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)

Intravascular lymphomatosis is an uncommon lymphoproliferative disorder characterised by intravascular distribution of neoplastic lymphoid cells (B- or T-lymphocites), what leads to occlusion of small vessels and causes signs and symptoms of disorder. Its initial predilection sites are the brain or the skin, and hematopoetic organs are usually spared. The signs and symptoms of the disorder are attributed to vascular occlusion. Clinical course is fatal, besides therapy. In our case disorder has started with partial epileptic seizures with secundar generalisation, and after that with motor aphasia, right hemiparesis, urinary incontinence and cognitive changes. She was hospitalized for several times, completely investigated, but with no conclusion. Seven months after apperance of symptoms, she died. And diagnosis was determined at autopsy. A myriad of neurological and systemic manifestations are putting us in an unequal position with this fatal disease. Early diagnosis can increase survival and decrease intensity of symptoms. So every new case is important as warning that we must not forget about this disease.
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PMID:[Intravascular lymphoma of the central nervous system]. 1469 48

A 47-year-old Hispanic male presented with visual field disturbances, memory impairment, and a seizure. CT and MRI were consistent with meningioma. Both neurologic exam and routine laboratory tests were within normal limits. The patient underwent craniotomy and subtotal resection of the tumor. On H&E, the lesion was composed of a lymphoid mass with well-defined irregularly shaped follicles surrounded by a monomorphic population of small lymphocytes. Marginal zones stained for B-cell markers, CD20 and CD79a, one T-cell marker, CD43, and kappa light chains. While other markers did not stain the majority of tumor cells, they did identify other lymphoid and plasma cell elements. A diagnosis of marginal zone B-cell lymphoma of dura, mucosa-associated lymphoid tissue (MALT)-type (extranodal) was made. MALT-type lymphomas are unusual in the nervous system; this is the first such case reported in a male and serves to emphasize the wide diversity of presentation of a neoplasm originally described in the GI tract and thus far described in the CNS only in females.
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PMID:Primary dural mucosa-associated lymphoid tissue-type lymphoma: case report and review of the literature. 1517 17

An 86-year-old African-American man presented with tonic-clonic seizures. Intravenous phenytoin was urgently administered into the dorsum of the right hand. The patient developed a raised purple area of discoloration around the intravenous insertion site within 2 h and edema and vesiculobullous lesions of the distal forearm, hands, and fingers within 8 h. Microscopic sections from a biopsy at 12 h revealed epidermal necrosis, superficial ulceration, and a mild superficial and deep perivascular lymphoid infiltrate, associated with numerous thrombi of small vessels throughout the dermis. The findings were judged to be consistent with soft-tissue injury associated with intravenous administration of phenytoin, also termed purple glove syndrome. Purple glove syndrome, named for its distinctive purple discoloration and swelling of the hands in the distribution of a glove, is an uncommon complication of intravenous phenytoin administration through small dorsal veins of the hands. It is comprised by pain, discoloration, and edema in the vicinity of intravenous infusion of phenytoin through dorsal veins of the hand. The histopathologic features of fully developed lesions have been reported; however, early-stage findings have not been previously described, and the histogenesis of this lesion is controversial. The presence of thrombi in this early-stage lesion suggests that thrombosis plays a role in the initial pathogenesis of this condition.
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PMID:Early histopathologic changes in purple glove syndrome. 1523 84

A progressive neurological disease affecting twenty-two young adult dogs of smaller breeds is reported. The disease was most often acute in onset and the neurological signs included cervical pain, seizures, behavioural changes, ataxia, head tilt, muscle tremor and paresis. The lesions were those of a disseminated granulomatous meningoencephalomyelitis in which perivascular cuffs of macrophages and lymphoid cells were the predominant finding. Severe lesions were most often seen in the cerebral white matter although the brain stem, mid-brain and spinal cord were also commonly affected.
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PMID:Granulomatous meningoencephalomyelitis of dogs in New Zealand. 1603 Sep 79

Extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) lymphomas are a well-described type of low-grade B-cell non-Hodgkin lymphoma. They typically arise adjacent to mucosal surfaces in the gastrointestinal tract, lung and conjunctiva, and, less frequently, in the skin, salivary gland and thyroid gland. Unusual locations, such as the genitourinary tract, thymus and meninges, have also been reported. We recently encountered a case of an intracranial MALT lymphoma in a 53-year-old man who presented with persistent headaches and a seizure. The lesion developed as a mass within the lateral ventricle, appeared to be arising from the choroid plexus, and was not associated with meninges. Histologically, there was a vaguely nodular, dense lymphoid infiltrate with occasional benign follicles colonized by marginal zone lymphoma, suggesting derivation from a focus of prior inflammation. Translocations involving the MALT1 gene were not identified but karyotypic evaluation highlighted a complex cytogenetic profile with many chromosomal abnormalities. This rare case provides insight into the pathophysiology of MALT lymphomas.
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PMID:Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle. 1619 87

EBV infection in tumor cells is generally restricted to the latent forms of viral infection. Switching the latent form of viral infection into the lytic form may induce tumor cell death. We have previously reported that certain chemotherapy agents can increase the amount of lytic viral gene expression in EBV-positive tumor cells. In this report, we have explored the potential utility of valproic acid (VPA), an anti-seizure drug that also has strong histone deacetylase inhibitory activity, for activating lytic viral gene expression in EBV-positive tumors. Although VPA treatment alone induced only a modest increase in the level of lytic viral gene expression, it strongly enhanced the ability of chemotherapeutic agents to induce lytic EBV gene expression in EBV-positive epithelial and lymphoid cells in vitro. Furthermore, VPA enhanced cell killing in vitro by chemotherapeutic agents in lymphoblastoid cells and gastric cells (AGS) containing wild-type EBV. In contrast, VPA did not enhance the cytotoxicity of chemotherapy in lymphoblastoid cells containing a lytic-defective (BZLF1-knockout) form of EBV or in EBV-negative AGS cells. Finally, we found that the combination of VPA and chemotherapy was significantly more effective in inhibiting EBV-driven lymphoproliferative disease in severe combined immunodeficient mice than chemotherapy alone. These results suggest that VPA could potentiate the efficacy of chemotherapy for EBV-positive tumors in patients.
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PMID:Valproic acid enhances the efficacy of chemotherapy in EBV-positive tumors by increasing lytic viral gene expression. 1695 Nov 92

A 15-year-old female harbor seal (Phoca vitulina) was referred to the Nantes Veterinary School, Nantes, France, with a clinical history of anorexia, seizures, and left hemiplegia. Cerebrospinal fluid analysis revealed large numbers of neoplastic lymphoid cells. After injection of a contrast agent into the cerebrospinal space, radiographs demonstrated an asymmetry of the right lateral ventricle. Necropsy examination revealed a marked edema of the right frontal lobe, extending to the basal nuclei and thalamus in the brain. Histological examination of the brain revealed leptomeningeal lymphoma. Immunohistochemical labeling demonstrated that the neoplasm was of T-cell origin. No significant macroscopic or microscopic lesions were observed in the other organs examined, including lymphoid organs. This is the first report of primary leptomeningeal lymphoma in a harbor seal.
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PMID:Primary meningeal T-cell lymphoma in a harbor seal (Phoca vitulina). 1767 18

The prion protein (PrP) plays a key role in the pathogenesis of prion diseases. However, the normal function of the protein remains unclear. The cellular isoform (PrP(C)) is expressed most abundantly in the brain, but has also been detected in other non-neuronal tissues as diverse as lymphoid cells, lung, heart, kidney, gastrointestinal tract, muscle, and mammary glands. Cell biological studies of PrP contribute to our understanding of PrP(C) function. Like other membrane proteins, PrP(C) is post-translationally processed in the endoplasmic reticulum and Golgi on its way to the cell surface after synthesis. Cell surface PrP(C) constitutively cycles between the plasma membrane and early endosomes via a clathrin-dependent mechanism, a pathway consistent with a suggested role for PrP(C) in cellular trafficking of copper ions. Although PrP(-/-) mice have been reported to have only minor alterations in immune function, PrP(C) is up-regulated in T cell activation and may be expressed at higher levels by specialized classes of lymphocytes. Furthermore, antibody cross-linking of surface PrP(C) modulates T cell activation and leads to rearrangements of lipid raft constituents and increased phosphorylation of signaling proteins. These findings appear to indicate an important but, as yet, ill-defined role in T cell function. Recent work has suggested that PrP(C) is required for self-renewal of haematopoietic stem cells. PrP(C) is highly expressed in the central nervous system, and since this is the major site of prion pathology, most interest has focused on defining the role of PrP(C) in neurones. Although PrP(-/-) mice have a grossly normal neurological phenotype, even when neuronal PrP(C) is knocked out postnatally, they do have subtle abnormalities in synaptic transmission, hippocampal morphology, circadian rhythms, and cognition and seizure threshold. Other postulated neuronal roles for PrP(C) include copper-binding, as an anti- and conversely, pro-apoptotic protein, as a signaling molecule, and in supporting neuronal morphology and adhesion. The prion protein may also function as a metal binding protein such as copper, yielding cellular antioxidant capacity suggesting a role in the oxidative stress homeostasis. Finally, recent observations on the role of PrP(C) in long-term memory open a challenging field.
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PMID:Physiological role of the cellular prion protein. 1807 96

