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)

Neurological disorders associated with a malignant neoplasm, which is not caused by a direct effect such as metastasis, infiltration or compression, is called carcinomatous neuromyopathy. Subacute cerebellar degeneration recognized in this category is characterized by acutely or subacutely progressive cerebellar ataxia and widespread loss of Purkinje cells. There have been several reports of subacute cerebellar degeneration in lung carcinoma, ovarian carcinoma and Hodgkin's disease, but rare in urogenital malignancies. We present a patient with neurological disorder considered subacute cerebellar degeneration associated with HCG-beta positive seminoma. A 29-year-old man noticed a left intrascrotal mass in the summer of 1984. The mass began to grow in April, 1985 and diplopia, gait disturbance and dysarthria appeared late in May. He consulted our hospital on July 20, 1985. Serum human chorionic gonadotropin (HCG)-beta was elevated to 200 ng/ml but alpha-fetoprotein and carcinoembryonic antigen were normal. Left high orchiectomy was performed and the tumor was diagnosed histologically as typical seminoma. Bulky metastatic tumor was recognized in retroperitoneum on abdominal CT but brain CT was normal. VAB VI chemotherapy was performed. The retroperitoneal metastatic tumor disappeared and HCG-beta was normalized and complete remission achieved, but cerebellar symptoms still remain 14 months after remission. This case is considered to be subacute cerebellar degeneration associated with seminoma and is the second case with testicular carcinoma reported.
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PMID:[Subacute cerebellar degeneration with HCG-beta positive seminoma of the testis]. 245 60

Autopsy findings for two patients with the Nijmegen breakage syndrome (NBS) are presented. This syndrome has the same type of immunologic and cytogenetic abnormalities as ataxia telangiectasia (AT). In NBS, however, microcephaly is found and progressive cerebellar ataxia and oculocutaneous telangiectasia are lacking. We demonstrate a clear neuropathologic difference between these two syndromes, as the diffuse cortical cerebellar degeneration characteristic of AT was absent in NBS. In the thymus the histologic picture was suggestive of simple dysplasia. Lymphoid tissues were slightly atrophic but otherwise structurally normal. In one of the two presented cases an extranodal diffuse large cell malignant non-Hodgkin lymphoma of B cell immunoblastic type was found in Waldeyer's ring, in the small and large intestines, and in the brain, whose sequelae had caused death. Six of the 19 patients known with certainty to have this syndrome have developed lymphoid malignancy, which indicates that these patients are prone to develop malignancies.
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PMID:Postmortem findings in the Nijmegen breakage syndrome. 780 77

Neuron-restricted autoantibodies are important markers of neurological autoimmunity related to cancer. We identified a new paraneoplastic IgG, PCA-2 (Purkinje cell cytoplasmic antibody type 2), in 10 patients. Nine had mixed subacute neurological presentations (5 brainstem or limbic encephalitis, 3 cerebellar ataxia, 2 Lambert-Eaton myasthenic syndrome, 1 autonomic neuropathy, and 1 motor neuropathy). All 9 were smokers, and 8 had definite or probable lung cancer (7 with biopsy-confirmed small cell lung carcinoma [SCLC]; 1 imaged only). One patient had no follow-up information. A 10th patient was among 58 with uncomplicated SCLC. PCA-2 binds to a cytoplasmic antigen in neurons and SCLC cells. Its immunostaining pattern in mouse tissues is distinct from that of the paraneoplastic autoantibodies PCA-1 (anti-Yo, marker of immune response initiated by ovarian or breast carcinoma) and PCA-Tr (anti-Tr, immune response marker of Hodgkin's lymphoma). PCA-2 binds to cerebellar Purkinje somata and dendrites, neurons in internal granular layer and dentate nucleus, and neuronal elements in gut and kidney. Western blots of reduced/denatured cerebellar and SCLC proteins reveal a common antigenic band, of approximately 280 kd. PCA-2 is the seventh IgG neuronal autoantibody marker of paraneoplastic autoimmunity identifiable unambiguously by standardized immunofluorescence criteria.
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PMID:New Purkinje cell antibody (PCA-2): marker of lung cancer-related neurological autoimmunity. 1071 48

An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. Hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and Horner syndrome.
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PMID:Paraneoplastic cerebellar degeneration and Horner syndrome: association of two uncommon findings in a child with Hodgkin disease. 1077 31

