Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A paraneoplastic syndrome associated with anti-N-methyl-D-asparate (NMDA) receptors can initially present as a neurologic or psychiatric disturbance. Removal of the tumor is usually curative, and the syndrome is associated with the presence, rather than the history, of tumor. We present a case in which a 25-year-old, Hispanic woman presented with seizures, memory loss, and unusual behavioral changes. The woman had a teratoma removed 2 months earlier. Because of the time course, a paraneoplastic syndrome was initially considered unlikely. Brain imaging, electroencephalography (EEG) and neurologic work-up were negative. The patient was treated for a suspected somatoform disorder and psychosis. Based on the clinical picture, the working diagnosis was changed to delirium due to paraneoplastic limbic encephalitis. A course of intravenous immunoglobins (IVIg), and high dose steroids was administered. The patient's symptoms improved, and she was discharged home. After discharge, studies came back positive for antibodies against NR1/NR2 of the NMDA receptor.
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PMID:Limbic encephalitis presenting with seizures, anterograde amnesia, and psychosis in a patient seven weeks status post immature ovarian teratoma removal. 2073 Dec 68

Anti-N-methyl-D-aspartate receptor encephalitis is a rare and serious autoimmune disorder, usually part of a paraneoplastic syndrome. Patients present with a change in mental status, bizarre behavior, and seizures. There are many neoplastic and infectious etiologies, with 90 percent of documented cases in young women, frequently with an ovarian teratoma. Damage to limbic brain receptors is most often induced by teratoma cell-produced anti-N-methyl-D-aspartate antibodies. Treatment targets the specific oncologic or infectious cause and/or employs steroids, plasmapheresis, or other anti-inflammatory therapies. Neurological sequellae persist 75 percent of the time, relapse occurs in 20 percent of cases, and the mortality rate is 25 percent.
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PMID:Anti-N-methyl-D encephalitis. 2201 62

Encephalitis associated with autoantibodies directed against the N-methyl-D-aspartate receptor (NMDAR) is usually a paraneoplastic syndrome that presents in young females with ovarian teratomas. We report a case of a previously healthy 14-year-old girl with sudden-onset paranoia, hallucinations, hyperactivity, increased speech, decreased sleep, seizures, and violent behavior deteriorating to catatonia. Her cerebrospinal fluid tested positive for anti-NMDAR antibodies. She was treated with five sessions of therapeutic plasma exchange (TPE) after having failed therapy with antibiotics, intravenous steroids, intravenous immunoglobulin (IVIG), one dose of rituximab, and seven sessions of electroconvulsive therapy (ECT). The American Society for Apheresis assigns a Category III (Grade 2C) recommendation for TPE in paraneoplastic neurologic syndromes; however, apheresis specifically for anti-NMDAR encephalitis has not been well studied. Literature review revealed two case reports describing outstanding improvement in patients with anti-NMDAR encephalitis following TPE. We report no improvement in our patient's symptoms after plasma exchange and discuss possible reasons for why it failed along with review of the literature.
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PMID:Adjunct therapeutic plasma exchange for anti-N-methyl-D-aspartate receptor antibody encephalitis: a case report and review of literature. 2241 18

Anti N-methyl-D-aspartate receptor encephalitis (ANMDARE), also known as limbic encephalitis (LE), is a treatable rare disorder characterized by personality changes, irritability, depression, seizures, memory loss and sometimes dementia. It is classified under paraneoplastic syndrome (PNS) and produces antibodies against NR1 and NR2 subunits of glutamate aspartate receptor. It is thought to be closely related with malignancies like small cell lung cancer, ovarian teratoma and Hodgkin's lymphoma, apart from testis, breast and rarely gastric malignancies. Non-paraneoplastic encephalitis cases are the ones with no detectable malignancy and may be triggered by severe infection. As nuclear medicine physicians, we must be aware of the diverse presentation of ANMDARE or LE and should include a whole body positron emission tomography / computed tomography (PET/CT) and not just brain PETCT during imaging. We describe the first case of PET/CT in an idiopathic ANMDARE Indian adolescent girl.
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PMID:PET/CT in the evaluation of anti-NMDA-receptor encephalitis: What we need to know as a NM physician. 2217 17

Limbic encephalitis is a syndrome characterised by irritability, depression, sleeping disturbance, convulsion, hallucination and short-period memory loss that is commonly associated with a malignancy even if there is no evidence of it by the time of presentation. Most reported cases of limbic encephalitis as a paraneoplastic syndrome are associated with small-cell lung cancer and lymphoma. This article is a case report of a patient with limbic encephalitis associated with an oesophageal adenocarcinoma. The patient is a middle-aged man who presented apathy and unstable mood. After months, developed diplopia, reduced visual acuity and involuntary movements. Later, gait disability, disorientation, memory loss and aggressive behaviour were detected, associated with seizures. After investigation, limbic encephalitis was diagnosed and, as the patient developed dysphagia, oesophageal adenocarcinoma was detected. Oesophageal carcinoma usually does not have neurological symptoms associated.
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PMID:Limbic encephalitis as the presenting symptom of oesophageal adenocarcinoma: another cancer to search? 2359 72

