Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Limbic encephalitis refers to an inflammatory process involving the hippocampi. amygdala and less frequently frontobasal and insular regions. This disorder used to be considered extremely rare, invariably associated with cancer, and unresponsive to treatment. However, recent studies suggest that limbic encephalitis is more frequent than it was previously thought, and a substantial number of patients may recover. This is due in part to the development of clinical diagnostic criteria and identification of antibodies directed against two broad categories of antigens: (1) intracellular or classical paraneoplastic antigens, including Hu, Ma2, and CV2 CRMP5, among others, and (2) cell surface antigens including, voltage-gated potassium channels (VGKC), N-methyl-D-aspartate receptor (NMDAR), and others expressed in the neuropil of the hippocampus. While the disorders related to the first category of antibodies associate with cancer (lung, testis and other), prominent brain infiltrates of T-cells, and limited response to treatment, the disorders related to the second category of antibodies associate with other tumors (thymoma. teratoma, Hodgkin's lymphoma), appear to be antibody-mediated, and respond better to immunotherapy. Of particular interest in the later group is the disorder that associates with antibodies to extracellular epitopes of NR1/NR2 heteromers of the NMDA receptor. Patients with this syndrome may present as limbic encephalitis but more frequently manifest severe psychiatric symptoms, seizures, dyskinesias, autonomic instability or hypoventilation. In all, the study of these disorders provides a link between immunologic processes and neuronal events involved in memory, cognition, seizures, and neuronal degeneration.
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PMID:[Limbic encephalitis and variants related to neuronal cell membrane autoantigens]. 1919 3

Limbic encephalitis is an inflammatory disease localized to the "grand lobe limbique" defined by Broca in 1878, sometimes restricted to the hippocampus, but sometimes including extralimbic abnormalities. The main features are subacute onset, short-term memory disorders and cognitive impairment, temporal seizures, and hippocampic changes on MRI. A list of underlying causes has recently been published Infectious causes used to be frequent (mainly herpes simplex virus). Paraneoplastic limbic encephalitis is characterized by the presence of various onconeural antibodies, such as AntiHu and ANNA3 (bronchial small cell carcinoma), AntiMa2 (testicular tumor), AntiCV2 (lymphoma, thymoma,...). No such antibodies are detected in 40% of patients. The prognosis of these forms is poor. Voltage-gated potassium channel-associated limbic encephalopathies are due to antibodies targeting potassium channels. Mutations of the genes encoding the Kv11 and Kv12 subunits are responsible for several Shaker syndromes, including neuromyotonia, Morvan's disease, type I episodic ataxia, and limbic encephalitis with hyponatremia. Plasma exchanges and immunotherapy are effective. In patients without detectable antibodies, hippocampic anti-neuropil antibodies should be sought, particularly those targeting N-methyl-D-aspartate receptors. Ovarian teratoma is the usual cause of this type of encephalitis. Surgery and immunotherapy are effective. These disorders have been categorized into those associated with antibodies targeting intracellular antigens (poor-prognosis paraneoplastic encephalitis) and those associated with antibodies targeting antigens reacting with cellular membranes (potassium channelopathies and antineuropil antibodies), which respond to immunotherapy and carry a better prognosis. Limbic encephalitis can also reveal Hodgkin's disease, as in a case observed by the authors.
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PMID:[Limbic encephalitis--evolving concepts]. 1944 70

Treatment-related malignancies are widely reported in the medical literature. They are usually chemotherapy-related leukemias or radiation-induced solid tumors. Here we present a case of a 47-year-old woman treated with mantle radiation for Hodgkin's lymphoma who later developed a thymoma in the setting of a venous thrombosis. A review of the literature including surveillance recommendations for secondary malignancies will be discussed.
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PMID:Thymoma developing 30 years after mantle radiation for Hodgkin's lymphoma. 1986 Feb 71

Tumors of the mediastinum represent a very heterogeneous group of primary and metastatic tumors. Most tumors of the mediastinum are detected on chest radiography, but for more precise view additional imaging methods are necessary. The most common causes of tumor mass in anterior mediastinum are thymoma, germ cell tumors, retrosternal goitre and Hodgkin's lymphoma. In the middle mediastinum most commonly tumors are congenital cysts, such as bronhogenic and pericardial cysts, and enlarged lymph nodes, while in the posterior mediastinum dominantly present neurogenic tumors. Complex anatomy of mediastinum infrequently makes difficulties in radiological diagnosis of these tumors. Computerized tomography (CT) is a radiological method of choice in the diagnosis of primary and/or secondary mediastinal lesions. Localization and structure of the tumor masses are very important for diagnosis. Knowledge of anatomy, with the standard topographic divisions on the anterior, middle and posterior mediastinum is necessary prerequisite for a valid CT diagnostic. Lesions in all parts of the mediastinum are systematically presented from the aspect of practical recommendations, with a view of the typical pathological findings.
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PMID:[Computerized tomography of mediastinal tumors]. 2042

