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Sample records for multifocal leukoencephalopathy epidemiology

  1. Progressive multifocal leukoencephalopathy: new concepts

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    Marco A. Lima

    2013-09-01

    Full Text Available Progressive multifocal leukoencephalopathy (PML is a demyelinating disease of the CNS caused by reactivation of JC virus (JCV in a setting of cellular immunosuppression. Originally, PML was observed in patients with advanced HIV infection, lymphoproliferative disorders and transplant recipients. However, the widespread use of HIV antiretroviral drugs and the new selective immunomodulatory and immunosuppressive medications, such as Rituximab and Natalizumab, has recently modified the epidemiology, clinical presentation and prognosis of PML. Herein, we discuss the new concepts on PML, emphasizing the recent modification in the epidemiology; the impact of new immunomodulatory treatments in the disease, PML-IRIS (Immune reconstitution inflammatory síndrome, new treatment strategies and other JCV related CNS diseases.

  2. Rituximab-Associated Inflammatory Progressive Multifocal Leukoencephalopathy

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    Schofield, Christina; Harris, Penelope

    2016-01-01

    Progressive multifocal leukoencephalopathy (PML) is a rare disease of the immunosuppression that results from neurotropic invasion of the JC virus which leads to demyelination of oligodendrocytes. Immune reconstitution inflammatory syndrome (IRIS), on the other hand, is a condition of inflammation that develops as the immune system reconstitutes. This case report describes a case of a 35-year-old HIV-negative male who presented with three weeks of right lower extremity paresthesias as well as right upper extremity apraxia. He was diagnosed with PML complicated by IRIS secondary to Rituximab, which he had completed four months prior to presentation. Despite the condition's poor prognosis, the patient recovered with only minor deficits. PMID:27965904

  3. Drug-induced progressive multifocal leukoencephalopathy

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    Vermeer, N S; Straus, S M J M; Mantel-Teeuwisse, A K;

    2015-01-01

    Progressive multifocal leukoencephalopathy (PML) has been identified as a serious adverse drug reaction (ADR) of several immunomodulatory biologicals. In this study, we contrasted the reporting patterns of PML for two biologicals for which the risk was identified at different points...... in their lifecycle: natalizumab (before reapproval) and rituximab (nine years postapproval). We found that, apart from the differences in clinical characteristics (age, gender, indication, time to event, fatality), which reflect the diversity in context of use, PML reports for natalizumab were more complete and were...... received sooner after occurrence. This study serves as an important reminder that spontaneous reports should only be used with great caution to quantify and compare safety profiles across products over time. The observed variability in reporting patterns and heterogeneity of PML cases presents challenges...

  4. Smoldering myeloma presenting as progressive multifocal leukoencephalopathy: a case report

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    Troppmann Martina

    2012-07-01

    Full Text Available Abstract Introduction Progressive multifocal leukoencephalopathy is an opportunistic infection occurring in patients with severe cellular immunodeficiency. This case highlights the role of cellular immunodeficiency in the reactivation of John Cunningham virus in a case of an early stage plasmacytoma. Case presentation A 76-year-old Caucasian woman presented with progressive left-sided hemiparesis, accompanied by hypoesthesia, hypoalgesia and neuropsychological symptoms. Magnetic resonance imaging demonstrated new hyperattenuating lesions in the right thalamus and left-sided subcortically. A polymerase chain reaction test revealed 4500 copies of John Cunningham virus-deoxyribonucleic acid/ml in cerebrospinal fluid. Human immunodeficiency virus infection was ruled out. A bone marrow biopsy showed an early stage immunoglobulin G-kappa plasmacytoma. Cidofovir (5mg/kg weekly for three weeks was started. A significant improvement of her neuropsychological symptoms was achieved, but motor system and sensory symptoms did not change. Conclusions This case shows a rapid course of progressive multifocal leukoencephalopathy with severe residual deficits. In the diagnostic workup of all patients with atypical neurologic symptoms or immunodeficiency, progressive multifocal leukoencephalopathy should be included as a differential diagnosis.

  5. A role for susceptibility weighted imaging in progressive multifocal leukoencephalopathy

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    Yap, SM

    2017-04-01

    We report a radiologic finding on magnetic resonance imaging (MRI) of the brain of two cases of progressive multifocal leukoencephalopathy (PML) of hypointense signal of subcortical U-fibres on susceptibility weighted (SW) sequence. The first case is a 50-year-old man recently treated with chemotherapy including rituximab for non-Hodgkin\\'s lymphoma. The second case is a 64-year-old woman with human immunodeficiency virus (HIV) infection. Iron deposition is a likely causative factor. We propose that SWI may be especially useful in the assessment of indeterminate cases to reduce the likelihood of a missed diagnosis of PML

  6. Progressive Multifocal Leukoencephalopathy: Endemic Viruses and Lethal Brain Disease.

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    Haley, Sheila A; Atwood, Walter J

    2017-06-21

    In 1971, the first human polyomavirus was isolated from the brain of a patient who died from a rapidly progressing demyelinating disease known as progressive multifocal leukoencephalopathy. The virus was named JC virus after the initials of the patient. In that same year a second human polyomavirus was discovered in the urine of a kidney transplant patient and named BK virus. In the intervening years it became clear that both viruses were widespread in the human population but only rarely caused disease. The past decade has witnessed the discovery of eleven new human polyomaviruses, two of which cause unusual and rare cancers. We present an overview of the history of these viruses and the evolution of JC polyomavirus-induced progressive multifocal leukoencephalopathy over three different epochs.Wereview what is currently known about JC polyomavirus, what is suspected, and what remains to be done to understand the biology of how this mostly harmless endemic virus gives rise to lethal disease. Expected final online publication date for the Annual Review of Virology Volume 4 is September 29, 2017. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.

  7. Multifocal inflammatory leukoencephalopathy induced by accidental consumption of levamisole: A case report

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    Sariaslani, Payam; Ghanbari, Parvin

    2012-01-01

    Levamisole is an anthelmintic agent and also immunostimulant drug which is used to treat colorectal cancer. The present study aimed to show accidental consumption of levamisole alone induced multifocal inflammatory leukoencephalopathy. A 53-year-old male was admitted to the Neurology Department of Farabi Hospital (Kermanshah, Iran) with walking inability and recognition disorder. Following clinical examinations, the patient diagnosed as multifocal inflammatory leukoencephalopathy following levamisole consumption.The patient was treated with intravenous methylprednisolone followed by prednisolone. The magnetic resonance imaging (MRI) was done 1 month later and did not show a reduction or remission in the lesions. History of the patient showed that he had accidentally consumed levamisole 8 months ago. It seems that the consumption of levamisole can induce multifocal inflammatory leukoencephalopathy and delayed treatment of the patient with corticosteroid cannot diminish the neurotoxicity of levamisole. In addition, the cytotoxic dose of levamisole induces irreversible multifocal inflammatory leukoencephalopathy. PMID:24250864

  8. Multifocal inflammatory leukoencephalopathy induced by accidental consumption of levamisole: A case report.

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    Sariaslani, Payam; Ghanbari, Ali; Ghanbari, Parvin

    2012-01-01

    Levamisole is an anthelmintic agent and also immunostimulant drug which is used to treat colorectal cancer. The present study aimed to show accidental consumption of levamisole alone induced multifocal inflammatory leukoencephalopathy. A 53-year-old male was admitted to the Neurology Department of Farabi Hospital (Kermanshah, Iran) with walking inability and recognition disorder. Following clinical examinations, the patient diagnosed as multifocal inflammatory leukoencephalopathy following levamisole consumption.The patient was treated with intravenous methylprednisolone followed by prednisolone. The magnetic resonance imaging (MRI) was done 1 month later and did not show a reduction or remission in the lesions. History of the patient showed that he had accidentally consumed levamisole 8 months ago. It seems that the consumption of levamisole can induce multifocal inflammatory leukoencephalopathy and delayed treatment of the patient with corticosteroid cannot diminish the neurotoxicity of levamisole. In addition, the cytotoxic dose of levamisole induces irreversible multifocal inflammatory leukoencephalopathy.

  9. Multifocal Inflammatory Leukoencephalopathy Induced by Accidental Consumption of Levamisole: A Case Report

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    Ali Ghanbari

    2012-01-01

    Full Text Available Levamisole is an anthelmintic agent and also immunostimulant drug which is used to treat colorectal cancer. The present study aimed to show accidental consumption of levamisole alone induced multifocalinflammatory leukoencephalopathy. A 53-year-old male was admitted to the Neurology Department of Farabi Hospital(Kermanshah, Iran with walking inability and recognition disorder. Following clinical examinations, the patient diagnosed as multifocal inflammatory leukoencephalopathy following levamisole consumption.The patient was treated with intravenous methylprednisolone followed by prednisolone. The magnetic resonance imaging (MRI was done 1 month later and did not show a reduction or remission in the lesions. History of the patient showed that he had accidentally consumed levamisole 8 months ago. It seems that the consumption of levamisole can induce multifocal inflammatory leukoencephalopathy and delayed treatment of the patient with corticosteroid cannot diminish the neurotoxicity of levamisole. In addition, the cytotoxic dose of levamisole induces irreversible multifocal inflammatory leukoencephalopathy.

  10. Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology

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    Shala Ghaderi Berntsson

    2016-03-01

    Full Text Available Progressive multifocal leukoencephalopathy (PML caused by reactivation of the JC virus (JCV, a human polyomavirus, occurs in autoimmune disorders, most frequently in systemic lupus erythematosus (SLE. We describe a HIV-negative 34-year-old female with SLE who had been treated with immunosuppressant therapy (IST; steroids and azathioprine since 2004. In 2011, she developed decreased sensation and weakness of the right hand, followed by vertigo and gait instability. The diagnosis of PML was made on the basis of brain MRI findings (posterior fossa lesions and JCV isolation from the cerebrospinal fluid (700 copies/ml. IST was immediately discontinued. Cidofovir, mirtazapine, mefloquine and cycles of cytarabine were sequentially added, but there was progressive deterioration with a fatal outcome 1 year after disease onset. This report discusses current therapeutic choices for PML and the importance of early infection screening when SLE patients present with neurological symptoms. In the light of recent reports of PML in SLE patients treated with rituximab or belimumab, we highlight that other IST may just as well be implicated. We conclude that severe lymphopenia was most likely responsible for JCV reactivation in this patient and discuss how effective management of lymphopenia in SLE and PML therapy remains an unmet need.

  11. Risk stratification for progressive multifocal leukoencephalopathy in patients treated with natalizumab

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    Sørensen, Per Soelberg; Bertolotto, Antonio; Edan, Gilles

    2012-01-01

    Natalizumab is a highly effective immunomodulator in the treatment of multiple sclerosis (MS). Treatment with natalizumab has been associated with progressive multifocal leukoencephalopathy (PML), an infection of the central nervous system (CNS) caused by a pathogenic form of the normally benign JC...

  12. Diffusion-weighted imaging in patients with progressive multifocal leukoencephalopathy

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    Cosottini, M. [University of Pisa, Department of Neuroscience, Pisa (Italy); Service of Neuroradiology AO, Pisa (Italy); Tavarelli, C.; De Cori, S.; Bartolozzi, C. [University of Pisa, Department of Radiology, Pisa (Italy); Del Bono, L.; Doria, G. [Unit of Infectious Diseases AO, Pisa (Italy); Giannelli, M. [Unit of Medical Physics, Pisa (Italy); Michelassi, M.C. [Service of Neuroradiology AO, Pisa (Italy); Murri, L. [University of Pisa, Department of Neuroscience, Pisa (Italy)

    2008-05-15

    Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system due to JC polyoma virus infection of oligodendrocytes. PML develops in patients with impaired T-cell function as occurs in HIV, malignancy or immunosuppressive drugs users. Until now no imaging methods have been reported to correlate with clinical status. Diffusion-weighted imaging (DWI) is a robust MRI tool in investigating white matter architecture and diseases. The aim of our work was to assess diffusion abnormalities in focal white matter lesions in patients with PML and to correlate the lesion load measured with conventional MRI and DWI to clinical variables. We evaluated eight patients with a biopsy or laboratory-supported diagnosis of PML. All patients underwent MRI including conventional sequences (fluid attenuated inversion recovery-FLAIR) and DWI. Mean diffusivity (MD) maps were used to quantify diffusion on white matter lesions. Global lesion load was calculated by manually tracing lesions on FLAIR images, while total, central core and peripheral lesion loads were calculated by manually tracing lesions on DWI images. Lesion load obtained with the conventional or DWI-based methods were correlated with clinical variables such as disease duration, disease severity and survival. White matter focal lesions are characterized by a central core with low signal on DWI images and high MD (1.853 x 10{sup -3} mm2/s), surrounded by a rim of high signal intensity on DWI and lower MD (1.1 x 10{sup -3} mm2/s). The MD value of normal-appearing white matter is higher although not statistically significant (0.783 x 10{sup -3} mm2/s) with respect to control subjects (0.750 x 10{sup -3} mm2/s). Inter-rater correlations of global lesion load between FLAIR (3.96%) and DWI (3.43%) was excellent (ICC =0.87). Global lesion load on FLAIR and DWI correlates with disease duration and severity (respectively, p = 0.037, p = 0.0272 with Karnofsky scale and p = 0.0338 with

  13. Progressive multifocal leukoence-phalopathy presenting as homonymous hemianopia in a patient with acquired immunodeficiency syndrome

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    Amit Pandey

    2012-01-01

    Full Text Available We present a case of a Human Immunodeficiency Virus (HIV positive patient who was referred for retinal evaluation to rule out ophthalmic manifestations of Acquired Immunodeficiency Syndrome (AIDS. She complained of some disturbance in vision in both eyes. Fundus examination showed no abnormality. Perimetry, done to rule out optic nerve pathology, showed a left homonymous hemianopia. Magnetic Resonance Imaging (MRI scan showed features of Progressive Multifocal Leukoencephalopathy (PML. She had no other neurological symptoms or signs.

  14. Strategy in Regulatory Decision-Making for Management of Progressive Multifocal Leukoencephalopathy

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    Segec, A; Keller-Stanislawski, B; Vermeer, N S

    2015-01-01

    Progressive multifocal leukoencephalopathy (PML) has been observed after the use of several medicines, including monoclonal antibodies. As these drugs play important roles in the therapeutic armamentarium, it is important to address the challenges that this severe adverse reaction poses to the sa...... use of medicines. Considering the need for consistent outcomes of regulatory decisions, the European Medicines Agency Pharmacovigilance Risk Assessment Committee (PRAC) used PML as an example to develop a systematic approach to labeling and risk minimization....

  15. Capecitabine Induced Multifocal Leukoencephalopathy: Do We Have Always to Switch off the Chemotherapy?

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    Anastasia Bougea

    2016-01-01

    Full Text Available Capecitabine is a well tolerated and safe 5-fluorouracil agent for adjuvant, neoadjuvant chemotherapy or metastatic cases. Neurological side effects require discontinuation of chemotherapy. We report this unique case of a 50-year-old female, who presented an isolated episode of dysarthria and ataxia under bevacizumab, capecitabine, and oxaliplatin treatment due to reversible multifocal leukoencephalopathy that did not recur after readministration of chemotherapy.

  16. Progressive multifocal leukoencephalopathy restricted to the posterior fossa in a patient with systemic lupus erythematosus

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    Goncalves, Fabricio Guimaraes; Lamb, Leslie; Del Carpio-O' Donovan, Raquel, E-mail: goncalves.neuroradio@gmail.com [McGill University Health Center Montreal General Hospital (Canada)

    2011-11-15

    Progressive multifocal leukoencephalopathy is a neurological infectious disease caused by the John Cunningham polyoma virus (JCV), an opportunistic agent with worldwide distribution. This disease is frequently seen in immunosuppressed patients and rarely associated with systemic lupus erythematosus. In the central nervous system PML demyelinating lesions occur in the supratentorial compartment. The authors describe a rare case of PML secondary to SLE treatment with atypical presentation restricted to the posterior fossa (author)

  17. Visual loss due to progressive multifocal leukoencephalopathy in a heart transplant patient.

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    Lewis, A R; Kline, L B; Pinkard, N B

    1993-12-01

    A 59-year-old male heart transplant recipient experienced progressive visual loss following routine cataract surgery with intraocular lens implantation. Cranial magnetic resonance (MR) imaging suggested and brain biopsy confirmed the cause to be progressive multifocal leukoencephalopathy (PML). The patient died 2 months following cataract surgery. This case illustrates that visual failure may be the initial manifestation of PML, and the ophthalmologist should be aware of this central demyelinating disorder when dealing with immunocompromised patients.

  18. Recurrence of progressive multifocal leukoencephalopathy despite immune recovery in two HIV seropositive individuals.

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    Crossley, Kate M; Agnihotri, Shruti; Chaganti, Joga; Rodriguez, Michael L; McNally, Leon Patrick; Venna, Nagagopal; Turbett, Sarah E; Gutman, Matthew; Morey, Adrienne; Koralnik, Igor J; Brew, Bruce J

    2016-08-01

    We present two cases of recurrent progressive multifocal leukoencephalopathy (PML) in patients with long standing virally suppressed human immunodeficiency virus (HIV) and normal CD4+ T cell count who were taking stable regimens of highly active antiretroviral therapy (HAART). This has significant implications for other patients with a past history of PML, not just those with HIV but also those on medications such as natalizumab or fumarates.

  19. Progressive multifocal leukoencephalopathy after stem cell transplantation, unsuccessfully treated with cidofovir.

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    Osorio, S; de la Cámara, R; Golbano, N; Marti, E; Fedele, C G; Nieto, S; Manzanares, R; Fernández-Rañada, J M

    2002-12-01

    We report a patient with progressive multifocal leukoencephalopathy (PML) after autologous stem cell transplantation (SCT) for non-Hodgkin's lymphoma (NHL). This is an unusual association, and to date only seven cases have been reported. This is the first case of PML after SCT treated with cidofovir, and the fifth case treated with this drug in a patient without human immunodeficiency virus (HIV) infection. In the previous four patients treated with cidofovir the outcome was discouraging, as was the case in this patient.

  20. Confirmed case of levamisole-associated multifocal inflammatory leukoencephalopathy in a cocaine user.

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    Vitt, Jeffrey R; Brown, Ethan G; Chow, Daniel S; Josephson, S Andrew

    2017-04-15

    Levamisole is a common adulterant in cocaine and has previously been associated with a variety of serious complications including multifocal inflammatory leukoencephalopathy (MIL). There have been several reports of MIL in patients taking cocaine and, though suspected, the presence of levamisole was not confirmed. We present a case of a 63-year-old woman presenting with stupor and spastic quadraparesis found to have urine positive for cocaine and levamisole. An MRI brain revealed innumerable FLAIR hyperintensities with restricted diffusion and incomplete ring-enhancement. This is the first case to confirm the presence of levamisole in a patient with MIL associated with cocaine use.

  1. The effects of cidofovir on progressive multifocal leukoencephalopathy: an MRI case study

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    Cardenas, R.L.; Cheng, K.H. [Physics Dept., Texas Technical University, Lubbock (United States); Sack, K. [Mount Diablo Medical Center, Concord, CA (United States)

    2001-05-01

    MRI was used to study the effects of introducing cidofovir (HPMPC, Vistide) to the antiretroviral therapy of a 33-year-old white man diagnosed as having progressive multifocal leukoencephalopathy (PML) secondary to AIDS. In response to combined cidofovir and antiretroviral therapy he showed significant clinical improvement. MRI showed a decrease in extent of existing lesions, without new ones. Blood chemistry information obtained indicated some involvement of immunologic mechanisms: the CD4:8 ratio showed improvement from an average of 0.08 before treatment to 0.13 during therapy. (orig.)

  2. Dual Therapy with Cidofovir and Mirtazapine for Progressive Multifocal Leukoencephalopathy in a Sarcoidosis Patient

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    Jae Hyun Park

    2011-10-01

    Full Text Available Background: Progressive multifocal leukoencephalopathy (PML is a demyelinating central nervous system disease caused by JC virus (JCV reactivation in immunocompromised patients. The disease course of PML is often progressive, fatal and at present, there are few reports on successful treatment outcomes. Case Report: A 45-year-old man with systemic sarcoidosis presented with rapidly progressive dementia and right hemiparesis. The patient was diagnosed with PML as confirmed via brain biopsy and JCV PCR. With a combination treatment of cidofovir and mirtazapine, there was significant improvement of neurological symptoms without measurable functional deficit. Conclusion: This case suggests that dual therapy with cidofovir and mirtazapine might be an effective treatment option in PML patients with sarcoidosis.

  3. Mirtazapine and mefloquine therapy for non-AIDS-related progressive multifocal leukoencephalopathy.

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    Epperla, Narendranath; Medina-Flores, Rafael; Mazza, Joseph J; Yale, Steven H

    2014-12-01

    Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection of the human nervous system caused by the JC virus. We report what is, to the best of our knowledge, the second reported case using a combination of mefloquine and mirtazapine in a patient with non-AIDS-related PML with a good clinical outcome. Conversely, the recent trial of mefloquine in 21 patients with AIDS and 3 without AIDS failed to show a reduction of JC viral DNA levels in the cerebral spinal fluid. However, the positive clinical response seen in our patient after the initiation of this combination therapy suggests that further studies in the form of randomized controlled trials for the treatment of non-AIDS-related PML are warranted.

  4. Posterior reversible encephalopathy syndrome masquerading as progressive multifocal leukoencephalopathy in rituximab treated neuromyelitis optica.

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    Berger, Joseph R; Neltner, Janna; Smith, Charles; Cambi, Franca

    2014-11-01

    Both progressive multifocal leukoencephalopathy (PML) and posterior reversible encephalopathy syndrome (PRES) have been reported as complications of rituximab therapy. These disorders may appear indistinguishable on magnetic resonance imaging (MRI). We report on a 42 year old woman with neuromyelitis optica (NMO) of 10 years duration who developed extensive white matter disease affecting chiefly both parietal lobes 6 months after her first and only dose of rituximab. The MRI findings suggested the diagnosis of PML, but her history was more consistent with PRES. Ultimately, a brain biopsy was performed which was consistent with the diagnosis of PRES. PRES and PML may have overlapping symptomatology and be indistinguishable on MRI. An approach to distinguishing between these two disorders is addressed.

  5. Progressive multifocal leukoencephalopathy in a patient with acquired immunodeficiency syndrome (AIDS) manifesting Gerstmann's syndrome.

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    Saito, H; Sakai, H; Fujihara, K; Fujihara, K; Itoyama, Y

    1998-11-01

    We reported a case of acquired immunodeficiency syndrome (AIDS) via multiple blood transfusions, who manifested progressive multifocal leukoencephalopathy (PML) about 18 months after the development of AIDS. PML initiated with right hemiparesis, dysphasia, and Gerstmann's syndrome and resulted in death within 2 months after the onset. Neuroimaging examinations revealed white matter lesions mainly in the left posterior parietal lobe. The cortical gray matter also showed abnormal signal intensity. Peripheral CD4+ lymphocyte count was 81/microl. Routine cerebrospinal fluid (CSF) examinations were negative. CSF antibodies against herpes simplex virus, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus as well as serum antibody against toxoplasma gondii were negative. Though autopsy or biopsy of the brain was not performed, JC virus genomes were detected in the CSF sample by a polymerase chain reaction, and their sequencing showed unique alterations of the regulatory regions, characteristic to PML-type JC virus.

  6. Progressive Multifocal Leukoencephalopathy: Recent Advances and a Neuro-Ophthalmological Review.

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    Sudhakar, Padmaja; Bachman, David M; Mark, Alexander S; Berger, Joseph R; Kedar, Sachin

    2015-09-01

    Progressive multifocal leukoencephalopathy (PML) is a severe often fatal opportunistic infection of the central nervous system caused by reactivation of a ubiquitous polyoma virus, JC virus. Although typically characterized by multifocal asymmetric subcortical white matter lesions, it may be monofocal and affect the cortical gray matter. Among the broad spectrum of clinical manifestations that occurs with PML, visual complaints are common. Combination of representative personally observed cases of PML and comprehensive review of case series of PML from 1958 through 2014. Neuro-ophthalmic signs and symptoms were reported in approximately 20%-50% of patients with PML and can be the presenting manifestation in half of these. A majority of these presentations occur from damage to cerebral visual pathways resulting in visual field defects, cortical blindness, and other disorders of visual association. Given the decreased frequency of infratentorial and cerebellar involvement, ocular motility disorders are less common. Visual complaints occur in patients with PML and are often the presenting sign. Awareness of this condition is helpful in avoiding unnecessary delays in the diagnosis of PML and management of the underlying condition. Recent guidelines have established criteria for diagnosis of PML in the high-risk patient population and strategies to mitigate the risk in these populations.

  7. CT and MRI in progressive multifocal leukoencephalopathy. CT und MRT der progressiven multifokalen Leukenzephalopathie (PML)

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    Lanfermann, H. (Inst. und Poliklinik fuer Radiologische Diagnostik, Koeln Univ. (Germany)); Heindel, W. (Inst. und Poliklinik fuer Radiologische Diagnostik, Koeln Univ. (Germany)); Schroeder, R. (Neuropathologie des Inst. fuer Pathologie, Koeln Univ. (Germany)); Lackner, K. (Inst. und Poliklinik fuer Radiologische Diagnostik, Koeln Univ. (Germany))

    1994-07-01

    Radiological findings and course of progressive multifocal leukoencephalopathy in 14 patients (1 woman, 13 men; 13 HIV seropositive, 1 chronic lymphatic leukaemia) were analysed retrospectively and correlated with clinical symptoms. A total of 21 CT and 16 MRI studies were evaluated. CT scans and MR images of 9 patients, which had been obtained in less than two weeks, could be compared to each other. MRI was superior to CT: 6 lesions with a diameter of 1 cm and below were not detected on CT scans, in 5 patients the extent of lesions was underestimated. Cortical involvement, mass effect or signs of atrophy were missing. Only 1 of 65 lesions showed a tiny enhancement after Gd injection. Due to the pattern and spread of lesions, which showed a close correlation to the neurologic symptoms, three different types of PML are suggested: 1. Initial precentral demyelinisation with contralateral hemiparesis (n=8); 2. lesions in temporo-occipital locations with visual disturbances (n=2); 3. predominantly bilateral lesions of cerebellar white matter with ataxia (n=4). (orig.)

  8. Stratification and monitoring of natalizumab-associated progressive multifocal leukoencephalopathy risk: recommendations from an expert group.

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    McGuigan, C; Craner, M; Guadagno, J; Kapoor, R; Mazibrada, G; Molyneux, P; Nicholas, R; Palace, J; Pearson, O R; Rog, D; Young, C A

    2016-02-01

    The use of natalizumab for highly active relapsing-remitting multiple sclerosis (MS) is influenced by the occurrence of progressive multifocal leukoencephalopathy (PML). Through measurement of the anti-JCV antibody index, and in combination with the presence or absence of other known risk factors, it may be possible to stratify patients with MS according to their risk of developing PML during treatment with natalizumab and detect early suspected PML using MRI including a diffusion-weighted imaging sequence. This paper describes a practical consensus guideline for treating neurologists, based on current evidence, for the introduction into routine clinical practice of anti-JCV antibody index testing of immunosuppressant-naïve patients with MS, either currently being treated with, or initiating, natalizumab, based on their anti-JCV antibody status. Recommendations for the frequency and type of MRI screening in patients with varying index-associated PML risks are also discussed. This consensus paper presents a simple and pragmatic algorithm to support the introduction of anti-JCV antibody index testing and MRI monitoring into standard PML safety protocols, in order to allow some JCV positive patients who wish to begin or continue natalizumab treatment to be managed with a more individualised analysis of their PML risk.

  9. Cidofovir in combination with HAART and survival in AIDS-associated progressive multifocal leukoencephalopathy.

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    Kraemer, Christoffer; Evers, Stefan; Nolting, Thorsten; Arendt, Gabriele; Husstedt, Ingo W

    2008-04-01

    Progressive multifocal leukoencephalopathy is a demyelinating disease with a high mortality caused by the JC virus and occurs in about 5% of HIV-infected patients. Highly active anti-retroviral therapy (HAART) has a proven efficacy in prolonging the survival of patients with AIDS-associated PML, but there are differing opinions about adding cidofovir to the treatment of PML. To investigate the benefit of HAART combined with cidofovir, we retrospectively analysed the survival of 33 patients with AIDS-associated PML proven by PCR in CSF, biopsy or at autopsy. Additionally, we also analysed 37 patients with probable PML. Seventeen (51.5%) of the patients with confirmed PML were treated with HAART and 14 (42.4%) with cidofovir in any combination. Of these patients, 13 (39.4%) were treated with HAART and cidofovir in combination, four (12.1%) patients received only HAART without cidofovir and one (3%) patient received only cidofovir without HAART. Fifteen patients did not receive HAART or cidofovir. The cumulative survival was significantly longer in patients with HAART than in patients without HAART (p = 0.006), independent whether cidofovir was given or not. In comparison with single therapy with HAART, the combination of HAART and cidofovir showed no significant increase in survival (p = 0.435). Therefore, a benefit for cidofovir in addition to HAART in the treatment of PML in HIV-infected patients could not be proven.

  10. Mefloquine improved progressive multifocal leukoencephalopathy in a patient with immunoglobulin A nephropathy.

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    Shin, Jung-Won; Jung, Keun-Hwa; Lee, Soon-Tae; Moon, Jangsup; Lim, Jung-Ah; Byun, Jung-Ick; Park, Kyung-Il; Lee, Sang Kun; Chu, Kon

    2014-10-01

    We describe a patient with immunoglobulin A nephropathy who was diagnosed with progressive multifocal leukoencephalopathy (PML) and successfully treated with mefloquine, an antimalarial medication. A 67-year-old man with immunoglobulin A nephropathy presented to the hospital emergency room with fever and generalized tonic-clonic seizure. Cerebrospinal fluid (CSF) nested polymerase chain reaction (PCR) was positive for John Cunningham virus and brain MRI displayed high signal intensity in the white matter in the right parietal lobe without gadolinium enhancement. Tapering of prednisone did not arrest the disease progression and a new lesion was detected on the cerebellum. Administration of mefloquine stopped lesion progression and resulted in dramatic clinical improvement. The CSF nested PCR for the John Cunningham virus also became negative. In reviewing the literature, mefloquine has had a heterogeneous effect in PML patients, and P-glycoprotein polymorphism and proper dosage could contribute to the various effects seen. Mefloquine may be a favorable treatment option in some patients with PML, and P-glycoprotein polymorphism may play an important role in its efficacy. More large studies in other ethnic groups including polymorphism studies for the gene encoding P-glycoprotein (ABCB1/MDR1) and taking into account various underlying conditions with secondary immunosuppression should be carried out to investigate whether mefloquine is effective for treating PML.

  11. Remission of progressive multifocal leukoencephalopathy following highly active antiretroviral therapy in a man with AIDS

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    Yoganathan K

    2012-04-01

    Full Text Available Katie Yoganathan1, David Brown2, Kathir Yoganathan31Cardiff Medical School, Cardiff, Wales, UK; 2Virus Reference Department, Microbiology Services, Health Protection Agency, London, UK; 3Singleton Hospital, Abertawe Bro Morgannwg University Health Board, Swansea, UKAbstract: A 43-year-old Caucasian homosexual man with AIDS presented with blurring of vision, change of personality, and memory loss in March 1999. He had first been admitted 2 months previously for treatment of Pneumocystis jiroveci pneumonia. A magnetic resonance imaging scan on admission showed multiple white matter lesions involving both subcortical cerebral hemispheres and cerebellar regions, with no mass effect or surrounding edema. JC virus was detected by nested polymerase chain reaction in the cerebrospinal fluid. These findings were diagnostic of progressive multifocal leukoencephalopathy (PML. His CD4 count was 34 cells/mL, and his HIV ribonucleic acid level was 800,789 copies/mL. He was treated with a combination antiretroviral therapy. He was last reviewed in October 2011. He was fully independent socially and mentally, but he still had some residual neurologic signs with right-sided homonymous hemianopia and visual agnosia. His HIV ribonucleic acid level was undetectable, and his CD4 count was 574 cells/mm3. Although the median survival of patients with PML was poor before the antiretroviral therapy era, our patient, who is now aged 55 years, is still alive 12 years after the diagnosis. The diagnosis of PML and differential diagnosis of focal neurologic signs in HIV-positive patients are discussed in this case report.Keywords: HIV, focal neurologic signs, cerebral toxoplasmosis, primary brain lymphoma, ischaemic stroke

  12. Progressive multifocal leukoencephalopathy in common variable immunodeficiency: mitigated course under mirtazapine and mefloquine.

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    Kurmann, Rebekka; Weisstanner, Christian; Kardas, Piotr; Hirsch, Hans H; Wiest, Roland; Lämmle, Bernhard; Furrer, Hansjakob; Du Pasquier, Renaud; Bassetti, Claudio L; Sturzenegger, Mathias; Krestel, Heinz

    2015-12-01

    Demonstration of survival and outcome of progressive multifocal leukoencephalopathy (PML) in a 56-year-old patient with common variable immunodeficiency, consisting of severe hypogammaglobulinemia and CD4+ T lymphocytopenia, during continuous treatment with mirtazapine (30 mg/day) and mefloquine (250 mg/week) over 23 months. Regular clinical examinations including Rankin scale and Barthel index, nine-hole peg and box and block tests, Berg balance, 10-m walking tests, and Montreal Cognitive Assessment (MoCA) were done. Laboratory diagnostics included complete blood count and JC virus (JCV) concentration in cerebrospinal fluid (CSF). The noncoding control region (NCCR) of JCV, important for neurotropism and neurovirulence, was sequenced. Repetitive MRI investigated the course of brain lesions. JCV was detected in increasing concentrations (peak 2568 copies/ml CSF), and its NCCR was genetically rearranged. Under treatment, the rearrangement changed toward the archetype sequence, and later JCV DNA became undetectable. Total brain lesion volume decreased (8.54 to 3.97 cm(3)) and atrophy increased. Barthel (60 to 100 to 80 points) and Rankin (4 to 2 to 3) scores, gait stability, and box and block (7, 35, 25 pieces) and nine-hole peg (300, 50, 300 s) test performances first improved but subsequently worsened. Cognition and walking speed remained stable. Despite initial rapid deterioration, the patient survived under continuous treatment with mirtazapine and mefloquine even though he belongs to a PML subgroup that is usually fatal within a few months. This course was paralleled by JCV clones with presumably lower replication capability before JCV became undetectable. Neurological deficits were due to PML lesions and progressive brain atrophy.

  13. Pre-existing T- and B-cell defects in one progressive multifocal leukoencephalopathy patient.

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    Alessandra Sottini

    Full Text Available Progressive multifocal leukoencephalopathy (PML usually occurs in patients with severe immunosuppression, hematological malignancies, chronic inflammatory conditions or receiving organ transplant. Recently, PML has also been observed in patients treated with monoclonal antibodies. By taking advantage of the availability of samples from a multiple sclerosis (MS patient treated with natalizumab, the antibody anti-α4 integrin, who developed PML and was monitored starting before therapy initiation, we investigated the fate of T and B lymphocytes in the onset of PML. Real-time PCR was used to measure new T- and B-cell production by means of T-cell receptor excision circle (TREC and K-deleting recombination excision circle (KREC analysis and to quantify transcripts for CD34, terminal-deoxynucleotidyltransferase, and V pre-B lymphocyte gene 1. T- and B-cell subsets and T-cell heterogeneity were measured by flow cytometry and spectratyping. The data were compared to those of untreated and natalizumab-treated MS patients and healthy donors. Before therapy, a patient who developed PML had a low TREC and KREC number; TRECs remained low, while KRECs and pre-B lymphocyte gene 1 transcripts peaked at 6 months of therapy and then decreased at PML diagnosis. Flow cytometry confirmed the deficient number of newly produced T lymphocytes, counterbalanced by an increase in TEMRA cells. The percentage of naive B cells increased by approximately 70% after 6 months of therapy, but B lymphocyte number remained low for the entire treatment period. T-cell heterogeneity and immunoglobulins were reduced. Although performed in a single patient, all results showed that an immune deficit, together with an increase in newly produced B cells a few months after therapy initiation, may predispose the patient to PML. These findings indicate the TREC/KREC assay is a potential tool to identify patients at risk of developing PML and may provide insights into the immunological

  14. Maraviroc Failed to Control Progressive Multifocal Leukoencephalopathy-Associated IRIS in a Patient with Advanced HIV Infection

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    Mónica Rodríguez

    2014-01-01

    Full Text Available Due to the lack of therapeutic options for patients with progressive multifocal leukoencephalopathy-associated immune reconstitution inflammatory syndrome (PML-associated IRIS, maraviroc has generated expectations among the medical community. However, we report a patient with advanced HIV infection, who developed PML-associated IRIS and had a fatal outcome despite the addition of maraviroc to suppressive ART. Future studies are required to define the therapeutic role of maraviroc in PML-associated IRIS and differentiate individuals who may benefit from maraviroc from those who may develop neurological deterioration.

