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)

With the advent of chemotherapy, mortality rates in acute lymphoblastic leukaemia (ALL) have decreased, but complications in the central nervous system have appeared. These include direct involvement of the brain itself and the development of chemotherapy-related encephalopathy as a delayed reaction. In most reported cases, this encephalopathy is believed to be due to necrotising angiitis arising from the combination of chemotherapy with adjuvant radiotherapy. We report the cases of four children with ALL who had been treated with high-dose intravenous and intrathecal chemotherapy but no radiation therapy, and who were admitted to hospital because of seizures. CT of the brain revealed the presence of diffuse periventricular white matter hypodensities in all cases and subcortical hyperdense foci in three cases. MRI showed diffuse hyperintense white matter lesions on T2-weighted images in all four patients; hypointense changes were observed on susceptibility-sensitive FLASH sequences in the hyperdense foci seen on CT as well as changes that were hyperintense on T1-weighted images. It was, therefore, concluded that the lesions corresponded to a leukoencephalopathy with calcific deposits. These findings are of a pure form of methotrexate encephalopathy causing seizures.
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PMID:Pure methotrexate encephalopathy presenting with seizures: CT and MRI features. 947 50

Mice infected with the LP-BM5 murine leukemia virus (MuLV) develop an immune deficiency syndrome together with an encephalopathy characterized by impairments in spatial learning and memory. These cognitive deficits are evident before the appearance of neuron loss and lymphoid cell invasion of the brain. Nonetheless, a prominent gliosis and a variety of neurochemical changes precede the development of cognitive deficits. The neurochemical abnormalities include significant decreases in striatal Met-enkephalin and substance P (but not somatostatin), increases in concentrations of quinolinic acid and platelet-activating factor, and alterations in brain fyn kinase. At this stage of the infection, some of these neurochemical changes can be reversed by glutamate receptor antagonists, cytokine inhibitors, and anti-retroviral agents. In later stages of the infection, however, the infected mice develop irreversible neuronal loss, invasion of hematopoietic cells, and increased viral burden in the CNS. In addition, motor-neuron dysfunction (hindlimb paralysis, weakness, and ataxia) and seizures are sometimes observed during the late stages of infection. Thus, the LP-BM5 MuLV-infected mouse is a useful model for studying the chronology of neurodegenerative changes, ranging from reversible neuron dysfunction to irreversible neuron loss, that are associated with retrovirus-induced immunodeficiency.
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PMID:The encephalopathy associated with murine acquired immunodeficiency syndrome. 962 8

A 16 year old boy with epilepsy and learning difficulties is reported. At 3 years of age he was diagnosed with common acute lymphoblastic leukaemia, and received therapy according to the UK protocol, UKALL VIII. This included prophylactic CNS radiotherapy and chemotherapy. He did not develop CNS leukaemia, and complete remission was achieved. At age 7, he began to experience lethargy and learning difficulties, especially problems with hand-writing, concentration and memory. Furthermore, he began experiencing atypical absence seizures, which were provoked by concentration at times of tiredness. EEG showed bilateral non-specific abnormalities, with some epileptiform features. Over the following 9 years, several anti-epileptic drugs were prescribed. Although with the changes in therapy initial remissions have been achieved, the seizures have, each time, continued to relapse. At age 12, EEG was very abnormal, showing frequent generalized slow or sharp waves. At age 13, MRI revealed multiple discrete small high-intensity lesions in the subcortical white matter of both hemispheres. Problems with lethargy, concentration and memory persist and although multiple anti-epileptic drugs have been prescribed, seizures continue to occur almost daily.
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PMID:Leukoencephalopathy after CNS prophylaxis for acute lymphoblastic leukaemia. 966 10

