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)

We report the case of a 23-year-old female with severe neurologic dysfunction without a clear cause at the time of initial presentation. The search for an underlying malignancy revealed a slightly enlarged cervical lymph node with Hodgkin's disease (HD). There was no evidence of a brain tumor despite nonspecific bright changes in proton density in the basal ganglia of the right hemisphere of the cerebellum, right cerebellar tonsil, posterior limb of the internal capsule, and the right side of the medulla spinae as shown by magnetic resonance imaging (MRI) as well as reactive lymphocytosis with slightly elevated protein levels in the cerebrospinal fluid (CSF). The findings suggested a cerebellar disorder, with main differential diagnosis between neurologic paraneoplastic syndrome (NPS) and HD involving the CNS. Based on limited experience with NPS and HD in the CNS, possible diagnostic and therapeutic options are discussed.
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PMID:Diagnostic and therapeutic quandaries in primary manifestation of Hodgkin's disease in the central nervous system. 1202 40

The presence of antineuronal anti-Tr antibodies is associated to paraneoplastic cerebellar degeneration due to Hodgkin's disease. The anti-Tr can become negative after successful and early treatment of the tumor, and there could even be remission of the cerebellar symptoms in some patients. There are few cases in which no tumor is found when there are anti-Tr. We report the case of a 66 year old man with a severe cerebellar syndrome and anti-Tr in serum detected by immunohistochemistry. After a 4 year follow-up, no underlying tumor has been found. In addition, anti-Tr spontaneously disappeared. The cerebellar degeneration persists and is incapacitating. This case suggests that in a few instances the origin of anti-Tr is not a tumor but another unknown cause. Alternatively the anti-Tr mediated immune response could have eradicated the underlying lymphoma.
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PMID:[Cerebellar degeneration associated with anti-Tr antibodies without Hodgkin's disease. Four years follow-up]. 1547 May 88

Paraneoplastic cerebellar degeneration (PCD) can present as a severe and (sub)acute cerebellar syndrome. PCD can accompany different kinds of neoplasms including small cell lung cancer, adenocarcinoma of the breast and ovary, and Hodgkin's lymphoma. A 34-year-old patient is described with acute dysarthria, gait ataxia and diplopia. Despite extensive laboratory and radiological evaluations in this patient with rapidly deteriorating cerebellar syndrome, the diagnosis of a paraneoplastic syndrome was only made after several months, when an anti-Tr antibody was detected in his serum. The search for Hodgkin's disease as concomitant disorder was then started and resulted in stage II B disease. The patient was successively treated with six courses of etoposide, bleomycin, vinblastine and dexamethasone and radiotherapy, which resulted in a complete remission of the Hodgkin's disease. After starting therapy the cerebellar degeneration stabilised. The pathogenesis of neuronal damage in central nervous system paraneoplastic disorders such as the one we describe is not completely understood. Antitumour therapy is assumed to be the important cornerstone in stabilising the neurological condition. Improvement of the cerebellar syndrome in anti-Tr autoantibody paraneoplastic disease is a rare achievement. Early recognition of the concomitant disorders (anti-Tr autoantibody disease and Hodgkin's lymphoma) is of crucial importance.
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PMID:Paraneoplastic cerebellar degeneration preceding the diagnosis of Hodgkin's lymphoma. 1692 86

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological syndrome associated with lung cancer, breast adenocarcinoma,ovarian adenocarcinoma, and Hodgkin disease. It is rarely seen in pediatrics. We report a case of a 10-year-old boy with a 2-year prodrome that led to a diagnosis of PCD in association with stage IV Hodgkin disease. He received radiation and chemotherapy for his Hodgkin disease with resolution of his lymphoma. Based on promising data in adults on the efficacy of rituximab over other immuno suppressive agents in paraneoplastic disorders, he was treated with rituximab with marked improvement of the cerebellar syndrome.
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PMID:Rituximab as potential therapy for paraneoplastic cerebellar degeneration in pediatric Hodgkin disease. 2253 86

Paraneoplastic cerebellar degeneration (PCD) is a type of paraneoplastic neurological disorder (PND) that is associated with many solid tumors, Hodgkin's lymphoma (HL) and very rarely with non-Hodgkin's lymphoma (NHL). We report a case of PCD associated with gastric diffuse large B-cell lymphoma (DLBCL) in a patient who presented with acute onset of giddiness and double vision and had complete remission of the gastric lesion and marked improvement of cerebellar syndrome with rituximab-based combination chemotherapy. A brief review of the literature is also presented.
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PMID:Gastric diffuse large B cell lymphoma presenting as paraneoplastic cerebellar degeneration: case report and review of literature. 2420 96