  15. Characteristics of progressive multifocal leukoencephalopathy clarified through internet-assisted laboratory surveillance in Japan

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    Nakamichi Kazuo

    2012-10-01

    Full Text Available Abstract Background Progressive multifocal leukoencephalopathy (PML, a rare but fatal demyelinating disease caused by JC virus (JCV, occurs mainly in immunocompromised patients. As PML develops in individuals with various underlying disorders sporadically and infrequently, a nationwide survey of PML is difficult. This study was conducted to elucidate the characteristics of PML in Japan through an internet-assisted laboratory surveillance program. Methods A diagnostic support system for PML was established using a real-time PCR assay of JCV DNA in cerebrospinal fluid (CSF, and requests for testing were received from clinicians via specialized websites. Medical histories of patients were collected through standardized questionnaires, and a database of CSF JCV loads and clinical information was created and analyzed. Results For 4 years from April 2007 to March 2011, CSF specimens from 419 patients were tested. Forty-eight individuals were found positive for JCV DNA in their CSF and were diagnosed with PML. PML primarily occurred not only in HIV-positive patients (33.3% but also in patients with hematologic disorders after receiving stem cell transplantation, chemotherapy, and/or immunosuppressive treatment (39.6%. The frequencies of PML cases among the subjects in these two categories were 20.3% and 23.5%, respectively. Although no significant features were observed with respect to CSF JCV loads in PML patients with an HIV infection or hematologic disorder, males were predominant in both groups (100% and 89.5%, respectively. The proportion of PML cases with autoimmune disorders (6.3% or solid-organ transplants (2.1% was smaller than those with HIV infection or hematologic disorders, probably due to the limited availability of therapeutic monoclonal antibodies and transplantation from brain dead donors. Conclusions The results suggest that the internet-assisted laboratory surveillance program might be a useful strategy for collecting precise real

  16. Insight into Metabolic 1H-MRS Changes in Natalizumab Induced Progressive Multifocal Leukoencephalopathy Brain Lesions

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    Ruth Schneider

    2017-09-01

    Full Text Available BackgroundProgressive multifocal leukoencephalopathy (PML is a severe complication of immunosuppressive therapies, especially of natalizumab in relapsing–remitting multiple sclerosis (MS. Metabolic changes within PML lesions have not yet been described in natalizumab-associated PML in MS patients.ObjectiveTo study metabolic profiles in natalizumab-associated PML lesions of MS patients by 1H magnetic resonance spectroscopy (1H-MRS at different stages during the PML course. To assess changes associated with the occurrence of the immune reconstitution inflammatory syndrome (IRIS.Methods20 patients received 1H-MRS and imaging at 3 T either in the pre-IRIS, IRIS, early-post-PML, or late post-PML setting. Five of these patients received individual follow-up examinations, including the pre-IRIS or IRIS phase. Clinical worsening was described by changes in the Karnofsky Performance Scale (KPS and the expanded disability status scale (EDSS 1 year before PML and scoring at the time of 1H-MRS.ResultsIn PML lesions, increased levels of the Lip/Cr ratio, driven by rising of lipid and reduction of Creatine, were found before the occurrence of IRIS (p = 0.014 with a maximum in the PML–IRIS group (p = 0.004. By contrast, marked rises of Cho/Cr in PML lesions were detected exclusively during the IRIS phase (p = 0.003. The Lip/Cr ratio decreased to above-normal levels in early-post-PML (p = 0.007, compared to normal appearing white matter (NAWM and to normal levels in the late-post-PML group. NAA/Cho was reduced compared to NAWM in the pre-IRIS, IRIS, and early-post-PML group. In NAA/Cr, the same effect was seen in the pre-IRIS and early-post-PML group. These cross-sectional results were confirmed by the individual follow-up examinations of four patients. NAA/Cho, Cho/Cr, and the lipid rise relative to NAWM in PML lesions were significantly correlated with the residual clinical worsening (KPS change in post-PML patients (Spearman

  17. Rituximab-associated progressive multifocal leukoencephalopathy derived from non-Hodgkin lymphoma: neuropathological findings and results of mefloquine treatment.

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    Sano, Yasuteru; Nakano, Yuta; Omoto, Masatoshi; Takao, Masaki; Ikeda, Eiji; Oga, Atsunori; Nakamichi, Kazuo; Saijo, Masayuki; Maoka, Takashi; Sano, Hironori; Kawai, Motoharu; Kanda, Takashi

    2015-01-01

    A 66-year-old man with non-Hodgkin lymphoma (NHL) developed progressive multifocal leukoencephalopathy (PML) after undergoing chemotherapy including rituximab. Although the administration of mefloquine at a dose of 500 mg weekly temporarily led to a dramatic decrease in the copy number of JC Virus DNA in the cerebrospinal fluid, the patient's symptoms gradually worsened. The CD4(+) T count remained continuously low, at least until approximately five months after the last cycle of chemotherapy. A postmortem examination performed 10 months after the onset of PML disclosed a severe condition associated with rituximab-treated PML originating from NHL and a high mefloquine concentration in the brain. The accumulation of further data regarding mefloquine treatment in PML cases may help to elucidate the optimal dosage and time window for effectively treating PML.

  18. Progressive Multifocal Leukoencephalopathy in a HIV-Negative Patient with Small Lymphocytic Leukemia following Treatment with Rituximab

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    Subhankar Chakraborty

    2011-03-01

    Full Text Available We describe a case of progressive multifocal leukoencephalopathy (PML caused by infection with the human polyomavirus JC virus in a patient with B-cell small lymphocytic leukemia who was treated with rituximab. The first symptoms of PML appeared immediately following the last of five cycles of rituximab, cyclophosphamide and pentostatin. Magnetic resonance imaging revealed changes consistent with PML, although JC virus DNA was not detected by polymerase chain reaction assay of the cerebrospinal fluid. A stereotactic biopsy of the brain showed histological changes consistent with PML, while electron microscopy revealed JC virus particles attached to the nuclei of astrocytes. The patient was treated supportively but died 53 days after the initial onset of symptoms.

  19. Leucoencefalopatia multifocal progressiva confirmada por PCR para vírus JC no líquor: relato de caso Progressive multifocal leukoencephalopathy confirmed by PCR: case report

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    Alessandro Comarú Pasqualotto

    2004-06-01

    Full Text Available Relatamos um caso de leucoencefalopatia multifocal progressiva, doença infecciosa desmielinizante do sistema nervoso central, seguido de revisão da literatura. Comenta-se a importância diagnóstica dos métodos de imagem, bem como da análise do líquor e da técnica de reação em cadeia da polimerase (PCR. Até o presente momento, não há terapia efetiva para a condição, com sobrevida média de 1-6 meses nos pacientes não tratados; evolução favorável pode ocorrer no subgrupo de pacientes que obtém melhora da imunidade com o uso de terapia anti-retroviral.A case of progressive multifocal leukoencephalopathy (PML is presented, with literature review. PML diagnosis and its differential diagnosis are presented, with emphasis on neuroradiology, cerebrospinal fluid analysis and polymerase chain reaction studies. The prognosis of PML is usually poor, with a median survival of 1-6 months. There is yet no proven effective treatment for this condition; HAART has become the standard of care for these patients.

  20. Failure of mefloquine therapy in progressive multifocal leukoencephalopathy: report of two Japanese patients without human immunodeficiency virus infection.

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    Kobayashi, Zen; Akaza, Miho; Numasawa, Yoshiyuki; Ishihara, Shoichiro; Tomimitsu, Hiroyuki; Nakamichi, Kazuo; Saijo, Masayuki; Morio, Tomohiro; Shimizu, Norio; Sanjo, Nobuo; Shintani, Shuzo; Mizusawa, Hidehiro

    2013-01-15

    Although progressive multifocal leukoencephalopathy (PML) cases showing responses to mefloquine therapy have been reported, the efficacy of mefloquine for PML remains unclear. We report on the failure of mefloquine therapy in two Japanese patients with PML unrelated to human immunodeficiency virus. One of the patients was a 47-year-old male who had been treated with chemotherapy for Waldenström macroglobulinemia, and the other was an 81-year-old male with idiopathic CD4(+) lymphocytopenia. Diagnosis of PML was established based on MRI findings and increased JC virus DNA in the cerebrospinal fluid in both patients. Mefloquine was initiated about 5 months and 2 months after the onset of PML, respectively. During mefloquine therapy, clinical and radiological progression was observed, and JC virus DNA in the cerebrospinal fluid was increased in both patients. Both patients died about 4 months and 2 months after initiation of mefloquine, respectively. Further studies are necessary to clarify the differences between mefloquine responders and non-responders in PML.

  1. A study of mefloquine treatment for progressive multifocal leukoencephalopathy: results and exploration of predictors of PML outcomes.

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    Clifford, David B; Nath, Avindra; Cinque, Paola; Brew, Bruce J; Zivadinov, Robert; Gorelik, Leonid; Zhao, Zhenming; Duda, Petra

    2013-08-01

    Immune reconstitution has improved outcomes for progressive multifocal leukoencephalopathy (PML), a potentially lethal brain disease caused by JC virus (JCV). However, an antiviral treatment to control JCV is needed when immune reconstitution is delayed or not possible. On the basis of in vitro efficacy, this study evaluated the effect of mefloquine on PML and factors that may predict PML outcomes. This 38-week, open-label, randomized, parallel-group, proof-of-concept study compared patients with PML who received standard of care (SOC) with those who received SOC plus mefloquine (250 mg for 3 days, then 250 mg weekly). Patients randomized to SOC could add mefloquine treatment at week 4. The primary endpoint was change from baseline to weeks 4 and 8 in JCV DNA copy number (load) in cerebrospinal fluid (CSF). Exploratory analyses evaluated factors that might correlate with clinical outcome. The majority of enrolled patients were HIV positive. Preplanned interim data analyses suggested that the study was unlikely to successfully demonstrate a significant difference between groups; therefore, the study was terminated prematurely. There was no significant difference between groups in CSF JCV DNA loads or clinical/MRI findings. Decrease in CSF JCV DNA load from baseline to week 4 was associated with a better clinical outcome at 16 weeks, as measured by Karnofsky scores. This study found no evidence of anti-JCV activity by mefloquine. An early decrease of CSF JCV DNA load appears to be associated with a better clinical outcome.

  2. Presymptomatic diagnosis with MRI and adequate treatment ameliorate the outcome after natalizumab-associated progressive multifocal leukoencephalopathy (PML.

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    Hans eLindå

    2013-02-01

    Full Text Available Natalizumab (Tysabri® is a monoclonal antibody that prevents inflammatory cells from binding to brain endothelial cells and passing into the brain parenchyma. Natalizumab is a highly effective treatment for relapsing-remitting multiple sclerosis (MS. Progressive multifocal leukoencephalopathy (PML is an opportunistic brain JC virus infection that has been shown to be associated with natalizumab treatment. We describe PML in a patient with MS after 44 monthly infusions of natalizumab. With the aid of a routine MRI scan, PML was detected before any unambiguous clinical manifestations had emerged. PML was treated with plasma exchange to accelerate removal of natalizumab. Mirtazapine and mefloquine was promptly added and approximately one month after plasma exchange, when an immune-reconstitution-inflammatory-syndrome (IRIS appeared, steroid treatment was initiated. Steroid treatment was then continued until no virus could be detected in the cerebrospinal fluid. The outcome was favourable. We believe that this case clearly illustrates the importance of an early, presymptomatic, detection of PML and an adequate treatment. We also propose that surveillance with MRI scans, every 3 months after 24 months of treatment, should be performed in JC virus antibody positive natalizumab-treated MS patients in order to detect PML in an early phase.

  3. Changes in JC virus-specific T cell responses during natalizumab treatment and in natalizumab-associated progressive multifocal leukoencephalopathy.

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    Molly R Perkins

    Full Text Available Progressive multifocal leukoencephalopathy (PML induced by JC virus (JCV is a risk for natalizumab-treated multiple sclerosis (MS patients. Here we characterize the JCV-specific T cell responses in healthy donors and natalizumab-treated MS patients to reveal functional differences that may account for the development of natalizumab-associated PML. CD4 and CD8 T cell responses specific for all JCV proteins were readily identified in MS patients and healthy volunteers. The magnitude and quality of responses to JCV and cytomegalovirus (CMV did not change from baseline through several months of natalizumab therapy. However, the frequency of T cells producing IL-10 upon mitogenic stimulation transiently increased after the first dose. In addition, MS patients with natalizumab-associated PML were distinguished from all other subjects in that they either had no detectable JCV-specific T cell response or had JCV-specific CD4 T cell responses uniquely dominated by IL-10 production. Additionally, IL-10 levels were higher in the CSF of individuals with recently diagnosed PML. Thus, natalizumab-treated MS patients with PML have absent or aberrant JCV-specific T cell responses compared with non-PML patients, and changes in T cell-mediated control of JCV replication may be a risk factor for developing PML. Our data suggest further approaches to improved monitoring, treatment and prevention of PML in natalizumab-treated patients.

  4. Aids-related progressive multifocal leukoencephalopathy: a retrospective study in a referral center in São Paulo, Brazil.

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    Vidal, José E; Penalva de Oliveira, Augusto C; Fink, Maria Cristina D S; Pannuti, Cláudio S; Trujillo, J Roberto

    2008-01-01

    Few data are available about progressive multifocal leukoencephalopathy (PML) in patients with acquired immunodeficiency syndrome (AIDS) from Brazil. The objectives of this study were to describe the main features of patients with PML and estimate its frequency among AIDS patients with central nervous system (CNS) opportunistic diseases admitted to the Instituto de Infectologia Emílio Ribas, São Paulo, Brazil, from April 2003 to April 2004. A retrospective and descriptive study was performed. Twelve (6%) cases of PML were identified among 219 patients with neurological diseases. The median age of patients with PML was 36 years and nine (75%) were men. Nine (75%) patients were not on antiretroviral therapy at admission. The most common clinical manifestations were: focal weakness (75%), speech disturbances (58%), visual disturbances (42%), cognitive dysfunction (42%), and impaired coordination (42%). The median CD4+ T-cell count was 45 cells/microL. Eight (67%) of 12 patients were laboratory-confirmed with PML and four (33%) were possible cases. Eleven (92%) presented classic PML and only one case had immune reconstitution inflammatory syndrome (IRIS)-related PML. In four (33%) patients, PML was the first AIDS-defining illness. During hospitalization, three patients (25%) died as a result of nosocomial pneumonia and nine (75%) were discharged to home. Cases of PML were only exceeded by cases of cerebral toxoplasmosis, cryptococcal meningoencephalitis, and CNS tuberculosis, the three more frequent neurologic opportunistic infections in Brazil. The results of this study suggest that PML is not an uncommon HIV-related neurologic disorder in a referral center in Brazil.

  5. Deep-Sequence Identification and Role in Virus Replication of a JC Virus Quasispecies in Patients with Progressive Multifocal Leukoencephalopathy.

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    Takahashi, Kenta; Sekizuka, Tsuyoshi; Fukumoto, Hitomi; Nakamichi, Kazuo; Suzuki, Tadaki; Sato, Yuko; Hasegawa, Hideki; Kuroda, Makoto; Katano, Harutaka

    2017-01-01

    JC virus (JCV) is a DNA virus causing progressive multifocal leukoencephalopathy (PML) in immunodeficient patients. In the present study, 22 genetic quasispecies with more than 1.5% variant frequency were detected in JCV genomes from six clinical samples of PML by next-generation sequencing. A mutation from A to C at nucleotide (nt) 3495 in JCV Mad1 resulting in a V-to-G amino acid substitution at amino acid (aa) position 392 of the large T antigen (TAg) was identified in all six cases of PML at 3% to 19% variant frequencies. Transfection of JCV Mad1 DNA possessing the V392G substitution in TAg into IMR-32 and human embryonic kidney 293 (HEK293) cells resulted in dramatically decreased production of JCV-encoded proteins. The virus DNA copy number was also reduced in supernatants of the mutant virus-transfected cells. Transfection of the IMR-32 and HEK293 cells with a virus genome containing a revertant mutation recovered viral production and protein expression. Cotransfection with equal amounts of wild-type genome and mutated JCV genome did not reduce the expression of viral proteins or viral replication, suggesting that the mutation did not have any dominant-negative function. Finally, immunohistochemistry demonstrated that TAg was expressed in all six pathological samples in which the quasispecies were detected. In conclusion, the V392G amino acid substitution in TAg identified frequently in PML lesions has a function in suppressing JCV replication, but the frequency of the mutation was restricted and its role in PML lesions was limited.

  6. [Progressive multifocal leukoencephalopathy associated to natalizumab: the importance of magnetic resonance imaging in its early diagnosis].

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    Martí, Glòria; Río, Jordi; Rovira, Àlex; Auger, Cristina; Tintoré, Mar; Sastre-Garriga, Jaume; Vidal, Anka; Castilló, Joaquín; Montalban, Xavier

    2015-02-16

    Introduccion. El natalizumab es un farmaco utilizado en la esclerosis multiple (EM), cuyo principal efecto adverso es el desarrollo de una leucoencefalopatia multifocal progresiva (LMP). Como esta es potencialmente mortal o discapacitante, el tratamiento debe suspenderse inmediatamente ante su sospecha, teniendo en cuenta el posible desarrollo posterior de un sindrome de reconstitucion inmune o rebrote de la EM. Caso clinico. Se describe un caso de LMP, inicialmente asintomatico, en el contexto del tratamiento con natalizumab en una paciente con EM. Como factores de riesgo se determinaron titulos altos de anticuerpos contra el virus John Cunningham (VJC) y mas de dos años de tratamiento. La reaccion en cadena de la polimerasa para el VJC en el liquido cefalorraquideo resulto negativa en dos determinaciones. El periodo entre el diagnostico radiologico y el inicio de la clinica fue de dos meses. Durante el curso de la enfermedad, la paciente desarrollo un sindrome inflamatorio de reconstitucion inmune y rebrotes de su EM. Presento una buena respuesta tras el inicio de tratamiento con fingolimod, una vez estabilizada la LMP. Conclusion. Este caso ilustra la importancia de una estrecha vigilancia clinicorradiologica en pacientes con EM tratados con natalizumab, sobre todo cuando presentan factores de riesgo para el desarrollo de LMP, asi como su potencial incidencia en la supervivencia y estado funcional final.

  7. Progressive Multifocal Leukoencephalopathy in a 62-Year-Old Immunocompetent Woman

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    Venkata C. Gourineni

    2014-01-01

    Full Text Available Progressive multifocal encephalopathy (PML is a rare demyelinating disease that typically presents in immunodeficient patients. We report a case of a previously healthy 62-Year-Old woman who suffered from an unsteady gait, throbbing headaches, and progressive left-sided weakness and numbness. Stroke was initially suspected based on imaging and symptoms. A series of follow-up magnetic resonance images of the brain showed a right parietal lesion growing in size as the patient became unable to walk and experienced increasing lethargy and confusion. A biopsy of the lesion was positive for the John Cunningham virus (JCV. A diagnosis of PML was made and she was started on mefloquine. No improvement was seen on this treatment and her condition worsened. Although PML remains uncommon in immunocompetent individuals, it cannot be ruled out based on their immune status. Although the exact cause remains uncertain, underlying or transient states of immunosuppression may be responsible for reactivation of the JCV in these patients.

  8. Progressive Multifocal Leukoencephalopathy

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    ... of the U.S. Food and Drug Administration. Hexadecyloxypropyl-Cidofovir (CMX001) is currently being studied as a treatment ... of the U.S. Food and Drug Administration. Hexadecyloxypropyl-Cidofovir (CMX001) is currently being studied as a treatment ...

  9. Progressive multifocal leukoencephalopathy secondary to rituximab-induced immunosuppression and the presence of John Cunningham virus: a case report and literature review.

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    Kelly, Deirdre; Monaghan, Bernadette; McMahon, Eileen; Watson, Geoffrey; Kavanagh, Eoin; O'Rourke, Killian; McCaffrey, John; Carney, Desmond

    2016-09-01

    We present the case of a 60-year-old man who developed subacute neurologic changes, in the setting of stage III non-Hodgkin's follicular lymphoma, and was treated with induction chemotherapy, followed by a year of maintenance rituximab. Magnetic resonance imaging of the brain with gadolinium was pathognomonic for progressive multifocal leukoencephalopathy (PML). He was treated with sequential plasmapheresis and intravenous immunoglobulin with clinical improvement. A literature review of the diagnostic workup of rituximab-induced PML was undertaken. This case and the literature review demonstrate the important role of magnetic resonance imaging of the brain in diagnosis and follow-up of rituximab-induced PML. Specific radiologic features in combination with cerebrospinal fluid can be diagnostic and avoid the morbidity and mortality of a diagnostic brain biopsy. Plasmapheresis and intravenous immunoglobulin have a therapeutic role and demonstrate symptom improvement and disease control. Follow-up imaging in combination with clinical response is important in demonstrating a treatment response.

  10. Efficient propagation of progressive multifocal leukoencephalopathy-type JC virus in COS-7-derived cell lines stably expressing Tat protein of human immunodeficiency virus type 1.

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    Nukuzuma, Souichi; Nakamichi, Kazuo; Kameoka, Masanori; Sugiura, Shigeki; Nukuzuma, Chiyoko; Miyoshi, Isao; Takegami, Tsutomu

    2010-12-01

    The high incidence of progressive multifocal leukoencephalopathy (PML) in AIDS patients compared with many other immunosuppressive diseases suggests that HIV-1 infection is strictly related to the activation of JC virus (JCV) propagation. In this report, propagation of PML-type JCV in COS-7-derived cell lines stably expressing HIV-1 Tat (COS-tat cells) has been examined. In COS-tat cells, production of viral particles and replication of genomic DNA were markedly increased compared to COS-7 cells, as judged by HA and real-time PCR analyses. These results demonstrate that COS-tat cells provide a useful model system for studying HIV-1 Tat-mediated propagation of PML-type JCV.

  11. Head tremor and progressive multifocal leukoencephalopathy in AIDS patients: report of two cases Tremor cefálico e leucoencefalopatia multifocal progressiva em pacientes com SIDA: relato de dois casos

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    Carlos R.M. Rieder

    2005-03-01

    Full Text Available Progressive multifocal leukoencephalopathy (PML is caused by replication of JC virus in oligodendrocytes of immunocompromised patients. Common manifestations are focal motor and sensory deficits, gait abnormalities, speech and language disturbances, cognitive disorders, headache, and visual impairment. Although the occurrence of movement disorders is rare in PML, bradykinesia, rigidity, dystonia, myoclonic jerks and myoclonic ataxia have been described. Head tremor associated with PML has not been previously reported. We report two cases of PML in whom head tremor was present.A leucoencefalopatia multifocal progressiva (LMP é causada pela replicação do vírus JC em oligodendrócitos de pacientes imunocomprometidos. As manifestações mais comuns incluem déficits motores e sensitivos, alterações da marcha, da fala e da linguagem, cefaléia e distúrbios visuais e cognitivos. Embora a presença de distúrbios do movimento não seja tão freqüente na LMP, bradicinesia, rigidez, abalos mioclônicos e ataxia mioclônica já foram descritos. Nós relatamos dois casos de LMP associados com tremor cefálico.

  12. Progressive multifocal leukoencephalopathy secondary to rituximab-induced immunosuppression and the presence of John Cunningham virus: a case report and literature review

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    Deirdre Kelly, MBBCH

    2016-09-01

    Full Text Available We present the case of a 60-year-old man who developed subacute neurologic changes, in the setting of stage III non-Hodgkin's follicular lymphoma, and was treated with induction chemotherapy, followed by a year of maintenance rituximab. Magnetic resonance imaging of the brain with gadolinium was pathognomonic for progressive multifocal leukoencephalopathy (PML. He was treated with sequential plasmapheresis and intravenous immunoglobulin with clinical improvement. A literature review of the diagnostic workup of rituximab-induced PML was undertaken. This case and the literature review demonstrate the important role of magnetic resonance imaging of the brain in diagnosis and follow-up of rituximab-induced PML. Specific radiologic features in combination with cerebrospinal fluid can be diagnostic and avoid the morbidity and mortality of a diagnostic brain biopsy. Plasmapheresis and intravenous immunoglobulin have a therapeutic role and demonstrate symptom improvement and disease control. Follow-up imaging in combination with clinical response is important in demonstrating a treatment response.

  13. Progressive Multifocal Leukoencephalopathy (PML) Development Is Associated With Mutations in JC Virus Capsid Protein VP1 That Change Its Receptor Specificity

    Science.gov (United States)

    Reid, Carl; Testa, Manuela; Brickelmaier, Margot; Bossolasco, Simona; Pazzi, Annamaria; Bestetti, Arabella; Carmillo, Paul; Wilson, Ewa; McAuliffe, Michele; Tonkin, Christopher; Carulli, John P.; Lugovskoy, Alexey; Lazzarin, Adriano; Sunyaev, Shamil; Simon, Kenneth; Cinque, Paola

    2011-01-01

    Progressive multifocal leukoencephalopathy (PML), a fatal demyelinating disease caused by JC virus (JCV) infection of oligodendrocytes, may develop in patients with immune disorders following reactivation of chronic benign infection. Mutations of JCV capsid viral protein 1 (VP1), the capsid protein involved in binding to sialic acid cell receptors, might favor PML onset. Cerebrospinal fluid sequences from 37/40 PML patients contained one of several JCV VP1 amino acid mutations, which were also present in paired plasma but not urine sequences despite the same viral genetic background. VP1-derived virus-like particles (VLPs) carrying these mutations lost hemagglutination ability, showed different ganglioside specificity, and abolished binding to different peripheral cell types compared with wild-type VLPs. However, mutants still bound brain-derived cells, and binding was not affected by sialic acid removal by neuraminidase. JCV VP1 substitutions are acquired intrapatient and might favor JCV brain invasion through abrogation of sialic acid binding with peripheral cells, while maintaining sialic acid–independent binding with brain cells. PMID:21628664

  14. Progressive multifocal leuko-encephalopathy studied by magnetization transfer imaging; Leucoencephalopathie multifocale progressive. Etude de la demyelinisation par transfert de magnetisation

    Energy Technology Data Exchange (ETDEWEB)

    Armand, J.P.; Dousset, V.; Mieze, S.; Caille, J.M. [Hopital Pellegrin, 33 - Bordeaux (France); Franconi, J.M. [Siemens SA, 92 - Saint-Denis (France). Service Instrumentation Scientifique; Lacoste, D. [Gecsa, Groupe d`Epidemiologie Clinique du Sida en Aquitaine, CHU, 33 - Bordeaux (France); Letenneur, L. [Bordeaux-2 Univ., 33 (France)

    1997-09-01

    Purpose: magnetization transfer imaging (MT) has bees used to study the degree of demyelination in progressive multifocal leuko-encephalopathy (PML). Material and method: two groups were studied: a group of 10 HIV + patients with clinical, MR features, biological and/or biopsy proven PLM, and a group of 11 normal volunteers with matched age. MT ratio (MTR) were obtained from the center of the PLM lesions and 11 areas of normal appearing white matter (NAWM) in the control group. Results: the mean MTR of NAWM in the control group was 46.6% (SD = 2,3). PLM lesions demonstrated a strong and significant (p = 0) decreased of the MTR with mean MTR value of 22.4% (SD = 2,3). Conclusion: MT characterized the demyelinating process in PLM, and can be to used to improve diagnosis. Furthermore, MT allowed a quantification of the degree of the demyelination which can be helpful in other demyelinating process of CNS such multiple sclerosis. (authors). 11 refs.

  15. Polyomavirus JC in the Context of Immunosuppression: A Series of Adaptive, DNA Replication-Driven Recombination Events in the Development of Progressive Multifocal Leukoencephalopathy

    Directory of Open Access Journals (Sweden)

    Edward M. Johnson

    2013-01-01

    Full Text Available Polyomavirus JC (JCV is the etiological agent of progressive multifocal leukoencephalopathy (PML, a demyelinating infection of oligodendrocytes in the brain. PML, a frequently fatal opportunistic infection in AIDS, has also emerged as a consequence of treatment with several new immunosuppressive therapeutic agents. Although nearly 80% of adults are seropositive, JCV attains an ability to infect glial cells in only a minority of people. Data suggest that JCV undergoes sequence alterations that accompany this ability, and these changes can be derived from an archetype strain by mutation, deletion, and duplication. While the introductory source and primary tissue reservoir of JCV remain unknown, lymphoid cells have been identified as potential intermediaries in progression of JCV to the brain. This review is focused on sequence changes in the noncoding control region (NCCR of the virus. We propose an adaptive mechanism that involves a sequential series of DNA replication-driven NCCR recombination events involving stalled DNA replication forks at NCCR palindromic secondary structures. We shall describe how the NCCR sequence changes point to a model in which viral DNA replication drives NCCR recombination, allowing JCV adaptation to different cell types in its progression to neurovirulence.

  16. Progressive multifocal leukoencephalopathy and natalizumab.

    Science.gov (United States)

    Hellwig, Kerstin; Gold, Ralf

    2011-11-01

    Natalizumab (TYSABRI(®)), a specific α4-integrin antagonist, is approved as a second-line treatment of relapsing-remitting MS (RRMS) patients who fail therapy with interferons or as first-line treatment of patients with highly active relapsing-remitting disease. Since the market introduction of natalizumab as a monotherapy in July of 2006, 111 cases of PML have been reported in natalizumab-treated MS patients as of April 2011. This review focuses on the available data regarding risk stratification for PML under long-term natalizumab therapy, and summarizes the current approach for PML management, as a natalizumab treatment complication is not necessarily associated with a fatal outcome. There is a need for development of surrogate markers that would help to better define the risk of PML in individual patients.

  17. Clinical and Radiological Characterization of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients: A Retrospective Analysis and Review of the Literature.

    Science.gov (United States)

    Augusto, Luís; Neves, Nélia; Reis, Carina; Abreu, Cândida; Sarmento, António

    2015-01-01

    Introdução: A leucoencefalopatia multifocal progressiva é uma patologia desmielinizante causada pelo vírus John Cunningham, geralmente associada a estados de imunodepressão, em particular a infeção pelo vírus da imunodeficiência humana. Pode apresentar múltiplas manifestações clínicas e tem habitualmente um padrão imagiológico típico. A evolução clínica é geralmente progressiva, podendo ocorrer uma melhoria do prognóstico associada à recuperação imunológica.Material e Métodos: Foi conduzida uma análise retrospetiva dos dados clínicos e imagiológicos de doentes admitidos no nosso Hospital entre janeiro de 2005 e abril de 2014 com o diagnóstico de leucoencefalopatia multifocal progressiva (ICD10:A81.2) associado a infeção por vírus da imunodeficiência humana.Resultados: Vinte e um doentes foram incluídos, sendo 20 do sexo masculino (95,2%). A idade média na altura do diagnóstico foi 39 anos. A forma de apresentação mais frequente foi défice motor. O vírus John Cunningham foi identificado no líquido cefalorraquidiano em 20 doentes (95,2%). Nos estudos de imagem verificou-se um predomínio de lesões supratentoriais, assimétricas e bilaterais. Quatro doentes (19%) desenvolveram síndrome inflamatória de resposta imunológica. A abordagem terapêutica incluiu início ou otimização de terapêutica anti-retrovirica, associada a corticoterapia em quatro casos. Dezassete (81%) doentes morreram no período do estudo, sendo a sobrevida mediana após diagnóstico de três meses (intervalo 1 a13).Discussão: Os resultados do nosso estudo são concordantes com os dados previamente publicados relativamente à leucoencefalopatia multifocal progressiva, evidenciando a sua associação à infecção pelo vírus da imunodeficiência humana, particularmente nos doentes com imunossupressão grave, o predomínio de sinais e sintomas motores e cognitivos, e a existência de um atingimento bilateral e assimétrico evidente nas sequ

  18. Aids-related progressive multifocal leukoencephalopathy: a retrospective study in a referral center in São Paulo, Brazil Leucoencefalopatia multifocal progressiva em pacientes com aids: estudo retrospectivo em um centro de referência de São Paulo, Brasil

    Directory of Open Access Journals (Sweden)

    José E. Vidal

    2008-08-01

    Full Text Available Few data are available about progressive multifocal leukoencephalopathy (PML in patients with acquired immunodeficiency syndrome (AIDS from Brazil. The objectives of this study were to describe the main features of patients with PML and estimate its frequency among AIDS patients with central nervous system (CNS opportunistic diseases admitted to the Instituto de Infectologia Emílio Ribas, São Paulo, Brazil, from April 2003 to April 2004. A retrospective and descriptive study was performed. Twelve (6% cases of PML were identified among 219 patients with neurological diseases. The median age of patients with PML was 36 years and nine (75% were men. Nine (75% patients were not on antiretroviral therapy at admission. The most common clinical manifestations were: focal weakness (75%, speech disturbances (58%, visual disturbances (42%, cognitive dysfunction (42%, and impaired coordination (42%. The median CD4+ T-cell count was 45 cells/µL. Eight (67% of 12 patients were laboratory-confirmed with PML and four (33% were possible cases. Eleven (92% presented classic PML and only one case had immune reconstitution inflammatory syndrome (IRIS-related PML. In four (33% patients, PML was the first AIDS-defining illness. During hospitalization, three patients (25% died as a result of nosocomial pneumonia and nine (75% were discharged to home. Cases of PML were only exceeded by cases of cerebral toxoplasmosis, cryptococcal meningoencephalitis, and CNS tuberculosis, the three more frequent neurologic opportunistic infections in Brazil. The results of this study suggest that PML is not an uncommon HIV-related neurologic disorder in a referral center in Brazil.Existe informação limitada sobre a presença da leucoencefalopatia multifocal progressiva (LEMP em pacientes com aids no Brasil. Os objetivos do presente estudo foram descrever as principais características dos pacientes com LEMP e estimar a freqüência desta doença em pacientes com aids e doen

  19. [Fatal toxic leukoencephalopathy associated with consumption of pasta base of cocaine: Report of three cases].

    Science.gov (United States)

    Cartier R, Luis; González L, Daniela; Harán D, Jorge

    2015-11-01

    The prevalence of drug-associated toxic encephalopathy is unknown, but it is an uncommon condition. Toxic leukoencephalopathy was described associated with heroin consumption, it has been less commonly described with the use of cocaine and there are no reports of its association with consumption pasta base of cocaine (PBC). We report two females aged 31 years and a male aged 19 years, consumers of PBC who developed a fatal toxic leukoencephalopathy. They initiated their disease with severe and persistent headache, sequential focal neurologic deficits and a progressive impairment of consciousness that culminated with their death. Laboratory parameters such as blood count, cerebrospinal fluid analyses or infectious biological indices were normal. MRI showed multifocal lesions in brain white matter of both hemispheres confirming the leukoencephalopathy. There was no response to the use of methylprednisolone.

