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)

Two young patients with subacute measles encephalitis are described: a 20-year-old male hemophiliac infected with human immunodeficiency virus (HIV) and a 4-year-old girl with acute leukemia. Both patients were afebrile and had persistent focal seizures and slurred speech beginning 2 and 7 months, respectively, after the onset of uncomplicated acute measles. The diagnosis of subacute measles encephalitis was established by demonstration of paramyxovirus nucleocapsid on electron microscopy of brain tissue in one case and by detection of measles virus genome with the polymerase chain reaction in both. Treatment of the HIV-infected man with intravenous ribavirin was begun when the patient lost consciousness after several weeks of seizures; he died. The girl with leukemia was treated early after the onset of symptoms and recovered after a 15-week course. Review of 31 previously published cases revealed a typical clinical presentation. Cerebrospinal fluid (CSF) analysis, electroencephalography, measurement of measles antibody in serum and CSF, and computed tomography of the brain were not helpful in the diagnosis of subacute measles encephalitis. In contrast, histologic examination of brain tissue proved useful in establishing the diagnosis. On the basis of our experience and our literature review, we conclude that histologic and polymerase chain reaction studies of brain tissue are required for the early diagnosis of subacute measles encephalitis and that therapy with intravenous ribavirin is effective when administered early.
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PMID:Subacute measles encephalitis in the young immunocompromised host: report of two cases diagnosed by polymerase chain reaction and treated with ribavirin and review of the literature. 832 78

The mechanisms of endothelial cell damage that lead to cerebral hemorrhage are not completely understood. In this study, a cloned murine retrovirus, TR1.3, that uniformly induced stroke in neonatal BALB/c mice is described. Restriction digest mapping suggests that TR1.3 is part of the Friend murine leukemia virus (FMuLV) family. However, unlike mice exposed to other FMuLVs, mice infected with TR1.3 virus developed tremors and seizures within 8 to 18 days postinoculation. This was uniformly followed by paralysis and death within 1 to 2 days. Postmortem examination of TR1.3-inoculated mice revealed edematous brain tissue with large areas of intracerebral hemorrhage. Histologic analysis revealed prominent small vessel pathology including syncytium formation of endothelial cells. Immunohistochemical analysis of frozen brain sections using double fluorescence staining demonstrated that TR1.3 virus specifically infected small vessel endothelial cells. Although infection of vessel endothelial cells was detected in several organs, only brain endothelial cells displayed viral infection associated with hemorrhage. The primary determinant of TR1.3-induced neuropathogenicity was found to reside within a 3.0-kb fragment containing the 3' end of the pol gene, the env gene, and the U3 region of the long terminal repeat. The restricted tropism and acute pathogenicity of this cloned murine retrovirus provide a model for studying virus-induced stroke and for elucidating the mechanisms involved in syncytium formation by retroviruses in vivo.
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PMID:Intracerebral hemorrhages and syncytium formation induced by endothelial cell infection with a murine leukemia virus. 839 66

Feline leukemia viruses carrying transduced v-myc genes (myc-FeLV) induce tumors of clonal origin, suggesting that activated myc alone is not sufficient for tumorigenesis. To investigate the hypothesis that insertional mutagenesis plays a role by activating genes which collaborate with v-myc, we looked for evidence of common proviral integration sites in these tumors. By inverse polymerase chain reaction we identified a 6-kb domain, designated fit-1, in which FeLV proviruses were inserted in four of nine (44%) T-cell tumors induced by myc-FeLV. The fit-1 locus was mapped to feline chromosome B2 and appears to be distinct from known oncogenes located on this chromosome. Fit-1 represents a novel common proviral integration region which may harbor a cellular gene which acts in concert with the myc gene in T-cell tumorigenesis.
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PMID:A common proviral integration region, fit-1, in T-cell tumors induced by myc-containing feline leukemia viruses. 839 12