Patients with Hodgkin's disease can develop paraneoplastic cerebellar ataxia because of the generation of autoantibodies against mGluR1 (mGluR1-Abs). Yet, the pathophysiological mechanisms underlying their motor coordination deficits remain to be elucidated. Here, we show that application of IgG purified from the patients' serum to cerebellar slices of mice acutely reduces the basal activity of Purkinje cells, whereas application to the flocculus of mice in vivo evokes acute disturbances in the performance of their compensatory eye movements. In addition, the mGluR1-Abs block induction of long-term depression in cultured mouse Purkinje cells, whereas the cerebellar motor learning behavior of the patients is affected in that they show impaired adaptation of their saccadic eye movements. Finally, postmortem analysis of the cerebellum of a paraneoplastic cerebellar ataxia patient showed that the number of Purkinje cells was significantly reduced by approximately two thirds compared with three controls. We conclude that autoantibodies against mGluR1 can cause cerebellar motor coordination deficits caused by a combination of rapid effects on both acute and plastic responses of Purkinje cells and chronic degenerative effects.
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PMID:Mechanisms underlying cerebellar motor deficits due to mGluR1-autoantibodies. 1260

Paraneoplastic cerebellar degeneration (PCD) is a heterogeneous group of disorders characterized by subacute cerebellar ataxia, specific tumour types and (often) associated antineuronal antibodies. Nine specific antineuronal antibodies are associated with PCD. We examined the relative frequency of the antineuronal antibodies associated with PCD and compared the neurological symptoms and signs, associated tumours, disability and survival between groups of PCD with different antibodies. Also, we attempted to identify patient-, tumour- and treatment-related characteristics associated with functional outcome and survival. In a 12-year period, we examined >5000 samples for the presence of antineuronal antibodies. A total of 137 patients were identified with a paraneoplastic neurological syndrome and high titre (> or =400) antineuronal antibodies. Fifty (36%) of these patients had antibody-associated PCD, including 19 anti-Yo, 16 anti-Hu, seven anti-Tr, six anti-Ri and two anti-mGluR1. Because of the low number, the anti-mGluR1 patients were excluded from the statistical analysis. While 100% of patients with anti-Yo, anti-Tr and anti-mGluR1 antibodies suffered PCD, 86% of anti-Ri and only 18% of anti-Hu patients had PCD. All patients presented with subacute cerebellar ataxia progressive over weeks to months and stabilized within 6 months. The majority of patients in all antibody groups had both truncal and appendicular ataxia. The frequency of nystagmus and dysarthria was lower in anti-Ri patients (33 and 0%). Later in the course of the disease, involvement of non-cerebellar structures occurred most frequently in anti-Hu patients (94%). In 42 patients (84%), a tumour was detected. The most commonly associated tumours were gynaecological and breast cancer (anti-Yo and anti-Ri), lung cancer (anti-Hu) and Hodgkin's lymphoma (anti-Tr and anti-mGluR1). In one anti-Hu patient, a suspect lung lesion on CT scan disappeared while the PCD evolved. Seven patients improved by at least 1 point on the Rankin scale, while 16 remained stable and 27 deteriorated. All seven patients that improved received antitumour treatment for their underlying cancer, resulting in complete remission. The functional outcome was best in the anti-Ri patients, with three out of six improving neurologically and five were able to walk at the time of last follow-up or death. Only four out of 19 anti-Yo and four out of 16 anti-Hu patients remained ambulatory. Also, survival from time of diagnosis was significantly worse in the anti-Yo (median 13 months) and anti-Hu (median 7 months) patients compared with anti-Tr (median >113 months) and anti-Ri (median >69 months). Patients receiving antitumour treatment (with or without immunosuppressive therapy) lived significantly longer [hazard ratio (HR) 0.3; 95% confidence interval (CI) 0.1-0.6; P = 0.004]. Patients > or =60 years old lived somewhat shorter from time of diagnosis, although statistically not significant (HR 2.9; CI 1.0-8.5; P = 0.06).
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PMID:Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. 1276 61