  20. Reversible posterior leukoencephalopathy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Ja; Yu, Won Jong; Ahn, Kook Jin; Jung, So Lyung; Lee, Yeon Soo; Kim, Ji Chang; Kang, Si Won [The Catholic Univ. of Korea, Taejon (Korea, Republic of); Song, Chang Joon [Chungnam National Univ. School of Medicine, Cheonju (Korea, Republic of); Song, Soon-Young; Koo, Ja Hong [Kwandong Univ. College of Medicine, Myungji Hospital, Seoul (Korea, Republic of); Kim, Man Deuk [College of Medicine Pochon CHA Univ., Seoul (Korea, Republic of)

    2001-10-01

    To review reversible posterior leukoencephalopathy syndrome. We reviewed 22 patients (M:F=3:19; age, 17-46 years) with the characteristic clinical and imaging features of reversible posterior leukoencephalopathy syndrome. All underwent brain MRI, and in three cases both CT and MRI were performed. In one, MRA was obtained, and in eleven, follow-up MR images were obtained. We evaluated the causes of this syndrome, its clinical manifestations, and MR findings including the locations of lesions, the presence or absence of contrast enhancement, and the changes seen at follow-up MRI. Of the 22 patients, 13 had eclampsia (six during pregnancy and seven during puerperium). Four were receiving immunosuppressive therapy (three, cyclosporine ; one, FK 506). Four suffered renal failure and one had complicated migraine. The clinical manifestations included headache (n=12), visual disturbance (n=13), seizure (n=15), focal neurologic sign (n=3), and altered mental status (n=2). Fifteen patients had hypertension and the others normotension. MRI revealed that lesions were bilateral (n=20) or unilateral (n=2). In all patients the lesion was found in the cortical and subcortical areas of the parieto-occipital lobes ; other locations were the basal ganglia (n=9), posterior temporal lobe (n=8), frontal lobe (n=5), cerebellum (n=5), pons (n=2), and thalamus (n=1). All lesions were of high signal intensity on T2-weighted images, and of iso to low intensity on T1-weighted images. One was combined with acute hematoma in the left basal ganglia. In eight of 11 patients who underwent postcontrast T1-weighted MRI, there was no definite enhancement ; in one, enhancement was mild, and in tow, patchy. CT studies showed low attenuation, and MRA revealed mild vasospasm. The symptoms of all patients improved. Follow-up MRI in nine of 11 patients depicted complete resolution of the lesions ; in two, small infarctions remained but the extent of the lesions had decreased. Reversible posterior

  1. MRI in methotrexate-related leukoencephalopathy: Disseminated necrotising leukoencephalopathy in comparison with mild leukoencephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Oka, M. [Department of Radiology, Hokkaido Graduate University School of Medicine, 060-8638, Sapporo (Japan); Department of Radiology, St Luke' s International Hospital, 9-1 Akashi-cho Chuo-ku, 104-8560, Tokyo (Japan); Terae, S.; Kudoh, K.; Tha, K.K.; Miyasaka, K. [Department of Radiology, Hokkaido Graduate University School of Medicine, 060-8638, Sapporo (Japan); Kobayashi, R.; Yoshida, M.; Suzuki, Y. [Department of Paediatrics, Hokkaido Graduate University School of Medicine, 060-8638, Sapporo (Japan); Sawamura, Y.; Kaneda, M. [Department of Neurosurgery, Hokkaido Graduate University School of Medicine, 060-8638, Sapporo (Japan)

    2003-07-01

    We report two fatal cases of methotrexate (MTX)-induced disseminated necrotising leukoencephalopathy (DNL) in which MRI was repeated from the onset. Initial T2-weighted images showed multiple areas of high signal, mainly in deep cerebral white matter, which on follow-up, spread and coalesced to involve the entire white matter. Small irregular low-signal foci on T2-weighted images were seen within the high-signal lesions. Multiple areas of contrast enhancement corresponded to these low-signal foci. The condition of both patients deteriorated and they died. We compared their MRI findings with those of seven patients with mild MTX-related leukoencephalopathy, six of whom were asymptomatic; one had transient neurological symptoms. They showed no contrast enhancement, but rather mild-to-moderate diffuse high signal in deep white matter, which later disappeared. These findings suggest that multiple low-signal foci on T2-weighted images with contrast enhancement may be characteristic of DNL, and that contrast-enhanced imaging is useful to differentiate this condition from mild leukoencephalopathy. (orig.)

  2. Megalencephalic Leukoencephalopathy with Subcortical Cysts-a New Child Leukoencephalopathy

    Institute of Scientific and Technical Information of China (English)

    YuanBao-Qiang; MarjoSvanderKnaap

    2004-01-01

    Here we review a new variety of leukoencephalopathy with infantile megalencephaly and discrepant clinical course (MLC, MIM: 604004). These children had megalencephaly in the first year of life, with or without mild delay of motor function and/or seizures. After a few years, motor handicap was slowly progressive with unsteady gait, serious cerebeliar ataxia and mild plasticity. Eventually most of patients were confined to a wheelchair. Meanwhile mental development was relatively preserved, although the learning problems was increased from the midway of elementary school. Most of patients had tonic-clonic seizure and some might advance to status epilepticus. Antiepileptic drugs may effectively control seizure. The disorders of known metabolic defects were excluded. Neurophysiological examination showed that EEG had interictal epileptic discharges on the generalized slow wave background in most patients. The cerebral white matter had diffuse abnormality, with swelling of white matter, and cysts in the frontoparietal and anterior-temporal lobes on MRI examination. Some central white matter structures were spared, such as corpus caliosum. The severity of lesions on MRI is inconsistent with the clinical signs. Pathogenesis of this disease was unknown. The pathological findings found a spongiform leukoencephalopathy due to myelin splitting and intramyelinic vacuole formation but without myelin loss, This disease had probably an autosomal recessive inheritance, The geneKIAA027 on 22qtel was responsible for M LC.

  3. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported.

  4. Delayed Radiation-Induced Vasculitic Leukoencephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Rauch, Philipp J. [Departments of Pathology and Neurosurgery, Yale University School of Medicine, New Haven, Connecticut (United States); Faculty of Medicine, University of Heidelberg, Heidelberg (Germany); Park, Henry S. [Departments of Pathology and Neurosurgery, Yale University School of Medicine, New Haven, Connecticut (United States); Knisely, Jonathan P.S. [Department of Radiation Medicine, North Shore University Hospital, Manhasset, New York (United States); Chiang, Veronica L. [Departments of Pathology and Neurosurgery, Yale University School of Medicine, New Haven, Connecticut (United States); Vortmeyer, Alexander O., E-mail: alexander.vortmeyer@yale.edu [Departments of Pathology and Neurosurgery, Yale University School of Medicine, New Haven, Connecticut (United States)

    2012-05-01

    Purpose: Recently, single-fraction, high-dosed focused radiation therapy such as that administered by Gamma Knife radiosurgery has been used increasingly for the treatment of metastatic brain cancer. Radiation therapy to the brain can cause delayed leukoencephalopathy, which carries its own significant morbidity and mortality. While radiosurgery-induced leukoencephalopathy is known to be clinically different from that following fractionated radiation, pathological differences are not well characterized. In this study, we aimed to integrate novel radiographic and histopathologic observations to gain a conceptual understanding of radiosurgery-induced leukoencephalopathy. Methods and Materials: We examined resected tissues of 10 patients treated at Yale New Haven Hospital between January 1, 2009, and June 30, 2010, for brain metastases that had been previously treated with Gamma Knife radiosurgery, who subsequently required surgical management of a symptomatic regrowing lesion. None of the patients showed pathological evidence of tumor recurrence. Clinical and magnetic resonance imaging data for each of the 10 patients were then studied retrospectively. Results: We provide evidence to show that radiosurgery-induced leukoencephalopathy may present as an advancing process that extends beyond the original high-dose radiation field. Neuropathologic examination of the resected tissue revealed traditionally known leukoencephalopathic changes including demyelination, coagulation necrosis, and vascular sclerosis. Unexpectedly, small and medium-sized vessels revealed transmural T-cell infiltration indicative of active vasculitis. Conclusions: We propose that the presence of a vasculitic component in association with radiation-induced leukoencephalopathy may facilitate the progressive nature of the condition. It may also explain the resemblance of delayed leukoencephalopathy with recurring tumor on virtually all imaging modalities used for posttreatment follow-up.

  5. Global epidemiology of capsular group W meningococcal disease (1970-2015): Multifocal emergence and persistence of hypervirulent sequence type (ST)-11 clonal complex.

    Science.gov (United States)

    Mustapha, Mustapha M; Marsh, Jane W; Harrison, Lee H

    2016-03-18

    Following an outbreak in Mecca Saudi Arabia in 2000, meningococcal strains expressing capsular group W (W) emerged as a major cause of invasive meningococcal disease (IMD) worldwide. The Saudi Arabian outbreak strain (Hajj clone) belonging to the ST-11 clonal complex (cc11) is similar to W cc11 causing occasional sporadic disease before 2000. Since 2000, W cc11 has caused large meningococcal disease epidemics in the African meningitis belt and endemic disease in South America, Europe and China. Traditional molecular epidemiologic typing suggested that a majority of current W cc11 burden represented global spread of the Hajj clone. However, recent whole genome sequencing (WGS) analyses revealed significant genetic heterogeneity among global W cc11 strains. While continued spread of the Hajj clone occurs in the Middle East, the meningitis belt and South Africa have co-circulation of the Hajj clone and other unrelated W cc11 strains. Notably, South America, the UK, and France share a genetically distinct W cc11 strain. Other W lineages persist in low numbers in Europe, North America and the meningitis belt. In summary, WGS is helping to unravel the complex genomic epidemiology of group W meningococcal strains. Wider application of WGS and strengthening of global IMD surveillance is necessary to monitor the continued evolution of group W lineages.

  6. Chronic recurrent multifocal osteomyelitis

    NARCIS (Netherlands)

    Wedman, Jan; van Weissenbruch, Ranny

    2005-01-01

    We report what is, to our best knowledge, the first case of chronic recurrent multifocal osteomyelitis (CRMO) in which the frontal and sphenoid bones were involved. Characterized by a prolonged and fluctuating course of osteomyelitis at different sites, CRMO is self-limited, although sequelae can oc

  7. A new leukoencephalopathy with vanishing white matter

    NARCIS (Netherlands)

    vanderKnaap, MS; Barth, PG; Gabreels, FJM; Franzoni, E; Begeer, JH; Stroink, H; Rotteveel, JJ; Valk, J.

    1997-01-01

    We identified nine children with a leukoencephalopathy of similar type according to clinical and MRI findings. The patients included three affected sibling pairs. The age range was 3 to 19 years. The onset of the disease was in childhood; the course was both chronic-progressive and episodic. There w

  8. Cerebral lymphoma presenting as a leukoencephalopathy

    Science.gov (United States)

    Ayuso-Peralta, L; Orti-Pareja, M; Zurdo-Hernandez, M; Jimenez-Jimenez, F; Tejeiro-Martinez, J; Ricoy, J; de la Lama, A; Bernardo, A

    2001-01-01

    Cerebral lymphoma is infrequent in immunocompetent patients. This tumour usually appears on CT and MRI as a single lesion or as multiple lesions with mass effect and homogeneous enhancement after contrast administration. A patient is described with a cerebral lymphoma, confirmed by histopathological examination, who presented as a progressive leukoencephalopathy.

 PMID:11459903

  9. A new leukoencephalopathy with vanishing white matter

    NARCIS (Netherlands)

    vanderKnaap, MS; Barth, PG; Gabreels, FJM; Franzoni, E; Begeer, JH; Stroink, H; Rotteveel, JJ; Valk, J.

    1997-01-01

    We identified nine children with a leukoencephalopathy of similar type according to clinical and MRI findings. The patients included three affected sibling pairs. The age range was 3 to 19 years. The onset of the disease was in childhood; the course was both chronic-progressive and episodic. There w

  10. Multifocal recurrent periostitis

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Anderson, R.; Tink, A.

    1981-11-01

    Two case reports of recurrent multifocal periostitis in two girls aged 15 and 16 are added to the eight cases already reported in the literature. The disease is characterised clinically by recurrent mesomelic swelling of the extremities and radiologically by periosteal thickening and sclerosis of underlying bone. Hyperglobulinaemia is the most constant biochemical finding. The bone biopsy shows no typical features. The possibility of a viral etiology is discussed.

  11. Reversible Posterior Leukoencephalopathy Syndrome Induced by Pazopanib

    Directory of Open Access Journals (Sweden)

    Chelis Leonidas

    2012-10-01

    Full Text Available Abstract Background The reversible posterior leukoencephalopathy syndrome is a clinical/radiological syndrome characterized by headache, seizures, impaired vision, acute hypertension, and typical magnetic resonance imaging findings. There are several reports in the literature that depict its occurrence in cancer patients. The list of common anticancer and supportive care drugs that predispose to reversible posterior leukoencephalopathy syndrome is expanding and includes not only a large number of chemotherapeutic agents but also an increased number of new targeted drugs, particularly angiogenesis inhibitors such as bevacizumab,sorefenib and sunitinib. Pazopanib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor, platelet-derived growth factor receptor, and c-Kit which after a positive phase III randomized clinical trial in patients with advanced renal cell cancer received FDA approval for the treatment of advanced renal cell carcinoma. Until now no cases of reversible posterior leukoencephalopathy syndrome induced by pazopanib have been reported. Case report We present the case of a 40 years old female patient with heavily pre-treated metastatic renal cell carcinoma who received pazopanib as salvage treatment. After 21 days of pazopanib therapy the patient referred to the emergency department with epileptic seizure, impaired vision at both eyes and headache. MRI of the brain revealed subcortical oedema at the occipital and parietal lobes bilaterally. She was treated with anticonvulsants, i.v. administration of mannitol and antihypertensives and she recovered completely from her symptoms and was discharged on the tenth hospital day. A brain MRI performed 3 weeks after showed that the subcortical oedema had been subsided. Conclusion In conclusion this is the first case of pazopanib induced reversible posterior leukoencephalopathy syndrome. Although usually reversible, this syndrome is a serious and

  12. Adaptive mutations in the JC virus protein capsid are associated with progressive multifocal leukoencephalopathy (PML.

    Directory of Open Access Journals (Sweden)

    Shamil R Sunyaev

    2009-02-01

    Full Text Available PML is a progressive and mostly fatal demyelinating disease caused by JC virus infection and destruction of infected oligodendrocytes in multiple brain foci of susceptible individuals. While JC virus is highly prevalent in the human population, PML is a rare disease that exclusively afflicts only a small percentage of immunocompromised individuals including those affected by HIV (AIDS or immunosuppressive drugs. Viral- and/or host-specific factors, and not simply immune status, must be at play to account for the very large discrepancy between viral prevalence and low disease incidence. Here, we show that several amino acids on the surface of the JC virus capsid protein VP1 display accelerated evolution in viral sequences isolated from PML patients but not in sequences isolated from healthy subjects. We provide strong evidence that at least some of these mutations are involved in binding of sialic acid, a known receptor for the JC virus. Using statistical methods of molecular evolution, we performed a comprehensive analysis of JC virus VP1 sequences isolated from 55 PML patients and 253 sequences isolated from the urine of healthy individuals and found that a subset of amino acids found exclusively among PML VP1 sequences is acquired via adaptive evolution. By modeling of the 3-D structure of the JC virus capsid, we showed that these residues are located within the sialic acid binding site, a JC virus receptor for cell infection. Finally, we go on to demonstrate the involvement of some of these sites in receptor binding by demonstrating a profound reduction in hemagglutination properties of viral-like particles made of the VP1 protein carrying these mutations. Collectively, these results suggest that a more virulent PML causing phenotype of JC virus is acquired via adaptive evolution that changes viral specificity for its cellular receptor(s.

  13. Modeling probability of additional cases of natalizumab-associated JCV sero-negative progressive multifocal leukoencephalopathy.

    Science.gov (United States)

    Carruthers, Robert L; Chitnis, Tanuja; Healy, Brian C

    2014-05-01

    JCV serologic status is used to determine PML risk in natalizumab-treated patients. Given two cases of natalizumab-associated PML in JCV sero-negative patients and two publications that question the false negative rate of the JCV serologic test, clinicians may question whether our understanding of PML risk is adequate. Given that there is no gold standard for diagnosing previous JCV exposure, the test characteristics of the JCV serologic test are unknowable. We propose a model of PML risk in JCV sero-negative natalizumab patients. Using the numbers of JCV sero-positive and -negative patients from a study of PML risk by JCV serologic status (sero-positive: 13,950 and sero-negative: 11,414), we apply a range of sensitivities and specificities in order calculate the number of JCV-exposed but JCV sero-negative patients (false negatives). We then apply a range of rates of developing PML in sero-negative patients to calculate the expected number of PML cases. By using the binomial function, we calculate the probability of a given number of JCV sero-negative PML cases. With this model, one has a means to establish a threshold number of JCV sero-negative natalizumab-associated PML cases at which it is improbable that our understanding of PML risk in JCV sero-negative patients is adequate.

  14. Progressive multifocal leukoencephalopathy complicating untreated chronic lymphatic leukemia: case report and review of the literature.

    Science.gov (United States)

    Di Pauli, Franziska; Berger, Thomas; Walder, Alois; Maier, Hans; Rhomberg, Paul; Uprimny, Christian; Steurer, Michael; Stockhammer, Guenther

    2014-08-01

    A 58-year old female with a four-year history of previously untreated CLL at Binet stage A complained about word finding problems, impaired vision, and gait unsteadiness. Concerning her CLL she was asymptomatic and had never required any specific treatment. Her neurological examination disclosed cognitive alterations, homonyme hemianopia to the right, aphasia, and mild right-sided hemiparesis. Cerebral MRI showed a hyperintense lesion on T2 weighted images without contrast enhancement. CSF examination revealed normal findings, including CSF protein, cell count, cytology and PCR-analysis was negative for the presence of JC virus DNA. On follow-up MRI, performed 2 weeks later, the T2 lesion was further enlarging. Subsequent stereotactic brain biopsy was diagnostic for PML revealing abnormal oligodendrocytes staining positive against antibodies specific for simian vacuolating virus 40. In addition, repeated CSF analyses for JC-Virus DNA in the course of the disease became positive. After confirmation of diagnosis treatment with mirtazapine (30 mg/d) and mefloquine (250 mg/d) was initiated. Rapid clinical progression correlated to further worsening on MRI. Therefore this treatment was terminated after 16 days and the regime was changed to a five-day courses of cytarabine (2 mg/kg/d) combined with intrathecal administration of liposomal cytarabine (50 mg). Due to further clinical progression with global aphasia, blindness and severe right-sided hemiparesia, medication was stopped. The Patient died three and a half months after onset of symptoms.

  15. Fatal Progressive Multifocal Leukoencephalopathy in a Kidney Transplant Recipient 19 Years After Successful Renal Allograft Transplantation

    DEFF Research Database (Denmark)

    Carlson, N; Hansen, Jesper Melchior

    2014-01-01

    in circumstances of extreme immunodeficiency. Development of fulminant PML is rare and treatment options are limited. CASE REPORT: We have presented a case of JCV reactivation resulting in PML 19 years after renal allograft transplantation and after recent conversion of immunosuppressive treatment. One year after...

  16. Multifocal contact lens myopia control.

    Science.gov (United States)

    Walline, Jeffrey J; Greiner, Katie L; McVey, M Elizabeth; Jones-Jordan, Lisa A

    2013-11-01

    Previous studies on soft multifocal contact lens myopia control published in the peer-reviewed literature reported findings of noncommercial contact lenses worn for 1 year or less. This study sought to determine the progression of myopia and axial elongation of children fitted with commercially available distance center soft multifocal contact lenses for 2 years. Eight- to eleven-year-old children with -1.00 D to -6.00 D spherical component and less than 1.00 D astigmatism were fitted with soft multifocal contact lenses with a +2.00 D add (Proclear Multifocal "D"; CooperVision, Fairport, NY). They were age- and gender-matched to participants from a previous study who were fitted with single-vision contact lenses (1 Day Acuvue; Vistakon, Jacksonville, FL). A-scan ultrasound and cycloplegic autorefraction were performed at baseline, after 1 year, and after 2 years. Multilevel modeling was used to compare the rate of change of myopia and axial length between single-vision and soft multifocal contact lens wearers. Forty participants were fitted with soft multifocal contact lenses, and 13 did not contribute complete data (5 contributed 1 year of data). The adjusted mean ± standard error spherical equivalent progression of myopia at 2 years was -1.03 ± 0.06 D for the single-vision contact lens wearers and -0.51 ± 0.06 for the soft multifocal contact lens wearers (p < 0.0001). The adjusted mean axial elongation was 0.41 ± 0.03 and 0.29 ± 0.03 for the single-vision and soft multifocal contact lens wearers, respectively (p < 0.0016). Soft multifocal contact lens wear resulted in a 50% reduction in the progression of myopia and a 29% reduction in axial elongation during the 2-year treatment period compared to a historical control group. Results from this and other investigations indicate a need for a long-term randomized clinical trial to investigate the potential for soft multifocal contact lens myopia control.

  17. [Chronic recurrent multifocal osteomyelitis].

    Science.gov (United States)

    Schilling, F; Eckardt, A; Kessler, S

    2000-01-01

    The aim of this paper is to give a detailed description of the so-called "chronic recurrent multifocal osteomyelitis" (CRMO). The clinical, radiological and histopathological results of an analysis of 29 cases (15 children/adolescents and 14 adults) are presented and correlated to current data from the literature. We could delinate the following points: 1. CRMO is a systemic aseptic inflammation of the bone marrow (Osteitis), it can occur polytopically and association with pustulous dermatologic symptoms is possible. 2. It is not a rare disease 3. Osteomyelitis is probably "reactive" and a plasma-cell sclerotic process with ist own characteristic histologic three-phase course. 4. We could observe 5 specific types of localization which can be documented by X-ray or bone scan. 5. Accompanying arthritis os often present, especially "sympathetic coxitis". 6. The use of drugs in treatment of CRMO (i.e. azithromycin, calcitonin, and bisphosphonates) is discussed. In conclusion we want to point out, that 1. 99mTC bone scan should always be performed when there is suspicion for CRMO to reveal the pattern of affection, 2. the rheumatologist and dermatologist should be contacted, 3. operation is normally not necessary for treatment of the mostly self-limitin disease, and 4. the term "SAPHO syndrome" should be avoided, further differentiation of the diagnosis is necessary.

  18. Chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Roderick, Marion R; Ramanan, Athimalaipet V

    2013-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease occurring primarily in children and adolescents. Episodes of systemic inflammation occur due to immune dysregulation without autoantibodies, pathogens or antigen-specific T cells. CRMO is characterised by the insidious onset of pain with swelling and tenderness over the affected bones. Clavicular involvement was the classical description; however, the metaphyses and epiphyses of long bones are frequently affected. Lesions may occur in any bone, including vertebrae. Characteristic imaging includes bone oedema, lytic areas, periosteal reaction and soft tissue reaction. Biopsies from affected areas display polymorphonuclear leucocytes with osteoclasts and necrosis in the early stages. Subsequently, lymphocytes and plasma cells predominate followed by fibrosis and signs of reactive new bone forming around the inflammation. Diagnosis is facilitated by the use of STIR MRI scanning, potentially obviating the need for biopsy and unnecessary long-term antibiotics due to incorrect diagnosis. Treatment options include non-steroidal anti-inflammatory drugs and bisphosphonates. Biologics have been tried in resistant cases with promising initial results. Gene identification has not proved easy although research in this area continues. Early descriptions of the disease suggested a benign course; however, longer-term follow up shows that it can cause significant morbidity and longer-term disability. Although it has always been thought of as very rare, the prevalence is likely to be vastly underestimated due to poor recognition of the disease.

  19. Leukoencephalopathy with spheroids (HDLS) and pigmentary leukodystrophy (POLD)

    Science.gov (United States)

    Wider, C; Van Gerpen, J A.; DeArmond, S; Shuster, E A.; Dickson, D W.; Wszolek, Z K.

    2009-01-01

    Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and familial pigmentary orthochromatic leukodystrophy (POLD) present as adult-onset dementia with motor impairment and epilepsy. They are regarded as distinct diseases. We review data from the literature that support their being a single entity. Apart from a slightly older age at onset, a more rapid course, and more prominent pyramidal tract involvement, familial POLD is clinically similar to HDLS. Moreover, the pathologic hallmarks of the two diseases, axonal spheroids in HDLS and pigmented macrophages in POLD, can be identified in both conditions. This supports HDLS and POLD being referred collectively as adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP). GLOSSARY ALSP = adult-onset leukoencephalopathy with axonal spheroids and pigmented glia; HDLS = hereditary diffuse leukoencephalopathy with axonal spheroids; OLD = orthochromatic leukodystrophy; POLD = pigmentary orthochromatic leukodystrophy. PMID:19487654

  20. Cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome.

    Science.gov (United States)

    Abenza-Abildua, Maria Jose; Fuentes, Blanca; Diaz, Domingo; Royo, Aranzazu; Olea, Teresa; Aguilar-Amat, Maria Jose; Diez-Tejedor, Exuperio

    2009-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical radiological syndrome, characterised by acute headache, altered consciousness, seizures and hypertension. The most frequent causes are hypertensive encephalopathy, eclampsia and some immunosuppressive therapies. The pathogenesis remains unclear, but it appears to be related to altered cerebral circulation, producing oedema that can be seen on MRI, and it resolves in 2 or 3 weeks. In the present report, a possible first reported case of cyclophosphamide-induced RPLS in a 27-year-old man with high blood pressure (HBP) and glomerulonephritis caused by Goodpasture syndrome, treated with cyclophosphamide during the last month and prednisone for glomerulonephritis resulting from Goodpasture syndrome without other immunosuppressive drugs, is described.Symptoms appeared during a hypertensive crisis, but when cyclophosphamide was replaced by rituximab and hypertension was controlled, the patient did not have neurological symptoms. Almost all reported cases induced by immunosuppressive therapy or other causes were associated with hypertension as well.

  1. Genetics Home Reference: leukoencephalopathy with thalamus and brainstem involvement and high lactate

    Science.gov (United States)

    ... Health Conditions LTBL leukoencephalopathy with thalamus and brainstem involvement and high lactate Enable Javascript to view the expand/collapse ... Close All Description Leukoencephalopathy with thalamus and brainstem involvement and high lactate ( LTBL ) is a disorder that affects the ...

  2. [Multifocal tuberculosis in immunocompetent patients].

    Science.gov (United States)

    Rezgui, Amel; Fredj, Fatma Ben; Mzabi, Anis; Karmani, Monia; Laouani, Chadia

    2016-01-01

    Multifocal tuberculosis is defined as the presence of lesions affecting at least two extrapulmonary sites, with or without pulmonary involvement. This retrospective study of 10 cases aims to investigate the clinical and evolutionary characteristics of multifocal tuberculosis. It included 41 cases with tuberculosis collected between 1999 and 2013. Ten patients had multifocal tuberculosis (24%): 9 women and 1 man, the average age was 50 years (30-68 years). Our patients were correctly BCG vaccinated. The evaluation of immunodepression was negative in all patients. 7 cases had lymph node tuberculosis, 3 cases digestive tuberculosis, 2 cases pericardial tuberculosis, 2 cases osteoarticular tuberculosis, 1 case brain tuberculosis, 2 cases urinary tuberculosis, 4 cases urogenital tuberculosis, 1 case adrenal tuberculosis, 1 case cutaneous and 1 case muscle tuberculosis. All patients received anti-tuberculosis treatment for a mean duration of 10 months, with good evolution. Multifocal tuberculosis is difficult to diagnose. It can affect immunocompetent patients but often has good prognosis. Anti-tuberculosis therapy must be initiated as soon as possible to avoid sequelae.

  3. Marjolin ulcer with multifocal origin

    Directory of Open Access Journals (Sweden)

    Das Sudip

    2009-01-01

    Full Text Available Marjolin ulcer developed from a twenty years old post burn scar. The patient presented with chronic ulceration followed by multifocal development of squamous cell carcinoma with different growth pattern. One nodular lesion grew rapidly to produce a large lesion with history of a little bleeding after trauma but without any pain. Excision followed by skin grafting resulted in good cosmetic scar.

  4. Posterior Reversible Leukoencephalopathy Syndrome Associated with Pazopanib

    Directory of Open Access Journals (Sweden)

    Robert Foerster

    2013-04-01

    Full Text Available A 62-year-old female patient with metastatic renal cell carcinoma under third-line treatment with pazopanib for 8 weeks suddenly developed severe headaches, grand mal seizures and paresis of the left arm in combination with gait instability as well as nausea and vomiting during her vacation abroad. The emergency physician measured systolic blood pressure values over 300 mm Hg and suspected a stroke. The CT imaging without contrast agent in a local hospital did not show any pathologic findings despite bone metastases. The colleagues suspected cerebral metastases or meningeosis carcinomatosa and referred the patient to our department for further diagnostics and treatment planning. An MRI scan ruled out the suspected cerebral metastases or meningeosis carcinomatosa, but showed signs of reversible posterior leukoencephalopathy syndrome (RPLS in the form of band-like hyperintensities as a sign of cytotoxic edema in the gray and white matter of the left parietal lobe. The patient then reported that similar blood pressure values had been measured shortly after the start of a first-line therapy with sunitinib, so that we discontinued the current treatment with pazopanib. Within 6 days the neurologic symptoms vanished and the patient was discharged. An intermittent hypertension persisted. A follow-up MRI 3 weeks later showed an RPLS-typical cortical infarction in the affected area. RPLS should be considered as the actual reason for neurologic findings in hypertensive patients with known metastatic cancers under tyrosine kinase inhibitor therapy.

  5. Methotrexate-induced acute toxic leukoencephalopathy.

    Science.gov (United States)

    Salkade, Parag R; Lim, Teh Aun

    2012-01-01

    Acute lymphoblastic leukemia (ALL) is one of the most common malignancies of childhood, which is treated with high doses of methotrexate (MTX), as it crosses the blood-brain barrier and can be administered intravenously and via intrathecal route to eradicate leukemic cells from central nervous system (CNS). Additionally, high doses of MTX not only prevent CNS recurrence but also hematologic relapses. Although, standard treatment protocol for ALL includes multimodality therapy, MTX is usually associated with neurotoxicity and affects periventricular deep white matter region. Methotrexate-induced 'acute toxic leukoencephalopathy' has varying clinical manifestations ranging from acute neurological deficit to seizures or encephalopathy. Diffusion weighted magnetic resonance imaging (DW-MRI) is widely available and routinely used in clinical practice to identify acute stroke and also to distinguish acute stroke from non-stroke like conditions. We report a local teenage Chinese girl who developed 2 discrete episodes of left upper and lower limb weakness with left facial nerve paresis after receiving the 2 nd and 3 rd cycle of high dose of intravenous and intrathecal methotrexate, without having cranial irradiation. After each episode of her neurological deficit, the DW-MRI scan showed focal restricted diffusion in right centrum semiovale. Her left sided focal neurological deficit and facial nerve paresis almost completely subsided on both these occasions within 3 days of symptom onset. Follow-up DW-MRI, after her neurological recovery, revealed almost complete resolution of previously noted restricted diffusion in right centrum semiovale, while the lesion was not evident on concurrent T2W (T2-weighted) and FLAIR (Fluid-Attenuated Inversion recovery) sequences, nor showed any post contrast enhancement on post gadolinium enhanced T1W (T1-weighted) sequences. No residual neurological deficit or intellectual impairment was identified on clinical follow up over a 2 year

  6. Primary multifocal osseous lymphoma in a child

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Takashi S.P. [University of Iowa, Carver College of Medicine, Iowa City, IA (United States); Ferguson, Polly J. [University of Iowa, Department of Pediatrics, Iowa City, IA (United States); Khanna, Geetika [Washington University, Mallinckrodt Institute of Radiology, St Louis, MO (United States)

    2008-12-15

    We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention. (orig.)

  7. Chasing the dragon-Toxic leukoencephalopathy(Heroin-Induced Spongiform Leukoencephalopathy)

    Institute of Scientific and Technical Information of China (English)

    初建平

    2012-01-01

    @@ "Chasing the dragon" is a toxic leukoencephalopathy caused by inhalation of heroin fumes.The term"chasing the dragon"appears to have originated in China in the 1920s and became a popular mechanism of heroin administration in the 1950s in Hong Kong because the drug was cheap but impure.A small quantity of powder is placed on aluminium foil,which is then heated underneath with a lighter or matches.The heroin liquefies into a reddish brown glob, which moves around on the foil and emits a white vapor.The glob or"dragon"is"chased"with the lighter underneath while the vapor is sucked through a straw or pipe.

  8. Mice with megalencephalic leukoencephalopathy with cysts : a developmental angle

    NARCIS (Netherlands)

    Dubey, Mohit; Bugiani, Marianna; Ridder, Margreet C; Postma, Nienke L; Brouwers, Eelke; Polder, Emiel; Jacobs, J Gerbren; Baayen, Johannes C; Klooster, Jan; Kamermans, M.; Aardse, Romy; de Kock, Christiaan P J; Dekker, Marien P; van Weering, Jan R T; Heine, Vivi M; Abbink, Truus E M; Scheper, Gert C; Boor, Ilja; Lodder, Johannes C; Mansvelder, Huibert D; van der Knaap, Marjo S

    OBJECTIVE: Megalencephalic leukoencephalopathy with cysts (MLC) is a genetic disease characterized by infantile onset white matter edema and delayed onset neurological deterioration. Loss of MLC1 function causes MLC. MLC1 is involved in ion-water homeostasis, but its exact role is unknown. We

  9. [Treatment of multifocal motor neuropathies].

    Science.gov (United States)

    Azulay, J P; Pouget, J; Rihet, P; Serratrice, G

    1996-05-01

    Multifocal motor neuropathy is characterized by a progressive asymetrical weakness, predominantly affecting the upper limbs with persistent conduction blocks on motor but not sensory nerves. Treatment woth prednisone and plasma exchanges have failed to demonstrate any positive effects. Some improvements have been reported with cyclophosphamide. Mainly immunoglobulin therapy has been evaluated with a beneficial response in almost 70% of the cases. These benefits obtained over periods of less than six months have recently been confirmed by a long-term evaluation of 18 patients treated by repeated infusions.

  10. Multifocal fibrosclerosis with intracranial pachymeningitis.

    Science.gov (United States)

    Kitano, A; Shimomura, T; Okada, A; Takahashi, K

    1995-04-01

    A 29-year-old woman with a 4-year history of multifocal fibrosclerosis showed unique neurologic complications. Episcleritis, orbital pseudotumor, and eosinophilic phlegmon preceded intracranial inflammatory pachymeningitis. The pachymeningitis was associated with disturbance of the visual field, incomplete Gerstmann's syndrome, and pseudotumor cerebri. T2-weighted magnetic resonance images revealed a high signal intensity lesion in the left temporal and occipital lobes, and gadolinium-enhanced T1-weighted images revealed the enhancement of the thickened left tentorial leaf. The laboratory data suggested that the etiology might be autoimmunological. The disease and MRI abnormalities improved following administration of corticosteroids.

  11. Ocular Straylight with Different Multifocal Contact Lenses

    NARCIS (Netherlands)

    Łabuz, Grzegorz; López-Gil, Norberto; van den Berg, Thomas J T P; Vargas-Martín, Fernando

    2017-01-01

    PURPOSE: Multifocal contact lenses have been growing in popularity as a modality to correct presbyopic eyes, although visual side effects such as disability glare have been reported. The objective of this study was to investigate the effect of multifocal contact lenses on disability glare by means

  12. Multifocal Osteonecrosis Secondary to Chronic Alcohol Ingestion

    OpenAIRE

    Kazu Matsumoto; Hiroyasu Ogawa; Haruhiko Akiyama

    2015-01-01

    Multifocal osteonecrosis is a relatively rare disorder with an estimated incidence of around 3% among patients diagnosed as having osteonecrosis. Multifocal osteonecrosis is caused by the several conditions including corticosteroid treatment, coagulation disorders, connective tissue disorders including systemic lupus erythematosus (SLE), inflammatory bowel disease, renal transplantation, and underlying malignancies. Alcohol abuse is one of the risk factors for osteonecrosis, and alcohol-induc...