Between 1984 and 1993, monoclonal antibodies (MAbs) radiolabelled with (131)I were administered into the CSF of 52 patients with neoplastic meningitis (meningosis) with progressive disease despite active conventional therapy. Selection of MAbs was based on immunoreactivity with patients' tumour and lack of binding to normal central nervous system (CNS) tissue. Following full clinical assessment and neuro-imaging which included isotope flow study of CSF pathways, (131)I-MAb was administered via a ventricular access device, lumbar catheter or both. Radioisotope activity varied from 25 mCi to 160 mCi in adults. Dose escalation was carried out and some patients received multiple doses. Distribution of (131)I-MAb and clearance kinetics were derived from serial scintigraphy and CSF/blood sampling. Evidence of localisation to tumour was frequently observed. Toxicity was minimal and easily treated, although one death occurred, possibly due to a seizure. The best results were obtained in primitive neuroectodermal tumour (n=22), where 53% of evaluable cases had responses and 11% had stable disease, adults responding better than children. Three exceptional survivals have been recorded; one patient leads a normal life at 10 years 11 months, one case is alive and normal at 3 years, 2 months. A third case survived in good condition for 8 years. The mean survival of responders was 39 months and non-responders 4 months. In the total series, 50% of patients survived for at least one year with 2 long term survivors. CSF therapy with (131)I-MAb appears to be valuable as a single agent or when used in combination with other modalities. Results of treating leukaemia and carcinoma cases suggest that re-seeding into the CSF compartment from active systemic disease may account for early relapse in the CNS. One carcinoma case with no apparent systemic disease made a remarkable response and survival for 4 years following a single treatment. Neoplastic meningitis generally carries a dismal prognosis. The results obtained in this initial trial are sufficiently encouraging to stimulate further attempts at CSF therapy with (131)I-MAbs.
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PMID:Treatment of neoplastic meningitis by targeted radiation using (131)I-radiolabelled monoclonal antibodies. Results of responses and long term follow-up in 40 patients. 969 76

Uncertainty remains about the quantitative effects of doses of ionising radiation less than 0.2 Sv. Estimates of hereditary effects, based on the atomic bomb survivors, suggest that the mutation doubling dose is about 2 Sv for acute low LET radiation, but the confidence limits are wide. The idea that paternal gonadal irradiation might explain the Seascale cluster of childhood leukaemia has been disproved. Fetal irradiation may lead to a reduction in IQ and an increase in seizures in childhood proportional to dose. Estimates that doses to a whole population cause a risk of cancer proportional to dose, with 0.1 Sv given acutely causing a risk of 1%, will need to be modified as more information is obtained, but the idea that there is a threshold for risk above this level is not supported by observations on the irradiated fetus or the effect of fallout. The idea, based on ecological observations, that small doses protect against the development of cancer is refuted by the effect of radon in houses. New observations on the atomic bomb survivors have raised afresh the possibility that small doses may also have other somatic effects.
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PMID:Effects of small doses of ionising radiation. 1032 98

It is well known that Down's syndrome is sometimes associated with leukemia. However, there have been only a few case reports of a relationship between Down's syndrome and brain tumors. We report 2 cases with histological diagnoses of germinoma. The 1st case was a 10-year-old boy with Down's syndrome complaining of seizure and left hemiparesis. Computed tomographic (CT) scan and magnetic resonance imaging (MRI) showed a mass lesion in the right basal ganglia and thalamus. Histological examination indicated two cell pattern germinomas. The 2nd case was a 20-year-old man with Down's syndrome complaining of headache and vomiting. CT scan and MRI showed a pineal region tumor with marked hydrocephalus. Surgical specimens showed typical germinoma. Only 13 cases of brain tumors associated with Down's syndrome have been reported. A higher incidence of germ cell tumors seems to be related to chromosomal abnormalities.
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PMID:Intracranial germinoma associated with Down's syndrome. Report of 2 cases. 987 49

The frequency and severity of neurologic symptoms in children with systemic cancer is unknown. The authors reviewed the records of children with systemic cancer for whom a neurologic consultation was requested between 1993 and 1996. The 157 patients had 161 malignancies and 205 consultations. Leukemia (59) and lymphoma (34) were the most common malignancies. The 68 solid tumors included neuroblastoma (13), Ewing's sarcoma, and rhabdomyosarcoma (10 each). In contrast to adults, in whom back pain and altered mental status are the most common reasons for neurologic consultation, headache (33) and seizures (29) were the most common symptoms in children. Structural lesions were present in 84% of patients with headache and focal deficit and in 14% of patients with isolated headache. Structural disease was identified in 37% of children with seizures. Neurologic signs were caused by complications of cancer therapy in 70 instances and to direct tumor invasion of the nervous system in 60. In 71 consultations, neurologic symptoms could not be attributed to cancer or its treatment. The spectrum of neurologic symptoms in children with cancer differs from adults and requires the consulting neurologist to have a thorough knowledge of childhood cancer and its effects on the nervous system.
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PMID:Neurologic consultations in children with systemic cancer. 1008 40

