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Biomedical subjects

Banu Anlar

Publications and source records attributed to Banu Anlar.

At least 19 recordsLinked to original sources
PubMed2007

Signaling lymphocyte activating molecule (SLAM) expression in subacute sclerosing panencephalitis.

Signaling lymphocyte activating molecule (SLAM) is a receptor for measles virus which also has immunomodulatory activity. We analyzed SLAM expression in mononuclear cells (MNC) of patients with SSPE (n=7) and control subjects (n=7) from the same population. Native 10% PAGE analysis in cell and brain tissue extracts followed by Western blotting using monoclonal anti-human SLAM showed four types of bands. Differences in the type and amount of SLAM expression were observed between SSPE and control cases. Lymphocytes of SSPE patients showed two types of SLAM bands in comparison to only one in control lymphocytes. Stimulation of cells with lipopolysaccharide (80 u/ml) and concanavalin A (1 microg/ml) in vitro led to the appearance of a second isoform in both groups. Brain homogenates of SSPE patients (n=2) displayed all four types of SLAM isoforms at significantly higher levels than those of control brains (n=2). Our results show native PAGE enables the detection of all SLAM isotypes. The expression of SLAM is increased in lymphocytes, monocytes, and brain tissues of SSPE patients.

Antigens, CD↗
PubMed2006

MR imaging, diffusion-weighted imaging and MR spectroscopy findings in acute rapidly progressive subacute sclerosing panencephalitis.

We are presenting two cases with subacute sclerosing panencephalitis of acute and rapid form, which were initially diagnosed as acute viral encephalitis. We obtained both diffusion-weighted imaging and magnetic resonance spectroscopy, whose findings suggested the presence of acute inflammatory and metabolic changes with peculiar distribution, which were not observed on conventional MR imaging. These techniques might help to demonstrate the extent and progression of the panencephalitis.

Anticonvulsants↗
PubMed2006

Molecular genetic analyses in neurofibromatosis type 1 patients with tumors.

Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant disorders. NF1 is clinically characterized by neurofibromas, pigmentation anomalies, and an increased risk of malignant tumors. The NF1 gene product, neurofibromin, has a GTPase-activating protein domain (GRD) that interacts with the Ras protein, which is crucial in regulating signal transduction and cell proliferation/differentiation. We performed mutation analyses in the NF1-GRD region (exons 21-27a) and in exons 4b, 16, 29, and 37, and intron 28 in 17 NF1 patients with tumors. We identified a large deletion in the NF1 gene in a patient with a rhabdomyosarcoma as well as a variation in intron 22 in a patient with an optic glioma. We also found a 4-base pair deletion in another patient with optic glioma. In addition, allelic loss of the NF1 locus was shown in a pilocytic astrocytoma. Functional analyses of mutations in the NF1 gene may provide further insights into the pathogenesis of NF1 tumors.

Adolescent↗
PubMed2006

Atypical clinical course in juvenile metachromatic leukodystrophy involving novel arylsulfatase A gene mutations.

A male and female with juvenile metachromatic leukodystrophy (MLD) with unusual manifestations are presented, each involving a novel arylsulfatase A gene mutation. One patient demonstrated acute intermittent encephalopathic episodes for 1 year after having received the diagnosis of MLD at the age of 6 years. The other patient presented at the age of 5 years with acute hemiparesis, which was diagnosed as acute disseminated encephalomyelitis and resolved in 3 weeks. After 2 years of remission he started to show progressive neurological deterioration. The episodic manifestations in both patients were associated with acute, resolving cerebral lesions on magnetic resonance imaging accompanying or preceding the classical demyelinating lesions of MLD. The diagnosis of MLD was based on arylsulfatase A enzyme activity levels and genetic analysis, and after the exclusion of neurological conditions such as encephalitis, vasculopathy, or mitochondrial disorders. The pathogenesis of this previously undescribed finding in MLD is unknown but might be related to a susceptibility of myelin to acute damage.

Acyclovir↗
PubMed2006

Phenylketonuria in pediatric neurology practice: a series of 146 cases.