  13. Fatal atypical reversible posterior leukoencephalopathy syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Golombeck Stefanie Kristin

    2013-01-01

    Full Text Available Abstract Introduction Reversible posterior leukoencephalopathy syndrome – a reversible subacute global encephalopathy clinically presenting with headache, altered mental status, visual symptoms such as hemianopsia or cortical blindness, motor symptoms, and focal or generalized seizures – is characterized by a subcortical vasogenic edema symmetrically affecting posterior brain regions. Complete reversibility of both clinical signs and magnetic resonance imaging lesions is regarded as a defining feature of reversible posterior leukoencephalopathy syndrome. Reversible posterior leukoencephalopathy syndrome is almost exclusively seen in the setting of a predisposing clinical condition, such as pre-eclampsia, systemic infections, sepsis and shock, certain autoimmune diseases, various malignancies and cytotoxic chemotherapy, transplantation and concomitant immunosuppression (especially with calcineurin inhibitors as well as episodes of abrupt hypertension. We describe for the first time clinical, radiological and histological findings in a case of reversible posterior leukoencephalopathy syndrome with an irreversible and fatal outcome occurring in the absence of any of the known predisposing clinical conditions except for a hypertensive episode. Case presentation A 58-year-old Caucasian woman presented with a two-week history of subacute and progressive occipital headache, blurred vision and imbalance of gait and with no evidence for raised arterial blood pressure during the two weeks previous to admission. Her past medical history was unremarkable except for controlled arterial hypertension. Cerebral magnetic resonance imaging demonstrated cortical and subcortical lesions with combined vasogenic and cytotoxic edema atypical for both venous congestion and arterial infarction. Routine laboratory and cerebrospinal fluid parameters were normal. The diagnosis of reversible posterior leukoencephalopathy syndrome was established. Within hours after

  14. Reversible posterior leukoencephalopathy syndrome : a report of 2 cases.

    Directory of Open Access Journals (Sweden)

    Arora A

    2001-07-01

    Full Text Available Reversible posterior leukoencephalopathy syndrome (RPLE is an increasingly recognised disorder, most commonly associated with malignant hypertension, toxaemia of pregnancy or the use of immunosuppressive agents. Two cases of RPLE syndrome occurring in the setting of accelerated hypertension and eclampsia are described. Both patients had seizures, altered sensorium and typical findings on neuroimaging. They had complete clinical and radiological recovery. The clinical course, pathophysiology and neuroimaging features of RPLE syndrome are discussed.

  15. Acute fever and delayed leukoencephalopathy following low dose intraventricular methotrexate.

    OpenAIRE

    Boogerd, W.; vd Sande, J J; Moffie, D

    1988-01-01

    Nine out of 14 patients treated with intraventricular methotrexate (MTX) for meningeal carcinomatosis from breast carcinoma and surviving more than 4 months developed disseminated necrotising leukoencephalopathy (DNL). All four patients who had received both intraventricular MTX and whole brain radiotherapy developed DNL. Five of the six patients who experienced an acute febrile reaction with mild encephalopathic signs following intraventricular administration of MTX developed DNL after a mea...

  16. Primary multifocal osseous Hodgkin's lymphoma

    Directory of Open Access Journals (Sweden)

    Kohler Janice

    2008-03-01

    Full Text Available Abstract Background Hodgkin's disease (HD most commonly presents with progressive painless enlargement of peripheral lymph nodes, especially around the cervical region. A few children have systemic symptoms and weight loss. At the time of diagnosis, osseous involvement is uncommon Case presentation A case is described of Primary Multifocal Osseous Hodgkin's Lymphoma in a seven-year-old boy. He presented with a painful swelling in the sternum, and further investigations revealed deposits in his L1 vertebra, the left sacro-iliac joint and the right acetabulum. Conclusion The clinical, radiological and histological features of this disease can mimic other medical conditions, including Tuberculosis, making the diagnosis difficult and often leading to delays in treatment. This is a very rare condition and we believe this to be the youngest reported case in the literature.

  17. Manifestation of leukoencephalopathy in a patient with advanced non-small cell lung cancer following treatment with gefitinib

    Institute of Scientific and Technical Information of China (English)

    HUANG Yi-sheng; HUANG Biao; WU Yi-long

    2011-01-01

    The present case is a patient with advanced non-small cell lung cancer (NSCLC) who developed leukoencephalopathy following radiotherapy and gefitinib treatments.There are rarely reports of such incidences because the median survival period of advanced NSCLC is only ten months.The features of leukoencephalopathy in this case were atypical for radiation leukoencephalopathy,so it was suspected that the leukoencephalopathy was associated with gefitinib.

  18. Leukoencephalopathy Associated with Parvovirus Infection in Cretan Hound Puppies▿

    Science.gov (United States)

    Schaudien, D.; Polizopoulou, Z.; Koutinas, A.; Schwab, S.; Porombka, D.; Baumgärtner, W.; Herden, C.

    2010-01-01

    Leukoencephalopathies in dogs encompass presumably inherited conditions such as leukodystrophies, hypomyelination or spongiform degeneration, but other causes, such as virus infections and toxic or nutritional factors, might also play a contributory role. In this report, we provide evidence of parvovirus infection and replication in the brains of five 6-week-old Cretan hound puppies suffering from a puppy shaker syndrome and leukoencephalopathy. Although these puppies belonged to two different litters, they were closely related, tracing back two generations to the same sire. Histologically, a mild to moderate lymphohistiocytic meningitis, with focal lymphohistiocytic leukoencephalitis in two animals, and a mild to moderate vacuolation with myelin loss, mainly in the white matter of the cerebellum was detected. Vacuolation was also found in the corpus callosum, fimbria hippocampi, mesencephalon, capsula interna, basal ganglia, and hypothalamus. By immunohistology and in situ hybridization, either parvoviral antigen, DNA, mRNA, or replicative intermediate DNA were detected in the cerebellum, hippocampus, periventricular areas, corpus callosum, cerebral cortex, medulla oblongata, and spinal cord. Parvovirus antigen, DNA, and mRNA were present in cells of the outer granular layer of the cerebellum and in periventricular cells, most likely representing spongioblasts, glial cells, neurons, endothelial cells, occasional macrophages, and ependymal cells. Sequencing revealed canine parvovirus type 2 stretches. Thus, an association of parvovirus infection with the leukoencephalopathy seems likely, possibly facilitated by a genetic predisposition due to the mode of inbreeding in this particular dog breed. PMID:20592142

  19. Multifocal Osteonecrosis Secondary to Chronic Alcohol Ingestion

    Directory of Open Access Journals (Sweden)

    Kazu Matsumoto

    2015-01-01

    Full Text Available Multifocal osteonecrosis is a relatively rare disorder with an estimated incidence of around 3% among patients diagnosed as having osteonecrosis. Multifocal osteonecrosis is caused by the several conditions including corticosteroid treatment, coagulation disorders, connective tissue disorders including systemic lupus erythematosus (SLE, inflammatory bowel disease, renal transplantation, and underlying malignancies. Alcohol abuse is one of the risk factors for osteonecrosis, and alcohol-induced osteonecrosis is 5% among all the osteonecrosis. Furthermore, the overall incidence of alcohol-induced multifocal osteonecrosis was approximately 6% among all the osteonecrosis induced by the alcohol. Therefore, here, we report an extremely rare case of alcohol-induced multifocal osteonecrosis involving three joints (two knees and one hip and review the related literature.

  20. Use of interleukin-2 for management of natalizumab-associated progressive multifocal leukoencephalopathy: case report and review of literature.

    Science.gov (United States)

    Dubey, Divyanshu; Zhang, Yinan; Graves, Donna; DeSena, Allen D; Frohman, Elliot; Greenberg, Benjamin

    2016-05-01

    A 51-year-old woman with relapsing-remitting multiple sclerosis (RRMS) and 3-year history of natalizumab use developed expressive aphasia. A brain magnetic resonance image (MRI) showed left frontotemporal and right parietal lesion with mild contrast enhancement and cerebrospinal fluid (CSF) was positive for John Cunningham virus (JCV) by polymerase chain reaction (PCR). The patient received five cycles of plasmapheresis followed by intravenous immunoglobulin. Despite this intervention, her speech deteriorated and she developed right hemiparesis. Upon referral to our institution, CSF quantitative JCV PCR was notable for 834 copies/ml. The patient was given an initial dose of 50,000 units of interleukin-2 (IL-2) subcutaneously (SQ) followed by 1 million units IL-2 SQ daily. Due to concern for immune reconstitution inflammatory syndrome (IRIS), the patient also received intravenous methylprednisone weekly. The regimen was tolerated well by the patient with no severe adverse effects. Clinically, the patient showed some improvement, and became more responsive and regained right lower extremity antigravity strength. After 12 weeks of IL-2 therapy, JCV quantitative PCR was notable for 31 copies/ml and the patient was more responsive. Due to persistence of JCV, IL-2 therapy was changed to mefloquine. At follow up after 6 months, the patient showed no clinical deterioration.

  1. Multi-focal multiphoton lithography.

    Science.gov (United States)

    Ritschdorff, Eric T; Nielson, Rex; Shear, Jason B

    2012-03-01

    Multiphoton lithography (MPL) provides unparalleled capabilities for creating high-resolution, three-dimensional (3D) materials from a broad spectrum of building blocks and with few limitations on geometry, qualities that have been key to the design of chemically, mechanically, and biologically functional microforms. Unfortunately, the reliance of MPL on laser scanning limits the speed at which fabrication can be performed, making it impractical in many instances to produce large-scale, high-resolution objects such as complex micromachines, 3D microfluidics, etc. Previously, others have demonstrated the possibility of using multiple laser foci to simultaneously perform MPL at numerous sites in parallel, but use of a stage-scanning system to specify fabrication coordinates resulted in the production of identical features at each focal position. As a more general solution to the bottleneck problem, we demonstrate here the feasibility for performing multi-focal MPL using a dynamic mask to differentially modulate foci, an approach that enables each fabrication site to create independent (uncorrelated) features within a larger, integrated microform. In this proof-of-concept study, two simultaneously scanned foci produced the expected two-fold decrease in fabrication time, and this approach could be readily extended to many scanning foci by using a more powerful laser. Finally, we show that use of multiple foci in MPL can be exploited to assign heterogeneous properties (such as differential swelling) to micromaterials at distinct positions within a fabrication zone.

  2. [Multifocal epileptic crises following mumps].

    Science.gov (United States)

    Parain, D; Boulloche, J

    1988-04-01

    A 15 years-old girl with no previous history of epilepsy or neurological disease presented three types of epileptic symptoms the same day: 1) clusters of rhythmic myoclonus of the left hemiface; 2) episodes of painful paresthesias of the left arm followed by secondary generalised seizures; 3) episodes of elementary visual hallucinations of the right hemifield. She had several seizures each hour and some were recorded. There were no EEG abnormalities during the facial myoclonus but rapid rhythms were seen during the sensory and visual partial seizures on the right parietal and left occipital lobes. There was no fever and no drowsiness. The CSF tap showed pleocytosis. Serological studies indicated recent mumps. The drugs were initially inefficient. The seizures disappeared after a month. The drugs were stopped after three months and the seizures had not relapsed after a one year's follow-up. Though there were no other sign of encephalitis, we believe that episode of multifocal seizures was due to mumps encephalitis.

  3. Reversible acute methotrexate leukoencephalopathy: atypical brain MR imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Ziereisen, France; Damry, Nash; Christophe, Catherine [Queen Fabiola Children' s University Hospital, Department of Radiology, Brussels (Belgium); Dan, Bernard [Queen Fabiola Children' s University Hospital, Department of Neurology, Brussels (Belgium); Azzi, Nadira; Ferster, Alina [Queen Fabiola Children' s University Hospital, Department of Paediatrics, Brussels (Belgium)

    2006-03-15

    Unusual acute symptomatic and reversible early-delayed leukoencephalopathy has been reported to be induced by methotrexate (MTX). We aimed to identify the occurrence of such atypical MTX neurotoxicity in children and document its MR presentation. We retrospectively reviewed the clinical findings and brain MRI obtained in 90 children treated with MTX for acute lymphoblastic leukaemia or non-B malignant non-Hodgkin lymphoma. All 90 patients had normal brain imaging before treatment. In these patients, brain imaging was performed after treatment completion and/or relapse and/or occurrence of neurological symptoms. Of the 90 patients, 15 (16.7%) showed signs of MTX neurotoxicity on brain MRI, 9 (10%) were asymptomatic, and 6 (6.7%) showed signs of acute leukoencephalopathy. On the routine brain MRI performed at the end of treatment, all asymptomatic patients had classical MR findings of reversible MTX neurotoxicity, such as abnormal high-intensity areas localized in the deep periventricular white matter on T2-weighted images. In contrast, the six symptomatic patients had atypical brain MRI characterized by T2 high-intensity areas in the supratentorial cortex and subcortical white matter (n=6), cerebellar cortex and white matter (n=4), deep periventricular white matter (n=2) and thalamus (n=1). MR normalization occurred later than clinical recovery in these six patients. In addition to mostly asymptomatic classical MTX neurotoxicity, MTX may induce severe but reversible unusual leukoencephalopathy. It is important to recognize this clinicoradiological presentation in the differential diagnosis of acute neurological deterioration in children treated with MTX. (orig.)

  4. Reversible posterior leukoencephalopathy syndrome in a child treated with bevacizumab.

    Science.gov (United States)

    Levy, Carolyn Fein; Oo, Khine Zin; Fireman, Fernando; Pierre, Louisdon; Bania, Marita A; Sadanandan, Swayamprabha; Yamashiro, Darrell J; Glade Bender, Julia L

    2009-05-01

    Bevacizumab is a monoclonal antibody targeting vascular endothelial growth factor (VEGF). Hypertension is a well-recognized, common side effect of VEGF blocking agents. The reversible posterior leukoencephalopathy syndrome (RPLS) has been described as a rare but serious consequence of bevacizumab administration. We present a case of a 6-year-old child with refractory hepatoblastoma who developed hypertensive crisis, seizures and MRI changes consistent with RPLS while receiving bevacizumab with gemcitabine and oxaliplatin. Findings completely resolved without neurologic sequelae with stringent blood-pressure control. Better understanding of risk for RPLS, prompt recognition and aggressive management will be required as bevacizumab gains wider use in pediatrics.

  5. Insights into the dynamics of hereditary diffuse leukoencephalopathy with axonal spheroids

    Science.gov (United States)

    Van Gerpen, J A.; Wider, C; Broderick, D F.; Dickson, D W.; Brown, L A.; Wszolek, Z K.

    2008-01-01

    Objective: To report a new American family with hereditary diffuse leukoencephalopathy with spheroids (HDLS), including serial, presymptomatic and symptomatic, cranial MRIs from the proband. Methods: We report clinical and genealogic investigations of an HDLS family, sequential brain MRIs of the proband, and autopsy slides of brain tissue from the proband’s father. Results: We identified seven affected family members (five deceased). The mean age at symptomatic disease onset was 35 years (range: 20–57), and the mean disease duration was 16 years (range: 3–46). Five affected individuals initially manifested memory disturbance and behavioral changes, whereas two experienced a mood disorder as their presenting symptom. Our proband’s father had been diagnosed clinically with vascular dementia, but his brain autopsy was consistent with HDLS. The proband had a cranial MRI prior to symptom onset, with two subsequent MRIs performed during follow-up. These serial images reveal a progressive, confluent, frontal-predominant leukoencephalopathy with symmetric cortical atrophy. Conclusions: The proband of our newly identified hereditary diffuse leukoencephalopathy with spheroids (HDLS) kindred had subtle evidence of an incipient leukoencephalopathy on a presymptomatic cranial MRI. Conceivably, MRI may facilitate identifying affected presymptomatic individuals within known HDLS kindreds, increasing the likelihood of isolating the causative genes. GLOSSARY DLS = diffuse leukoencephalopathy with spheroids; FLAIR = fluid-attenuated inversion recovery; HDLS = hereditary diffuse leukoencephalopathy with spheroids; LENAS = leukoencephalopathy with neuroaxonal spheroids; LFB = Luxol fast blue; NAL = neuroaxonal leukodystrophy; POLD = pigmentary type of orthochromatic leukodystrophy. PMID:18794495

  6. Broadband Multifocal Conic-Shaped Metalens

    CERN Document Server

    Bao, Yanjun; Fang, Zheyu

    2016-01-01

    Compared with lens with one focal point,multifocal lens has a lower focusing quality with high background noise. This is arisen from the construction of multifocal lens, which is usually divided into several zones, with each corresponds to one focal point.Light passing through different zones cannot constructively interfere at the foci, resulting in a decreased optical performance. Here, we propose two multifocal metalenses with nanoslits arranged in an ellipse and a hyperbola, with both are able to focus incident light at their multifoci constructively, giving a better focusing properties than that designed by conventional methods.We theoretically and experimentally demonstrate that, within a broadband wavelength range (600-900 nm), the ellipse-shaped metalens (ESM) can focus the lights with opposite circular polarizations (CP) at its two focal points, respectively,while a hyperbola-shaped metalens (HSM) can only focus one particular CP light at its both foci, simultaneously.This types of conic-shaped metale...

  7. Additional multifocal sulcus-based intraocular lens: alternative to multifocal intraocular lens in the capsular bag.

    Science.gov (United States)

    Schrecker, Jens; Kroeber, Sandra; Eppig, Timo; Langenbucher, Achim

    2013-04-01

    To compare the visual outcomes of additional multifocal intraocular lenses (IOLs) for sulcus fixation with those of standard multifocal IOLs in the capsular bag. Department of Ophthalmology, Rudolf-Virchow-Klinikum Glauchau, Glauchau, Germany. Prospective controlled clinical trial. Eyes had phacoemulsification and implantation of a monofocal IOL in the capsular bag and an additional aberration-free diffractive IOL in the ciliary sulcus (multifocal add-on IOL group). Measurements of uncorrected and distance-corrected distance, intermediate, and near visual acuities; contrast sensitivity; and defocus curve were performed 3 months postoperatively. Results were compared with those in eyes with an aberration-correcting diffractive posterior chamber IOL (multifocal PC IOL group). The multifocal add-on IOL group comprised 34 eyes of 20 patients and the multifocal PC IOL group, 31 eyes of 17 patients. Cataract surgery, IOL implantation, and the postoperative course were uneventful in all cases. There were no statistically significant differences in uncorrected and distance-corrected distance, intermediate, or near visual acuities between the 2 groups. The median uncorrected distance visual acuity was 0.00 logMAR in both groups, and the median uncorrected near visual acuity was 0.10 logMAR in both groups. Contrast sensitivity testing yielded significantly better results in the multifocal add-on IOL group, especially at spatial frequencies over 1.5 cycles per degree. Defocus curves were similar in the 2 groups. Visual performance with a multifocal diffractive add-on IOL was equivalent to that achieved with a commonly used multifocal diffractive PC IOL. Copyright © 2013 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.

  8. A case of leukoencephalopathy, cerebral calcifications and cysts

    Directory of Open Access Journals (Sweden)

    Ajay Gulati

    2011-01-01

    Full Text Available Triad of leukoencephalopathy, cerebral calcifications and cysts (LCC is a recently reported rare disease named ′Labrune syndrome′ after the first case was reported in 1996 by Labrune et al. Herein, we report a case of a 36-year-old man with mild right-sided weakness and seizures for 5 years. CT of brain revealed extensive calcification involving bilateral basal ganglia, right thalamus and bilateral deep cerebellar nuclei. A supratentorial cystic lesion with blood fluid level was seen in left occipitotemporal region. MRI examination revealed diffuse symmetric white matter hyperintensity suggesting leukoencephalopathy. On follow-up, patient reported improvement in the weakness and no further seizure episodes. However, follow-up of MRI revealed persistence of lesions. Differential diagnosis considered were parasitic infections (hydatid, cysticercosis, Coat′s plus disease and causes of diffuse cerebral calcification like Fahr′s disease and post-radiotherapy/chemotherapy. Serology for parasitic infections was negative. No history of radiotherapy or chemotherapy in the past could be elicited in the history. Another close differential is Coat′s plus disease which can mimic LCC pathologically.

  9. Toxic leukoencephalopathy due to yam bean seeds poisoning.

    Science.gov (United States)

    Fu, Pin-Kuei; Wang, Pao-Yu

    2012-07-01

    Toxic leukoencephalopathy is attributed to exposure to a wide variety of agents, including systemic chemotherapy, cranial irradiation, illicit drug abuse, and toxins from the environment. Diagnosis of this disease requires documented exposure to a toxin, neurobehavioral deficits, and typical neuroimaging abnormalities. Intoxication by compounds extracted from yam bean seeds may mimic cyanide poisoning but fail to respond to antidotal therapy. We report a 54-year-old Chinese woman who developed disturbed consciousness after eating 40 pieces of yam bean seeds. Head computed tomography obtained 24 hours after the episode was normal. However, magnetic resonance imaging obtained 20 days after the episode revealed symmetrical faint high signal over the bilateral periventricular white matter on T1-weighted image, which turned into diffuse and symmetrical bright high signal on FLAIR. The diagnosis of this patient was toxic leukoencephalopathy by yam bean seeds intoxication. The changes in brain images after yam bean seeds intoxication have not ever been reported. Physicians in Asia and the Pacific islands should have a high index of suspicion when they care for patients with acute confusion and a high anion gap metabolic acidosis but normal serum cyanide level.

  10. Clinical features and magnetic resonance image analysis of 15 cases of demyelinating leukoencephalopathy induced by levamisole

    Science.gov (United States)

    YAN, RUIFANG; WU, QINGWU; REN, JIPENG; CUI, HONGKAI; ZHAI, KAIHUA; ZHAI, ZHANSHENG; DUAN, QINGHONG

    2013-01-01

    The aim of this study was to explore the diagnostic value of magnetic resonance imaging (MRI) for levamisole-induced demyelinating leukoencephalopathy. The clinical features and MRI findings of 15 patients with levamisole-induced demyelinating leukoencephalopathy were retrospectively analyzed. The abnormality rate of the patients was demonstrated to be 100% by MRI, and scattered multiple cerebral foci were observed in all of the patients. The majority of the foci were located at the centrum ovale, peri-lateral cerebral ventricles and basal ganglia, while the remainder were located in the brain stem and cerebellum, as well as in the white matter regions of the temporal, frontal, apical and occipital lobes. In addition, mottling and ring-shaped enhancements were observed. The study demonstrated that MRI effectively displays demyelinating leukoencephalopathy, and that the combination of MRI with the medical history of the patient is of significance for the early diagnosis, differentiation and treatment of demyelinating leukoencephalopathy. PMID:23935721

  11. Genetics Home Reference: cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy

    Science.gov (United States)

    ... Encyclopedia: Stroke Health Topic: Dementia Health Topic: Ischemic Stroke Genetic and Rare Diseases Information Center (1 link) Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy Additional NIH ... Educational Resources (6 links) ...

  12. Permanent bilateral cortical blindness due to reversible posterior leukoencephalopathy syndrome.

    Science.gov (United States)

    Iwama, Mayumi; Takahashi, Hiroshi; Takagi, Ryo; Hiraoka, Miki

    2011-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is induced by acute cerebral edema. Its symptoms include seizures, headache, altered mental status, and visual disturbances. The clinical and radiological findings are usually transient. This report describes a case of RPLS resulting in bilateral total blindness. A 40-year-old man presented with lethargy and bilateral visual loss. He had a 20-year history of hypertension, but had never been treated. On presentation, the left eye was able to perceive light, but the right eye was not. Radiological examination showed diffuse edema in the brain, and ocular fundus examination revealed severe bilateral hypertensive retinopathy. Antihypertensive therapy improved the patient's general condition, including blood pressure. Radiological findings 5 months later showed resolution of most of the abnormal signal areas. However, total blindness had developed in both eyes by day 15, and two courses of pulsed corticosteroid therapy failed to restore the visual loss.

  13. Multifocal epithelial hyperplasia: A potentially precancerous disease? (Review)

    Science.gov (United States)

    BASCONES-MARTÍNEZ, A.; COK, S.; BASCONES-ILUNDÁIN, C.; ARIAS-HERRERA, S.; GOMEZ-FONT, R.; BASCONES-ILUNDÁIN, J.

    2012-01-01

    Multifocal epithelial hyperplasia (MEH), also known as Heck’s disease, manifests as a papulonodular lesion in the oral mucosa and has been associated with the human papillomavirus, a virus related to various precancerous diseases in the oral cavity. It has a predisposition for the female gender and for children. Although the majority of reported cases have been among American Indians and Eskimos, it has been described in multiple ethnic groups in various geographical locations. The objective of this review was to report on the clinical characteristics and epidemiology of MEH and its possible correlation with oral cancer. It is based on a search of articles in international journals published prior to April 2011, using the PubMed database and selecting articles related to the epidemiology and clinical characteristics of MEH. The review revealed a higher number of cases in individuals of American Indian origin and a predilection of the disease for the female gender and for patients between the 1st and 2nd decades of life. The most frequent lesion site was the lower lip. The disease has been associated with socio-economic and genetic factors, among others. No cases of malignant transformation have been reported. PMID:22740890

  14. Postinfectious encephalitis with multifocal white matter lesions.

    Science.gov (United States)

    Boulloche, J; Parain, D; Mallet, E; Tron, P

    1989-08-01

    Two cases of multifocal white matter lesions occurring after viral illness are reported. Evoked potentials study and cranial magnetic resonance imaging (T2-weighted image) showed early abnormalities while CT scan was initially normal. Patients improved dramatically with steroid therapy. It would seem that because of a considerable responsiveness to steroids this affection should be differentiated from other types of encephalitis. Relations with multiple sclerosis are discussed.

  15. Imaging of chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Khanna, Geetika; Sato, Takashi S P; Ferguson, Polly

    2009-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder of children and young adults that is characterized by nonbacterial osteomyelitis. Patients typically present with multifocal bone pain secondary to sterile osseous inflammation, and the disease has a relapsing and remitting course. The cause of CRMO remains unclear, although the results of several studies have suggested a genetic component. The typical imaging findings of CRMO include lytic and sclerotic lesions in the metaphyses of long bones and the medial clavicles. Other common sites of disease are the vertebral bodies, pelvis, ribs, and mandible. CRMO is often bilateral and multifocal at presentation. Owing to the lack of a diagnostic test, CRMO remains a diagnosis of exclusion. Although generally a self-limiting disease, CRMO can have a prolonged course and result in significant morbidity. Radiologists can be the first to suggest this diagnosis given its characteristic radiographic appearance and distribution of disease. Radiologists should be familiar with the typical imaging findings of CRMO to prevent unnecessary multiple biopsies and long-term antibiotic treatment in children with CRMO.

  16. Multifocal pigmented villonodular synovitis in a child

    Science.gov (United States)

    Zhao, Liang; Zhou, Kaiyu; Hua, Yimin; Li, Yifei; Mu, Dezhi

    2016-01-01

    Abstract Introduction: Pigmented villonodular synovitis (PVNS) is a rare, benign proliferative disorder of the synovial membrane that typically presents in adults and affects a single joint. Multifocal PVNS is very rare, particularly in childhood. We reported a rare case of multifocal PVNS affecting over 20 joints in a child. Clinical procedure: A 7-year-old female patient had a 6-month history of multifocal joints swelling with mild pain. She was diagnosed as polyarticular juvenile idiopathic arthritis at a local hospital. Naproxen, methotrexate, infliximab, and pavlin were used to treat the patient for 2 months. However, the treatment had no effect, the joints swelling remained. The patient was then transferred to our hospital. Physical examination revealed multiple joints swelling, especially in the shoulders joints. Puncture fluid from a shoulder joint was bloody. Magnetic resonance imaging (MRI) revealed synovial thickening and hemosiderin deposition. Biopsy of joint synovium found villous nodules, the invasion of foam cells, and hemosiderin deposition. By collecting all of the evidence, the diagnosis of PVNS was confirmed. Conclusions: PVNS was easily misdiagnosed as rheumatoid arthritis and the formal treatment was usually delayed. This case described here is the first case of PVNS involving such a large numbers of joints that has been reported in the literature. PMID:27537585

  17. Multifocal epithelial hyperplasia. Report of nine cases.

    Science.gov (United States)

    Ledesma-Montes, Constantino; Vega-Memije, Elisa; Garcés-Ortíz, Maricela; Cardiel-Nieves, Maritza; Juárez-Luna, Claudia

    2005-01-01

    Multifocal epithelial hyperplasia (MEH) is also known as focal epithelial hyperplasia, Heck's disease or multifocal papillomavirus-induced epithelial hyperplasia. It is characterised by the presence of multiple lesions in the oral mucosa of children and it has been associated with the presence of the human papillomavirus. The aim of this study was to determine the clinico-pathological features of the cases diagnosed as MEH in the Service of Dermatology of the Hospital Manuel Gea González (SDHMGG). The files of the SDHMGG were reviewed and all cases diagnosed as MEH were retrieved. Nine MEH cases were found. Most of the patients were 20 year-old or younger (67%) and females were more commonly affected (78%). All patients presented multiple lesions and always, close relatives with similar lesions were found. Lesions were located most commonly in the buccal mucosa, lower lip and commissures. MEH is a soft tissue intraoral condition that needs treatment solely of the traumatised lesions or those with cosmetic problems. Remaining lesions will disappear with the age of the patients. It is suggested that this entity should be named multifocal epithelial hyperplasia since this name describes better the clinico-pathological and microscopic features of the disease.

  18. Pediatric multifocal liver lesions evaluated by MRI

    Directory of Open Access Journals (Sweden)

    Majed Almotairi

    2015-01-01

    Full Text Available Objective: The purpose of this study is to present our experience with MRI evaluation of multifocal liver lesions in children and describe the MRI characteristics of these lesions. Patients and Methods: A retrospective review of consecutive MRI exams performed for the evaluation of multiple liver lesions between 1 January 2007 and 31 December 2012 was done to note the number of lesions, the size of the largest lesion, MR signal characteristics, and background liver. Final diagnosis was assigned to each case based on pathology in the available cases and a combination of clinical features, imaging features, and follow-up in the remaining cases. Results: A total of 48 children (22 boys, 26 girls; age between 3 months and 18 years with average age 10.58 years and median age 11 years were included in the study. Totally 51 lesion diagnoses were seen in 48 children that included 17 focal nodular hyperplasia (FNH, 8 hemangiomas, 7 metastases, 6 regenerative nodules, 3 adenomas, 3 abscesses, and one each of angiomyolipoma, epithelioid hemangioendothelioma, focal fatty infiltration, hepatocellular carcinoma, hepatic infarction, nodular regenerative hyperplasia, and hepatic cyst. Background liver was normal in 33, cirrhotic in 10, fatty in 3, and siderotic in 2 children. Most FNH, hemangiomas, and regenerative nodules showed characteristic MRI features, while metastases were variable in signal pattern. Conclusion: Many commonly seen multifocal liver lesions in children have characteristic MRI features. MRI can help to arrive at reasonable differential diagnoses for multifocal liver lesions in children and guide further investigation and management.

  19. Lente intra-ocular multifocal difrativa apodizada: resultados Diffractive apodized multifocal intraocular lens: results

    Directory of Open Access Journals (Sweden)

    Virgilio Centurion

    2007-12-01

    Full Text Available OBJETIVO: Mostrar os resultados visuais e refracionais com lente intra-ocular multifocal difrativa apodizada. MÉTODOS: Estudo de 100 olhos de 50 pacientes com catarata, submetidos à facoemulsificação com implante bilateral de lente intra-ocular (LIO multifocal difrativa apodizada. Foi avaliada a acuidade visual binocular sem e com correção para longe e perto, a previsibilidade refracional e a freqüência de uso de óculos. RESULTADOS: A acuidade visual sem correção para longe foi de e " 20/30 em 97,56% dos olhos operados e e" J2 em 100%, sendo que 82% dos pacientes nunca usam óculos e 16% usam de forma esporádica. CONCLUSÃO: A LIO multifocal difrativa apodizada mostrou ser uma opção previsível, reproduzível e segura na correção dos vícios de refração para longe e perto durante a cirurgia da catarata, permitindo elevado índice de independência ao uso de óculos.OBJECTIVE: To show visual and refraction results using multifocal diffractive apodized intraocular lens. METHODS: The study of 100 eyes of 50 patients with cataract, submitted to phacoemulsification with bilateral implant of multifocal diffractive apodized intraocular lens (IOL. Binocular visual acuity was evaluated with and without correction for near and distance, and refraction previsibility and frequency of wearing glasses. RESULTS: Visual acuity without correction for distance was e" 20/30 in 97.56% of eyes operated on and e" J2 in 100%, of these 82% of patients never wear glasses and 16% wear glasses sporadically. CONCLUSION: Multifocal diffractive apodized IOL proved to be a foreseeable option, reproducible and safe in the correction of refraction errors for distance and near during cataract surgery, enabling a high rate of independence from the use of glasses.

  20. Fibrose sub-retiniana progressiva e corioretinite granulomatosa multifocal

    OpenAIRE

    Miguel Hage Amaro; Cristina Muccioli; Mario Martins dos Santos Motta

    2006-01-01

    We describe a case of progressive subretinal fibrosis and multifocal chorioretinitis along with its findings on both fluorescein and indocyanine green angiography. The progressive subretinal fibrosis syndrome is a severe subset of multifocal choroiditis. The clustering of lesions around the nerve optic head may mean that the disease is spread through the flow in and out of the eye around the optic nerve.Descrevemos um caso de fibrose sub-retiniana progressiva e corrioretinite multifocal granu...

  1. Posterior reversible leukoencephalopathy syndrome in children with hematologic disorders

    Directory of Open Access Journals (Sweden)

    Barış Malbora

    2010-09-01

    Full Text Available Objective: Posterior reversible leukoencephalopathy syndrome (PRES is characterized by headache, altered mental status, cortical blindness, and seizures associated with neuroradiological findings. It involves predominantly white matter of the parieto-occipital lobes. Several medications and disorders play a role in the etiology of PRES. In this study, we aimed to show how the prognosis of PRES in hematological diseases of childhood might be according to the etiological factors.Materials and Methods: Here, we report PRES in six patients, aged 4 to 14 years, with diagnoses of leukemia and aplastic anemia. Results: Suggested causes in our patients were chemotherapeutics, hypertension, infection and antimicrobial drug administration, tumor lysis syndrome, acute renal failure and hemodialysis, immunosuppressive drug administration, and hypomagnesemia. One of the patients died of sepsis, renal failure and pulmonary hemorrhage and another died of relapse after total recovery from PRES. The other four patients are under follow-up without problems. Conclusion: We suggest that PRES can recover fully with early diagnosis and treatment whereas it can show poor prognosis depending on the etiology.

  2. Elevation of blood ciclosporin levels by voriconazole leading to leukoencephalopathy

    Directory of Open Access Journals (Sweden)

    Qu Caihong

    2013-01-01

    Full Text Available We report that one 18-year-old female patient with no epilepsia history developed severe epileptiform seizures while she was receiving "ciclosporin A (CsA-mycophenolate-methylprednisolone" antirejection therapy after combining one week′s voriconazole administration following allogeneic hematopoietic stem cell transplantation (allo-HSCT for myelodysplastic syndromes (MDS. Her blood concentration of CsA was 378 ng/ml (elevated ↑64%, contrasted with the level before the addition of voriconazole on the second day of admission, and the MRI of head showed leukoencephalopathy in bilateral occipital and left frontal lobe on the 4 th day of admission. The most likely mechanism is that because of voriconazole′s enzyme inhibition and CsA as the substrate of hepatic enzymes, voriconazole elevated the blood concentration of CsA and enhanced its toxicity. This case highlights the importance of clinical pharmacists joining the medical team and optimizing the patients′ treatment protocols by performing a systematic literature research, accumulating the knowledge of the potential drug interaction and examining prescriptions.