Patients with limbic encephalitis usually present with rapidly progressive short-term memory deficits, psychiatric symptoms, and seizures. The recent concept of limbic encephalitis has been expanded. Especially, various types of autoimmune limbic encephalitis are associated with autoantibodies of intracellular or cell membrane antigens. Sine autoimmune limbic encephalitis is also associated with some types of tumors, it has also an aspect of paraneoplastic syndrome. Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a new category of treatment-responsive limbic encephalitis associated with anti-NMDAR antibodies, which is the most frequent autoantibody to cell membrane antigen. The autoantibodies are detected in the CSF and serum of young women with ovarian teratoma, who typically develop schizophrenia-like psychiatric symptoms. There is a highly characteristics syndrome evolving in 5 stages; the prodromal, psychotic, unresponsive, hyperkinetic, and gradual recovery phases. The hyperkinetic phase is the most prolonged and crucial. This disorder is usually severe and can be fatal, but it is potentially reversible. Although the pathogenesis remains unclear, this disorder is considered to be the autoantibody-mediated encephalitis. This review focuses in the recent concept of limbic encephalitis and clinical characteristics of anti-NMDA receptor encephalitis.
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PMID:[Recent concept of limbic encephalitis: progress in anti-NMDA receptor encephalitis]. 2362 28

Limbic encephalitis is an inflammatory disease of the central nervous system characterized by diverse neurologic symptoms including mnestic disturbances, hallucinations, and seizures as well as behavioral symptoms like depression, personality changes, and acute confusional states resembling dementia. Several antibodies have been described in the pathogenesis of limbic encephalitis. It is often a paraneoplastic syndrome associated with small cell lung cancer, breast cancer, or Hodgkin's lymphoma among others. Here, we report a patient with B-cell chronic lymphocytic leukemia (B-CLL), presenting with otherwise unexplained neurologic symptoms consistent with limbic encephalitis. Despite intensive diagnostic procedures, no causing agent could be identified. Pleocytosis consisting of T cells was detected in the cerebrospinal fluid (CSF). We initiated anti-B-cell therapy with Rituximab for B-CLL with quick and durable resolution of symptoms. We speculate that disruption of interaction between autoreactive T and malignant B cells is responsible for the therapeutic effect of Rituximab.
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PMID:Improvement of paraneoplastic limbic encephalitis after systemic treatment with rituximab in a patient with B-cell chronic lymphocytic leukemia. 2398 26

An 8-year-old Thoroughbred mare presented for decreased appetite, ataxia, and weakness. Abdominal ultrasound revealed a large volume of anechoic fluid along with multiple masses involving the spleen, liver, and diaphragm. Pleural fluid was identified via ultrasonography and thoracic radiography. Thoracic radiographs also identified pulmonary interstitial nodules, an undulant dorsal diaphragmatic margin and enlargement of tracheobronchial lymph nodes. Clinical signs of weakness and mild seizures were concurrent with hypoglycemic episodes. The final diagnosis was cholangiocarcinoma with extensive metastasis. Clinical signs of weakness, ataxia, and seizures were attributed to a paraneoplastic syndrome of tumor-associated hypoglycemia that has been infrequently reported in horses.
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PMID:Imaging diagnosis-hypoglycemia associated with cholangiocarcinoma and peritoneal carcinomatosis in a horse. 2410 84

Introduction. Paraneoplastic limbic encephalitis (PLE) is a rare disorder that typically follows a chronic or subacute course of personality changes, memory loss, seizures, and hallucinations. Early diagnosis is difficult and characteristic symptoms can be mimicked by a variety of conditions. We present a case of PLE, initially presenting as acute herpetic encephalitis. Case Presentation. A 56-year-old male was admitted for evaluation of acute onset headache, fever, and confusion. On neurological examination he was confused with MMSE score of 15/30. CSF analysis revealed marked lymphocytic pleocytosis. A possible diagnosis of acute herpetic encephalitis was rendered and patient was treated with acyclovir. CSF PCR was negative. Cranial MRI revealed bilateral hyperintense lesions in medial temporal lobes with contrast enhancement. Despite treatment with acyclovir patient was deteriorated; thus, a paraneoplastic syndrome was suspected. Chest CT showed a right paratracheal lymph node mass, while a biopsy revealed neuroendocrine lung cancer. Auto antibodies to Hu were also detected. The patient was treated with steroids and chemotherapy. Six months later, he had complete tumour remission and marked neurological improvement. Discussion. PLE can rarely invade acutely, being indistinguishable from herpetic encephalitis. Inclusion of PLE in the differential diagnosis of acute encephalitis is of great clinical significance.
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PMID:Paraneoplastic limbic encephalitis resembling acute herpetic encephalitis. 2437 54

Herein is presented an interesting case of small-cell neuroendocrine carcinoma of the cervix which initially manifests as seizures due to hyponatremia caused by paraneoplastic syndrome of inappropriate anti diuretic hormone (SIADH). Awareness of a paraneoplastic syndrome at presentation can lead to early diagnosis and early initiation of treatment. The management is also unique in that it combines treating the paraneoplastic aspects as well as targeting the tumour itself. Multimodality treatment gives the best outcome in this aggressive tumour.
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PMID:Neuroendocrine carcinoma of the cervix presenting as intractable hyponatremic seizures due to paraneoplastic SIADH-a rare case report and brief review of the literature. 2511 22


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