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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
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PMID:Paraneoplastic syndromes associated with anti-Hu antibodies. 1134 32

The anti-Hu antibody (HuAb) is directed against RNA-associated neuronal proteins and is known to cause paraneoplastic encephalomyelitis/sensory neuronopathy syndrome mostly when associated with small cell lung cancer (SCLC). Paraneoplastic encephalomyelitis/sensory neuronopathy syndrome with concurrent autonomic neuropathy has been reported to occur in paraneoplastic syndromes, although its occurrence concomitant with acute pandysautonomia is less frequent. The authors describe the clinical, neuropathologic, and serologic features of two cases with an anti-Hu-related paraneoplastic syndrome presenting with progressive autonomic neuropathy. Both patients showed features of dysautonomia, including postural dizziness, abdominal pain, and diarrhea, and symptoms of sensory neuropathy. Investigations disclosed severe sensory and autonomic neuropathy and positive HuAb titers. The disease of patient 1 had a very rapid progression, and the patient died of cardiac arrest within 2 months of the onset of symptoms. The autopsy revealed SCLC. In contrast, the disease of patient 2 had a less aggressive course. An extensive tumor search disclosed SCLC only 28 months after onset of symptoms, and the patient died 1 month later of cardiorespiratory arrest. Autopsies in both cases showed inflammation involving the intermediolateral columns and the dorsal root ganglia. These two cases illustrate the association of early dysautonomia with HuAb-related paraneoplastic syndrome and the variations of clinical, neuropathologic, and serologic findings in these types of cases.
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PMID:Phenotypic and neuropathologic heterogeneity of anti-Hu antibody-related paraneoplastic syndrome presenting with progressive dysautonomia: report of two cases. 1157 Jun

Paraneoplastic syndromes rarely affect patients with head and neck cancer. Four patients with different histological types of head and neck cancer are presented in which the primary malignancy was preceded and/or accompanied by a paraneoplastic syndrome. In the first patient erythrodermia preceded the diagnosis of a nasopharyngeal carcinoma. The second patient presented with a B cell lymphoma of the nasopharynx in association with the syndrome of inappropriate secretion of arginine vasopressine (Schwartz-Bartter syndrome). In the third patient paraneoplastic polyarthritis had been diagnosed 5 months before a hypopharyngeal carcinoma was diagnosed. In the last patient the paraneoplastic anti-Hu positive encephalomyelitis was associated with a primary malignancy in the larynx with neck metastases. Diagnostic procedures, treatment and follow-up of these patients are reported and accompanied by a review of the literature.
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PMID:Paraneoplastic syndromes in patients with primary malignancies of the head and neck. Four cases and a review of the literature. 1598 84

Anti-Hu encephalomyelitis is one of the most frequent paraneoplastic syndromes, classically presenting with diffuse neurological involvement. We report a 69-year-old man presenting with a three-month isolated, progressive gait disorder with normal neurological examination, except for loss of balance and gait failure reminding frontal disequilibrium, only accompanied by a very mild rigidity of his right foot. MRI of the brain showed hyperintensities in both amygdale and left putamen. EMG study showed no abnormal continuous spontaneous fiber activity. Because of fast progression and MRI findings, anti-Hu antibodies were tested, resulting positive. Mediastinal biopsy of two adenopathies detected by body-PET, confirmed an oat-cell carcinoma. The patient received oral steroids and oncological therapy. One year later, the tumor is in remission. His gait and abnormal posture of right leg are normal. Only mild residual hyperintensities persist on follow-up MRI. A paraneoplastic syndrome should be considered in the differential diagnosis of subacute, fast progressive gait disorders.
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PMID:Isolated frontal disequilibrium as presenting form of anti-Hu paraneoplastic encephalomyelitis. 1726 86

Paraneoplastic neurological syndromes (PNS) are infrequent disorders that are associated with cancer. The syndromes are highly heterogeneous and often affect several areas of the nervous system. Among the most well-known syndromes are paraneoplastic encephalomyelitis, cerebellar degeneration, sensory neuronopathy, and Lambert-Eaton myastenic syndrome. There are various associated tumors, in particular small cell lung cancer, cancer of the breast and ovary, and thymoma. The onset of neurological symptoms often precedes the cancer diagnosis, and the recognition of a paraneoplastic syndrome should lead to immediate search for cancer. The etiology of the paraneoplastic syndromes is believed to be autoimmune. Antibodies to onconeural antigens, expressed in the tumor of the affected individual and in normal neurons, are found in many of the patients. These antibodies are useful markers for paraneoplastic etiology. The pathogenesis of the PNS is uncertain, but cellular immune responses are thought to be the main effector mechanism. The cornerstone of therapy is the identification and treatment of the underlying malignancy. In some of the disorders, immunosuppressive therapy is of additional benefit. The prognosis of the different PNS varies depending on the level of affection and the degree of neuronal death.
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PMID:Paraneoplastic neurological syndromes and onconeural antibodies: clinical and immunological aspects. 1768 42