  3. Acute fever and delayed leukoencephalopathy following low dose intraventricular methotrexate.

    Science.gov (United States)

    Boogerd, W; vd Sande, J J; Moffie, D

    1988-10-01

    Nine out of 14 patients treated with intraventricular methotrexate (MTX) for meningeal carcinomatosis from breast carcinoma and surviving more than 4 months developed disseminated necrotising leukoencephalopathy (DNL). All four patients who had received both intraventricular MTX and whole brain radiotherapy developed DNL. Five of the six patients who experienced an acute febrile reaction with mild encephalopathic signs following intraventricular administration of MTX developed DNL after a mean time of 5 months and a low mean dose of 44 mg MTX. DNL was also noted in two patients without a previous febrile reaction or whole brain radiotherapy, following prolonged intraventricular MTX therapy after a mean time of 19.5 months and a mean dose of 147 mg MTX. These findings confirm the hazards of (1) high cumulative doses of intrathecal MTX and (2) combined intrathecal chemotherapy and whole brain radiotherapy. This study also suggests a possible relationship between an early and transient febrile reaction during intraventricular administration of MTX and the development of DNL.

  4. Acute fever and delayed leukoencephalopathy following low dose intraventricular methotrexate.

    Science.gov (United States)

    Boogerd, W; vd Sande, J J; Moffie, D

    1988-01-01

    Nine out of 14 patients treated with intraventricular methotrexate (MTX) for meningeal carcinomatosis from breast carcinoma and surviving more than 4 months developed disseminated necrotising leukoencephalopathy (DNL). All four patients who had received both intraventricular MTX and whole brain radiotherapy developed DNL. Five of the six patients who experienced an acute febrile reaction with mild encephalopathic signs following intraventricular administration of MTX developed DNL after a mean time of 5 months and a low mean dose of 44 mg MTX. DNL was also noted in two patients without a previous febrile reaction or whole brain radiotherapy, following prolonged intraventricular MTX therapy after a mean time of 19.5 months and a mean dose of 147 mg MTX. These findings confirm the hazards of (1) high cumulative doses of intrathecal MTX and (2) combined intrathecal chemotherapy and whole brain radiotherapy. This study also suggests a possible relationship between an early and transient febrile reaction during intraventricular administration of MTX and the development of DNL. Images PMID:3225584

  5. A brainstem variant of reversible posterior leukoencephalopathy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kitaguchi, H.; Tomimoto, H.; Terada, K. [Kyoto University, Department of Neurology, Graduate School of Medicine, Sakyo-ku, Kyoto (Japan); Miki, Y.; Yamamoto, A. [Kyoto University, Department of Nuclear Medicine and Diagnostic Imaging, Graduate School of Medicine, Sakyo-ku, Kyoto (Japan); Satoi, H.; Kanda, M. [Ijinkai Takeda General Hospital, Department of Neurology, Fushimi-ku, Kyoto (Japan); Fukuyama, H. [Kyoto University, Human Brain Research Center, Graduate School of Medicine, Sakyo-ku, Kyoto (Japan)

    2005-09-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is caused by various heterogeneous factors, the commonest being hypertension, followed by nonhypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. Patients with RPLS exhibit bilateral white and gray matter abnormalities in the posterior aspects of the cerebral hemispheres. However, this syndrome may affect the brainstem predominantly, and these cases are designated as hypertensive brainstem encephalopathy. We present here two patients with reversible brainstem encephalopathy: one with hypertension and the other without hypertension. These patients presented with swelling and diffuse hyperintensities of the brainstem in fluid-attenuated inversion-recovery (FLAIR) and T2-weighted MRI, but with relatively mild clinical symptoms. They recovered without major neurological deficits, but had residual lacunar lesions in the pons. Reversible brainstem encephalopathy with characteristic MRI features was found in both hypertensive and nonhypertensive patients. These patients were diagnosed with a brainstem variant of RPLS, which is potentially fully reversible after an adequate treatment, and therefore should be carefully differentiated from other brainstem disease conditions. (orig.)

  6. Reversible posterior leukoencephalopathy in the course of Goodpasture syndrome.

    Science.gov (United States)

    Ozkok, Abdullah; Elcioglu, Omer Celal; Bakan, Ali; Atilgan, Kadir Gokhan; Alisir, Sabahat; Odabas, Ali Riza

    2012-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by headache, altered consciousness, seizures, and cortical blindness. The most frequent etiological factors are hypertension, kidney diseases, and immunosuppressive drugs such as steroids and cyclophosphamide. Herein we present a case of a 22-year-old female patient presented with alveolar hemorrhage and acute renal failure necessitating hemodialysis. In renal biopsy, necrotizing crescentic glomerulonephritis and immunofluorescence pattern compatible with Goodpasture syndrome were found. Anti-glomerular basement membrane antibody result was positive. At follow-up, respiratory failure ensued, steroid pulse treatment was started, and she was transferred to intensive care unit (ICU). In the ICU, she had visual disturbances and blindness together with seizures. Cranial magnetic resonance imaging (MRI) revealed irregular T2- and fluid-attenuated inversion recovery (FLAIR)-weighted lesions in bilateral occipital lobes. On clinical and radiological grounds, RPLS was diagnosed. With the supportive and anti-hypertensive treatment, RPLS was resolved without a sequela. Subsequent cranial MRI was totally normal. In the literature, RPLS associated with Goodpasture syndrome was reported only once. Hypertension and methylprednisolone might be the responsible etiologies in this case.

  7. [Multifocal-motor neuropathy and motor neuropathy with multifocal conduction block (Lewis-Sumner syndrome)].

    Science.gov (United States)

    Finsterer, J; Mamoli, B

    1995-01-01

    Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory involvement, but without upper motor neuron signs. The cardinal feature and primary pathophysiological basis for the weakness is the multifocal motor conduction block which remains stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal stimulus response without any change in the negative peak duration. Nerves at the site of the conduction block show demyelination, endoneural edema, rudimentary onion bulbs and lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid antibodies, which may block the Na+ channels, produce demyelination or interfere with remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and cyclophosphamide.

  8. Progressive subretinal fibrosis and multifocal granulomatous chorioretinitis Fibrose sub-retiniana progressiva e corioretinite granulomatosa multifocal

    Directory of Open Access Journals (Sweden)

    Miguel Hage Amaro

    2006-06-01

    Full Text Available We describe a case of progressive subretinal fibrosis and multifocal chorioretinitis along with its findings on both fluorescein and indocyanine green angiography. The progressive subretinal fibrosis syndrome is a severe subset of multifocal choroiditis. The clustering of lesions around the nerve optic head may mean that the disease is spread through the flow in and out of the eye around the optic nerve.Descrevemos um caso de fibrose sub-retiniana progressiva e corrioretinite multifocal granulomatosa junto com os achados de angiografia fluoresceínica e com indocianina verde e propomos uma nova fisiopatologia para a fibrose em anel justa-papilar. A síndrome de fibrose sub-retiniana progressiva é uma doença grave, rara e constitui um subtipo grave da coroidite multifocal. As múltiplas lesões com fibrose circundando o disco óptico podem significar que a doença é oriunda do fluxo do líquido céfalo-raqueano, ao redor do nervo óptico.

  9. Incidence of Leukoencephalopathy After Whole-Brain Radiation Therapy for Brain Metastases

    Energy Technology Data Exchange (ETDEWEB)

    Ebi, Junko, E-mail: junkoe@fmu.ac.jp [Department of Radiology, Fukushima Medical University, Fukushima (Japan); Sato, Hisashi; Nakajima, Masaru; Shishido, Fumio [Department of Radiology, Fukushima Medical University, Fukushima (Japan)

    2013-04-01

    Purpose: To evaluate the incidence of leukoencephalopathy after whole-brain radiation therapy (WBRT) in patients with brain metastases. Methods and Materials: We retrospectively reviewed 111 patients who underwent WBRT for brain metastases from April 2001 through March 2008 and had evaluable computed tomography (CT) and/or magnetic resonance imaging (MRI) at least 1 month after completion of WBRT. We evaluated the leukoencephalopathy according to the Common Terminology Criteria for Adverse Events, version 3.0. The patients who had brain tumor recurrence after WBRT were censored at the last follow-up CT or MRI without recurrence. To evaluate the risk factors for leukoencephalopathy, bivariate analysis was performed using a logistic regression analysis adjusted for follow-up time. Factors included in the analysis were age, gender, dose fractionation, 5-fluorouracil, methotrexate, cisplatin, and other chemotherapeutic agents. Results: The median age of the 111 patients was 60.0 years (range, 23-89 years). The median follow-up was 3.8 months (range, 1.0-38.1 months). Leukoencephalopathy developed in 23 of the 111 patients. Grades 1, 2, and 3 were observed in 8, 7, and 8 patients, respectively. The incidence was 34.4% (11 of 32), 42.9% (6 of 14), 66.7% (2 of 3), and 100% (2 of 2) of the patients who were followed up for ≥6, ≥12, ≥24, and ≥36 months, respectively. In the bivariate analysis, older age (≥65 years) was significantly correlated with higher risk of leukoencephalopathy (odds ratio 3.31; 95% confidence interval 1.15-9.50; P=.03). Conclusions: The incidence of leukoencephalopathy after WBRT was 34.4% with ≥6 months follow-up, and increased with longer follow-up. Older age was a significant risk factor. The schedule of WBRT for patients with brain metastases should be carefully determined, especially for favorable patients.

  10. Disseminated coccidioidomycosis with multifocal musculoskeletal disease involvement

    Directory of Open Access Journals (Sweden)

    Michael F. McConnell, DO

    2017-03-01

    Full Text Available We report a rare case of disseminated coccidioidomycosis with multifocal musculoskeletal involvement. The patient presented to the emergency department with left shoulder pain and swelling. Magnetic resonance imaging of the left shoulder revealed enhancing soft tissue masses, bony lesions, and fluid collections in and around the glenohumeral joint with involvement of the proximal humerus, glenoid, and rotator cuff musculature. Multiple additional areas of involvement were subsequently discovered. Fungal cultures confirmed coccidioidomycosis infection at all surgical sites with superimposed polymicrobial bacterial infection in the left shoulder.

  11. A case of leukoencephalopathy caused by radiation and chemotherapy for brain metastasis of breast cancer

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Shigeru; Sonoo, Hiroshi; Nomura, Tsunehisa; Ohkubo, Sumiko; Yamamoto, Yutaka; Tanaka, Katsuhiro; Kurebayashi, Junichi; Hiratsuka, Junichi [Kawasaki Medical School, Kurashiki, Okayama (Japan)

    2002-08-01

    A case of treatment-related leukoencephalopathy is presented. A patient with breast cancer metastasis to the brain, liver, bone and distant lymph nodes was treated with whole brain radiation and docetaxcel. Eleven months after radiation, magnetic resonance imaging showed diffuse leukoencephalopathy. Twenty-two months after radiation, the patient had gait disturbance, parkinsonism, dementia and urinary incontinence. From this experience, stereotactic radiosurgery such as cyber knife and gamma knife therapy, representing a new modality for delivering intense focal radiation, should be come preferred techniques for treating patients with brain metastases, to avoid the potential cognitive side effects of fractionated whole-brain radiotherapy. (author)

  12. Functional outcomes after home-based rehabilitation for heroin-induced spongiform leukoencephalopathy

    Institute of Scientific and Technical Information of China (English)

    Xuhong Li; Liming Deng; Bin Ye

    2012-01-01

    A 22-year-old man with a 2-year history of heroin vapor inhalation developed spongiform leukoencephalopathy and underwent clinical and home-based rehabilitative treatments.Activities of daily living were measured using the Functional Independence Measure at discharge and at 6, 12, and 24 months after discharge.His neurological symptoms gradually disappeared with rehabilitative treatment, and the functional scale scores increased from 55 on admission to 105 at 24 months after discharge.These results suggest that home-based rehabilitation was effective in ameliorating the pathology and improving activities of daily living in this patient with heroin-induced spongiform leukoencephalopathy.

  13. Leukoencephalopathy with spheroids (HDLS) and pigmentary leukodystrophy (POLD): a single entity?

    Science.gov (United States)

    Wider, C; Van Gerpen, J A; DeArmond, S; Shuster, E A; Dickson, D W; Wszolek, Z K

    2009-06-02

    Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and familial pigmentary orthochromatic leukodystrophy (POLD) present as adult-onset dementia with motor impairment and epilepsy. They are regarded as distinct diseases. We review data from the literature that support their being a single entity. Apart from a slightly older age at onset, a more rapid course, and more prominent pyramidal tract involvement, familial POLD is clinically similar to HDLS. Moreover, the pathologic hallmarks of the two diseases, axonal spheroids in HDLS and pigmented macrophages in POLD, can be identified in both conditions. This supports HDLS and POLD being referred collectively as adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).

  14. Evolving paradigms in multifocal breast cancer.

    Science.gov (United States)

    Salgado, Roberto; Aftimos, Philippe; Sotiriou, Christos; Desmedt, Christine

    2015-04-01

    The 7th edition of the TNM defines multifocal breast cancer as multiple simultaneous ipsilateral and synchronous breast cancer lesions, provided they are macroscopically distinct and measurable using current traditional pathological and clinical tools. According to the College of American Pathologists (CAP), the characterization of only the largest lesion is considered sufficient, unless the grade and/or histology are different between the lesions. Here, we review three potentially clinically relevant aspects of multifocal breast cancers: first, the importance of a different intrinsic breast cancer subtype of the various lesions; second, the emerging awareness of inter-lesion heterogeneity; and last but not least, the potential introduction of bias in clinical trials due to the unrecognized biological diversity of these cancers. Although the current strategy to assess the lesion with the largest diameter has clearly its advantages in terms of costs and feasibility, this recommendation may not be sustainable in time and might need to be adapted to be compliant with new evolving paradigms in breast cancer.

  15. Comparison of a new-generation sectorial addition multifocal intraocular lens and a diffractive apodized multifocal intraocular lens.

    NARCIS (Netherlands)

    Linden, J.W.M. van der; Velthoven, M. van; Meulen, I. van der; Nieuwendaal, C.; Mourits, M.; Lapid-Gortzak, R.

    2012-01-01

    PURPOSE: To compare visual, refractive, and satisfactory outcomes between a new-generation sectorial addition multifocal intraocular lens (IOL) (Lentis Mplus LS-312; study group) and a diffractive apodized multifocal IOL (Restor SN6AD1; control group). SETTING: Private practice, Driebergen, and Depa

  16. Reversible posterior leukoencephalopathy syndrome in children; MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang Kwon; Kim, Yong Sun [Kyungpook National Univ. Taegu (Korea, Republic of); Kwon, Soon Hak [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    2001-02-01

    To find out the characteristic MR findings of reversible posterior leukoen-cephalopathy syndrome (RPLS) due to various causes in chldren. Eigh children with RPLS underwent MR imaging, and the findings were retrospectively analyzed. All eight were acutely hypertensive at the time of a neurotoxic episode. Three had intra-abdominal tumors (one adrenal pheochromo-cytoma, one para-aortic paraganglioma and one para-aortic ganglioneuroma encasing the left renal artery): three were being treated with cyclosporine: one was being treated with steroid: and one had hemolytric uremic syndrome. Initial cranial MR images were analyzed with particular emphasis on the distribution of the lesions. To assess possible sequelae, follow-up MR images were obtained in seven patients at least one week after the treatment of hypertension. Four underwent proton MR spectroscopy. Characteristic distribution of lesions in the occipital and posterior parietal lobes was identified in all cases regardless of the causes of RPLS. The cerebellum, basal ganglia, anterior parietal, and frontal lobe were involved in four, two, one, and one case, respectively. Cortical gray matter involvement was predominant in six and subcortical white matter involvement predominated in two patients. The distribution of lesions was bilateral and asymmetric. Gyriform enhancement was identified in six cases, and small hemorrhage was noted in one. In seven patients, the clinical and MR findings improved without sequelae on follow-up study. In one, proton MR spectroscopy demonstrated a high lactate peak at the time of the neurologic event. Nearnormal spectra were noted in three children who underwent proton MR spectroscopy after recovery. The MR findings of RPLS are characteristic in that lesions are distributed in the posterior region of the brain and they are reversible on follow-up study. In children with RPLS due to unknown causes, the possibility of intra-abdominal tumors should also be consiodered.

  17. Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST.

    Science.gov (United States)

    Leena, J B; Fernandes, Hilda; Swethadri, G K

    2013-06-01

    Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.

  18. Why we use AT.Lisa multifocals?

    Science.gov (United States)

    Filip, M; Nicolae, Miruna; Filip, A; Dragne, Carmen; Triantafyllidis, G; Antonescu, Cristina

    2014-01-01

    In this paper, the authors try to motivate their preference for implanting AT.Lisa Multifocals from all other premium IOL's from the market. It is emphasized, through clinical examples, that their choice comes after a long experience with this type of mul- tifocals IOL's. We make a short presentation of this particular type of MIOL's with their good but also weak points and try to motivate our decision to change from other types. We present the steps that each patient has to follow in our clinic prior to surgery itself, stressing out the idea that the discussion with the patient is very important in taking a decision regarding the implantation of a Premium IOL.

  19. Multifocal stenosing ulceration of the small intestine

    Institute of Scientific and Technical Information of China (English)

    Hugh James Freeman

    2009-01-01

    Several reports have described an apparently uncommon clinicopathological disorder that is characterized by multifocal stenosing small-intestinal ulceration. Compared to Crohn's disease, the ulcers are not transmural and typically remain shallow, and involve only the mucosa and submucosa. The disorder seems to be localized in the jejunum and proximal ileum only, and not the distal ileum or colon. Only nonspecific inflammatory changes are present without giant cells or other typical features of granulomatous inflammation. Most patients present clinically with recurrent obstructive events that usually respond to steroids, surgical resection, or both. With the development of newer imaging modalities to visualize the small-intestinal mucosa, such as double-balloon enteroscopy, improved understanding of the long-term natural history of this apparently distinctive disorder should emerge.

  20. Parent-reported cognitive function is associated with leukoencephalopathy in children with brain tumors.

    Science.gov (United States)

    Lai, Jin-Shei; Bregman, Corey; Zelko, Frank; Nowinski, Cindy; Cella, David; Beaumont, Jennifer J; Goldman, Stewart

    2017-04-26

    Cognitive dysfunction is a major concern for children with brain tumors. A valid, user-friendly screening tool could facilitate prompt referral for comprehensive neuropsychological assessments and therefore early intervention. Applications of the pediatric perceived cognitive function item bank (pedsPCF) such as computerized adaptive testing can potentially serve as such a tool given its brevity and user-friendly nature. This study aimed to evaluate whether pedsPCF was a valid indicator of cerebral compromise using the criterion of structural brain changes indicated by leukoencephalopathy grades. Data from 99 children (mean age = 12.6 years) with brain tumors and their parents were analyzed. Average time since diagnosis was 5.8 years; time since last treatment was 4.3 years. Leukoencephalopathy grade (range 0-4) was based on white matter damage and degree of deep white matter volume loss shown on MRI. Parents of patients completed the pedsPCF. Scores were based on the US general population-based T-score metric (mean = 50; SD = 10). Higher scores reflect better function. Leukoencephalopathy grade distributions were as follows: 36 grade 0, 27 grade 1, 22 grade 2, 13 grade 3, and 1 grade 4. The mean pedsPCF T-score was 48.3 (SD = 8.3; range 30.5-63.7). The pedsPCF scores significantly discriminated patients with different leukoencephalopathy grades, F = 4.14, p = 0.0084. Effect sizes ranged from 0.09 (grade 0 vs. 1) to 1.22 (grade 0 vs. 3/4). This study demonstrates that the pedsPCF is a valid indicator of leukoencephalopathy and provides support for its use as a screening tool for more comprehensive neurocognitive testing.

  1. Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation.

    NARCIS (Netherlands)

    Scheper, G.C.; Klok, T. van der; Andel, R.J. van; Berkel, C.G. van; Sissler, M.; Smet, J.; Muravina, T.I.; Serkov, S.V.; Uziel, G.; Bugiani, M.; Schiffmann, R.; Krageloh-Mann, I.; Smeitink, J.A.M.; Florentz, C.; Coster, R. van; Pronk, J.C.; Knaap, M.S. van der

    2007-01-01

    Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) has recently been defined based on a highly characteristic constellation of abnormalities observed by magnetic resonance imaging and spectroscopy. LBSL is an autosomal recessive disease, most often manifesti

  2. Posterior reversible leukoencephalopathy syndrome presenting in a post-partum, 25-year-old-female with concomitant subarachnoid hemorrhage

    Directory of Open Access Journals (Sweden)

    Daniel M. Aronovich

    2014-01-01

    Full Text Available Hinchey et al., first described that posterior reversible leukoencephalopathy syndrome has having a unique neuroradiographical finding of vasogenic edema and clinical symptoms including headache, altered mental status, seizure and visual disturbances in 1996. We present a rare case of posterior reversible leukoencephalopathy syndrome in a 2-week, post-partum G2P2A0 (normal spontaneous vaginal delivery at forty-weeks, without complications 25-year-old-female with subarachnoid hemorrhage.

  3. Posterior reversible leukoencephalopathy syndrome presenting in a post-partum, 25-year-old-female with concomitant subarachnoid hemorrhage

    Institute of Scientific and Technical Information of China (English)

    Daniel M Aronovich; Kirsten L Ritchie; Alexander J Scumpia

    2014-01-01

    Hincheyet al., first described that posterior reversible leukoencephalopathy syndrome has having a unique neuroradiographical finding of vasogenic edema and clinical symptoms including headache, altered mental status, seizure and visual disturbances in1996.We present a rare case of posterior reversible leukoencephalopathy syndrome in a2-week, post-partumG2P2A0(normal spontaneous vaginal delivery at forty-weeks, without complications)25-year-old-female with subarachnoid hemorrhage.

  4. Patient acceptability of the Tecnis® multifocal intraocular lens

    Directory of Open Access Journals (Sweden)

    Sood P

    2011-03-01

    Full Text Available Priyanka Sood1, Maria A Woodward21Emory Eye Center, Atlanta, GA, USA; 2Kellogg Eye Center, Ann Arbor, MI, USAAbstract: Cataract surgery has evolved. The goal of the surgeon includes both restoration of vision and refinement of vision. Patients' desire for spectacle independence has driven the market for presbyopia-correcting cataract surgery and development of novel intraocular lens (IOL designs. The Tecnis® Multifocal Intraocular Lens incorporates an aspheric, modified anterior prolate IOL with a diffractive multifocal lens design. The design aims to minimize spherical aberration and improve range of focus. The purpose of this review is to assess patient acceptability of the Tecnis® multifocal intraocular lens.Keywords: Tecnis®, intraocular lens, multifocal, presbyopia 

  5. Adult multifocal pigmented villonodular synovitis--clinical review.

    Science.gov (United States)

    Botez, Paul; Sirbu, Paul Dan; Grierosu, Carmen; Mihailescu, Dan; Savin, Liliana; Scarlat, Marius M

    2013-04-01

    Pigmented villonodular synovitis (PVNS) is a rare, benign proliferative disease of the synovial tissue that affects a single joint or a tendon sheath. Data from the literature present only a few cases of multifocal PVNS. This paper presents multifocal PVNS in the adult. This disease can affect bilateral shoulders, hips and knees. The diagnosis may be delayed by the slow evolution of the disease (up to ten years); some patients may be seen with late-stage degenerative joints, serious complications, painful and functionally uncompensated, with significant locomotion deficit. PVNS requires a radical treatment with prosthetic arthroplasty associated with synovectomy. Complex imaging (X-Rays, magnetic resonance imaging (MRI), ultrasound) and macroscopic appearance of the lesions during surgery confirms the clinical diagnosis of multifocal PVNS with secondary bone lesions. Histology marks the final diagnosis of multifocal PVNS. The postoperative results are good, with recovery in functional parameters of the joints with endoprosthesis.

  6. Proliferative multifocal leukoplakia better name that proliferative verrucous leukoplakia

    OpenAIRE

    Aguirre-Urizar Jose M

    2011-01-01

    Abstract In this letter I propose the name "Proliferative Multifocal Leukoplakia" with the goal of reducing under-diagnosis of this disease, improve the early diagnosis, try to make an early therapy and control, and prevent its malignant transformation.

  7. Chronic Ocular Hypertension after Treated Multifocal Bacterial Keratitis

    Directory of Open Access Journals (Sweden)

    Theodoros Athanassios Papadopoulos

    2013-02-01

    Full Text Available Purpose: To report an unusual case of multifocal bacterial keratitis that despite success-ful treatment caused chronic ocular hypertension. Methods: A 67-year-old woman with unilateral multifocal keratitis and no previous ocular pathology was admitted to our hospital. Corneal scrapings and conjunctival samples were obtained for culture and the patient received intensive therapy with fortified vancomycin and tobramycin eye drops. Results: The cultures demonstrated two strains of Staphylococcus epidermidis, one resistant to ciprofloxacin and both sensitive to vancomycin. Treatment was effective and gradually discontinued after total cessation of the inflammatory activity. During the follow-up period, the patient developed late and persistent ocular hypertension of unknown etiology, in absence of any detectable inflammation or complication, and received permanent antiglaucoma therapy. Conclusion: Differential diagnosis between fungal and bacterial infection is critical in cases of multifocal keratitis. Patients with multifocal bacterial keratitis may need intraocular pressure monitoring, even after complete infection healing.

  8. Multifocal bacterial osteomyelitis in a renal allograft recipient following urosepsis.

    Science.gov (United States)

    Valson, A T; David, V G; Balaji, V; John, G T

    2014-05-01

    Non-tubercular bacterial osteomyelitis is a rare infection. We report on a renal allograft recipient with osteomyelitis complicating urosepsis, manifesting as a multifocal infection poorly responsive to appropriate antibiotics and surgical intervention and culminating in graft loss.

  9. Orthostatic intolerance in multifocal acquired demyelinating sensory and motor neuropathy.

    Science.gov (United States)

    Tramontozzi, Louis A; Russell, James A

    2012-09-01

    We report a patient with orthostatic intolerance and syncope as a major clinical manifestation of an acquired multifocal neuropathy with the clinical, electrodiagnostic, and cerebrospinal fluid features of multifocal acquired demyelinating sensory and motor neuropathy or the Lewis-Sumner syndrome. Immunomodulatory therapy led to clinical remission of both somatic and autonomic signs and symptoms. We are unaware of a previous description of symptomatic dysautonomia in this disorder.

  10. Melanoma conjuntival multifocal recidivado originado de nevus pigmentado preexistente

    OpenAIRE

    Marcos Leandro Pereira; Dulcídio de Barros Moreira Júnior

    2014-01-01

    O melanoma conjuntival multifocal recidivado originado de nevus preexistente é extremamente raro, ocorrendo em uma pessoa para cinco milhões de habitantes. Seu estudo é de extrema relevância, devido sua potencial letalidade. Este estudo objetiva descrever um caso de melanoma conjuntival multifocal recidivado proveniente de nevus pigmentado preexistente ocorrido em Patos de Minas, MG. Este é um estudo de caso com revisão de literatura. O diagnóstico histopato...

  11. Suspected Perinatal Depression Revealed to be Hereditary Diffuse Leukoencephalopathy with Spheroids

    Science.gov (United States)

    Blume, Josefine; Weissert, Robert

    2017-01-01

    Early motor symptoms of neurodegenerative diseases often appear in combination with psychiatric symptoms, such as depression or personality changes, and are in danger of being misdiagnosed as psychogenic in young patients. We present the case of a 32-year-old woman who presented with rapid-onset depression, followed by a hypokinetic movement disorder and cognitive decline during pregnancy. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor gene, which led to the diagnosis of hereditary diffuse leukoencephalopathy with spheroids. Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is probably an under-recognized disease. HDLS should be considered in patients with rapidly progressing parkinsonian symptoms and dementia accompanied by white matter lesions. PMID:28122429

  12. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in a Chinese pedigree

    Institute of Scientific and Technical Information of China (English)

    Erhe Xu; Huiqing Dong; Milan Zhang; Min Xu

    2012-01-01

    The present study enrolled a Chinese family that comprised 34 members and spanned three generations. Eight members were diagnosed with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, and disease diagnoses corresponded with autosomal incomplete dominance inheritance. The primary clinical manifestations included paralysis, dysarthria, and mild cognitive deficits. Magnetic resonance imaging revealed diffuse leukoencephalopathy with involvement of bilateral anterior temporal lobes, in particular the pons. In addition, multiple cerebral infarction was identified in the proband. Sural nerve biopsy findings of the proband revealed granular osmophilic material deposits in the extracellular matrix, which were adjacent to smooth muscle cells of dermal arterioles. Screening exons 2-4 for NOTCH 3 mutations by direct sequencing did not reveal any abnormalities.

  13. A Founder Mutation in VPS11 Causes an Autosomal Recessive Leukoencephalopathy Linked to Autophagic Defects

    Science.gov (United States)

    Schaffner, Adam; Fedick, Anastasia; Kaye, Lauren E.; Liao, Jun; Yachelevich, Naomi; Chu, Mary-Lynn; Boles, Richard G.; Moran, Ellen; Tokita, Mari; Gorman, Elizabeth; Zhang, Wei; Xia, Fan; Leduc, Magalie; Yang, Yaping; Eng, Christine; Wong, Lee-Jun; Schiffmann, Raphael; Diaz, George A.; Kornreich, Ruth; Thummel, Ryan; Wasserstein, Melissa; Yue, Zhenyu; Edelmann, Lisa

    2016-01-01

    Genetic leukoencephalopathies (gLEs) are a group of heterogeneous disorders with white matter abnormalities affecting the central nervous system (CNS). The causative mutation in ~50% of gLEs is unknown. Using whole exome sequencing (WES), we identified homozygosity for a missense variant, VPS11: c.2536T>G (p.C846G), as the genetic cause of a leukoencephalopathy syndrome in five individuals from three unrelated Ashkenazi Jewish (AJ) families. All five patients exhibited highly concordant disease progression characterized by infantile onset leukoencephalopathy with brain white matter abnormalities, severe motor impairment, cortical blindness, intellectual disability, and seizures. The carrier frequency of the VPS11: c.2536T>G variant is 1:250 in the AJ population (n = 2,026). VPS11 protein is a core component of HOPS (homotypic fusion and protein sorting) and CORVET (class C core vacuole/endosome tethering) protein complexes involved in membrane trafficking and fusion of the lysosomes and endosomes. The cysteine 846 resides in an evolutionarily conserved cysteine-rich RING-H2 domain in carboxyl terminal regions of VPS11 proteins. Our data shows that the C846G mutation causes aberrant ubiquitination and accelerated turnover of VPS11 protein as well as compromised VPS11-VPS18 complex assembly, suggesting a loss of function in the mutant protein. Reduced VPS11 expression leads to an impaired autophagic activity in human cells. Importantly, zebrafish harboring a vps11 mutation with truncated RING-H2 domain demonstrated a significant reduction in CNS myelination following extensive neuronal death in the hindbrain and midbrain. Thus, our study reveals a defect in VPS11 as the underlying etiology for an autosomal recessive leukoencephalopathy disorder associated with a dysfunctional autophagy-lysosome trafficking pathway. PMID:27120463

  14. Chlorfenapyr-Induced Toxic Leukoencephalopathy with Radiologic Reversibility: A Case Report and Literature Review.

    Science.gov (United States)

    Baek, Byung Hyun; Kim, Seul Kee; Yoon, Woong; Heo, Tae Wook; Lee, Yun Young; Kang, Heoung Keun

    2016-01-01

    Chlorfenapyr is a widely used, moderately hazardous pesticide. Previous reports have indicated that chlorfenapyr intoxication can be fatal in humans. We reported the first non-fatal case of chlorfenapyr-induced toxic leukoencephalopathy in a 44-year-old female with resolution of extensive and abnormal signal intensities in white matter tracts throughout the brain, brain stem, and spinal cord on serial magnetic resonance imaging.

  15. Chlorfenapyr-Induced Toxic Leukoencephalopathy with Radiologic Reversibility: A Case Report and Literature Review

    OpenAIRE

    Baek, Byung Hyun; Kim, Seul Kee; Yoon, Woong; Heo, Tae Wook; Lee, Yun Young; Kang, Heoung Keun

    2016-01-01

    Chlorfenapyr is a widely used, moderately hazardous pesticide. Previous reports have indicated that chlorfenapyr intoxication can be fatal in humans. We reported the first non-fatal case of chlorfenapyr-induced toxic leukoencephalopathy in a 44-year-old female with resolution of extensive and abnormal signal intensities in white matter tracts throughout the brain, brain stem, and spinal cord on serial magnetic resonance imaging.

  16. Chlorfenapyr-induced toxic leukoencephalopathy with radiologic reversibility: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Byung Hyun; Kim, Seul Kee; Yoon, Woong; Heo, Tae Wook; Lee, Yun Young [Dept. of Radiology, Chonnam National University Hospital, Gwangju (Korea, Republic of); Kang, Heonung Keun [Dept. of Radiology, Chonnam National University Hwasun Hospital, Hwasun (Korea, Republic of)

    2016-04-15

    Chlorfenapyr is a widely used, moderately hazardous pesticide. Previous reports have indicated that chlorfenapyr intoxication can be fatal in humans. We reported the first non-fatal case of chlorfenapyr-induced toxic leukoencephalopathy in a 44-year-old female with resolution of extensive and abnormal signal intensities in white matter tracts throughout the brain, brain stem, and spinal cord on serial magnetic resonance imaging.

  17. Methotrexate-Induced Neurotoxicity and Leukoencephalopathy in Childhood Acute Lymphoblastic Leukemia

    Science.gov (United States)

    Bhojwani, Deepa; Sabin, Noah D.; Pei, Deqing; Yang, Jun J.; Khan, Raja B.; Panetta, John C.; Krull, Kevin R.; Inaba, Hiroto; Rubnitz, Jeffrey E.; Metzger, Monika L.; Howard, Scott C.; Ribeiro, Raul C.; Cheng, Cheng; Reddick, Wilburn E.; Jeha, Sima; Sandlund, John T.; Evans, William E.; Pui, Ching-Hon; Relling, Mary V.