Primary meningeal lymphomas are very rare and those derived from T cells are even more infrequent (less than 5% of primary central nervous system lymphomas). Cerebellopontine angle involvement in the primary T-cell lymphoma is exceptional. Clinical presentation depends on the type of lesions, and histological diagnosis is needed. We present a rare case of a 50-year-old woman who presented with clinical cerebellar syndrome with posterior opsoclonus-myoclonus syndrome. Necropsy evaluation revealed primary diffuse leptomeningeal non-Hodgkin's T-cell lymphoma.
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PMID:T-cell primary leptomeningeal lymphoma in cerebellopontine angle. 2575 Feb 25

Paraneoplastic cerebellar degeneration (PCD) can occur severely and appear as subacute cerebellar syndrome. PCD may be associated with small cell lung cancer, adenocarcinoma, breast cancer, ovarian carcinoma, and Hodgkin's lymphoma. An 11-year-old male was admitted with acute cerebellar ataxia, dysarthria, and diplopia. Mediastinal conglomerated lymph nodes were depicted in a chest computed tomography (CT) examination, and diagnosis of stage IV Hodgkin's lymphoma was obtained after a lymph node biopsy. The antibodies against Purkinje cells (anti-Tr antibody) were positive immunohistochemically. Thus, paraneoplastic cerebellar degeneration depending on Hodgkin's disease was diagnosed. Despite the completion of chemotherapy, neurological recovery was not observed in the patient and plasmapheresis with immunoadsorption, and intravenous immunoglobulin (IVIG) was performed. Truncal ataxia, gait disturbance, and tremors decreased. Consequently, we thought that plasmapheresis with the immunoadsorption method and IVIG therapy might be a treatment option for cerebellar ataxia caused by a mechanism of immune ancestry.
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PMID:Hodgkin's lymphoma associated with paraneoplastic cerebellar degeneration in children: a case report and review of the literature. 2844 49

Progressive multifocal leukoencephalopathy (PML) is an uncommon opportunistic infection with high morbidity and mortality. This is an institutional review board-approved retrospective review of medical records identified by diagnostic coding for PML or John Cunningham virus (JCV) from 2000 to 2015. Inclusion criteria were cerebrospinal fluid (CSF) positive for JCV by polymerase chain reaction or brain biopsy-proven PML in non-HIV patients. There were 16 patients, 12 of whom were men (75%); the median age was 56 years (range, 31-71 years). All had hematologic malignancies (5 [31%] had chronic lymphocytic leukemia, 3 [19%] had acute myeloid leukemia, 3 had [19%] mantle cell lymphoma, and 1 patient each had acute lymphoblastic leukemia, Hodgkin lymphoma, myeloma, or B-cell lymphoma). One patient received no cancer-directed therapy. Of the remaining 15 patients, all received conventional chemotherapy, and 9 (60%) underwent transplant. Thirteen patients (87%) received immunomodulating therapy (predominantly rituximab). The median time from cancer diagnosis to PML diagnosis was 48.5 months. PML was diagnosed a median of 2.1 months from symptom onset; however, the median time to PML diagnosis was 5.4 months for the 4 patients presenting with a cerebellar syndrome. PML was diagnosed by CSF in 12 patients and brain biopsy in 4 following negative CSF test results. Median survival from PML diagnosis was 4.3 months for the 11 patients on treatment and 0.87 months for the 5 without treatment. PML still occurs in patients with hematologic malignancies in the absence of treatment. Twenty-five percent of our patients required brain biopsy for diagnosis, and diagnosis was delayed when the clinical presentation was unusual, such as a cerebellar syndrome.
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PMID:Progressive multifocal leukoencephalopathy and hematologic malignancies: a single cancer center retrospective review. 2929 50

BACKGROUND Paraneoplastic cerebellar degeneration (PCD) is a rare condition that can present as an acute or subacute cerebellar syndrome. PCD is most commonly associated with gynecological and breast cancer, small-cell lung cancer, and classical Hodgkin's lymphoma. The symptoms of PCD can arise several months before tumor diagnosis. This report is of a case of a 44-year-old man with PCD that preceded the diagnosis of classical Hodgkin's lymphoma by 16 months. CASE REPORT A 44-year-old man was admitted to hospital with a cerebellar syndrome that was initially diagnosed as vertebrobasilar insufficiency. Eight months later, cerebral magnetic resonance imaging (MRI) findings and serum anti-Tr antibodies supported the diagnosis of PCD, but no underlying malignancy was initially found. At 16 months after the initial diagnosis of PCD, the patient developed an enlarged inguinal lymph node. Histology of the excisional lymph node biopsy confirmed the diagnosis of classic mixed cellularity Hodgkin's lymphoma, Ann Arbor stage IIA. The patient responded to four cycles of adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy. CONCLUSIONS This case illustrates that in patients who present with PCD, an associated malignancy, such as classical Hodgkin's lymphoma, may emerge several months later, which supports long-term follow-up. The presence of anti-Tr antibodies may support a diagnosis of classical Hodgkin's lymphoma in a patient with a history of PCD who develops lymphadenopathy.
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PMID:A Case of Paraneoplastic Cerebellar Degeneration that Preceded the Diagnosis of Classical Hodgkin's Lymphoma by 16 Months. 3257 56