A 53-year-old-male patient was admitted to the Hiroshima University Hospital in August 2001, with a history of progressive cerebellar ataxia, notable by standing and gait disturbances. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed no signs of ischemic damage. Two weeks later, left axillary lymphadenopathy developed. The pathological finding was Hodgkin's disease (HD; a classical malignant lymphoma in an early stage). Serum antibodies against Purkinje's cells (anti-Tr antibody) were detected by immunohistochemical study. Thus, we diagnosed this as paraneoplastic cerebellar degeneration (PCD) associated HD. We immediately carried out chemotherapy (ABVD therapy), radiotherapy with 20-30 Gy, steroid pulse and intravenous immunoglobulin (IVIg) without neurologic improvement. The patient was subsequently treated by double filtration plasmapheresis (DFPP), leading to a marked improvement of standing and gait disturbances and to a reduction of autoantibodies, which became undetectable. We conclude that DFPP might be useful in treating patients with cerebellar ataxia, and should be considered as a therapeutic choice for it.
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PMID:A case report of plasmapheresis in paraneoplastic cerebellar ataxia associated with anti-Tr antibody. 1655 43

We review the neuronal antibodies described in CNS disorders in order to clarify their diagnostic value, emphasize potentials pitfalls and limitations in the diagnosis of paraneoplastic neurological syndromes (PNS), and examine the current evidence for a possible pathogenic role. We propose to classify the neuronal antibodies associated with syndromes resulting from CNS neuronal dysfunction into two groups according to the location of the antigen: inside the neuron or in the cell membrane. Group I includes antibodies which target intracellular antigens and probably are not pathogenic. They are further subdivided into three groups. Group Ia comprises well-characterized onconeural antibodies (Hu (ANNA1), Yo (PCA1), Ri (ANNA2), CV2 (CRMP5), amphiphysin, Ma2) that are useful for the diagnosis of PNS. Group Ib antibodies (SOX and ZIC) are cancer-specific but there is no evidence that the immune response is in any way pathogenically related to the PNS. Antibodies in group Ic (glutamic acid decarboxylase (GAD), adenylate kinase 5 and Homer 3) identify non-PNS: stiff-person syndrome (SPS), cerebellar ataxia, and limbic encephalitis (LE). Group II antibodies recognize neuronal surface antigens. Antibodies in group IIa associate with characteristic CNS syndromes but their detection does not indicate that the disorder is paraneoplastic. Antibodies to potassium channels, AMPA and GABA(B) receptors are associated with LE, NMDA receptor antibodies identify a well-defined encephalitis, and antibodies against glycine receptors associate with SPS with encephalitis. A pathogenic role of the antibodies is suggested by the response of symptoms to immunotherapy and the correlation between antibody titers and neurological outcome. Lastly, Group IIb includes antibodies that are found in patients with paraneoplastic cerebellar ataxia associated with lung cancer (P/Q type calcium channels antibodies) or Hodgkin disease (metabotropic glutamate receptor type 1 antibodies).
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PMID:Antibodies and neuronal autoimmune disorders of the CNS. 2003 30

Paraneoplastic cerebellar degeneration (PCD) is a rare disorder presenting typically with acute or subacute severe cerebellar ataxia. PCD is most commonly associated with small cell lung cancer followed by adenocarcinoma of breast and ovary, and Hogdkin's lymphoma. We report a case of a 54-year-old male with acute-onset pancerebellar syndrome with underlying Hodgkin's lymphoma. A high index of suspicion of PCD resulted in arriving at an early diagnosis of underlying Hodgkin's disease. The patient was managed with six cycles of chemotherapy, which resulted in clinical stabilization and reversal of magnetic resonance imaging abnormalities. Antitumor therapy appears to have a significant impact on reversing PCD and hence early diagnosis and intervention for the primary remains the corner stone in stabilizing the neurological condition.
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PMID:Paraneoplastic cerebellar degeneration in Hodgkin's lymphoma. 2291 95

Cerebellar dysfunction is a classic paraneoplastic syndrome associated with various types of cancer, including gynecological and breast tumors, small-cell lung cancer, thymoma, and Hodgkin's lymphoma. We present a 22-year-old woman with acute cerebellar ataxia that subsided upon removal of an ovarian teratoma. This patient may represent a new category of immune-mediated cerebellar ataxia that is reversible with removal of an underlying tumor.
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PMID:Reversible cerebellar ataxia due to ovarian teratoma. 2472 36


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