Paraneoplastic neurologic syndrome, a rare complication of carcinoma, includes various neurologic disorders, such as encephalomyelitis, paraneoplastic cerebellar degeneration, subacute sensory neuronopathy, retinal paraneoplastic syndrome, opsoclonus-myoclonus syndrome and stiff-person syndrome. Several antibodies to malignant tumor cells and neurons are detected in sera and cerebrospinal fluids of patients with this syndrome, however, there is no direct evidence of antiYo or antiHu antibodies' causative roles in neuronal loss. Recent studies showed cytotoxic T-cell activities against peptides of an antigen protein recognized by antibodies in the peripheral blood of patients with paraneoplastic cerebellar degeneration and antiYo antibodies, as well as in patients with antiHu syndrome. Treatment of paraneoplastic neurologic syndrome with plasmapheresis, immunosuppresive drugs, or intravenous immunoglobulin therapy has been attempted. Here, we discuss previous reports and theoretical treatments based on recent etiological hypothesis of paraneoplastic cerebellar degeneration.
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PMID:Pathogenesis and treatment of paraneoplastic neurologic syndrome. 1981 Sep 23

The diagnosis of anti-Hu-associated encephalomyelitis/sensory neuropathy may be particularly difficult when cranial nerve involvement represents the first clinical manifestation of the disease. We report a case of a patient who presented with facial pain as the first manifestation of an anti-Hu paraneoplastic syndrome, which needs a rapid detection and treatment of the underlying tumour. We suggest that paraneoplastic neuropathy should be considered during the management of trigeminal neuropathic pain, especially when brain imagery is normal.
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PMID:Facial pain as first manifestation of anti-Hu paraneoplastic syndrome. 2038 87

Limbic encephalitis is a paraneoplastic syndrome that is often associated with small cell lung cancer (SCLC), breast cancer, testicular tumors, teratoma, Hodgkin's lymphoma and thymoma. The common clinical manifestations of limbic encephalitis are subacute onset, cognitive dysfunction, seizures and psychiatric symptoms. Paraneoplastic neurological disorders are considered to occur because of cytotoxic T cell responses and antibodies against target neuronal proteins that are usually expressed by an underlying tumor. The main intracellular antigens related to limbic encephalitis are Hu, Ma2, and less frequently CV2/CRMP5 and amphiphysin. The anti-Hu antibody, which is involved in cerebellar degeneration and extensive or multifocal encephalomyelitis such as limbic encephalitis is closely associated with a history of smoking and SCLC. The anti-Ma2 antibody is associated with encephalitis of the limbic system, hypothalamus and brain-stem. For this reason, some patients with limbic encephalitis have sleep disorders (including REM sleep abnormalities), severe hypokinesis and gaze palsy in addition to limbic dysfunction. In men aged less than 50 years, anti-Ma2 antibody encephalitis is almost always associated with testicular germ-cell tumors that are occasionally difficult to detect. In older men and women, the most common tumors are non-SCLC and breast cancer. Limbic encephalitis associated with cell-surface antigens (e.g., voltage-gated potassium channels, NMDA receptors) is mediated by antibodies and often improves after a reduction in the antibody titer and after tumor resection. Patients with antibodies against intracellular antigens, except for those with anti-Ma2 antibodies and testicular tumors, are less responsive. Early diagnosis and treatment with immunotherapy, tumor resection or both are important for improving or stabilizing the condition of limbic encephalitis.
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PMID:[Limbic encephalitis with antibodies against intracellular antigens]. 2042 Jan 74

Encephalomyelitis occurs in paraneoplastic syndrome and acute disseminated encephalomyelitis through different autoimmune mechanisms. No postvaccinal encephalomyelitis other than acute disseminated encephalomyelitis has been reported in patients with malignancy. A 68-year-old woman was admitted because of a headache followed by a gait disturbance and psychomotor retardation 2 days after she had received an influenza vaccination followed by abulia, limb rigidity and hyperreflexia of both legs, and meningeal irritation. Cerebrospinal fluid studies showed increased intracranial pressure, elevated immunoglobulins G and A, and pleocytosis. Contrasted brain magnetic resonance imaging revealed ventriculomegaly and multiple symmetric leptomeningeal enhancement, without demyelinating changes or cortical ribbon signs. Somatosensory evoked potentials and nerve conduction velocity studies suggested myelitis. Encephalomyelitis was diagnosed on the basis of clinical and laboratory examinations. The etiological survey identified a lung adenocarcinoma. Both the encephalomyelitis and the lung adenocarcinoma simultaneously progressed after the vaccination and then, after targeted therapy for lung cancer, simultaneously subsided. In conclusion, postinfluenza-vaccination paraneoplastic encephalomyelitis may occur in patients with lung adenocarcinoma.
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PMID:Reversible postvaccination paraneoplastic encephalomyelitis in a patient with lung adenocarcinoma. 2096 57

This case report describes a patient with a locally advanced oropharyngeal cancer with a simultaneous paraneoplastic encephalomyelitis. To the best of our knowledge, a paraneoplastic neurological syndrome is a rare complication in head and neck cancer, and has previously not been reported in the literature. One year later, following initial treatment, a small cell lung cancer developed, a tumor frequently associated with this type of paraneoplastic syndrome. The dilemma, therefore, is whether this paraneoplastic symdrome was a secondary complication of the tonsilar concurrent cancer or a metachronous paraneoplastic syndrome prior to small cell lung cancer.
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PMID:Paraneoplastic encephalomyelitis: Is it an oropharyngeal or a lung cancer complication? 2287 Jan 48


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