The neurologic manifestations of patients with phenylketonuria treated at different ages are illustrated in this series of 146 cases, including 9 sib pairs. In addition to well-known findings such as mental retardation, autistic features, microcephaly, and tremor, motor retardation was common and responded promptly to dietary treatment. Hypotonia and diminished reflexes were more frequent findings than hypertonia. Four sib pairs showed divergent features, such as the later-treated sibling having higher function than the early-treated one. Because siblings have a similar genotype and similar environmental and dietary conditions, this observation can be explained by differences in phenylalanine transport to the brain or additional metabolic or perinatal factors influencing the neurologic outcome.

Age Factors↗
PubMed2005

Lymphocyte subsets, TNF alpha and interleukin-4 levels in treated and untreated subacute sclerosing panencephalitis patients.

Immunologic studies in relation to clinical status might help to understand the pathogenesis of subacute sclerosing panencephalitis (SSPE) and the effect of treatment. We measured lymphocyte subsets and intracellular TNFalpha and interleukin-4 levels in peripheral blood in SSPE patients. Patients had elevated percentages of CD8+ cells compared to age-matched control children. Rapidly progressive course was associated with increased CD4+ cells. Treatment with interferons and inosiplex altered the percentage of CD3+, CD4+ and CD19+ cells. TNFalpha and interleukin-4 levels had no correlation with course or treatment. The proportions of lymphocyte subsets appear to have a role in the evolution or manifestations of SSPE, if not in the pathogenesis.

Adolescent↗
PubMed2005

Serological follow-up in juvenile myasthenia: clinical and acetylcholine receptor antibody status of patients followed for at least 2 years.

Thirty cases of juvenile myasthenia with at least 2 years' follow-up and one measurement of acetylcholine receptor antibody titer were evaluated. Symptoms were initially ocular in 15 (50%) cases; only one developed generalized disease and others remained ocular in 2-24 years' follow-up. Antibodies to acetylcholine receptor (AchRAb) were absent in 12/30 (40%) of all patients initially, but became positive in 5/28 (18%) on further testing in 1-5 years. Spontaneous remission was observed in 6 cases (20%) of whom one became seropositive, and late relapses after 5-11 years' remission were observed in 3 patients with ocular myasthenia, of whom only one developed AchRAbs. Our results show ocular JM has an exceptional tendency to remain confined to these muscles, and delaying the classification of the disease as AchRAb-negative for a few years and repeating serological tests is advisable in the follow-up.

Adolescent↗
PubMed2005

Antibodies to borna disease virus in subacute sclerosing panencephalitis.

Mechanisms causing persistence and reactivation of measles virus in subacute sclerosing panencephalitis (SSPE) are unknown. Borna disease virus (BDV) frequently causes latent or persistent infection in the nervous system. We investigated a possible association of these viruses in SSPE. Although BDV seropositivity was similar in SSPE and control groups, SSPE patients with high antibodies to BDV had earlier and more rapid disease. The findings suggest that BDV might be involved in the course, but not in the etiopathogenesis, of SSPE.

Adolescent↗
PubMed2005

Childhood case of progressive multifocal leukoencephalopathy with improved clinical outcome.

A 6-year-old boy who had been in remission from acute lymphoblastic leukemia for 2.5 years presented with seizures, hemiparesis, visual loss, and white- and gray-matter lesions on cranial magnetic resonance imaging. The diagnosis of progressive multifocal leukoencephalopathy was established on the detection of JC virus DNA by polymerase chain reaction in brain tissue. The patient was administered several anticonvulsants, amantadine, acyclovir, and ganciclovir. He showed partial recovery. This case illustrates the possibility of long-term survival in progressive multifocal leukoencephalopathy with normal immunologic parameters.

Anticonvulsants↗
PubMed2005

Reversible posterior leukoencephalopathy syndrome: report of three cases.

Reversible posterior leukoencephalopathy syndrome is characterized clinically by headache, abnormalities of mental status and visual perception, and seizures. Despite its diverse causes, common precipitating factors are defined as abrupt elevations of blood pressure, renal decompensation, fluid retention, and immunosuppressive therapy. We report three children with reversible posterior leukoencephalopathy syndrome presenting with generalized seizures and headache. The causes of reversible posterior leukoencephalopathy syndrome were considered to be acute hypertension and immunosuppressive therapy in case 1 with systemic lupus erythematosus, chemotherapy (vincristine and/or actinomycin-D) and hyponatremia in case 2, and acute hypertension in case 3, admitted with a familial Mediterranean fever attack. In light of these cases, we review the literature for the etiology, clinical and laboratory findings, and pathogenetic mechanisms of the disease.