    2014-01-01

    Purpose Methotrexate (MTX) can cause significant clinical neurotoxicity and asymptomatic leukoencephalopathy. We sought to identify clinical, pharmacokinetic, and genetic risk factors for these MTX-related toxicities during childhood acute lymphoblastic leukemia (ALL) therapy and provide data on safety of intrathecal and high-dose MTX rechallenge in patients with neurotoxicity. Patients and Methods Prospective brain magnetic resonance imaging was performed at four time points for 369 children with ALL treated in a contemporary study that included five courses of high-dose MTX and 13 to 25 doses of triple intrathecal therapy. Logistic regression modeling was used to evaluate clinical and pharmacokinetic factors, and a genome-wide association study (GWAS) was performed to identify germline polymorphisms for their association with neurotoxicities. Results Fourteen patients (3.8%) developed MTX-related clinical neurotoxicity. Of 13 patients rechallenged with intrathecal and/or high-dose MTX, 12 did not experience recurrence of neurotoxicity. Leukoencephalopathy was found in 73 (20.6%) of 355 asymptomatic patients and in all symptomatic patients and persisted in 74% of asymptomatic and 58% of symptomatic patients at the end of therapy. A high 42-hour plasma MTX to leucovorin ratio (measure of MTX exposure) was associated with increased risk of leukoencephalopathy in multivariable analysis (P = .038). GWAS revealed polymorphisms in genes enriched for neurodevelopmental pathways with plausible mechanistic roles in neurotoxicity. Conclusion MTX-related clinical neurotoxicity is transient, and most patients can receive subsequent MTX without recurrence of acute or subacute symptoms. All symptomatic patients and one in five asymptomatic patients develop leukoencephalopathy that can persist until the end of therapy. Polymorphisms in genes related to neurogenesis may contribute to susceptibility to MTX-related neurotoxicity. PMID:24550419

  18. Stroke-like pattern in DTI and MRS of childhood mitochondrial leukoencephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Brockmann, K. [Department of Paediatrics and Neuropaediatrics, Georg August University, Goettingen (Germany); Department of Paediatrics and Neuropaediatrics, Children' s Hospital, Robert-Koch-Strasse 40, 37075, Goettingen (Germany); Finsterbusch, J.; Frahm, J. [Biomedizinische NMR Forschungs GmbH am Max-Planck-Institut fuer biophysikalische Chemie, Goettingen (Germany); Schara, U. [Department of Paediatrics and Neuropaediatrics, Ruhr University, Bochum (Germany); Wilichowski, E.; Hanefeld, F. [Department of Paediatrics and Neuropaediatrics, Georg August University, Goettingen (Germany)

    2004-04-01

    In a 13-month-old boy with recurrent motor deterioration provoked by fever MRI and proton MRS detected a leukoencephalopathy with reduced cerebral metabolites and elevated lactate. At follow-up 6 and 16 months later these abnormalities improved gradually. Serial diffusion tensor imaging revealed a stroke-like pattern with an initial strong reduction of the apparent diffusion coefficient followed by elevated values 6 months later. The relative diffusion anisotropy remained reduced. Muscle biopsy confirmed a mitochondrial encephalomyopathy. (orig.)

  19. Screening for GFAP rearrangements in a cohort of Alexander disease and undetermined leukoencephalopathy patients.

    Science.gov (United States)

    Ferreira, Marie-Céleste; Dorboz, Imen; Rodriguez, Diana; Boespflug Tanguy, Odile

    2015-09-01

    Alexander disease (AxD), a fatal degenerative leukoencephalopathy, is caused by de novo heterozygous missense mutations in the Glial Fibrillary Acidic Protein (GFAP) gene. The pathological hallmark of the disease is the presence of Rosenthal fibers, cytoplasmic inclusions in astrocytes, composed mainly of GFAP, αB-crystallin and HSP27. To date, several patients with a typical presentation of the disease or displaying characteristic Rosenthal fibers in brain material have been reported with no GFAP mutation. Recently, several studies have demonstrated a correlation between Rosenthal fiber formation and wild-type GFAP overexpression, despite the absence of mutations. We tested the hypothesis that a GFAP gene rearrangement could modulate AxD severity or promote GFAP overexpression and aggregation, resulting in leukoencephalopathy. A QMPSF assay was validated for 11 exonic fragments: 3 in control genes (CFTR, DSCR1, F9) and 8 corresponding to GFAP exons. A total of 97 patients suspected of AxD were analyzed: 28 with a GFAP mutation; 69 with clinical and magnetic resonance imaging criteria compatible with the disease. Neither duplications nor deletions of GFAP were found, suggesting that GFAP coding-region rearrangements may not be involved in AxD or Alexander-related leukoencephalopathies. In addition, 80 patients with undetermined leukodystrophies, and negative for PLP1, GJA12, Sox10 and MCT8 mutations and PLP1 and Lamin B1 rearrangements, were tested. These patients were also negative for GFAP rearrangements. Other hypotheses should be investigated for a molecular diagnosis in patients with undetermined leukoencephalopathy: mutations in GFAP isoforms, splicing sites or regulatory regions, or defaults in genes encoding molecular partners of GFAP. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  20. A Founder Mutation in VPS11 Causes an Autosomal Recessive Leukoencephalopathy Linked to Autophagic Defects.

    Science.gov (United States)

    Zhang, Jinglan; Lachance, Véronik; Schaffner, Adam; Li, Xianting; Fedick, Anastasia; Kaye, Lauren E; Liao, Jun; Rosenfeld, Jill; Yachelevich, Naomi; Chu, Mary-Lynn; Mitchell, Wendy G; Boles, Richard G; Moran, Ellen; Tokita, Mari; Gorman, Elizabeth; Bagley, Kaytee; Zhang, Wei; Xia, Fan; Leduc, Magalie; Yang, Yaping; Eng, Christine; Wong, Lee-Jun; Schiffmann, Raphael; Diaz, George A; Kornreich, Ruth; Thummel, Ryan; Wasserstein, Melissa; Yue, Zhenyu; Edelmann, Lisa

    2016-04-01

    Genetic leukoencephalopathies (gLEs) are a group of heterogeneous disorders with white matter abnormalities affecting the central nervous system (CNS). The causative mutation in ~50% of gLEs is unknown. Using whole exome sequencing (WES), we identified homozygosity for a missense variant, VPS11: c.2536T>G (p.C846G), as the genetic cause of a leukoencephalopathy syndrome in five individuals from three unrelated Ashkenazi Jewish (AJ) families. All five patients exhibited highly concordant disease progression characterized by infantile onset leukoencephalopathy with brain white matter abnormalities, severe motor impairment, cortical blindness, intellectual disability, and seizures. The carrier frequency of the VPS11: c.2536T>G variant is 1:250 in the AJ population (n = 2,026). VPS11 protein is a core component of HOPS (homotypic fusion and protein sorting) and CORVET (class C core vacuole/endosome tethering) protein complexes involved in membrane trafficking and fusion of the lysosomes and endosomes. The cysteine 846 resides in an evolutionarily conserved cysteine-rich RING-H2 domain in carboxyl terminal regions of VPS11 proteins. Our data shows that the C846G mutation causes aberrant ubiquitination and accelerated turnover of VPS11 protein as well as compromised VPS11-VPS18 complex assembly, suggesting a loss of function in the mutant protein. Reduced VPS11 expression leads to an impaired autophagic activity in human cells. Importantly, zebrafish harboring a vps11 mutation with truncated RING-H2 domain demonstrated a significant reduction in CNS myelination following extensive neuronal death in the hindbrain and midbrain. Thus, our study reveals a defect in VPS11 as the underlying etiology for an autosomal recessive leukoencephalopathy disorder associated with a dysfunctional autophagy-lysosome trafficking pathway.

  1. A Founder Mutation in VPS11 Causes an Autosomal Recessive Leukoencephalopathy Linked to Autophagic Defects.

    Directory of Open Access Journals (Sweden)

    Jinglan Zhang

    2016-04-01

    Full Text Available Genetic leukoencephalopathies (gLEs are a group of heterogeneous disorders with white matter abnormalities affecting the central nervous system (CNS. The causative mutation in ~50% of gLEs is unknown. Using whole exome sequencing (WES, we identified homozygosity for a missense variant, VPS11: c.2536T>G (p.C846G, as the genetic cause of a leukoencephalopathy syndrome in five individuals from three unrelated Ashkenazi Jewish (AJ families. All five patients exhibited highly concordant disease progression characterized by infantile onset leukoencephalopathy with brain white matter abnormalities, severe motor impairment, cortical blindness, intellectual disability, and seizures. The carrier frequency of the VPS11: c.2536T>G variant is 1:250 in the AJ population (n = 2,026. VPS11 protein is a core component of HOPS (homotypic fusion and protein sorting and CORVET (class C core vacuole/endosome tethering protein complexes involved in membrane trafficking and fusion of the lysosomes and endosomes. The cysteine 846 resides in an evolutionarily conserved cysteine-rich RING-H2 domain in carboxyl terminal regions of VPS11 proteins. Our data shows that the C846G mutation causes aberrant ubiquitination and accelerated turnover of VPS11 protein as well as compromised VPS11-VPS18 complex assembly, suggesting a loss of function in the mutant protein. Reduced VPS11 expression leads to an impaired autophagic activity in human cells. Importantly, zebrafish harboring a vps11 mutation with truncated RING-H2 domain demonstrated a significant reduction in CNS myelination following extensive neuronal death in the hindbrain and midbrain. Thus, our study reveals a defect in VPS11 as the underlying etiology for an autosomal recessive leukoencephalopathy disorder associated with a dysfunctional autophagy-lysosome trafficking pathway.

  2. Chasing the dragon - characterizing cases of leukoencephalopathy associated with heroin inhalation in British Columbia.

    Science.gov (United States)

    Buxton, Jane A; Sebastian, Renee; Clearsky, Lorne; Angus, Natalie; Shah, Lena; Lem, Marcus; Spacey, Sian D

    2011-01-21

    An association between leukoencephalopathy, a disease of the white matter of the brain, and smoking heroin is well recognized. This paper describes 27 cases of leukoencephalopathy identified in two cities in British Columbia, Canada 2001-2006; the largest number of geographically and temporally defined reported cases in North America.Twenty cases of leukoencephalopathy were identified in and around Vancouver with onset dates December 2001 to July 2003; seven further cases were identified in Victoria September 2005-August 2006. Twenty (74%) of all cases were male, two couples were reported and eleven cases (55%) had Asian ethnicity. One case reported smoking heroin on a single occasion and developed mild symptoms; all other cases were hospitalized. Thirteen (48%) cases died; all had smoked heroin for a minimum of 3 years. Testing of one available heroin sample identified no substance other than common cutting agents.Although a specific etiology was not identified our study supports the theory of an intermittent exposure to a toxic agent added to the heroin or a combustion by-product. It also suggests a dose response effect rather than genetic predisposition. Collaboration with public health, health professionals, law enforcement and persons who use illegal drugs, will facilitate the early identification of cases to enable timely and complete follow-up including obtaining samples. Testing of implicated heroin samples may allow identification of the contaminant and therefore prevent further cases. It is therefore important to ensure key stakeholders are aware of our findings.

  3. Chasing the dragon - characterizing cases of leukoencephalopathy associated with heroin inhalation in British Columbia

    Directory of Open Access Journals (Sweden)

    Angus Natalie

    2011-01-01

    Full Text Available Abstract An association between leukoencephalopathy, a disease of the white matter of the brain, and smoking heroin is well recognized. This paper describes 27 cases of leukoencephalopathy identified in two cities in British Columbia, Canada 2001-2006; the largest number of geographically and temporally defined reported cases in North America. Twenty cases of leukoencephalopathy were identified in and around Vancouver with onset dates December 2001 to July 2003; seven further cases were identified in Victoria September 2005-August 2006. Twenty (74% of all cases were male, two couples were reported and eleven cases (55% had Asian ethnicity. One case reported smoking heroin on a single occasion and developed mild symptoms; all other cases were hospitalized. Thirteen (48% cases died; all had smoked heroin for a minimum of 3 years. Testing of one available heroin sample identified no substance other than common cutting agents. Although a specific etiology was not identified our study supports the theory of an intermittent exposure to a toxic agent added to the heroin or a combustion by-product. It also suggests a dose response effect rather than genetic predisposition. Collaboration with public health, health professionals, law enforcement and persons who use illegal drugs, will facilitate the early identification of cases to enable timely and complete follow-up including obtaining samples. Testing of implicated heroin samples may allow identification of the contaminant and therefore prevent further cases. It is therefore important to ensure key stakeholders are aware of our findings.

  4. Reversible Posterior Leukoencephalopathy Syndrome Developing After Restart of Sunitinib Therapy for Metastatic Renal Cell Carcinoma

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    Shinji Fukui

    2016-01-01

    Full Text Available A 64-year-old Japanese man had started molecular-targeted therapy with sunitinib for lymph node metastasis 5 years after nephrectomy for left renal cell carcinoma (clear cell carcinoma, G2, pT2N0M0. He was transported to our emergency department because of generalized tonic-clonic seizure, vision loss, and impaired consciousness with acute hypertension after 8 cycles of treatment (2 years after the initiation of sunitinib therapy, including a drug withdrawal period for one year. MRI of the brain (FLAIR images showed multiple high-intensity lesions in the white matter of the occipital and cerebellar lobes, dorsal brain stem, and left thalamus. Reversible posterior leukoencephalopathy syndrome caused by sunitinib was suspected. In addition to the immediate discontinuation of sunitinib therapy, the administration of antihypertensive agents and anticonvulsants improved the clinical symptoms without neurological damage. Physicians should be aware that sunitinib causes reversible posterior leukoencephalopathy syndrome. The early recognition of reversible posterior leukoencephalopathy syndrome is critical to avoid irreversible neurological damage.

  5. Heroin-induced leukoencephalopathy: characterization using MRI, diffusion-weighted imaging, and MR spectroscopy

    Energy Technology Data Exchange (ETDEWEB)

    Offiah, C. [Department of Neuroradiology, St Bartholomew' s and the London Hospitals NHS Trust, London (United Kingdom); Hall, E. [Department of Neuroradiology, St Bartholomew' s and the London Hospitals NHS Trust, London (United Kingdom)], E-mail: curtis.offiah@bartsandthelondon.nhs.uk

    2008-02-15

    Aim: To describe the magnetic resonance imaging (MRI) characteristics of heroin-induced leukoencephalopathy or 'chasing the dragon syndrome' and, in particular, the diffusion-weighted imaging (DWI) and MR spectroscopy (MRS) features. Material and methods: Six patients with a clinical or histopathological diagnosis of heroin-induced leukoencephalopathy were identified and MRI examinations, including DWI and single-voxel MRS, reviewed. Results: Cerebellar white matter was involved in all six cases demonstrating similar symmetrical distribution with sparing of the dentate nuclei. Brain stem signal change was evident in five of the six patients imaged. Supratentorial brain parenchymal involvement, as well as brain stem involvement, correlated anatomically with corticospinal tract distribution. None of the areas of signal abnormality were restricted on DWI. Of those patients subjected to MRS, the areas of parenchymal damage demonstrated reduced N-acetylaspartate, reduced choline, and elevated lactate. Conclusion: Heroin-induced leukoencephalopathy results in characteristic and highly specific signal abnormalities on MRI, which can greatly aid diagnosis. DWI and MRS findings can be explained by known reported neuropathological descriptions in this condition and can be used to support a proposed mechanism for the benefit of current recommended drug treatment regimes.

  6. Incidence, clinical presentation, and outcome of progressive multifocal leukoencephalopathy in HIV-infected patients during the highly active antiretroviral therapy era: a nationwide cohort study

    DEFF Research Database (Denmark)

    Engsig, Frederik Neess; Hansen, Ann-Brit Eg; Omland, Lars Haukali;

    2009-01-01

    at presentation and follow-up. RESULTS: Among 4,649 patients, we identified 47 patients with PML. The incidence rates were 3.3, 1.8, and 1.3 cases per 1000 person-years at risk in 1995-1996, 1997-1999, and 2000-2006, respectively. The risk of PML was significantly associated with low CD4(+) cell count, and 47......-1996) and during the early HAART (1997-1999) and late HAART (2000-2006) periods. METHODS: Patients from a nationwide population-based cohort of adult HIV-1-infected individuals were included. We calculated incidence rates of PML and median survival times after diagnosis. We also described neurological symptoms...... interval [CI], 0.0-0.7) in 1995-1996 and 1.8 years (95% CI, 0.6-3.0) in both 1997-1999 and 2000-2006. CD4(+) cell count >50 cells/microL at diagnosis of PML was significantly associated with reduced mortality. Conclusions: The incidence of PML in HIV-infected patients decreased after the introduction...

  7. Objective perimetry in glaucoma: recent advances with multifocal stimuli.

    Science.gov (United States)

    Graham, S L; Klistorner, A; Grigg, J R; Billson, F A

    1999-06-01

    The introduction of multifocal stimulus recording has enhanced our ability to examine the human visual field with electrophysiologic techniques. We have adapted the multifocal pattern visual evoked potential (PVEP) to detect visual field loss. In glaucoma patients we sought to determine the extent to which the PVEP amplitudes correlate with perimetric thresholds. Multifocal pseudorandomly alternated pattern stimuli, which were cortically scaled in size, were presented with use of the VERIS-Scientific system. Bipolar occipital straddle electrode positions were used. The visual field up to 25 degrees of eccentricity was investigated. Forty-three glaucoma patients with reproducible visual field defects were tested. The bipolar PVEP corresponded well with Humphrey visual field defects, showing loss of signal in the scotoma area. For Humphrey quadrant threshold totals and PVEP quadrant amplitudes, the correlation coefficient was strong (r = 0.49, P < 0.0001). The multifocal PVEP demonstrates good correspondence with the topography of the visual field. This technique represents the first practical application of the multifocal PVEP to objective detection of visual field defects in glaucoma.

  8. Multifocal VEP assessment of optic neuritis evolution.

    Science.gov (United States)

    Alshowaeir, Daniah; Yannikas, Con; Garrick, Raymond; Van Der Walt, Anneke; Graham, Stuart L; Fraser, Clare; Klistorner, Alexander

    2015-08-01

    To evaluate multifocal visual evoked potentials (mfVEP) changes in optic neuritis (ON) and fellow eyes during first year after the attack. Eighty-seven patients and twenty-five controls were examined. Patients were classified as multiple sclerosis (MS) group, high risk (HR) or low risk (LR) groups for conversion to MS. mfVEP recordings and retinal nerve fiber layer (RNFL) thickness were analyzed. Recovery of amplitude and shortening of latency was fastest within the first 3months. The largest amplitude reduction and longest latency delay of the ON eye were recorded in the MS group. This was accompanied by deterioration of both parameters in fellow eyes (p<0.03). mfVEP remained stable in fellow eyes of the LR group. Inter-eye asymmetry showed similar amount of amplitude reduction and latency delay in all three groups. RNFL thickness strongly correlated with mfVEP amplitude as early as 3 months after ON (R(2)=0.6, p=0.001). mfVEP amplitude is an early predictor of post-ON axonal loss. The apparent more severe involvement of ON eyes in the MS subgroup may be due to subclinical inflammation along the visual pathway. Severity of amplitude reduction and latency delay after episode of ON is not MS-related. Retro-chiasmal demyelination is a possible factor contributing to amplitude and latency differences between MS and non-MS patients. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  9. Multifocal Group B Streptococcal Infection in a Newborn: Case Report

    Directory of Open Access Journals (Sweden)

    Osman Öztekin

    2011-12-01

    Full Text Available Introduction: Group B streptococci cause sepsis and other infections in newborns very early in life (early-onset sepsis and also later (late-onset sepsis in the newborn period.Case Report: A case of multifocal group B streptococcal infection with sepsis, meningitis, ventriculitis, osteomyelitis and abscesses in a newborn infant was reported. Ventriculitis is often thought of as a secondary complication of acute group B streptococcal meningitis.Conclusion: Multifocal group B streptococcal infection is rare and group B streptococcal ventriculitis should be suspected in cases that are refractory to treatment or in patients who later develop hydrocephalus. (Journal of Current Pediatrics 2011; 9: 141-4

  10. Research advances on multifocal electroretinogram in primary open angle glaucoma

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    Feng-Fei Mo

    2013-10-01

    Full Text Available Primary open angle glaucoma is a chronic and progressive optic neuropathy. It can lead to serious damage of visual impairment, and it is an important eye disease of blindness. Multifocal electroretinogram is a new way to measure visual electrophysiology. It can measure electroretinogram of the whole visual field of many small parts in a relatively short period of time, and it can reflect the function of regional retina. It has an extremely important value for early diagnosis of primary open angle glaucoma. The research advances on multifocal electroretinogram in diagnosing primary open angle glaucoma were summarized in this paper.

  11. Exploration of Multifocal Rod Electroretinograms Recording in Human

    Institute of Scientific and Technical Information of China (English)

    Changzheng Chen; Lezheng Wu; De-Zheng Wu; Shixian Long; Jiongji Liang; Futian Jiang; Libing Jiang

    2002-01-01

    Purpose:To test the feasibility of recording rod multifocal electroretinograms (ERG) in humans and observe appropriate recording conditions.Methods: Multifocal rod ERG were recorded using a stimulus array of 61 equalsized hexagons in two normal subjects after the dark adaptation. Flashes were blue (W47B). Blank frames between two successive flashes of hexagons varied from 0 to 14. Length of the m-sequence, bandwidth, flash frequency, flash intensities and background intensities were changed to obtain appropriate recording conditions for the clinical use.Results:Multifocal rod ERG were clearly recordable and well formed. They had an early implicit time, very small negative wave and a late implicit time, large positive wave. The positive wave was bimodal, whose timing and waveform were similar to the full-field rod ERG. The local response amplitudes can be suppressed with increase in flash frequency or background intensity, decrease in flash intensity or the size of stimulus elements.Conclusions: Multifocal rod ERG can be recorded in human and can provide topographical maps of retinal function that have clinical usage. 212-1 m-sequence length, 3F blank frames and 3~ 300 Hz bandwidth were suggested to appropriate recording conditions.

  12. The multifocal electroretinogram (mfERG) in the pig

    DEFF Research Database (Denmark)

    Voss Kyhn, Maria; Kiilgaard, Jens Folke; Lopez, Ana Garcia;

    2007-01-01

    To establish a method allowing multifocal electroretinography (mfERG) recording with simultaneous fundus monitoring on anaesthetized pigs. In addition we characterize the peaks of the porcine mfERG trace, and compare the visual streak area with the optic nerve head, a known non-response area...

  13. The multifocal electroretinogram (mfERG) in the pig

    DEFF Research Database (Denmark)

    Voss Kyhn, Maria; Kiilgaard, Jens Folke; Lopez, Ana Garcia

    2007-01-01

    To establish a method allowing multifocal electroretinography (mfERG) recording with simultaneous fundus monitoring on anaesthetized pigs. In addition we characterize the peaks of the porcine mfERG trace, and compare the visual streak area with the optic nerve head, a known non-response area. Fin...

  14. Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy

    DEFF Research Database (Denmark)

    Harbo, T; Andersen, Henning; Hess, A

    2009-01-01

    Background and purpose: For treatment of multifocal motor neuropathy (MMN), we hypothesized that (i) infusion of equivalent dosages of subcutaneous immunoglobulin (SCIG) is as effective as intravenous immunoglobulin (IVIG) and that (ii) subcutaneous infusion at home is associated with a better...

  15. Multifocal motor neuropathy and progressive atrophy : Pathophysiological similarities and differences

    NARCIS (Netherlands)

    Vlam, L.

    2015-01-01

    Progressive muscular atrophy (PMA) and multifocal motor neuropathy (MMN) share many clinical similarities. They are both characterized by progressive asymmetric muscle weakness with atrophy and fasciculations. Tendon reflexes are normally low or absent, although in some patients with MMN normal or e

  16. Diffractive multifocal intraocular lens interferes with intraoperative view

    Directory of Open Access Journals (Sweden)

    Mami Yoshino

    2010-05-01

    Full Text Available Mami Yoshino1, Makoto Inoue1,2, Nae Kitamura1, Hiroko Bissen-Miyajima11Department of Ophthalmology, Tokyo Dental College Suidobashi Hospital, Tokyo, Japan; 2Kyorin Eye Center, Kyorin University School of Medicine, Tokyo, JapanAbstract: We report an unusual finding during vitreous surgery in an eye implanted with a diffractive multifocal intraocular lens (IOL. A 70-year-old woman reported gradual visual deterioration to 20/40 in the left eye two and a half years after uneventful cataract surgery with implantation of a diffractive multifocal IOL. Funduscopic examination showed an epiretinal membrane (ERM in the left eye. Increased macular traction was believed to cause the visual deterioration. Vitreous surgery with removal of the ERM was performed and triamcinolone acetonide (TA was injected intravitreally to visualize the residual vitreous cortex. Although the ERM was peeled successfully, the ability to focus on the vitreoretinal interface through the IOL required great effort with decreased contrast sensitivity and ghost images of the intravitreal TA crystals. The vision improved to 20/25 4 months postoperatively. Macular surgery can be performed in an eye with a diffractive multifocal IOL; however, decreased contrast sensitivity and ghost images may interfere with the intraoperative view through the diffractive IOL in complicated cases.Keywords: diffractive multifocal intraocular lens, intraoperative view

  17. Megalencephalic leukoencephalopathy with subcortical cysts in all three siblings of a non-Aggarwal Indian family

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    Mani Kant Kumar

    2012-01-01

    Full Text Available Megalencephalic leukoencephalopathy with subcortical cysts (MLC is a rare neurodegenerative disease seen mainly in the Aggarwal community in India. It is characterized by early-onset macrocephaly with mild motor developmental delay, gradual onset ataxia, spasticity, seizures and usually late onset mild cognitive deterioration. Very few familial cases of MLC have been reported in the world literature, and to the best of our knowledge, there is no published study of all three siblings affected with MLC in a same family. Here, we are reporting three siblings belonging to a non-Aggarwal Hindu family, affected with MLC, who presented with early-onset macrocephaly and gradual onset ataxia.

  18. Right Hemispheric Leukoencephalopathy as an Incidental Finding Following a Lightning Strike.

    Science.gov (United States)

    Kruja, Jera; Kuqo, Altin; Grabova, Serla; Rroji, Arben; Vyshka, Gentian

    2016-12-15

    Lightning injuries may produce a variety of medical conditions, and specific neurological complications have been identified, with the character of immediate aftershock effects or even long-term consequences. The authors describe the incidental finding following a routine unenhanced brain MRI performed to a young female patient, suffering from a headache. Diffuse white matter changes with the character of a leukoencephalopathy were seen, which strictly interested only the right cerebral hemisphere. The parents referred that she suffered from an indoor lightning strike at age of seven months, although she survived with almost no external burns or signs, and recovered uneventfully at that time. A discussion over the effects of electrocution and lightning strike on the human body in general, and over the nervous system, is made. Particular attention must be shown when making the differential diagnosis of leukoencephalopathies with a strictly one-hemisphere extension since several other conditions might resemble each other under the radiological aspect, here including brain viral infections, genetic disorders, and so on. The particularity of the long-term aftershock effects of the lightning strike on the central nervous system raise again the necessity of collecting data and duly reporting every electrical accident, lightning events included.

  19. [A case of malignant syndrome with leukoencephalopathy due to cocaine abuse].

    Science.gov (United States)

    Yamazaki, K; Katayama, S; Iwai, T; Hirata, K

    1994-06-01

    We report a rare case of malignant syndrome with leukoencephalopathy due to cocaine abuse. The patient was a 22-year-old man. After nasally ingesting 4.2 g of cocaine, he developed coma, high grade fever of 39 degrees C, excessive sweating, muscular rigidity, and elevation of CK to 61,240 mU/ml. Although these symptoms improved in about 2 weeks, diffuse low density area of the cerebral white matter was observed in CT scan, and T2-weighted MRI revealed a high signal intensity area. Since disorientation and abnormal behavior appeared on the 24th hospital day after a lucid interval, sulpiride and haloperidol were administered. The patient again had fever, akinetic mutisn, severe muscular rigidity and excessive sweating. This patient has malignant syndrome associated with a wide-ranging lesion of the cerebral white matter as a result of cocaine abuse. And then antipsychotic drugs were administered for mental symptoms that recurred after a lucid interval, and second bouts of neuroleptic malignant syndrome occurred. There have been only a few reports on malignant syndrome due to cocaine, and there have been no reports on leukoencephalopathy due to cocaine or malignant syndrome.

  20. Posterior leukoencephalopathy following repair of an ileocecal anastomosis breakdown: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Kasper Ekkehard M

    2011-01-01

    Full Text Available Abstract Introduction Posterior reversible leukoencephalopathy syndrome refers to a constellation of neurologic symptoms related to temporary white matter changes. The disease typically presents in the context of an abrupt and drastic elevation in blood pressure (>180/110 mmHg. We report an unusual case of posterior reversible leukoencephalopathy syndrome in a post-operative setting, with a blood pressure parameter generally tolerated by most patients. Case presentation We report the case of a 22-year-old Caucasian man who suffered acute onset visual acuity loss four days after an ileocecal anastomosis. A head magnetic resonance imaging scan revealed findings typically associated with posterior reversible leukoencephalopathy syndrome. His symptoms developed in the context of blood pressure parameters that are typically well tolerated in a post-operative setting (150-160/80-90 mmHg. He did not have a history of renal failure or immunosuppression. His symptoms resolved with aggressive blood pressure management. Conclusions Posterior reversible leukoencephalopathy syndrome can occur in a post-operative setting with blood pressure parameters typically well-tolerated in most post-surgical patients. Timely diagnosis and treatment will minimize the risk of permanent neurologic injury.

  1. Diagnostic Criteria for Adult-onset Leukoencephalopathy With Axonal Spheroids and Pigmented Glia Due to CSF1R mutation.

    Science.gov (United States)

    Konno, Takuya; Yoshida, Kunihiro; Mizuta, Ikuko; Mizuno, Toshiki; Kawarai, Toshitaka; Tada, Masayoshi; Nozaki, Hiroaki; Ikeda, Shu-Ichi; Onodera, Osamu; Wszolek, Zbigniew K; Ikeuchi, Takeshi

    2017-09-18

    To establish and validate diagnostic criteria for adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) due to colony stimulating factor 1 receptor (CSF1R) mutation. We developed diagnostic criteria for ALSP based on a recent analysis of the clinical characteristics of ALSP. These criteria provide "probable" and "possible" designations for patients who do not have a genetic diagnosis. To verify its sensitivity and specificity, we retrospectively applied our criteria to 83 ALSP cases who had CSF1R mutations (24 of these were analyzed at our institutions, and the others were identified from the literature), 53 cases who had CSF1R mutation-negative leukoencephalopathies, and 32 cases who had cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with NOTCH3 mutations. Among the CSF1R mutation-positive cases, 50 cases (60%) were diagnosed as "probable" and 32 (39%) were diagnosed as "possible," leading to a sensitivity of 99% if calculated as a ratio of the combined number of cases who fulfilled "probable" or "possible" to the total number of cases. With regard to specificity, 22 cases (42%) with mutation-negative leukoencephalopathies and 28 (88%) with CADASIL were correctly excluded using these criteria. These diagnostic criteria are very sensitive for diagnosing ALSP with sufficient specificity for differentiating from CADASIL and moderate specificity for other leukoencephalopathies. Our results suggest that these criteria are useful for the clinical diagnosis of ALSP. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  2. Triple-stimulation technique in multifocal neuropathy with conduction block.

    Science.gov (United States)

    Deroide, Nicolas; Uzenot, David; Verschueren, Annie; Azulay, Jean-Philippe; Pouget, Jean; Attarian, Shahram

    2007-05-01

    In patients with multifocal neuropathy with conduction block (CB), CBs located between the root and Erb's point are not detected in nerve conduction studies. We therefore examined whether the triple-stimulation technique (TST) might provide a useful means of detecting CB proximal to Erb's point. Clinical assessments, extensive nerve conduction studies (NCS), conventional transcranial magnetic stimulation, and TST were performed on 10 patients with multifocal motor neuropathy with CB (MMNCB) and 6 patients with Lewis-Sumner syndrome. Conduction blocks located proximal to Erb's point were detected in 9 patients. Of the CBs, 58% were associated with muscle weakness. The use of TST to detect proximal CB improved the sensitivity of the American Association of Neuromuscular and Electrodiagnostic Medicine criteria for definite or probable MMNCB from 60% to 90%. Thus, the TST is a useful means for detection of proximal CB and gives NCS considerable additional diagnostic power.

  3. Multifocal epithelioid hemangioendothelioma of the phalanges of the hand

    Energy Technology Data Exchange (ETDEWEB)

    Bruegel, Melanie; Waldt, Simone; Woertler, Klaus; Rummeny, Ernst J. [Klinikum Rechts der Isar der Technischen Universitaet Muenchen, Department of Radiology, Munich (Germany); Weirich, Gregor [Klinikum Rechts der Isar der Technischen Universitaet Muenchen, Institute of Pathology, Munich (Germany)

    2006-10-15

    Epithelioid hemangioendothelioma (EH) of bone is a rare vascular neoplasm characterized by epithelioid endothelial cells and a variable biologic behaviour. The principal sites of occurrence of this osteolytic tumor are the lower extremity and the axial skeleton. Approximately half of the cases present with multifocal disease. The latter feature can be helpful in suggesting the diagnosis of a vascular tumor; on the other hand, it strengthens the need for a skeletal survey or whole-body MRI/CT. We report on the clinical, histologic and radiologic features - including CT and MRI findings - of EH in a case of multifocal disease of the phalanges of the hand, a very uncommon anatomic site of affliction. (orig.)

  4. Multifocal visual evoked responses to dichoptic stimulation using virtual reality goggles: Multifocal VER to dichoptic stimulation.

    Science.gov (United States)

    Arvind, Hemamalini; Klistorner, Alexander; Graham, Stuart L; Grigg, John R

    2006-05-01

    Multifocal visual evoked potentials (mfVEPs) have demonstrated good diagnostic capabilities in glaucoma and optic neuritis. This study aimed at evaluating the possibility of simultaneously recording mfVEP for both eyes with dichoptic stimulation using virtual reality goggles and also to determine the stimulus characteristics that yield maximum amplitude. ten healthy volunteers were recruited and temporally sparse pattern pulse stimuli were presented dichoptically using virtual reality goggles. Experiment 1 involved recording responses to dichoptically presented checkerboard stimuli and also confirming true topographic representation by switching off specific segments. Experiment 2 involved monocular stimulation and comparison of amplitude with Experiment 1. In Experiment 3, orthogonally oriented gratings were dichoptically presented. Experiment 4 involved dichoptic presentation of checkerboard stimuli at different levels of sparseness (5.0 times/s, 2.5 times/s, 1.66 times/s and 1.25 times/s), where stimulation of corresponding segments of two eyes were separated by 16.7, 66.7,116.7 & 166.7 ms respectively. Experiment 1 demonstrated good traces in all regions and confirmed topographic representation. However, there was suppression of amplitude of responses to dichoptic stimulation by 17.9+/-5.4% compared to monocular stimulation. Experiment 3 demonstrated similar suppression between orthogonal and checkerboard stimuli (p = 0.08). Experiment 4 demonstrated maximum amplitude and least suppression (4.8%) with stimulation at 1.25 times/s with 166.7 ms separation between eyes. It is possible to record mfVEP for both eyes during dichoptic stimulation using virtual reality goggles, which present binocular simultaneous patterns driven by independent sequences. Interocular suppression can be almost eliminated by using a temporally sparse stimulus of 1.25 times/s with a separation of 166.7 ms between stimulation of corresponding segments of the two eyes.

  5. The effect of multifocal soft contact lenses on peripheral refraction.

    Science.gov (United States)

    Kang, Pauline; Fan, Yvonne; Oh, Kelly; Trac, Kevin; Zhang, Frank; Swarbrick, Helen A

    2013-07-01

    To compare changes in peripheral refraction with single-vision (SV) and multifocal (MF) correction of distance central refraction with commercially available SV and MF soft contact lenses (SCLs) in young myopic adults. Thirty-four myopic adult subjects were fitted with Proclear Sphere and Proclear Multifocal SCLs to correct their manifest central refractive error. Central and peripheral refraction were measured with no lens wear and subsequently with the two different types of SCL correction. At baseline, refraction was myopic at all locations along the horizontal meridian. Peripheral refraction was relatively hyperopic compared with center at 30 and 35 degrees in the temporal visual field (VF) in low myopes, and at 30 and 35 degrees in the temporal VF, and 10, 30, and 35 degrees in the nasal VF in moderate myopes. Single-vision and MF distance correction with Proclear Sphere and Proclear Multifocal SCLs, respectively, caused a hyperopic shift in refraction at all locations in the horizontal VF. Compared with SV correction, MF SCL correction caused a significant relative myopic shift at all locations in the nasal VF in both low and moderate myopes and also at 35 degrees in the temporal VF in moderate myopes. Correction of central refractive error with SV and MF SCLs caused a hyperopic shift in both central and peripheral refraction at all positions in the horizontal meridian. Single-vision SCL correction caused the peripheral retina, which initially experienced absolute myopic defocus at baseline with no correction to experience an absolute hyperopic defocus. Multifocal SCL correction resulted in a relative myopic shift in peripheral refraction compared with SV SCL correction. This myopic shift may explain recent reports of reduced myopia progression rates with MF SCL correction.