Pathogenetic roles of human herpesvirus (HHV)-6 in lymphoproliferative diseases have been of continued interest. Many molecular studies have tried to establish a pathogenic role for HHV-6 in lymphoid malignancies. However, whether HHV-6 plays a role in these pathologies remains unclear, as positive polymerase chain reaction results for HHV-6 in those studies may reflect latent infection or reactivation rather than presence of HHV-6 in neoplastic cells. A small number of studies have investigated HHV-6 antigen expression in pathologic specimens. As a result, the lack of HHV-6 antigen expression on neoplastic cells argues against any major pathogenic role of HHV-6. The role of HHV-6 in childhood acute lymphoblastic leukemia (ALL) has also been of interest but remains controversial, with 2 studies documenting higher levels of HHV-6 antibody in ALL patients, and another 2 large-scale studies finding no significant differences in HHV-6 seroprevalences between ALL patients and controls. Alternatively, HHV-6 is increasingly recognized as an important opportunistic pathogen. HHV-6 reactivation is common among recipients of allogeneic stem cell transplantation (SCT), and is linked to various clinical manifestations. In particular, HHV-6 encephalitis appears to be significant, life-threatening complication. Most HHV-6 encephalitis develops in patients receiving transplant from an unrelated donor, particularly cord blood, typically around the time of engraftment. Symptoms are characterized by short-term memory loss and seizures. Magnetic resonance imaging typically shows limbic encephalitis. Prognosis for HHV-6 encephalitis is poor, but appropriate prophylactic measures have not been established. Establishment of preventive strategies against HHV-6 encephalitis represents an important challenge for physicians involved with SCT.
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PMID:Human herpesvirus 6 in hematological malignancies. 1990 10

Lymphomatoid granulomatosis is an Epstein-Barr virus-associated multisystem disease that combines granulomatous inflammatory process with lymphoproliferative potential. It predominantly affects lungs, skin, and brain and is characterized by multifocal, transmural, angiocentric, and angiodestructive pleomorphic lymphoid infiltrate in a perivascular distribution. Lymphomatoid granulomatosis is generally considered to be a neoplastic B-cell proliferation that has traditionally been associated with poor prognosis, evolving as a progressive multisystem disease transforming into B-cell lymphoma, with a median survival of 14 to 16 months only. Its lymphomatous nature explains prompt response to steroids and systemic chemotherapy, although appropriate optimal management still remains to be defined. The authors report on a young boy who presented with features of raised intracranial tension and sudden onset seizures. Neuroimaging showed 2 space-occupying lesions, larger in the left frontoparietal region with heterogeneous enhancement, moderate perifocal edema, compression, and mass effect. He underwent surgical decompression of the dominant lesion with prompt relief of symptoms. The diagnosis of lymphomatoid granulomatosis was confirmed on light microscopy and immunohistochemistry. An extensive systemic work-up ruled out other site(s) of involvement. He was successfully treated with aggressive systemic chemotherapy and moderate dose of whole-brain radiotherapy. Awareness of disease spectrum in the central nervous system may permit early diagnosis and thus allow institution of timely appropriate therapy.
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PMID:Isolated central nervous system involvement by lymphomatoid granulomatosis in an adolescent: a case report and review of literature. 2020 96


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