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

Thymomas are rare tumors of the mediastinum; a limited number of small studies have evaluated the outcomes in these patients. We identified 668 patients with thymoma from the Swedish Cancer Registry, and 2,719 population-based matched controls. We obtained information on autoimmunity from the nationwide inpatient/outpatient hospital discharge Registry. We constructed Kaplan-Meier curves for survival analysis, conditional regression and Cox proportional hazards models to evaluate the association between thymoma and autoimmune diseases, and standardized incidence ratios (SIRs) to evaluate the risk for second cancers following thymoma. Compared with controls, patients with benign or malignant thymoma had a poorer (p < 0.001) 5-year (79%, 53% vs. 91%), 10-year (65%, 39% vs. 78%) and 20-year (43%, 18% vs. 55%) overall survival. For thymoma patients, younger age at diagnosis and being diagnosed in recent years were associated with a better survival. Compared with controls, thymoma patients were more likely to have an autoimmune disease at some point during their lives (32.7% vs. 2.4%, respectively, p < 0.001), most frequently myasthenia gravis (24.5%), systemic lupus erythematosus (2.4%) and red cell aplasia (1.2%). Thymoma patients had twofold excess risk for second cancers compared with the general population, most notably: non-melanoma skin cancer (SIR = 10.6, 95% confidence intervals (CI) = 6.0-17.3), non-Hodgkin lymphoma (SIR = 6.8, 95% CI = 3.00-13.0), and cervical (SIR = 6.9, 95% CI = 1.4-20.1), endocrine (SIR = 4.7, 95% CI = 1.3-12.0), and prostate cancer (SIR = 3.0, 95% CI = 1.7-4.8). Despite the improved survival for thymoma patients over time, they have worse survival than controls. Thymoma patients are in need for follow-up to detect and manage autoimmune diseases and cancer.
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PMID:A population-based assessment of mortality and morbidity patterns among patients with thymoma. 2066 26

The aim of the study was to verify the possibility of treating patients with poor prognosis early-intermediate Hodgkin's disease with a combined modality therapy consisting of three cycles of ABVD followed by extended field irradiation (EFRT). No patient had bulky mediastinum or had previously been administered chemo- or radiotherapy. At pathological restaging, 40/44 (91%) evaluable patients achieved complete responses (CR). After a ten-year followup, freedom from progression (FFP), relapse-free survival (RFS) and overall survival (OS) were 80%, 83% and 81%, respectively. Of the prognostic factors, univariate analysis showed that only stage III negatively influenced RFS, but not OS. Toxicity was mild except for subclinical mediastinal fibrosis in 32.5% of CR patients. No patient reported reduced fertility. Two cases of second neoplasms were recorded: one ameboid glioma and one thymoma, both occurring within five years after discontinuing chemo-radiotherapy. Our data suggest that three cycles of ABVD preceeding EFRT is an effective treatment for poor prognosis early-intermediate stage Hodgkin's disease; nevertheless, stage III patients and some stage II patients with unfavorable prognostic factors should be treated with a more aggressive approach.
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PMID:Three cycles of ABDV plus extended field radiotherapy in patients with poor prognosis early-intermediate stage Hodgkin's disease. 2154 11

Stiff-person syndrome (SPS) is an uncommon yet under-diagnosed, neuromuscular disorder characterised by progressive muscle rigidity with superimposed painful muscle spasms triggered by tactile, auditory or emotional stimulation. We describe the world's first case report of typical SPS in a patient with previously excised thymoma and treated non-Hodgkin lymphoma. This is of relevance because of the increasingly elucidated autoimmune aetiology of SPS.
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PMID:Lymphoma, thymoma and the wooden man: stiff-person syndrome post-thymoma excision and non-Hodgkin lymphoma remission. 2235 95

We report a rare case of thymic Hodgkin lymphoma that occurred in an elderly patient and detected by 18F-FDG PET/CT. A 77-year-old woman with fever of unknown origin underwent 18F-FDG PET/CT scanning, which showed an area of increased radiopharmaceutical uptake in a mediastinal mass corresponding to a thymic lesion at CT scan. On the basis of the PET/CT findings, the patient was referred to surgery with the clinical suspicious of a thymoma. Histological examination demonstrated the presence of a primary thymic Hodgkin lymphoma instead.
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PMID:A rare case of primary thymic Hodgkin lymphoma in an elderly patient detected by 18F-FDG PET/CT. 2337 12

Stiff person syndrome (SPS) is a rare condition that causes rigidity in the muscles of the body and extremities, difficulty in walking, episodic spasms and progressive disability. SPS is generally seen together with autoimmune disorders such as diabetes mellitus, thyroiditis, vitiligo and pernicious anaemia. Rarely, it may develop as a paraneoplastic condition. SPS cases associated with breast cancer, small cell lung carcinoma, thymoma, Hodgkin's lymphoma and colorectal cancer have been reported in the literature. We present a case of a 58-year-old female patient who had malignant mesothelioma-associated SPS. Patients who have muscle spasms and difficulty in movement of joints should be evaluated for SPS before diagnosis of Parkinson's or other neurological disorders, and possible underlying malignancies should be excluded.
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PMID:The horses are the first thought but one must not forget the zebras even if they are rare: Stiff person syndrome associated with malignant mesothelioma. 2471 75


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