Adolescent↗
PubMed2004

Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis.

BACKGROUND: Few effective treatment methods are available for subacute sclerosing panencephalitis (SSPE),an infection associated with the measles virus. Interferons have shown some benefit in previous studies and clinical practice. OBJECTIVE: The purpose of this study was to compare the efficacy of 2 different regimens of interferon-beta(IFN-beta) in the treatment of SSPE in pediatric patients. METHODS: We retrospectively compared the results obtained with 2 regimens of IFN-beta1a: 60 microg administered intramuscularly once weekly (IFN-beta 1/wk), or 22 microg administered subcutaneously 3 times per week (IFN-beta 3/wk). All patients also received oral inosiplex 50 to 100 mg/kg daily, a treatment known to have partial efficacy in SSPE. Patients who continued treatment for at least 3 months and had at least 1 year of follow-up data were evaluated. Clinical parameters included the Neurological Disability Index (NDI), a measurement of mental, motor, and sensory functions; disease stage; and mental status. Data obtained at 6 and 12 months were compared with those at the time of diagnosis, and the percent change from baseline was calculated. A satisfactory clinical response was defined as reduction or stabilization of the NDI or stage improvement at 6 or 12 months. RESULTS: Patients treated with IFN-beta 3/wk had increased survival time (P < 0.02) and higher clinical response rates compared with those treated with IFN-beta 1/wk (P < 0.05). When stage 2 and stage 3 patients were evaluated separately, survival was significantly longer (P = 0.007) and the rate of progression slower in both stage groups with IFN-beta 3/wk. CONCLUSION: The results obtained for this patient sample suggest that IFN-beta administered subcutaneously times per week combined with inosiplex may be an effective treatment option in SSPE. This treatment regimen warrants further study.

Adjuvants, Immunologic↗
PubMed2004

Familial segmental neurofibromatosis.

Segmental neurofibromatosis is considered to be the result of postzygotic NF1 gene mutations. We present a family in which the proband has generalized neurofibromatosis 1, whereas members of previous generations manifest segmental skin lesions. All, including the clinically asymptomatic grandmother, carry the same haplotype. This is the only case in the literature in which a parent with segmental skin findings has a child with full-blown neurofibromatosis 1 disease. The genetic mechanisms underlying this association are discussed. This family can be further investigated by examination of tissue samples from affected and unaffected sites for mutations.

Brain Neoplasms↗
PubMed2004

Parvovirus B19 infection presenting as pre-B-cell acute lymphoblastic leukemia: a transient and progressive course in two children.

Parvovirus B19 is the causative agent of various forms of hematologic diseases such as aplastic crisis in patients with hemolytic anemia, aplastic anemia, hypoplastic anemia, and idiopathic thrombocytopenic purpura. In addition, parvovirus B19 infection may precede or be associated with acute lymphoblastic leukemia (ALL). The authors present two cases of parvovirus B19 infection and bone marrow infiltration with pre-B-cell lymphoblasts; one patients was diagnosed as having ALL, and the other patient, with neurologic findings, showed total resolution of the blastic morphology and phenotype.

Antibodies, Viral↗
PubMed2003

Transient intrauterine hypotension causes apoptosis in fetal rat brain and affects learning.

Hypotensive episodes are frequent during pregnancy, and their functional effect on fetal brain has not been studied. We produced systemic hypotension for 30 min during mid-gestation in pregnant rats and examined their offspring on postnatal days 1 and 28. When compared with sham controls, the brain of the hypotensive group contained more TUNEL-positive cells in the hippocampal and periventricular regions on both time points. Spatial learning assessed by water milk maze test was impaired in 28-day-old pups of the hypotensive mothers. According to these results, transient maternal hypotension can induce apoptotic cell death in fetal brain and affect learning. Similar mechanisms may be considered and investigated in the pathogenesis of human learning disorders.

Animals↗