Congenital errors of folate metabolism can be related either to defective transport of folate through various cells or to defective intracellular utilization of folate due to some enzyme deficiencies. Defective transport of folate across the intestine and the blood-brain barrier was reported in the condition 'Congenital Malabsorption of Folate'. This disease is characterized by a severe megaloblastic anaemia of early appearance associated with mental retardation. Anaemia is folate-responsive, but neurological symptoms are only poorly improved because of the inability to maintain adequate levels of folate in the CSF. A familial defect of cellular uptake was described in a family with a high frequency of aplastic anaemia or leukaemia. An isolated defect in folate transport into CSF was identified in a patient suffering from a cerebellar syndrome and pyramidal tract dysfunction. Among enzyme deficiencies, some are well documented, others still putative. Methylenetetrahydrofolate reductase deficiency is the most common. The main clinical findings are neurological signs (mental retardation, seizures, rarely schizophrenic syndromes) or vascular disease, without any haematological abnormality. Low levels of folate in serum, red blood cells and CSF associated with homocystinuria are constant. Methionine synthase deficiency is characterized by a megaloblastic anaemia occurring early in life that is more or less folate-responsive and associated with mental retardation. Glutamate formiminotransferase-cyclodeaminase deficiency is responsible for massive excretion of formiminoglutamic acid but megaloblastic anaemia is not constant. The clinical findings are a more or less severe mental or physical retardation. Dihydrofolate reductase deficiency was reported in three children presenting with a megaloblastic anaemia a few days or weeks after birth, which responded to folinic acid. The possible relationship between congenital disorders such as neural tube defects or dihydropteridine reductase deficiency and disturbances of folate metabolism are discussed. Neurological symptoms present in most of these congenital disorders highlight the role of folate in the central nervous system.
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PMID:Congenital errors of folate metabolism. 853 63

A prospective neurological and electroencephalographic (EEG) study was performed in sixteen leukemia patients receiving busulfan (BU) and cyclophosphamide before autologous bone marrow transplantation. All patients were given anticonvulsant prophylaxis with a combination of phenobarbital (PB) and clonazepam (CLZ). Neurological examination and EEG were performed prior to and soon after completion of BU treatment and were repeated two months later. No tonic-clonic and/or myoclonic convulsions were observed. In two patients, comparison of EEG recorded before and upon completion of BU administration revealed modification of features. EEG re-evaluated two months after BU showed normalization in one of the two patients. BU may trigger both generalized and myoclonic seizures together with EEG abnormalities; PB combined with CLZ may be useful prophylactic treatment.
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PMID:Clonazepam prophylaxis and busulfan-related myoclonic epilepsy in autografted acute leukemia patients. 864 20

A girl with acute lymphoblastic leukemia in remission had two seizures during the maintenance phase of her treatment. Magnetic resonance imaging with angiography identified a superior sagittal sinus thrombosis as the likely explanation for her symptoms. Possible causes are considered, and previous reports of the neurotoxicity of agents used in the treatment of leukemia are reviewed.
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PMID:Superior sagittal sinus thrombosis complicating maintenance treatment for acute lymphoblastic leukemia. 873 46

Involvement of CNS with leukemic cells is well recognized complication of acute lymphatic leukemia (ALL) in childhood, but with recent improvements in systemic treatment and longer survival the incidence of this complication has increased in adults. Neurological symptomatology in patients with CNS leukemia is due to meningeal infiltration, but sometimes also to diffuse and nodular cerebral infiltration. Between January 1991 and December 1994, 36 patients suffering of acute leukemia, 28 with ALL, and 8 with acute myeloid leukemia (AML) were demonstrated to have neuroleukemia by the following criteria: (1) the presence at lumbar puncture (LP) pleocytosis and blast cells on CSF sediment (without positive bacteriologic and fungal cultures), and (2) the presence of neurological symptoms and signs. All 36 patients had 46 episodes of CNS involvement. All patients had neurological examinations during every episode, and according to the neurological abnormalities were classified into four categories. LP was performed in all, and CSF sediments obtained by sedimentation in Sayk's chambers, were routinely stained by MGG and cytochemical stains to detection of leukemic cells (Fig. 1). EEG was done during 21 episodes, CT scan during 15. We divided patients into four groups according to the most prominent neurological symptoms and signs. First was the group included 23 episodes (50%), (18 ALL, 5 AML), where symptoms and signs of meningeal irritation predominated, mimicking the clinical picture of meningitis. This meningeal syndrome can sometimes produce differential diagnostic problems with CNS infections, when CSF examination is of primary importance. Second was the group of 9 patients (6 ALL and 3 AML) with 10 episodes (21.74%) where cranial nerve symptoms and signs-predominated, or were exclusively present. Most frequently affected were bulbomotors, facials and opticus. Third group consisted of 8 ALL patients (8 episodes, 17.39%) with dominant spinal root symptomatology, caused by pathological infiltration of either spinal roots or meninges surrounding them. This group includes also one patient with mononeuritis multiplex and the other with painful polyneuropathy. All patients in this group had pain on straight leg raising, but we stress here that all patients from other groups had positive Lazarevitsh's sign, too. So, it can be a good differential diagnostic parameter for distinguishing toxic medicamentous polyneuropathy from leukemic poliradiculoneuropathy. Fourth group included 5 patients (5 episodes, 10.87%). 4 ALL and 1 AML, where cerebral symptoms, such as seizures, hemiparesis and psychoorganic syndromes were prominent. CSF was obtained during all episodes by lumbar puncture. The protein concentration ranged from 21-3180 mg/dl, and was above normal (45 mg/dl) during 28 episodes. Mild hypoglycoracchia was present during 16 episodes. Cell count ranged from 11-4816 cells/cm3, malignant cells were identified during all episodes with same morphological and cytochemical characteristics of identified type of leukaemia. It has been established that the most valuable diagnostic procedure in CNS leukemia is CSF examination, and detection of blasts is sufficient for diagnosis. All other procedures like EEG, myelography and CT have only supplemental diagnostic significance. Finally, in this study we showed that neurological symptomatology in patients with acute leukemia is not dependent of the type of leukemia, moreover different types of AL can have same neurological manifestations. As others, we sometimes used the term CNS leukemia in this paper, although it is clear that meninges and peripheral nervous system are most often involved. This is the reason why we suggest that neuroleukemia, or NS leukemia should be used as more appropriate expressions.
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PMID:[Neuroleukemia in adults]. 910 25