  6. Multifocal cysticercosis with optical coherence tomography findings in a child

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    Manisha Agarwal

    2012-01-01

    Full Text Available We herein report a case with multifocal cysticercosis - sub-conjunctival cysticercus cyst, sub-retinal cysticercosis, and neurocysticercosis in a child. The optical coherence tomography (OCT findings of the sub-retinal cysticercus cyst are reported. He was treated with anti-helminthic drugs and oral prednisolone followed by surgical removal of the sub-retinal cyst. He subsequently underwent silicone oil removal with lens aspiration and intraocular lens implantation maintaining stable vision

  7. Retreatments after multifocal intraocular lens implantation: an analysis

    Science.gov (United States)

    Gundersen, Kjell Gunnar; Makari, Sarah; Ostenstad, Steffen; Potvin, Rick

    2016-01-01

    Purpose To determine the incidence and etiology of required retreatment after multifocal intraocular lens (IOL) implantation and to evaluate the methods and clinical outcomes of retreatment. Patients and methods A retrospective chart review of 416 eyes of 209 patients from one site that underwent uncomplicated cataract surgery with multifocal IOL implantation. Biometry, the IOL, and refractive data were recorded after the original implantation, with the same data recorded after retreatment. Comments related to vision were obtained both before and after retreatment for retreated patients. Results The multifocal retreatment rate was 10.8% (45/416 eyes). The eyes that required retreatment had significantly higher residual refractive astigmatism compared with those who did not require retreatment (1.21±0.51 D vs 0.51±0.39 D, P<0.01). The retreatment rate for the two most commonly implanted primary IOLs, blended bifocal (10.5%, 16/152) and bilateral trifocal (6.9%, 14/202) IOLs, was not statistically significantly different (P=0.12). In those requiring retreatment, refractive-related complaints were most common. Retreatment with refractive corneal surgery, in 11% of the eyes, and piggyback IOLs, in 89% of the eyes, was similarly successful, improving patient complaints 78% of the time. Conclusion Complaints related to ametropia were the main reasons for retreatment. Residual astigmatism appears to be an important determinant of retreatment rate after multifocal IOL implantation. Retreatment can improve symptoms for a high percentage of patients; a piggyback IOL is a viable retreatment option. PMID:27041983

  8. Patient acceptability of the Tecnis® multifocal intraocular lens

    OpenAIRE

    Woodward, Maria; Sood,Priyanka

    2011-01-01

    Priyanka Sood1, Maria A Woodward21Emory Eye Center, Atlanta, GA, USA; 2Kellogg Eye Center, Ann Arbor, MI, USAAbstract: Cataract surgery has evolved. The goal of the surgeon includes both restoration of vision and refinement of vision. Patients' desire for spectacle independence has driven the market for presbyopia-correcting cataract surgery and development of novel intraocular lens (IOL) designs. The Tecnis® Multifocal Intraocular Lens incorporates an aspheric, modified anter...

  9. Retreatments after multifocal intraocular lens implantation: an analysis

    Directory of Open Access Journals (Sweden)

    Gundersen KG

    2016-03-01

    Full Text Available Kjell Gunnar Gundersen,1 Sarah Makari,2 Steffen Ostenstad,1 Rick Potvin2 1Ifocus Eye Clinic, Haugesund, Norway; 2Science in Vision, Akron, NY, USA Purpose: To determine the incidence and etiology of required retreatment after multifocal intraocular lens (IOL implantation and to evaluate the methods and clinical outcomes of retreatment.Patients and methods: A retrospective chart review of 416 eyes of 209 patients from one site that underwent uncomplicated cataract surgery with multifocal IOL implantation. Biometry, the IOL, and refractive data were recorded after the original implantation, with the same data recorded after retreatment. Comments related to vision were obtained both before and after retreatment for retreated patients.Results: The multifocal retreatment rate was 10.8% (45/416 eyes. The eyes that required retreatment had significantly higher residual refractive astigmatism compared with those who did not require retreatment (1.21±0.51 D vs 0.51±0.39 D, P<0.01. The retreatment rate for the two most commonly implanted primary IOLs, blended bifocal (10.5%, 16/152 and bilateral trifocal (6.9%, 14/202 IOLs, was not statistically significantly different (P=0.12. In those requiring retreatment, refractive-related complaints were most common. Retreatment with refractive corneal surgery, in 11% of the eyes, and piggyback IOLs, in 89% of the eyes, was similarly successful, improving patient complaints 78% of the time.Conclusion: Complaints related to ametropia were the main reasons for retreatment. Residual astigmatism appears to be an important determinant of retreatment rate after multifocal IOL implantation. Retreatment can improve symptoms for a high percentage of patients; a piggyback IOL is a viable retreatment option. Keywords: piggyback IOL, Sulcoflex, toric, STAAR, symptoms, astigmatism

  10. Multifocal VEP in children: its maturation and clinical application.

    Science.gov (United States)

    Balachandran, C; Klistorner, A I; Billson, F

    2004-02-01

    To study the maturation of multifocal visual evoked potentials (multifocal VEP) in normal children between the ages of 5 and 16 years and to apply the results clinically in selected cases to the diagnosis of optic pathway diseases. 70 normal children were recruited from the community and multifocal VEP (Accumap ObjectiVision, Sydney, Australia) was recorded. The waveform of the evoked responses, the latency and amplitude were analysed. Using these data, an age matched comparison was made with three children with advanced optic nerve disease; two had optic nerve glioma and one had congenital glaucoma. The full field amplitude did not correlate with age and varied greatly within each age group (coefficient of variability 28%). When scaled with respect to the background electroencephalogram the intra-age group variability decreased to 15% and a sigmoid relation was found between amplitude and age. The scaled amplitude remained largely unchanged till 11 years, between 11 and 13 years there was a rapid increase (40%), and remained stable thereafter. This relation was seen at all eccentricities tested. The latency decreased gradually with age and plateaued at 13 years. In the three children with vision abnormalities this test was able to detect scotomas consistent with their condition. Multifocal VEP perimetry shows an age related maturation in the visual pathway, characterised by distinctive timeframe of development for amplitude and latency. It can be performed by children as young as 5 years of age and holds promise as a diagnostic test capable of documenting children's visual fields objectively, even before they are able to perform subjective field tests.

  11. Multifocal pattern VEP perimetry: analysis of sectoral waveforms.

    Science.gov (United States)

    Klistorner, A I; Graham, S L

    1999-01-01

    The objective detection of local visual field defects using multi-focal pattern visual evoked potentials (VEP) has recently been described. The individual waveforms show variable polarity in different parts of the visual field due to underlying cortical convolutions. Normal trace arrays were examined to determine if certain areas of similar waveform could be grouped for analysis, while minimising cancellation of data. The VEP was assessed using multi-focal pseudo-randomly alternated pattern stimuli which were cortically scaled in size. Bipolar occipital electrodes were used for recording. Waveforms were compared for different locations within the field up to 25 degrees of eccentricity. Analysis of sectors showing similarly shaped waveforms was performed. Twelve normal subjects were studied. Grouping waveforms by sectors of similar waveform increased the total calculated upper hemifield amplitude by 60%, compared with simple summations of responses for the whole hemifield. The inferior hemifield showed more consistent waveforms throughout, with the amplitude only increasing by 11% with sectoral summation. Intra-subject variability (10.6%) is less for sectors than for individual points (17.3%). Inter-subject amplitude differences are high, calculated at 56% for individual points and 45% for sectors. Due to differences in waveform as a result of underlying cortical anatomy, individual VEP responses from multifocal recordings should be grouped as sectors along the vertical meridian and above and below the horizontal, rather than by hemifields or quadrants. This finding is significant if one is considering within-field grouping strategies similar to the glaucoma hemifield test used in conventional perimetry, or reporting derived overall VEP amplitudes and latencies from a multifocal recording. Large amplitude variations between individuals and small signals from horizontal and upper field seen in single channel recording, still limit the application of this technique as

  12. Multifocal objective perimetry in the detection of glaucomatous field loss.

    Science.gov (United States)

    Goldberg, Ivan; Graham, Stuart L; Klistorner, Alexander I

    2002-01-01

    To test the ability of a new type of multifocal objective perimetry to identify glaucomatous visual field defects. A multichannel visual evoked potential was recorded using the ObjectiVision Accumap perimeter. One hundred patients (age, 62.2 +/- 9.8 years, mean MD -6.5 +/- 4.17 dB) with open-angle glaucoma and confirmed glaucomatous visual field defects were tested and compared with the normal database of 100 normal subjects (age, 58.9 +/- 10.7 years). Both eyes were tested, but for determining sensitivity the eye with the lesser field defect was chosen if both qualified. The amplitude and intereye asymmetry coefficient for each zone of the field were calculated. A mean amplitude and multifocal objective perimetry severity index was calculated for each subject. In 95 of 100 (95%) patients with glaucoma Humphrey field defects were correlated with visual evoked potential amplitude reductions identifying a cluster of three or more abnormal zones. In two of five remaining patients with glaucoma the defect was detected on the intereye asymmetry analysis. Topographic location was well correlated with Humphrey fields. Mean amplitude was significantly reduced in 86 of the glaucoma cases (86%). The glaucoma severity index was abnormal in 93 glaucoma cases and showed a correlation with Humphrey MD (r = 0.67 right eyes, 0.69 left eyes). In 37 glaucoma cases with no scotoma by definition in the fellow eye, 22 (59.4%) had an abnormal multifocal objective perimetry, whereas only eight had some other aspect of their Humphrey visual field flagged as abnormal. Multifocal objective perimetry can assess the visual field and identify glaucomatous visual field defects. It may have the potential for identifying defects earlier than conventional perimetry.

  13. Towards multifocal ultrasonic neural stimulation: pattern generation algorithms.

    Science.gov (United States)

    Hertzberg, Yoni; Naor, Omer; Volovick, Alexander; Shoham, Shy

    2010-10-01

    Focused ultrasound (FUS) waves directed onto neural structures have been shown to dynamically modulate neural activity and excitability, opening up a range of possible systems and applications where the non-invasiveness, safety, mm-range resolution and other characteristics of FUS are advantageous. As in other neuro-stimulation and modulation modalities, the highly distributed and parallel nature of neural systems and neural information processing call for the development of appropriately patterned stimulation strategies which could simultaneously address multiple sites in flexible patterns. Here, we study the generation of sparse multi-focal ultrasonic distributions using phase-only modulation in ultrasonic phased arrays. We analyse the relative performance of an existing algorithm for generating multifocal ultrasonic distributions and new algorithms that we adapt from the field of optical digital holography, and find that generally the weighted Gerchberg-Saxton algorithm leads to overall superior efficiency and uniformity in the focal spots, without significantly increasing the computational burden. By combining phased-array FUS and magnetic-resonance thermometry we experimentally demonstrate the simultaneous generation of tightly focused multifocal distributions in a tissue phantom, a first step towards patterned FUS neuro-modulation systems and devices.

  14. A multifocal angiosarcoma involving bones of foot: A case report

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    Başak DOĞAN AVŞARGİL

    2008-05-01

    Full Text Available Angiosarcomas of bone are rare tumors constituting 1% of all malignant bone tumors. They can be either solitary or multifocal and can easily be misinterpreted as multiple myeloma or metastasis radiologically. We present a case of multifocal angiosarcoma arrising in foot bones, the diagnosis of which was difficult clinically, radiologically and pathologically. The histologic findings observed in both bioptic and amputation specimens were provided.The patient was a 56 years-old man who has undergone coronary by-pass and saphenous vein surgery 3 months ago. He has been suffering from pain and swelling on left foot since then. The lesion was suspicious for atypical fungal infection, metastasis or lymphoma radiologically. “Tru-cut” biopsy revealed extensive necrosis and the lesion resembled an inflammatory and reactive process at first glance. Morphologic mimickers of the lesion like lymphoma, metastasis and small round cell tumors were excluded immunohistochemically and the diagnosis of “malignant vasculary tumor” was made upon CD- 31 positivity of tumor cells, presence of erytrocytes, cells with intracytoplasmic lumina and increased mitotic activity. In amputation specimen a multifocal tumor having areas of necrosis and hemorrhages was observed involving predominantly calcaneus but also infiltrating talus, distal tibia and achilles tendon. The maximum diameter of tumor was 5 cm in calcaneus. Adjuvant chemotherapy could not be given because of cardiac problems.The case was presented to increase awareness on this rare, diagnostically problematic issue, the classification of which is controversial.

  15. Multifocal pigmented villonodular synovitis in a child: A case report.

    Science.gov (United States)

    Zhao, Liang; Zhou, Kaiyu; Hua, Yimin; Li, Yifei; Mu, Dezhi

    2016-08-01

    Pigmented villonodular synovitis (PVNS) is a rare, benign proliferative disorder of the synovial membrane that typically presents in adults and affects a single joint. Multifocal PVNS is very rare, particularly in childhood. We reported a rare case of multifocal PVNS affecting over 20 joints in a child. A 7-year-old female patient had a 6-month history of multifocal joints swelling with mild pain. She was diagnosed as polyarticular juvenile idiopathic arthritis at a local hospital. Naproxen, methotrexate, infliximab, and pavlin were used to treat the patient for 2 months. However, the treatment had no effect, the joints swelling remained. The patient was then transferred to our hospital. Physical examination revealed multiple joints swelling, especially in the shoulders joints. Puncture fluid from a shoulder joint was bloody. Magnetic resonance imaging (MRI) revealed synovial thickening and hemosiderin deposition. Biopsy of joint synovium found villous nodules, the invasion of foam cells, and hemosiderin deposition. By collecting all of the evidence, the diagnosis of PVNS was confirmed. PVNS was easily misdiagnosed as rheumatoid arthritis and the formal treatment was usually delayed. This case described here is the first case of PVNS involving such a large numbers of joints that has been reported in the literature.

  16. Melanoma conjuntival multifocal recidivado originado de nevus pigmentado preexistente

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    Marcos Leandro Pereira

    2014-06-01

    Full Text Available O melanoma conjuntival multifocal recidivado originado de nevus preexistente é extremamente raro, ocorrendo em uma pessoa para cinco milhões de habitantes. Seu estudo é de extrema relevância, devido sua potencial letalidade. Este estudo objetiva descrever um caso de melanoma conjuntival multifocal recidivado proveniente de nevus pigmentado preexistente ocorrido em Patos de Minas, MG. Este é um estudo de caso com revisão de literatura. O diagnóstico histopatológico e o estadiamento precoce da lesão conjuntival é de fundamental importância para designar a conduta frente ao paciente. O procedimento terapêutico mais utilizado nos dias atuais é a excisão cirúrgica com crioterapia adjuvante associada à mitomicina C. O prognóstico do melanoma conjuntival multifocal recidivado originado de nevus preexistente é o pior dentre todos os melanomas oculares, apresentando alta taxa de mortalidade, 12% a 20% em 5 anos e 30% em 10 anos de desenvolvimento patológico.

  17. The Preliminary Clinical Observation of Array Multifocal lntraocular Lens Implantation

    Institute of Scientific and Technical Information of China (English)

    Zhende Lin; Bo Feng; Yizhi Liu; Bing Cheng

    2001-01-01

    Purpose: To evaluate the clinical effects of implantation of Array multifocal intraocular lenses. Methods: Thirty-one cases (37 eyes) of cataract patients, including 15 males( 19 eyes)and 16 females( 18 eyes), were involved in this study. All patients underwent standard phacoemulsification with Array multifocal intraocular lens implantation. The complications during operation, postoperative distant visual acuity, near visual acuity,corneal curvature and visual symptoms were observed. Results: the mean value of best postoperative visual acuity was recorded as follows:uncorrected distant visual acuity was 0.8, the best-corrected distant visual acuity was 0.9, uncorrected near visual acuity was 0.5, near visual acuity with distant-corrected was 0.6, the best-corrected near visual acuity wss 0.9. The astigmatism of cornea was less than 1.5 D pre-operatively and post-operatively. One patient complained of glare. Conclusion: Array multifocal intraocular lens can provide good distant and near visual acuity. With observation of more cases and follow-up of longer time, we can draw a further conclusion. Eye Science 2001; 17: 57 ~ 60.

  18. Neurosarcoidosis

    Science.gov (United States)

    ... illness. Complications resulting from immunosuppressive treatments, such as cryptococcal and tuberculous meningitis, progressive multifocal leukoencephalopathy, and inclusion body myositis, may ...

  19. MR imaging of acute intermittent porphyria mimicking reversible posterior leukoencephalopathy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Utz, N.; Kinkel, B.; Hedde, J.P.; Bewermeyer, H. [Staedtisches Krankenhaus Koeln-Merheim, Koeln (Germany). Neurologische Klinik

    2001-12-01

    Reversible posterior leukoencephalopathy syndrome (PLS) is characterized by headache, altered mental function, visual disturbances and seizures. Neuroimaging studies suggest a white-matter oedema, predominantly in the posterior parietal-temporal-occipital regions of the brain. We present the case of a 30-year-old woman who had suffered her first attack of acute intermittent porphyria (AIP). Following 1 week of abdominal pain she developed several generalized seizures, and hallucinations, and exhibited a progressive deterioration of the consciousness. T2-weighted images, especially fluid-attenuated inversion recovery (FLAIR) sequences showed bilateral lesions in the posterior frontal, parietal and occipital cortex and subcortical white matter. Following treatment with haematin and a high carbohydrate diet the patient's condition improved. Follow-up magnetic resonance imaging (MRI) revealed complete resolution of the lesions. To our knowledge, this is the first report concerning a completely reversible PLS in AIP. (orig.)

  20. Cerebral amyloid angiopathy presenting as a posterior leukoencephalopathy: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Maramattom Boby Varkey

    2004-10-01

    Full Text Available Cerebral amyloid angiopathy (CAA is well known to present with lobar intracerebral hemorrhage, dementia or transient neurological events. White matter changes with CAA have only been recently described and can be seen with either sporadic or familial CAA. We present a 50-year-old man with rapidly progressive dementia in whom MRI brain showed symmetrical white matter changes in the parieto-occipital regions. Brain biopsy revealed changes of CAA along with features of Alzheimer′s disease. Immunohistochemistry revealed amyloid beta protein. The subcortical lesions were thought to occur from hypoperfusion of the distal white matter. The role of amyloid in the pathogenesis of CAA and the mechanism of leukoencephalopathy are discussed.

  1. Severe Metabolic Acidosis and Hepatopathy due to Leukoencephalopathy with Thalamus and Brainstem Involvement and High Lactate.

    Science.gov (United States)

    Sellars, Elizabeth A; Balmakund, Tonya; Bosanko, Katherine; Nichols, Brandi L; Kahler, Stephen G; Zarate, Yuri A

    2017-04-01

    Leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL) is a recently described autosomal recessive mitochondrial disease characterized by early onset of neurological symptoms, a biphasic clinical course, and distinctive neuroimaging. Pathogenic variants in the EARS2 gene that encode for mitochondrial glutamyl-tRNA synthetase are responsible for LTBL. Here, we describe the clinical course of an infant diagnosed with an acute crisis of LTBL and severe liver disease. This article illustrates the utility of blood lactate quantification in addition to basic metabolic testing and brain imaging in a child with low tone and poor growth. In addition, this case demonstrates the utility of current genetic diagnostic testing, in lieu of more invasive procedures, in obtaining rapid answers in this very complicated group of disorders. Georg Thieme Verlag KG Stuttgart · New York.

  2. CSF1R mutations in hereditary diffuse leukoencephalopathy with spheroids are loss of function

    Science.gov (United States)

    Pridans, Clare; Sauter, Kristin A.; Baer, Kristin; Kissel, Holger; Hume, David A.

    2013-10-01

    Hereditary diffuse leukoencephalopathy with spheroids (HDLS) in humans is a rare autosomal dominant disease characterized by giant neuroaxonal swellings (spheroids) within the CNS white matter. Symptoms are variable and can include personality and behavioural changes. Patients with this disease have mutations in the protein kinase domain of the colony-stimulating factor 1 receptor (CSF1R) which is a tyrosine kinase receptor essential for microglia development. We investigated the effects of these mutations on Csf1r signalling using a factor dependent cell line. Corresponding mutant forms of murine Csf1r were expressed on the cell surface at normal levels, and bound CSF1, but were not able to sustain cell proliferation. Since Csf1r signaling requires receptor dimerization initiated by CSF1 binding, the data suggest a mechanism for phenotypic dominance of the mutant allele in HDLS.

  3. Megalencephalic leukoencephalopathy with subcortical cysts in an adult: quantitative proton MR spectroscopy and diffusion tensor MRI

    Energy Technology Data Exchange (ETDEWEB)

    Brockmann, K.; Hanefeld, F. [Dept. of Paediatrics and Neuropaediatrics, Children' s Hospital, Georg-August-Univ., Goettingen (Germany); Finsterbusch, J.; Frahm, J. [Biomedizinische NMR Forschungs GmbH am Max-Planck-Inst. fuer biophysikalische Chemie, Goettingen (Germany); Terwey, B. [Inst. fuer Magnet-Resonanz-Diagnostik, Zentralkrankenhaus, Bremen (Germany)

    2003-03-01

    A 37-year-old macrocephalic woman was investigated for increasing gait disturbance due to longstanding spasticity and ataxia. MRI showed widespread bilateral increase in signal from cerebral white matter on T2-weighted images. Numerous subcortical cysts were visible in anterior-temporal and parietal regions. These clinical and neuroradiological features are those of megalencephalic leukoencephalopathy with subcortical cysts (MLC), a recently delineated white-matter disease with onset in childhood. Quantitative localised proton MR spectroscopy of white matter revealed marked reduction of N-acetylaspartate, creatine, and choline with normal values for myoinositol, consistent with axonal loss and astrocytic proliferation. Diffusion tensor imaging showed an increased apparent diffusion coefficient and reduced anisotropy in affected white matter pointing to reduced cell density with an increased extracellular space. These findings are in line with histological changes alterations known to occur in MLC. (orig.)

  4. Leucoencefalopatía hipóxica tardía Delayed Hypoxic Leukoencephalopathy

    Directory of Open Access Journals (Sweden)

    Pablo Sartori

    2012-06-01

    Full Text Available La Leucoencefalopatía Hipóxica Tardía (LHT es una enfermedad desmielinizante de infrecuente presentación. Se produce como consecuencia de un fenómeno de hipoxia-anoxia y aparece días o semanas más tarde.Los síntomas y cambios imagenológicos remiten casi totalmente y tienen un buen pronóstico En este reporte se ilustra el caso de una joven que desarrolló LHT luego de sufrir un paro cardiorrespiratorio.Delayed Hypoxic Leukoencephalopathy (DHL is a rare demyelinating disease. This condition occurs days or weeks after a hypoxic-anoxic injury. Symptoms and imaging changes almost fully resolve, with a good prognosis. We report the case of a young woman who developed DHL after a cardiopulmonary arrest.

  5. A Case of Megalencephalic Leukoencephalopathy with Subcortical Cysts in an Iranian Consanguineous Family

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    Sepideh Amoeian

    2009-12-01

    Full Text Available Background:Megalencephalic leukoencephalopathy with subcortical cysts (MLC is an autosomal recessive disorder characterized by macrocephaly, and slowly progressive clinical course marked by ataxia, spasticity and mental decline. MLC is caused by mutations in the gene MLC1 which encodes a novel protein, MLC1. Conclusion:A 4-year-old girl with macrocephaly, spasticity, ataxia and abnormal cerebral white matter and subcortical cysts in brain MRI diagnosed with MLC. This is the first report of MLC in an Iranian family. Conclusion:MLC1 should be considered in children with macrocephaly and slowly progressive psychomotor decline. This disease can be prenatally diagnosed and genetic counseling offered for future pregnancies.

  6. Delayed post-hypoxic leukoencephalopathy: case report with a review of disease pathophysiology

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    Michael Andrew Meyer

    2013-07-01

    Full Text Available Delayed post-hypoxic leukoencephalopathy is a rare clinical phenomenon usually observed in a small number of carbon monoxide poisoning survivors. A similar phenomenon is reported here in a patient who successfully recovered from a large overdose of diazepam and methadone, but then abruptly declined 3 weeks after the initial event. Magnetic resnance revealed confluent white matter hyperintensity on fluid-attenuated inversion recovery and T2 weighted sequences, and spectroscopy revealed elevated peaks in choline, creatinine, and lactate. Analysis and review of the literature suggests this phenomenon occurs on average about 19 days after the initial event. Although the pathophysiology remains obscure, it is noted here that the mean lucid interval coincides approximately with the replacement half-life for myelin related lipids and proteins.

  7. Computer-aided diagnosis of leukoencephalopathy in children treated for acute lymphoblastic leukemia

    Science.gov (United States)

    Glass, John O.; Li, Chin-Shang; Helton, Kathleen J.; Reddick, Wilburn E.

    2005-04-01

    The purpose of this study was to use objective quantitative MR imaging methods to develop a computer-aided diagnosis tool to differentiate white matter (WM) hyperintensities as either leukoencephalopathy (LE) or normal maturational processes in children treated for acute lymphoblastic leukemia with intravenous high dose methotrexate. A combined imaging set consisting of T1, T2, PD, and FLAIR MR images and WM, gray matter, and cerebrospinal fluid a priori maps from a spatially normalized atlas were analyzed with a neural network segmentation based on a Kohonen Self-Organizing Map. Segmented regions were manually classified to identify the most hyperintense WM region and the normal appearing genu region. Signal intensity differences normalized to the genu within each examination were generated for two time points in 203 children. An unsupervised hierarchical clustering algorithm with the agglomeration method of McQuitty was used to divide data from the first examination into normal appearing or LE groups. A C-support vector machine (C-SVM) was then trained on the first examination data and used to classify the data from the second examination. The overall accuracy of the computer-aided detection tool was 83.5% (299/358) with sensitivity to normal WM of 86.9% (199/229) and specificity to LE of 77.5% (100/129) when compared to the readings of two expert observers. These results suggest that subtle therapy-induced leukoencephalopathy can be objectively and reproducibly detected in children treated for cancer using this computer-aided detection approach based on relative differences in quantitative signal intensity measures normalized within each examination.

  8. Chronic multifocal non-bacterial osteomyelitis in hypophosphatasia mimicking malignancy

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    Warmuth-Metz Monika

    2007-01-01

    Full Text Available Abstract Background Hypophosphatasia (HP is characterized by a genetic defect in the tissue-nonspecific alkaline phosphatase (TNSALP gene and predominantly an autosomal recessive trait. HP patients suffer from reduced bone mineralization. Biochemically, elevated concentrations of substrates of TNSALP, including pyridoxal-5'-phosphate and inorganic pyrophosphate occur in serum, tissues and urine. The latter has been associated with chronic inflammation and hyperprostaglandinism. Case presentation We report on 2 affected children presenting with multifocal inflammatory bone lesions mimicking malignancy: A 6 years old girl with short stature had been treated with human growth hormone since 6 months. Then she started to complain about a painful swelling of her left cheek. MRI suggested a malignant bone lesion. Bone biopsy, however, revealed chronic inflammation. A bone scan showed a second rib lesion. Since biopsy was sterile, the descriptive diagnosis of chronic non-bacterial osteomyelitis (CNO was established. The diagnostic tests related to growth failure were repeated and subsequent analyses demonstrated a molecular defect in the TNSALP gene. The second girl (10 years old complained about back pain after she had fallen from her bike. X rays of her spine revealed compressions of 2 thoracic vertebrae. At first these were considered trauma related, however a bone scan did show an additional lesion in the right 4th rib. A biopsy of this rib revealed a sterile lympho- plasmocytoid osteomyelitis suggesting multifocal CNO. Further analyses did show a decreased TNSALP in leukocytes and elevated pyridoxal phosphate in plasma, suggesting a heterozygous carrier status of HP. Conclusion Chronic bone oedema in adult HP and chronic hyper-prostaglandinism in childhood HP do suggest that in some HP patients bone inflammation is present in conjunction with the metabolic defect. Sterile multifocal osteomyelitis could be demonstrated. Non-steroidal anti

  9. Aggressive and multifocal pulmonary inflammatory myofiberblastic tumor in young woman

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    Choi, Yang Sean; Chung, Myung Hee; Kim, Hyun Jung; Park, Ki Hoon; Kim, Jeanna; Kwon, Soon Suck; Yoo, Won Jong [Bucheon St. Mary' s Hospital, The Catholic University of Korea, Bucheon (Korea, Republic of)

    2016-08-15

    We report a case of pulmonary inflammatory myofibroblastic tumor (IMT) showing aggressive and unusually rapid progression. A 27-year-old woman was admitted to the emergency room due to dry cough, fever and blood-tinged sputum that lasted one week. Initial chest radiograph and computed tomography scan revealed multifocal pulmonary nodules, which subsequently progressed into large necrotic masses within two months. She underwent a fine needle biopsy of the largest mass in the right middle lung zone which revealed inflammatory myofibroblastic cells consistent with IMT. The masses showed complete regression after six months of corticosteroid therapy. This unusual clinical manifestation could help explain the reactive inflammatory nature associated with IMTs.

  10. Multifocal fixed drug eruption with COX-2 inhibitor-celecoxib

    Directory of Open Access Journals (Sweden)

    Shikha Chugh

    2013-01-01

    Full Text Available Cyclooxygenase-2 (COX-2 inhibitors are rapidly becoming the first choice nonsteroidal anti-inflammatory drugs (NSAIDs for various rheumatological and other painful conditions. However, they might not be as safe or free of side effects as they are considered to be. These COX-2inhibitors may cause a variety of dermatological and systemic side effects of which we should be aware to avoid their indiscriminate use. We hereby report a case of multifocal fixed drug eruption (FDE with celecoxib which has not yet been reported in Indian settings.

  11. Multifocal Fixed Drug Eruption with COX-2 Inhibitor-Celecoxib

    Science.gov (United States)

    Chugh, Shikha; Sarkar, Rashmi; Garg, Vijay K; Singh, Avninder; Keisham, Chitralekha

    2013-01-01

    Cyclooxygenase-2 (COX-2) inhibitors are rapidly becoming the first choice nonsteroidal anti-inflammatory drugs (NSAIDs) for various rheumatological and other painful conditions. However, they might not be as safe or free of side effects as they are considered to be. These COX-2inhibitors may cause a variety of dermatological and systemic side effects of which we should be aware to avoid their indiscriminate use. We hereby report a case of multifocal fixed drug eruption (FDE) with celecoxib which has not yet been reported in Indian settings. PMID:23716804

  12. Chronic recurrent multifocal osteomyelitis--report of eight patients.

    Science.gov (United States)

    Handrick, W; Hörmann, D; Voppmann, A; Schille, R; Reichardt, P; Tröbs, R B; Möritz, R P; Borte, M

    1998-12-01

    The authors undertook a retrospective review of the clinical records and radiologic findings of eight children with chronic, recurrent multifocal osteomyelitis (CRMO). This disease is a recognized clinicopathologic entity with typical radiographic findings, mostly in the metaphyses of the long bones. The diagnosis is one of exclusion without pathognomonic findings. The patients were treated with non-steroidal anti-inflammatory drugs. There was no evidence of altered bone growth or abnormal joint development. It is concluded that CRMO is a distinct clinical entity that is different from acute or subacute bacterial osteomyelitis. Recognition of this condition is important to avoid treatment with antibiotics and repeated operations.

  13. Chronic recurrent multifocal osteomyelitis: a rare skeletal disorder.

    Science.gov (United States)

    Aygun, Deniz; Barut, Kenan; Camcioglu, Yildiz; Kasapcopur, Ozgur

    2015-08-25

    Chronic recurrent multifocal osteomyelitis (CRMO) is a rare non-infectious inflammatory bone disease of unknown aetiology. CRMO mainly affects the metaphyses of long bones and spine in children and young adolescents. It presents with recurrent episodes of bone pain and fever, resembling bacterial osteomyelitis, but cultures of lesions are sterile and it is unresponsive to antibiotic therapy. We report a case of a 3-year-old boy diagnosed with CRMO, who was initially treated for bacterial osteomyelitis, and received prolonged antibiotic therapy for chronic pain, and swelling of mandible and ulna. CRMO should be kept in mind in the differential diagnosis of chronic bone pain and osteomyelitis unresponsive to antibiotic treatment.

  14. [Chronic recurrent multifocal osteomyelitis of the spine : Children and adolescent].

    Science.gov (United States)

    von der Höh, N H; Völker, A; Jeszenszky, D; Heyde, C-E

    2016-06-01

    Chronic non-bacterial osteomyelitis (CNO) in childhood and adolescence is a non-infectious autoinflammatory disease of the bone with partial involvement of adjacent joints and soft tissue. The etiology is unknown. The disease can occur singular or recurrent. Individual bones can be affected and multiple lesions can occur. Chronic recurrent multifocal osteomyelitis (CRMO) shows the whole picture of CNO. Accompanying but temporally independent of the bouts of osteomyelitis, some patients show manifestations in the skin, eyes, lungs and the gastrointestinal tract. The article gives an overview of the clinical manifestations, diagnostic procedures, and treatment options for CRMO involvement of the spine based on the current literature and our own cases.

  15. Comparison of ocular straylight after implantation of multifocal intraocular lenses

    NARCIS (Netherlands)

    Łabuz, Grzegorz; Reus, Nicolaas J; van den Berg, Thomas J T P

    2016-01-01

    UNLABELLED: A comprehensive review of the effect of multifocal intraocular lens (IOL) designs on postoperative ocular straylight was performed. Studies reporting straylight values obtained with the natural pupil using the C-Quant device after uneventful multifocal IOL implantation were included. The

  16. Comparison of a hydrophilic and a hydrophobic apodized diffractive multifocal intraocular lens.

    NARCIS (Netherlands)

    Linden, J.W.M. van der; Meulen, I.J. van der; Mourits, M.P.; Lapid-Gortzak, R.

    2013-01-01

    To compare outcomes between a new design apodized diffractive hydrophilic multifocal intraocular lens (IOL) (Seelens MF; study group), and a well-known apodized diffractive hydrophobic multifocal IOL (SN6AD1; control group). A comparative case series comparing refractive and visual outcomes at dista

  17. Multifocal pattern electroretinogram does not demonstrate localised field defects in glaucoma.

    Science.gov (United States)

    Klistorner, A I; Graham, S L; Martins, A

    2000-01-01

    To determine if a multifocal PERG could be recorded in normals, and to examine changes in the multifocal PERG in glaucoma patients. To compare the ability of multifocal PERG and multifocal VEP responses in the same individuals to identify localised field defects in glaucoma. Using the VERIS Scientific system multifocal PERGs were recorded from 19 sites of the visual field according to pseudo-random binary m-sequence. Twenty normals and 15 glaucoma subjects were tested. Multifocal pattern VEPs were also recorded in the glaucoma cases using a cortically scaled stimulus. The second order kernel of the PERG shows a consistent signal. The overall PERG amplitude decreases with age in normals. In glaucoma the PERG amplitude was reduced across the field, but reductions did not correspond to the area of the scotoma. The VEP showed localised signal reductions in all 15 cases of glaucoma. A multifocal PERG can be recorded in normals. However it did not reflect localised ganglion cell losses, whereas the multifocal pattern VEP recorded to a very similar stimulus in the same individual did show losses in the scotoma area.

  18. Whole body MRI in the diagnosis of chronic recurrent multifocal osteomyelitis.

    LENUS (Irish Health Repository)

    Kennedy, M T

    2012-06-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is a diagnosis of exclusion primarily in children and adolescents. As part of the essential criteria for the diagnosis of CRMO, multifocal lesions must be identified. We present the case of an 11-year-old boy with CRMO, whose diagnosis was facilitated by the use of whole body magnetic resonance imaging (WBMR), but not isotope bone scanning.

