

Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Bruno, Michiko Kimura MD; Winterkorn, Jacqueline M.S. PhD, MD; Edgar, Mark A. MD; Kamal, Ayeesha MD; Stübgen, Patrick J. MD Author Information

Prevalence and Meaning of Anti-Hu Antibodies in Small Cell Lung Cancer

Neurologists are familiar with the anti-Hu paraneoplastic syndrome as a combination of limbic encephalopathy and sensory neuropathy, usually in

Association of small cell lung cancer and the anti-Hu paraneoplastic syndrome: radiographic and CT findings.

The anti-Hu syndrome (bearing the name of the patient in whom the antibody was first discovered) is characterized by a paraneoplastic encephalomyelitis or sensory neuronopathy and the presence of a specific antibody in the serum or CSF and is associated with lung cancer in most patients who have the syndrome. The objective of this study was to determine the relative merits of chest radiography and CT in the imaging of patients with the anti-Hu syndrome. Chest radiographic and CT findings of 11 patients with anti-Hu syndrome were reviewed and correlated with results of bronchoscopy, surgery, or autopsy. Ten of the 11 patients had small cell lung cancer; in the remaining patient, no cancer was found. Small cell lung cancer was first revealed on radiographs in one patient and solely on CT in the remaining nine patients with small cell lung cancer. In six of these nine patients, the initial CT findings were positive for lung cancer. Tumor was found on follow-up CT in 4-8 months when initial CT findings were negative. Mediastinal adenopathy was present in all 10 cancer patients. Hilar adenopathy was present in four. Parenchymal involvement was seen in three of the 10 patients with cancer. In two patients, the only CT finding of small cell lung cancer was one mediastinal lymph node of 10 mm in each patient. Chest CT should be recommended for patients with anti-Hu syndrome, even when chest radiographic findings are interpreted as normal.