Modern treatment of childhood acute lymphoblastic leukaemia (ALL) has dramatically improved the prognosis for children with this disease. Therapeutic approaches consist of multimodal chemotherapy and radiotherapy with significant long-term side-effects. We report on 4 children out of a group of 120 newly diagnosed patients with ALL, who survived the disease for more than 2 years and developed a cerebral haemorrhage after chemotherapy and fractionated cranial irradiation. Following a period of 2-12 years the four children presented with acute neurological signs and symptoms. i.e. seizures, ataxia and hemiparesis. CT and MRI revealed intracerebral mass lesions, interpreted as haemorrhage. After neurosurgery the patients neurological state improved. Histological examination confirmed the suspected diagnosis of bleeding cavernous haemangioma or capillary telangiectases. There are two possibilities to explain these rare alterations: they may be pre-existent to the disease and therapy or they may be caused by irradiation. CONCLUSION Acute neurological symptoms in patients treated for ALL may be caused by spontaneous cerebral haemorrhaging of cavernous haemangiomas or capillary telangiectases induced by chemotherapy and/or radiotherapy.
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PMID:Cerebral haemorrhage in long-term survivors of childhood acute lymphoblastic leukaemia. 917 78

An increased number of adults with mental retardation are living in the community and seeking health care from family physicians. When mentally retarded patients are enrolled in a medical practice, guardianship status should be determined, but these patients should be involved in their own care to as great an extent possible. Since a verbal history may be difficult to obtain, a systematic, thorough physical examination is important. Certain Illnesses, such as hepatitis B, recurrent aspiration syndrome, leukemia and atlantoaxial instability, are much more common in adults with Down syndrome then in adults with mental retardation from other causes. Seizures and mental illness are equally common in all mentally retarded adults. The behavior management and pharmacologic therapy of patients with mental retardation are best handled in close association with caregivers, as well as psychiatrists and neurologists who are familiar with the special needs of this population.
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PMID:Primary care of adults with mental retardation living in the community. 955 40

Refractory anemia with excess blasts in transformation (RAEB-T) is a pre-leukemic syndrome that can progress to acute myelogenous leukemia (AML). Although leptomeningeal disease in AML is rare and it is commonly associated with specific cytogenetic abnormalities, it has not been documented in RAEB-T. We report a woman who experienced seizures and had subarachnoid enhancement on MRI suggestive of leukemic infiltrates in the leptomeninges. Her bone marrow aspirate revealed 6.6% blasts and the presence of Auer rods, findings consistent with a diagnosis of RAEB-T. Subsequent cytogenetic analysis detected an uncommon deletion of chromosome 11q23, and the breakpoint on chromosome 11 is identical to that seen in AML patients having leptomeningeal disease and translocation between chromosome 9 and 11. Although RAEB-T is not considered as leukemia and is thought unlikely to involve the central nervous system, the presence of leptomeningeal disease and 11q23 deletion in our patient suggests that cytogenetic abnormality may be an important determinant of neurological complications, regardless of a diagnosis of RAEB-T or AML which is based on cellular morphology.
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PMID:Leptomeningeal disease in pre-leukemic syndrome: cytogenetic abnormality versus cellular morphology. 946 87


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