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

A case of an encephalomyelitis in a child with acute lymphoblastic leukemia is reported. The patient was a 5-year-old boy who developed seizures, progressive confusion, and coma after radiation and intrathecal methotrexate therapy. Computed tomography (CT) of the brain showed bilateral hypodensities in the posterior parietal and temporal regions. At autopsy, perivascular inflammation, microglial nodules without intranuclear viral inclusions, and bilateral necrosis of the temporoparietal and hippocampal regions were seen in the brain and spinal cord. Paraneoplastic encephalomyelitis is generally recognized in adult patients with underlying malignancy but, to our knowledge, has not been reported in children with leukemia. This report should alert the clinicians to an entity that must be included in the differential diagnosis of leukemic children with progressive neurologic disorder.
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PMID:Encephalomyelitis associated with leukemia in a child. 347 99

In the last decade, anti-neurons autoantibodies have been found in serum and cerebro spinal fluid of patients suffering from neurological paraneoplastic syndrome. This discovery has made possible to improve the knowledge of these syndromes as well as to characterize some proteins specific to the nervous system, unknown until now. Paraneoplastic encephalomyelitis can manifest together with an ataxic sensitive neuropathy, a limbic encephalitis, a brainstem or cerebellum syndrome. This encephalomyelitis is almost always associated with a small cells lung cancer and auto-antibodies, called anti-Hu, which recognize all the neurons of the nervous system. Patients with paraneoplastic cerebellar degeneration present an antibody, called anti-Yo, directed against Purkinje cells. In this case, the tumor is a gynecologic cancer. Patients presenting with an opso-myoclonus and a breast cancer have an antineurons anti-body, called anti-Ri, which is absent when the opso-myoclonus is associated with a lung cancer or a neuroblastoma. These three antibodies are the most frequently found and the best studied, but others, rarer, have been described. The genes coding for the proteins recognized by these three antibodies have been cloned. These proteins seem to play a major role in the neuronal maturation and homeostasis. These antibodies prove to be irreplaceable tools to study the phenomenons subtending the neuronal degeneration and the cellular proliferation.
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PMID:[Value of the detection of anti-nervous system autoantibodies in neurologic paraneoplastic syndromes]. 805 19

Paraneoplastic encephalomyelitis developed as the presenting feature of small-cell lung carcinoma in 3 patients. Two patients with paraneoplastic encephalomyelitis manifested predominantly as subacute sensory neuronopathy did not improve after prednisone treatment and chemotherapy. The third patient had severe axial and limb rigidity and myoclonus, which partially improved after chemotherapy and treatment with intravenous immunoglobulin and prednisone. Serum from each patient immunocytochemically stained the neuropil and to a lesser degree the neuronal cytoplasm in human cerebral and cerebellar cortex. On immunoblots of human neuronal extracts, each patient's serum contained high-titer IgG antibodies reacting with a protein band of apparent molecular mass 125 kd. This autoantibody pattern is indistinguishable from antibodies recently identified in several women with breast carcinoma and stiff-man syndrome. Screening of a human brain complementary DNA expression library with patient serum yielded clones whose sequence is identical to that of the synaptic vesicle-related protein amphiphysin. Reverse transcriptase-polymerase chain reaction demonstrated expression of amphiphysin in 8 of 10 small-cell lung carcinomas and in 5 of 14 breast carcinomas. These observations highlight the clinical and serological heterogeneity of paraneoplastic central nervous system disorders: Patients with a given clinical syndrome may have different antineuronal antibodies, and patients with a given autoantibody specificity have differing clinical presentations.
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PMID:Antiamphiphysin antibodies with small-cell lung carcinoma and paraneoplastic encephalomyelitis. 861 52

We report the case of a 62-year-old man affected by anti-Hu-associated paraneoplastic encephalomyelitis. The underlying tumor was a neuroendocrine cancer of the rectum expressing Hu antigen. The neurologic presentation was limited to moderate sensitive neuropathy associated with two complex partial seizures (dreamy state) without any further signs of limbic encephalopathy. A paraneoplastic etiology should be considered in patients with moderate symptomatology. Paraneoplastic encephalomyelitis with anti-Hu antibodies is not always associated with small-cell lung cancer.
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PMID:[Paraneoplastic encephalomyeloneuritis with anti-Hu antibodies and cancer of the rectum]. 929 26

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