Paraneoplastic syndrome is a rare neurologic disorder caused not by the direct invasion or metastasis of cancer, but by the remote effects of cancer. The central- and peripheral-nervous system or neuromuscular junction area were involved in this syndrome. The pathogenesis was thought as the autoimmune disease, the result of an immunologic response to cancer and to cross-react with self-cells of the nervous system or of the neuromuscular junction, causing neuronal dam-age. Specific forms of this syndrome are often associated with specific paraneoplastic autoantibodies and cancer. The onset of neurological symptoms and detection of these antibodies often precede the diagnosis of the cancer; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underly-ing cancer. Some paraneoplastic central neurological syndromes, such as cerebellar degeneration, limbic encephalitis, and necrotizing myelitis, were not improved by putative pathogenic autoantibodies, or by immunosuppresant or tumor removal, inspite of improvement in other peripheral neurologic syndrome, Lambert-Eaton myasthenic syndrome, neu-romyotonia, and Stiff-man syndrome. A more detailed understanding of the relationship between the cancer and the neural involvement from the molecular biological standpoint may lead to rational tumor therapy and elucidation of the mechanism of neural death. Here, major clinical forms with well-known antineuronal antibodies and specific cancers are reviewed.
Antibodies ; Autoantibodies ; Autoimmune Diseases ; Diagnosis ; Lambert-Eaton Myasthenic Syndrome ; Limbic Encephalitis ; Myelitis, Transverse ; Neoplasm Metastasis ; Nervous System ; Nervous System Diseases ; Neuromuscular Junction ; Neurons ; Paraneoplastic Syndromes* ; Small Cell Lung Carcinoma ; Stiff-Person Syndrome

No abstract available.
Limbic Encephalitis* ; Paraneoplastic Syndromes

BACKGROUND: Paraneoplastic neurological disorders associated with anti-Ri-antibodies, which are typically present with opsoclonus-myoclonus-ataxia. Most cases with anti-Ri-antibodyassociated paraneoplastic syndrome due to breast cancer occur in women - its occurrence in men is extremely rare. CASE REPORT: We present herein the case of a male patient with breast cancer who had atypical anti-Ri-antibody-associated paraneoplastic syndrome presenting as complete horizontal ophthalmoplegia, left trigeminal sensory symptoms, and truncal ataxia. Following the diagnosis of paraneoplastic syndrome, chemotherapy and immunomodulating treatment including intravenous immunoglobulin and oral prednisolone were administered. Although the patient was negative for serum anti-Ri-antibodies 14 weeks later, his symptoms persisted. CONCLUSIONS: To our knowledge, this is the first case report of ophthalmoplegia without opsoclonus-myoclonus in a male anti-Ri-antibody-positive patient with breast cancer.
Ataxia ; Breast ; Breast Neoplasms ; Female ; Humans ; Immunoglobulins ; Male ; Multiple Endocrine Neoplasia Type 1 ; Nervous System Diseases ; Ophthalmoplegia ; Paraneoplastic Syndromes ; Prednisolone

The Lambert-Eaton myasthenic syndrome (LEMS) heralds the occurrence of malignancy, especially small-cell lung cancer (SCLC), but it can also occur in the absence of cancer. Twelve patients were diagnosed as LEMS by clinical features and the classical electrophysiological triad, which includes a low amplitude of compound muscle action potentials (CMAP), decremental responses on low-rate stimulation, and incremental responses on high-rate stimulation on the repetitive nerve stimulation (RNS) test. There were 6 male and 6 female patients, ranging in age from 49 to 66 years. Malignancy(all were SCLC) was found in 7 patients. Males predominantly expressed the paraneoplastic form; whereas the primary autoimmune form was found only in women, who showed a good response to corticosteroid treatment. The neurological features were similar in both groups: proximal lower limb weakness, depressed muscle stretch reflexes, and dryness of mouth in nearly all patients. Bulbar dysfunction and limb paresthesia were a little more frequent in the paraneoplastic form. In RNS tests, the characteristic electrophysiological abnormalities were found in all patients and were more profound in the paraneoplastic form. We concluded that LEMS is commonly associated with malignancy, especially SCLC, but it should also be stressed that there are many female LEMS patients who do not harbor any malignancy at all, and that other treatment strategies such as immunotherapy should be considered for these patients.
Aged ; Carcinoma, Small Cell/complications ; Female ; Human ; Lambert-Eaton Myasthenic Syndrome/therapy ; Lambert-Eaton Myasthenic Syndrome/physiopathology* ; Lung Neoplasms/complications ; Male ; Middle Age ; Paraneoplastic Syndromes/physiopathology