Delirium is common among cancer patients, especially those with advanced disease. Typical treatment involves addressing the underlying cause if possible; eliminating nonessential and/or other drugs that can worsen confusion, manipulating the environment; and administering antipsychotic drugs to control symptoms and agitated behavior, and attempt to clear the patient's sensorium. The newer atypical antipsychotics may have potential in the treatment of delirium and also have the added benefit of causing less akithisia and other extrapyramidal side effects. This is illustrated by the case of a 59-year-old woman with leukemia and pain of unclear etiology who developed a delirium and a moderate to severe extrapyramidal syndrome (EPS) in the setting of escalation of her pain medications and concomitant escalation of prochlorperazine. The patient presented with confusion and moderate to severe cogwheeling rigidity, masked facies, bradykinesia, and tremor. Additionally, the patient had a relatively recent history of subdural hematoma and one seizure. Conservative management including eliminating multiple nonessential medications (including the prochlorperazine); changing her opioid analgesic; providing a 24-hour companion: and administering low doses of haloperidol (0.5 mg-2.0 mg) were not effective in treating the patient's delirium. The patient's EPS was dramatically worse following haloperidol doses. After approximately I week without improvement, the patient was started on olanzapine 5 mg daily with initial improvement but with residual confusion in the evenings and overnight. The dose was titrated up to 10 mg nightly with 2.5 mg as needed during the day. After 3 days on this regimen, the patient's mental status exam was normal and she was discharged home. We discuss the potential utility of this atypical antipsychotic in the palliative care setting.
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PMID:Complicated delirium in a cancer patient successfully treated with olanzapine. 1009 65

Rural Efforts to Assist Children at Home (REACH) is a service/demonstration and training project designed to provide specialized health care and case management services to medically dependent children in a 16-county area in central FLorida. REACH nurses, acting as Health Care Coordinators, provide instruction, consultation, coordination, and supervision of health care services in collaboration with tertiary care physicians and services at the University of Florida Health Center. Patients include infants, children, and adolescents with a wide variety of chronic illnesses including leukemia, muscular dystrophy, pulmonary disorders, failure to thrive, and seizure disorders. In addition to extensive work with families in the home, the REACH program incorporates a feedback system for its nurses, a progress-oriented record system, carefully designed agreements between tertiary care centers and community agencies, and an innovative training program for its nurses. REACH intends to serve about 1000 children during its 3-year demonstration phase. Success will be based on careful monitoring of costs, family functioning, and school attendance. If successful, the project will provide a model for the structured utilization of health and social services for families with medically dependent children.
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PMID:The REACH Project: an innovative health delivery model for medically dependent children. 1026 95

There is a growing body of information about the soluble forms of HLA in serum but there are only a few reports discussing sHLA in other body fluids. We quantitated sHLA-I and sHLA-II concentrations in sweat, saliva and tear samples from five normal individuals with known HLA-phenotypes. We also studied sweat samples from an additional 12 normal nonphenotyped subjects, as well as in CSF of 20 subjects with different illnesses, using solid phase enzyme linked immunoassay. Sweat, saliva and tears from normal subjects were found to contain very low or nondetectable amounts of sHLA-I. In contrast, sHLA-II molecules were found in each of these body fluids, although, with considerable variation between individuals. The presence of sHLA-II in saliva was further confirmed by Western-blotting. It was observed that sHLA-II having molecular mass of 43,900 and 18,100 daltons was comparable with that found in serum from normal individuals. In addition, no association of sHLA-II levels with allospecificities in either body fluid or in serum was apparent. The results of CSF sHLA concentrations in different diseases were as follows: (1) High CSF SHLA-I levels were measured during viral encephylitis (n = 3), while none of these patients contained sHLA-II in CSF; (2) The levels of sHLA-II, but not sHLA-I were elevated in CSF of patients during seizure (n = 6) and of patients with neonatal hepatitis (1 of 2) or with connective tissue disease accompanied with viral infection (n = 2); (3) No CSF sHLA-I or sHLA-II could be detected at polyneuropathy (n = 2), or in patients with syphilis (n = 3), or leukemia (n = 2) with evidence of neurologic involvement of central nervous system. Taken together, it may be concluded that the presence of sHLA in several body fluids is physiologically normal. It appears that sHLA-II is the predominant class of HLA molecules present in different body fluids. We propose that the system responsible for sHLA-II production in various body fluids must involve different mechanisms than those responsible for sHLA-I synthesis in serum.
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PMID:Soluble HLA in human body fluids. 1032 60


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