Small cell lung cancer (SCLC), which originated from neuroendocrine tissue, can develop into paraneoplastic endocrine syndromes, such as Cushing syndrome, because of an inappropriate secretion of ectopic adrenocorticotropic hormone (ACTH). This paraneoplastic syndrome is known to be a poor prognostic factor in SCLC. The reason for poor survival may be because of a higher risk of infection associated with hypercortisolemia. Therefore, early detection and appropriate treatment for this syndrome is necessary. But the diagnosis is challenging and the source of ACTH production can be difficult to identify. We report a 69-year-old male patient who had severe hypokalemia, metabolic alkalosis, and hypertension as manifestations of an ACTH-secreting small cell carcinoma of the lung. He was treated with ketoconazole and spironolactone to control the ACTH dependent Cushing syndrome. He survived for 15 months after chemotherapy, which is unusual considering the poor outcome of the ectopic ATH syndrome associated with SCLC.
ACTH Syndrome, Ectopic ; Adrenocorticotropic Hormone* ; Aged ; Alkalosis ; Carcinoma, Small Cell ; Cushing Syndrome ; Diagnosis ; Drug Therapy ; Humans ; Hypertension ; Hypokalemia ; Ketoconazole ; Lung ; Male ; Paraneoplastic Endocrine Syndromes ; Paraneoplastic Syndromes ; Small Cell Lung Carcinoma* ; Spironolactone

BACKGROUND: Lambert-Eaton myasthenic syndrome is well known to be a classical paraneoplastic syndrome of small cell lung carcinoma (SCLC). Three cases of seronegative myasthenia gravis (MG) and SCLC were previously reported. CASE REPORT: A 65-year-old man developed a severe progressive respiratory failure with clinical features of MG. Tests showed a decremental response in the repetitive nerve stimulation test, abnormal single-fiber electromyography, and positive acetylcholine receptor antibody. SCLC was confirmed by the lung biopsy. CONCLUSIONS: This case represents the first case of seropositive MG and SCLC.
Acetylcholine ; Aged ; Electromyography ; Humans ; Lambert-Eaton Myasthenic Syndrome ; Lung ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Respiratory Insufficiency ; Small Cell Lung Carcinoma

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.
Brain ; Dizziness ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Paraneoplastic Syndromes, Nervous System ; Prevalence

Adult ; Female ; Humans ; Limbic Encephalitis ; etiology ; Ovarian Neoplasms ; complications ; Paraneoplastic Syndromes, Nervous System ; etiology ; Receptors, N-Methyl-D-Aspartate ; immunology ; Seizures ; etiology ; Teratoma ; complications

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) has various causes including central nervous system disorders, pulmonary and endocrine diseases, paraneoplastic syndromes, and use of certain drugs. SIADH induced by chemotherapy with irinotecan-cisplatin is not a common complication. Here, we review a case of SIADH after treatment with irinotecan-cisplatin. A 45-year-old woman received adjuvant chemotherapy (paclitaxel-carboplatin) for ovarian clear cell carcinoma, but the cancer recurred within 9 months of chemotherapy. Subsequently, a second line of combination chemotherapy containing irinotecan-cisplatin was initiated. However, 5 days after chemotherapy administration, her general condition began to deteriorate; her hematological tests revealed hyponatremia. Therefore, it is imperative to consider the possibility of SIADH in patients being treated with irinotecan-cisplatin–based chemotherapy. Proper monitoring of serum sodium levels and assessment of clinical symptoms should be performed in such patients for early diagnosis and prompt management.
Central Nervous System Diseases ; Chemotherapy, Adjuvant ; Cisplatin ; Drug Therapy ; Drug Therapy, Combination ; Early Diagnosis ; Endocrine System Diseases ; Female ; Hematologic Tests ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Middle Aged ; Paraneoplastic Syndromes ; Sodium

Sensory neuropathy is characterized by pure sensory dysfunction, ultimately evolving into areflexia and severe sensory ataxia in spite of the preservation of the other functions in the nervous system such as motor function. This disorder is known to be commonly found in cancer patients as a paraneoplastic syndrome, but nowadays many reports are made that sensory neuropathy is not merely related to malignancy but also with autoimmune disorders such as Sjogren's syndrome or polyclonal gammopathy, etc. Hence we report two patients who have been diagnosed as chronic idiopathic ataxic sensory neuropathy through electrophysiological and histopathological studies without any evidence of cancer or any other known cause.
Ataxia ; Humans ; Nervous System ; Paraneoplastic Syndromes ; Sjogren's Syndrome