  19. Multifocality as a prognostic factor in breast cancer patients registered in Danish Breast Cancer Cooperative Group (DBCG) 1996-2001

    DEFF Research Database (Denmark)

    Joergensen, L.E.; Gunnarsdottir, K.A.; Lanng, C.;

    2008-01-01

    The purpose of this study was to investigate the prognostic influence of multifocality in breast cancer patients. In a cohort of 7196 patients there were 945 patients with multifocality. We found no prognostic influence of multifocality on overall survival when controlling for known prognostic......, Gunnarsdottir KA, Rasmussen BB, Moeller S, Lanng C. The prognostic influence of multifocality in breast cancer patients. Breast 2004;13:188-193]....

  20. Effect of pupil size on multifocal pattern visual evoked potentials.

    Science.gov (United States)

    Martins, Alessandra; Balachandran, Chandra; Klistorner, Alexander I; Graham, Stuart L; Billson, Francis A

    2003-08-01

    The purpose of this study was to investigate the influence of pupil diameter on the amplitude and latency of multifocal visual evoked potentials (mfVEP). The multifocal objective perimeter (Accumap; Objectivision) was used to stimulate the visual field at 56 sites extending to 32 degrees using a pseudo-random pattern stimulus. The mfVEP were recorded using bipolar occipital electrodes, 7 min/eye. Ten normal subjects were recruited from the community and one eye was randomly selected for testing. The mfVEP were recorded at four different pupil diameters (2 mm, 4 mm, 6 mm, 8 mm), obtained by applying tropicamide (0.5%) or pilocarpine (2%) in different dilutions. Appropriate refractive correction was provided to overcome cycloplegia and achieve a visual acuity of 6/7.5 or better. Analysis revealed that at most pupil diameters the normalized full field amplitude did not show significant variation, except at the most miotic pupil diameter (2 mm), where the amplitude became reduced, based on 2-way anova and Tukey's T method. There was, however, significant correlation between latency and pupil area (correlation coefficient: upper field -0.63, lower field -0.76). The results suggest that even in the presence of mydriatics or miotics, the mfVEP test can be used to assess diseases that affect amplitude, provided near correction is used. The interpretation of latency, however, must be made with caution, as a borderline conduction defect with a dilated pupil may appear normal.

  1. Effect of stimulus check size on multifocal visual evoked potentials.

    Science.gov (United States)

    Balachandran, Chandra; Klistorner, Alexander I; Graham, Stuart L

    2003-03-01

    In this study we examined the effects of varying stimulus check size on multifocal visual evoked potential (VEP). We also evaluated the currently used cortical scaling of stimulus segments. The ObjectiVision multifocal objective perimeter stimulates the eye with random check patterns at 56 cortically scaled segments within the visual field extending to a radius of 26 degrees. All cortically scaled segments have equal number of checks, which gradually increase in size from the center to the periphery, proportional to the size of the segment. Stimuli with 9, 16, 25, 36 and 49 checks/segment were tested on 10 eyes belonging to 10 normal subjects. The check size varied inversely with number of checks per segment. VEP was recorded using bipolar occipital cross electrodes (7 min/eye), the amplitude and latency of responses obtained were compared with the check size at different eccentricities. Our findings suggest that the existing setting with 16 checks/segment subtending 26' to 140' from center to periphery, is the most effective amongst all the check sizes. Decreasing the check size prolongs the latency in the central field only. Cortical scaling of segments generates responses of the same order of magnitude throughout the field, but could be improved slightly to enhance the signal from the outer two rings.

  2. Multifocal electroretinogram for assessing sun damage following the solar eclipse of 29 March 2006: multifocal electroretinography in solar maculopathy.

    Science.gov (United States)

    Arda, Hatice; Oner, Ayse; Mutlu, Sait; Köse, Ziya; Gumus, Koray; Karakucuk, Sarper; Mirza, Ertugrul

    2007-05-01

    To evaluate the clinical findings and multifocal electroretinography results of cases with solar maculopathy due to eclipse watching. Eight eyes of six patients (ages ranged 12-42) who presented to our clinic after the solar eclipse of 29 March 2006 were evaluated in the study. All patients underwent a full ophthalmologic examination and multifocal electroretinography (mfERG). Visual acuities at the initial examination were between 20/32 and 20/20; and at final examination between 20/25 and 20/20 respectively. Fundoscopic examination disclosed macular pigmentary changes in almost all patients. Fundus Fluorescein Angiography revealed a window defect in six eyes. The initial findings of the mfERG at the first visit showed a decrease in the P1 and N1 amplitudes of the central responses. The following mfERG recordings showed a recovery in central P1 and N1 amplitudes. Decrease in P1 and N1 amplitudes of central macular region can be detected by mfERG in patients with solar maculopathy. Follow-up mfERG test results may recover with the increase of visual acuity.

  3. Progression of solitary and multifocal papillary thyroid carcinoma— a retrospective study of 368 patients

    Institute of Scientific and Technical Information of China (English)

    ZHENG Xiang-qian; WANG Chen; XU Meng; YU Yang; YUN Xin-wei; JIA Yong-sheng; WEI Song-feng; REN Xiu-bao; GAO Ming

    2012-01-01

    Background Papillary thyroid carcinoma (PTC) represents one of the most frequent endocrine malignancies.Several factors have been found to be involved in determining the outcome of treatment for patients with PTC.Large tumor size,diagnosis at an early age,extra-thyroidal invasion,aggressive histological variants,and distant metastases are the most important determinants of a poor outcome.BRAFv600E mutation has been found to be a major genetic alteration in PTC.This study aimed to evaluate progression in patients with multifocal and solitary PTC.Methods We performed a retrospective study to analyze 368 patients with PTC who underwent surgery,including 282 patients with solitary PTC and 86 patients with multifocal PTC.The status of BRAFv600E mutation in all tumor foci from multifocal PTC was detected.Results Our study suggested that multifocal PTC was more related to lymph node metastasis and vascular invasion than solitary PTC.However,the distant metastasis rate and 10-year survival rate showed no difference between these two groups.The number of tumor foci did not affect progression of disease in multifocal PTC patients.Lymph node metastasis in multifocal PTC patients was associated with larger tumors,diagnosis at early stage,and extra-thyroidal invasion.Conclusion The status of BRAFv600E mutation was more frequent in multifocal PTC patients with lymph node metastasis and diagnosis at later age.

  4. Multifocal Renal Cell Carcinoma: Clinicopathologic Features and Outcomes for Tumors ≤4 cm

    Directory of Open Access Journals (Sweden)

    Paul L. Crispen

    2008-01-01

    Full Text Available A significant increase in the incidental detection of small renal tumors has been observed with the routine use of cross-sectional abdominal imaging. However, the proportion of small renal tumors associated with multifocal RCC has yet to be established. Here then, we report our experience with the treatment of multifocal RCC in which the primary tumor was ≤4 cm. In our series of 1113 RCC patients, 5.4% (60/1113 had multifocal disease at the time of nephrectomy. Discordant histology was present in 17% (10/60 of patients with multifocal RCC. Nephron sparing surgery was utilized more frequently in patients with solitary tumors. Overall, cancer-specific, and distant metastasis-free survival appeared to be similar between multifocal and solitary tumors. These findings are consistent with previous series which evaluated multifocal RCC with tumors >4 cm. With the known incidence of multifocality RCC, careful inspection of the entire renal unit should be performed when performing nephron sparing surgery.

  5. Bilateral implantation of +2.5 D multifocal intraocular lens and contralateral implantation of +2.5 D and +3.0 D multifocal intraocular lenses: Clinical outcomes.

    Science.gov (United States)

    Nuijts, Rudy M M A; Jonker, Soraya M R; Kaufer, Robert A; Lapid-Gortzak, Ruth; Mendicute, Javier; Martinez, Cristina Peris; Schmickler, Stefanie; Kohnen, Thomas

    2016-02-01

    To assess the clinical visual outcomes of bilateral implantation of Restor +2.5 diopter (D) multifocal intraocular lenses (IOLs) and contralateral implantation of a Restor +2.5 D multifocal IOL in the dominant eye and Restor +3.0 D multifocal IOL in the fellow eye. Multicenter study at 8 investigative sites. Prospective randomized parallel-group patient-masked 2-arm study. This study comprised adults requiring bilateral cataract extraction followed by multifocal IOL implantation. The primary endpoint was corrected intermediate visual acuity (CIVA) at 60 cm, and the secondary endpoint was corrected near visual acuity (CNVA) at 40 cm. Both endpoints were measured 3 months after implantation with a noninferiority margin of Δ = 0.1 logMAR. In total, 103 patients completed the study (53 bilateral, 50 contralateral). At 3 months, the mean CIVA at 60 cm was 0.13 logMAR and 0.10 logMAR in the bilateral group and contralateral group, respectively (difference 0.04 logMAR), achieving noninferiority. Noninferiority was not attained for CNVA at 40 cm; mean values at 3 months for bilateral and contralateral implantation were 0.26 logMAR and 0.11 logMAR, respectively (difference 0.15 logMAR). Binocular defocus curves suggested similar performance in distance vision between the 2 groups. Treatment-emergent ocular adverse events rates were similar between the groups. Bilateral implantation of the +2.5 D multifocal IOL resulted in similar distance as contralateral implantation of the +2.5 D multifocal IOL and +3.0 D multifocal IOL for intermediate vision (60 cm), while noninferiority was not achieved for near distances (40 cm). Copyright © 2016 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.

  6. Reversible posterior leukoencephalopathy syndrome and anti-neoplastic agents: a review

    Directory of Open Access Journals (Sweden)

    Farheen M. Shah-Khan

    2011-12-01

    Full Text Available Reversible Posterior Leukoencephalopathy Syndrome (RPLS is a well recognized entity with a variety of benign and malignant conditions. Recently it has been found to be associated with the use of anti-neoplastic agents including targeted therapies. RPLS occurs rapidly with the use of some drugs and more slowly with others. Combined therapies are associated with a more frequent and more rapid presentation. This review was based on a literature search for English Language articles concerning RPLS and chemotherapeutic agents published from June 1996 to March 2007. We used the PubMed database with keywords: “RPLS”, “Posterior reversible encephalopathy syndrome”, “(PRES”, “Chemotherapy” and “MRI”. This syndrome has classical Clinical-Radiologic features that are easy to recognize. Early recognition and withdrawal of the offending agent is all that is needed in most cases. This review highlights the features of the syndrome. It draws our attention to an entity which is being more frequently recognized and whose exact pathologic mechanisms need to be further studied. This syndrome is associated with the use of neurotoxic as well as non-neurotoxic agents and usually runs a benign course if there is an early diagnosis and management.

  7. Reversible posterior leukoencephalopathy syndrome and anti-neoplastic agents: a review

    Directory of Open Access Journals (Sweden)

    Farheen M. Shah-Khan

    2011-12-01

    Full Text Available Reversible Posterior Leukoencephalopathy Syndrome (RPLS is a well recognized entity with a variety of benign and malignant conditions. Recently it has been found to be associated with the use of anti-neoplastic agents including targeted therapies. RPLS occurs rapidly with the use of some drugs and more slowly with others. Combined therapies are associated with a more frequent and more rapid presentation. This review was based on a literature search for English Language articles concerning RPLS and chemotherapeutic agents published from June 1996 to March 2007. We used the PubMed database with keywords: “RPLS”, “Posterior reversible encephalopathy syndrome”, “(PRES”, “Chemotherapy” and “MRI”. This syndrome has classical Clinical-Radiologic features that are easy to recognize. Early recognition and withdrawal of the offending agent is all that is needed in most cases. This review highlights the features of the syndrome. It draws our attention to an entity which is being more frequently recognized and whose exact pathologic mechanisms need to be further studied. This syndrome is associated with the use of neurotoxic as well as non-neurotoxic agents and usually runs a benign course if there is an early diagnosis and management.

  8. Global Developmental Delay in a Mexican Patient With Megalencephalic Leukoencephalopathy With Subcortical Cysts

    Directory of Open Access Journals (Sweden)

    Cesar Misael Cerecedo Zapata

    2015-07-01

    Full Text Available Introduction: Megalencephalic leukoencephalopathy with subcortical cysts (MLC is a neurologic disorder characterized by macrocephaly within the first year of life and the delayed onset of motor function deterioration with ataxia and spasticity. Magnetic resonance imaging of the brain is diagnostic and shows diffusely abnormal, mildly swollen cerebral white matter and subcortical cysts. MLC exhibits an autosomal recessive mode of inheritance. Two genes have been associated with MLC. The first and most important gene is MLC1, The other gene involved is HEPACAM. Case Presentation: We studied a Mexican patient with a compatible diagnosis of MLC. The patient exhibited the c.353C > T, p.Thr118Met mutation, and both parents were carriers for the same mutation. To the best of our knowledge, no other cases of MLC have been reported in Mexican patient. This patient exhibited rapid deterioration of motor function. Conclusions: A diagnosis of MLC, which can be facilitated by imaging studies, should be considered in all patients who exhibit global developmental delay.

  9. 'Chasing the Dragon' - imaging of heroin inhalation leukoencephalopathy

    Energy Technology Data Exchange (ETDEWEB)

    Hagel, J. [Vancouver General Hospital, Dept. of Radiology, Vancouver, British Columbia (Canada)]. E-mail: jeff_hagel@hotmail.com; Andrews, G. [UBC Hospital, Dept. of Radiology, Vancouver, British Columbia (Canada); Vertinsky, T. [Vancouver General Hospital, Dept. of Radiology, Vancouver, British Columbia (Canada); Heran, M.K.S. [Vancouver General Hospital, Div. of Neuroradiology, Vancouver, British Columbia (Canada); Keogh, C. [BC Cancer Agency, Dept. of Radiology, Vancouver, British Columbia (Canada)

    2005-10-15

    'Chasing the dragon' refers to the inhalation of heroin pyrolysate vapors produced when the freebase form of heroin is heated. Inhalation of these vapors can result in a rare toxic spongiform leukoencephalopathy. The patients may progress through 3 defined clinical stages, with one-quarter reaching the terminal stage, which invariably leads to death. Imaging and, in particular, magnetic resonance imaging (MRI) demonstrates white matter findings that are felt to be specific for this entity and essential in its early diagnosis. We present the typical imaging findings in a pictorial essay format, using images taken from 9 patients who presented within an 18-month period at UBC-affiliated hospitals. These findings include low computed tomography (CT) attenuation and high T2 MRI signal most commonly in the posterior cerebral and cerebellar white matter, cerebellar peduncles, splenium of the corpus callosum, and posterior limb of the internal capsules. In addition, there is often selective, symmetric involvement of the corticospinal tract, the medial lemniscus, and the tractus solitarius. We also present the variable diffusion-weighted imaging arid apparent diffusion coefficient findings from 4 of our patients, which to our knowledge, have not been described in the literature. (author)

  10. Enlarging the nosological spectrum of hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS).

    Science.gov (United States)

    Hoffmann, Sarah; Murrell, Jill; Harms, Lutz; Miller, Kelly; Meisel, Andreas; Brosch, Thomas; Scheel, Michael; Ghetti, Bernardino; Goebel, Hans-Hilmar; Stenzel, Werner

    2014-09-01

    Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is an autosomal dominant disease clinically characterized by cognitive decline, personality changes, motor impairment, parkinsonism and seizures. Recently, mutations in the colony-stimulating factor-1 receptor (CSF1R) gene have been shown to be associated with HDLS. We report clinical, neuropathological and molecular genetic findings of patients from a new family with a mutation in the CSF1R gene. Disease onset was earlier and disease progression was more rapid compared with previously reported patients. Psychiatric symptoms including personality changes, alcohol abuse and severe depression were the first symptoms in male patients. In the index, female patient, the initial symptom was cognitive decline. Magnetic resonance imaging (MRI) showed bilateral, confluent white matter lesions in the cerebrum. Stereotactic biopsy revealed loss of myelin and microglial activation as well as macrophage infiltration of the parenchyma. Numerous axonal swellings and spheroids were present. Ultrastructural analysis revealed pigment-containing macrophages. Axonal swellings were detected by electron microscopy not only in the central nervous system (CNS) but also in skin nerves. We identified a heterozygous mutation (c.2330G>A, p.R777Q) in the CSF1R gene. Through this report, we aim to enlarge the nosological spectrum of HDLS, providing new clinical descriptions as well as novel neuropathological findings from the peripheral nervous system. © 2014 International Society of Neuropathology.

  11. [Diagnosis and treatment of multifocal motor neuropathy (Lewis-Sumner)].

    Science.gov (United States)

    Kaji, R

    1999-01-01

    We made a retrospective long-term follow-up study of 25 patients with multifocal motor neuropathy (Lewis-Sumner). The diagnosis was based upon criteria modified from those of AAEM (Sumner 1997). The electrophysiological findings indicating conduction block or focal demyelinative lesions were more diagnostic than anti-GM 1 antibody titers, which were elevated in only 40% of these patients. Demonstration of definite conduction block was not always possible in those patients who responded favorably to intravenous immunoglobulins (IVIg), whereas indirect pieces of evidence such as F-wave abnormalities or focal conduction delay or dispersion were equally helpful. IVIg had superior outcome to cyclophosphamide, which sometimes caused serious adverse effects. Three patients with severe axonal involvement showed elevated monospecific antibodies to GalNAc-GD1a.

  12. Reassignment of scattered emission photons in multifocal multiphoton microscopy.

    Science.gov (United States)

    Cha, Jae Won; Singh, Vijay Raj; Kim, Ki Hean; Subramanian, Jaichandar; Peng, Qiwen; Yu, Hanry; Nedivi, Elly; So, Peter T C

    2014-06-05

    Multifocal multiphoton microscopy (MMM) achieves fast imaging by simultaneously scanning multiple foci across different regions of specimen. The use of imaging detectors in MMM, such as CCD or CMOS, results in degradation of image signal-to-noise-ratio (SNR) due to the scattering of emitted photons. SNR can be partly recovered using multianode photomultiplier tubes (MAPMT). In this design, however, emission photons scattered to neighbor anodes are encoded by the foci scan location resulting in ghost images. The crosstalk between different anodes is currently measured a priori, which is cumbersome as it depends specimen properties. Here, we present the photon reassignment method for MMM, established based on the maximum likelihood (ML) estimation, for quantification of crosstalk between the anodes of MAPMT without a priori measurement. The method provides the reassignment of the photons generated by the ghost images to the original spatial location thus increases the SNR of the final reconstructed image.

  13. Spontaneous rupture of multifocal hepatocellular carcinoma: case report

    Directory of Open Access Journals (Sweden)

    Özen Ö

    2015-08-01

    Full Text Available Özkan Özen, Alptekin Tosun, Çiğdem Akgül Department of Radiology, Faculty of Medicine, Giresun University, Giresun, Turkey Abstract: Hemoperitoneum due to nontraumatic liver rupture is rare. The most common cause of nontraumatic rupture of the liver is hepatocellular carcinoma (HCC. The other causes of nontraumatic liver ruptures are peliosis hepatis, polyarteritis nodosa, systemic lupus erythematosus, preeclampsia, metastatic carcinoma, and other primary liver tumors. In this report, we present the computed tomography findings of spontaneous liver rupture in a 52-year-old male patient due to multifocal HCC, with the diagnosis proven by surgical specimen. Keywords: computed tomography, hemoperitoneum, liver, nontraumatic liver rupture

  14. Multifocal bone and bone marrow lesions in children - MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Raissaki, Maria; Demetriou, Stelios; Spanakis, Konstantinos; Skiadas, Christos; Karantanas, Apostolos H. [University of Crete, Faculty of Medicine, Department of Radiology, University Hospital of Heraklion, Heraklion, Crete (Greece); Katzilakis, Nikolaos; Stiakaki, Eftichia [University of Crete, Faculty of Medicine, Department of Pediatric Hematology-Oncology, University Hospital of Heraklion, Heraklion, Crete (Greece); Velivassakis, Emmanouil G. [University Hospital of Heraklion, Orthopedic Clinic, Heraklion, Crete (Greece)

    2017-03-15

    Polyostotic bone and bone marrow lesions in children may be due to various disorders. Radiographically, lytic lesions may become apparent after loss of more than 50% of the bone mineral content. Scintigraphy requires osteoblastic activity and is not specific. MRI may significantly contribute to the correct diagnosis and management. Accurate interpretation of MRI examinations requires understanding of the normal conversion pattern of bone marrow in childhood and of the appearances of red marrow rests and hyperplasia. Differential diagnosis is wide: Malignancies include metastases, multifocal primary sarcomas and hematological diseases. Benign entities include benign tumors and tumor-like lesions, histiocytosis, infectious and inflammatory diseases, multiple stress fractures/reactions and bone infarcts/ischemia. (orig.)

  15. Relatively spared central multifocal electroretinogram responses in acute quinine toxicity

    Science.gov (United States)

    Saeed, Muhammad Usman; Noonan, Carmel; Hagan, Richard; Brown, Malcolm

    2011-01-01

    A 71-year-old man was investigated with electrodiagnostic testing 4 months after a deliberate quinine overdose. Initially he was admitted to intensive care unit with visual acuity (VA) of perception of light in both eyes. VA recovered to 6/6 right eye and 6/12 left eye, though severely constricted fields were noted. Slow stimulus (base period of 83 ms) multifocal electroretinogram (ERG) showed electronegative responses outside the inner 5 degrees, with a reduced but electropositive response seen in this central area. It appears that in this case of bilaterally negative ERGs that the macula/fovea (which has a vascular supply through the choroid) is relatively spared as is seen in bilateral vascular electronegative ERGs. This may indicate that quinine toxicity to the retina may be secondary to effects similar to vascular occlusion or severe ischemia during the acute phase of quinine poisoning. PMID:22693278

  16. MONTE CARLO SIMULATION OF MULTIFOCAL STOCHASTIC SCANNING SYSTEM

    Directory of Open Access Journals (Sweden)

    LIXIN LIU

    2014-01-01

    Full Text Available Multifocal multiphoton microscopy (MMM has greatly improved the utilization of excitation light and imaging speed due to parallel multiphoton excitation of the samples and simultaneous detection of the signals, which allows it to perform three-dimensional fast fluorescence imaging. Stochastic scanning can provide continuous, uniform and high-speed excitation of the sample, which makes it a suitable scanning scheme for MMM. In this paper, the graphical programming language — LabVIEW is used to achieve stochastic scanning of the two-dimensional galvo scanners by using white noise signals to control the x and y mirrors independently. Moreover, the stochastic scanning process is simulated by using Monte Carlo method. Our results show that MMM can avoid oversampling or subsampling in the scanning area and meet the requirements of uniform sampling by stochastically scanning the individual units of the N × N foci array. Therefore, continuous and uniform scanning in the whole field of view is implemented.

  17. Chronic recurrent multifocal osteomyelitis exhibiting predominance of periosteal reaction.

    Science.gov (United States)

    Queiroz, Rodolfo Mendes; Rocha, Pedro Henrique Pereira; Lauar, Lara Zupelli; Costa, Mauro José Brandão da; Laguna, Claudio Benedini; Oliveira, Rafael Gouvêa Gomes de

    2017-04-01

    Chronic recurrent multifocal osteomyelitis is an idiopathic nonpyogenic autoinflammatory bone disorder involving multiple sites, with clinical progression persisting for more than 6 months and which may have episodes of remission and exacerbation in the long term. It represents up to 2-5% of the cases of osteomyelitis, with an approximate incidence of up to 4/1,000,000 individuals, and average age of disease onset estimated between 8-11 years, predominantly in females. The legs are the most affected, with a predilection for metaphyseal regions along the growth plate. We describe the case of a female patient, aged 2 years and 5 months, with involvement of the left ulna, right jaw and left tibia, showing a predominance of periosteal reaction as main finding.

  18. Cryptogenic Multifocal Ulcerous Stenosing Enteritis: An Exceptionally Rare Disease

    Directory of Open Access Journals (Sweden)

    Amandeep Kaur

    2016-01-01

    Full Text Available Cryptogenic Multifocal Ulcerous Stenosing Enteritis (CMUSE is a rare idiopathic disease of the small bowel. Its origin and pathophysiology has not been well described. Clinicopathologic features include unexplained ileal strictures with supercial ulceration. We present a case of a 31-year-old HIV positive lady who was admitted with complaints of recurrent abdominal pain and constipation. Laboratory investigations revealed iron deciency anemia. Ultrasonography of the abdomen showed dilated bowel loops and a subsequent barium follow through showed ve strictures in the ileum. Segmental small bowel resection with end-to-end anastomosis was performed. Grossly ileum showed ve ileal strictures. The diagnosis of CMUSE was made on histopathology after ruling out other causes of strictures. The present case highlights the importance of considering CMUSE in patients with chronic or recurrent episodes of intestinal obstruction with multiple small intestinal ulcers and strictures after other common causes have been ruled out.

  19. Practical rules for electrodiagnosis in suspected multifocal motor neuropathy.

    Science.gov (United States)

    Bromberg, Mark B; Franssen, Hessel

    2015-03-01

    Multifocal motor neuropathy (MMN) with conduction block (CB) is a rare chronic immune-mediated neuropathy, but important to diagnose as it is treatable. The key features in prototypic MMN are electrodiagnostic demonstration of focal CB away from common sites of entrapment and normal sensory conduction across these sites. However, there are challenges in distinguishing CB from the effects of abnormal temporal dispersion. Consensus electrodiagnostic criteria, reinforced by modeling studies, are available to support definite or probable CB. In addition, consideration of technical issues can guard against false-positive and false-negative conclusions. These include limb temperature, stimulus site, inadvertent stimulating electrode movement, and supramaximal and submaximal responses, as well as the possibility of Martin-Gruber anastamosis. Robust evidence supports the treatment of MMN with intravenous immunoglobulin, and guidelines have been developed. Application of practical and simple rules including a 4-step diagnostic algorithm can help practitioners correctly diagnose this treatable condition and improve patient outcomes.

  20. [Multifocal electroretinogram for assessing sun damage following the solar eclipse of 11 August 1999].

    Science.gov (United States)

    Mack, G; Uzel, J L; Sahel, J; Flament, J

    2002-04-01

    Following the eclipse of 11 August 1999, the ophthalmological clinic of Strasbourg cared for 4 patients who suffered from sun damage. The multifocal electroretinogram (ERG) carried out on our first patient enabled us to not only confirm the existence of a residual maculopathy as shown by the static visual field, but also, and above all to quantify the foveolar deficit. Moreover, with the other 3 patients, the multifocal ERG enabled us to detect a foveolar deficit completely overlooked by the other usual tests. In light of our clinical study, we can assert that the multifocal ERG has yet to be equaled by any other test for analysis of macular function.

  1. MULTIFOCAL BILATERAL METACHRONOUS BREAST CANCER IN MAN. CLINICAL CASE

    Directory of Open Access Journals (Sweden)

    E. K. Saribekyan

    2015-01-01

    Full Text Available Multifocal malignant tumors (MMT represent a comprehensive and multifaceted problem of clinical oncology.Today, as far as observations are accumulated, there are attempts to determine a real incidence rate of multifocal tumors. Research of MMT problem becomes more and more actual and significant in oncology. Several dozen thousands of MMT observations have been described all over the world for the recent 30 years. There is a stable trend towards the increase of the number of patients with such pathology. This is primarily concerned with the improvement of diagnostics, medical treatment and increased expectation of life.The increase in incidence of MMT is due to such factors as increased average expectation of life, increased intensity of carcinogenic effects, urbanization, accumulation of hereditary loading and improved diagnostics of oncologic diseases. Detection and record of such pathology are based first on detection of simultaneous tumors, second – on diagnostics of tumor and retrospective determination according to life record data of the first neoplasm, third – on determination of further tumor at regular follow-up, which requires prolonged and high quality examination of the patients.MMT most commonly develop in patients elder than 50 y. o., even in elder age in men than in women. The article describes a case of bilateral breast cancer in man. Clinicopathologic and immunohistochemical characteristics of the tumor were studied, the genetic polymorphisms were investigated by the method of polymerase chain reaction, associated with the risk of breast cancer development, genetic analysis of BRCA1 and BRCA2, blood cancer markers was done. Full equivalence of clinicopathologic and biological properties of tumor of the right and left mammae, witnessing the common etiology and pathogenesis of neoplastic process, was established. Each case of bilateral breast cancer in men shall be studied with particular care and referred in international

  2. Power profiles of multifocal contact lenses and their interpretation.

    Science.gov (United States)

    Plainis, Sotiris; Atchison, David A; Charman, W Neil

    2013-10-01

    Many contact lens (CL) manufacturers produce simultaneous-image lenses in which power varies either smoothly or discontinuously with zonal radius. We present in vitro measurements of some recent CLs and discuss how power profiles might be approximated in terms of nominal distance corrections, near additions, and on-eye visual performance. Fully hydrated soft, simultaneous-image CLs from four manufacturers (Air Optix AQUA, Alcon; PureVision multifocal, Bausch & Lomb; Acuvue OASYS for Presbyopia, Vistakon; Biofinity multifocal- "D" design, Cooper Vision) were measured with a Phase focus Lens Profiler (Phase Focus Ltd., Sheffield, UK) in a wet cell and powers were corrected to powers in air. All lenses had zero labeled power for distance. Sagittal power profiles revealed that the "low" add PureVision and Air Optix lenses exhibit smooth (parabolic) profiles, corresponding to negative spherical aberration. The "mid" and "high" add PureVision and Air Optix lenses have bi-aspheric designs, leading to different rates of power change for the central and peripheral portions. All OASYS lenses display a series of concentric zones, separated by abrupt discontinuities; individual profiles can be constrained between two parabolically decreasing curves, each giving a valid description of the power changes over alternate annular zones. Biofinity lenses have constant power over the central circular region of radius 1.5 mm, followed by an annular zone where the power increases approximately linearly, the gradient increasing with the add power, and finally an outer zone showing a slow, linear increase in power with a gradient being almost independent of the add power. The variation in power across the simultaneous-image lenses produces enhanced depth of focus. The through-focus nature of the image, which influences the "best focus" (distance correction) and the reading addition, will vary with several factors, including lens centration, the wearer's pupil diameter, and ocular

  3. Power Profiles of Commercial Multifocal Soft Contact Lenses

    Science.gov (United States)

    Kim, Eon; Bakaraju, Ravi C.; Ehrmann, Klaus

    2017-01-01

    ABSTRACT Purpose To evaluate the optical power profiles of commercially available soft multifocal contact lenses and compare their optical designs. Methods The power profiles of 38 types of multifocal contact lenses—three lenses each—were measured in powers +6D, +3D, +1D, −1D, −3D, and −6D using NIMO TR1504 (Lambda-X, Belgium). All lenses were measured in phosphate buffered saline across 8 mm optic zone diameter. Refractive index of each lens material was measured using CLR 12-70 (Index Instruments, UK), which was used for converting measured power in the medium to in-air radial power profiles. Results Three basic types of power profiles were identified: center-near, center-distance, and concentric-zone ring-type designs. For most of the lens types, the relative plus with respect to prescription power was lower than the corresponding spectacle add. For some lens types, the measured power profiles were shifted by up to 1D across the power range relative to their labeled power. Most of the lenses were designed with noticeable amounts of spherical aberration. The sign and magnitude of spherical aberration can either be power dependent or consistent across the power range. Conclusions Power profiles can vary widely between the different lens types; however, certain similarities were also observed between some of the center-near designs. For the more recently released lens types, there seems to be a trend emerging to reduce the relative plus with respect to prescription power, include negative spherical aberration, and keep the power profiles consistent across the power range. PMID:27748699

  4. Isolated neurosarcoidosis mimicking multifocal meningiomas: a diagnosis pitfall

    Science.gov (United States)

    Wang, Kun; He, Xiaoying; Wang, Wei; Niu, Huanjiang; Wang, Yirong; Cai, Xiujun; Yang, Shuxu

    2016-01-01

    Abstract Introduction: Neurosarcoidosis accounts for approximately 5% of the sarcoidosis, which develops exclusively in the nervous system and is always difficult to diagnose. We describe a rare case of isolated neurosarcoidosis mimicking as multifocal meningiomas. A 27-year-old male was admitted to our hospital with a history of unconsciousness and convulsion 1 month ago, which was suspected as a seizure. The results showed no abnormalities in complete blood count; serum electrolytes; erythrocyte sedimentation rate and ultrasonography of the liver, pancreas, spleen, kidney and parotid gland, and so on. Chest radiograph and electroencephalogram were also normal. Serum-angiotensin-converting enzyme slightly increased. Normal opening pressure was shown in cerebrospinal fluid sampling, which includes 8/μL white blood cells, 0.93 g/L protein, and 3.03 mmol/L glucose. Enhanced magnetic resonance imaging revealed multifocal enhancement lesions, including left sphenoid wing region, left temporal and bilateral occipitoparietal region, which were suspected as multiple “meningioma”. A left frontotemporal craniotomy was further performed. Both necrotizing and non-necrotizing granulomas were revealed in the pathological specimen, most of which were associated with multinucleated giant cells and macrophages. We could also see the fibrosis and inflammatory reaction in the sample composed of lymphocytes, histiocytes, and plasma cells. Histopathological examination showed that the cells were positive for human CD68 (KP1), CD68 (PGM1), and CD163; however, they were negative for the AF, epithelial membrane antigen, and glial fibrillary acidic protein. Tuberculosis-deoxyribonucleic acid test and special stains for acid-fast bacilli and fungi were negative. The diagnosis was finally made as isolated neurosarcoidosis. Then the patient was treated with additional corticosteroid therapy. Serial imaging examination 4 months later revealed that the lesions extremely decreased

  5. The effect of high- to low-altitude adaptation on the multifocal electroretinogram

    DEFF Research Database (Denmark)

    Kofoed, Peter Kristian; Sander, Birgit; Zubieta-Calleja, Gustavo

    2009-01-01

    markedly supernormal multifocal electroretinographic amplitudes that continued to increase during the 72-day period of observation where the highlanders' hematocrit normalized. The results suggest that acclimatization after a change in altitude and hence in ambient oxygen tension involves intrinsic retinal...

  6. Multifocal choroiditis following simultaneous hepatitis A, typhoid, and yellow fever vaccination

    Directory of Open Access Journals (Sweden)

    Escott S

    2013-02-01

    Full Text Available Sarah Escott, Ahmad B Tarabishy, Frederick H DavidorfHavener Eye Institute, The Ohio State University, Columbus, OH, USAAbstract: The paper describes the first reported case of multifocal choroiditis following simultaneous hepatitis-A, typhoid, and yellow fever vaccinations. A 33-year-old male developed sudden onset of flashing lights and floaters in his right eye 3 weeks following hepatitis A, typhoid, and yellow fever vaccinations. Fundus examination and angiography confirmed the presence of multiple peripheral chorioretinal lesions. These lesions demonstrated characteristic morphologic changes over a period of 8 weeks which were consistent with a diagnosis of self-resolving multifocal choroiditis. Vaccine-induced intraocular inflammation has been described infrequently. We demonstrate the first case of self-resolving multifocal choroiditis following simultaneous administration of hepatitis A, yellow fever, and typhoid immunizations.Keywords: multifocal choroiditis, vaccination, hepatitis A, typhoid, yellow fever

  7. A compound heterozygous EARS2 mutation associated with mild leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL).

    Science.gov (United States)

    Güngör, Olcay; Özkaya, Ahmet Kağan; Şahin, Yavuz; Güngör, Gülay; Dilber, Cengiz; Aydın, Kürşad

    2016-10-01

    Mitochondrial glutamyl-tRNA synthetase is a major component of protein biosynthesis that loads tRNAs with cognate amino acids. Mutations in the gene encoding this enzyme have been associated with a variety of disorders related to oxidative phosphorylation. Here, we present a case of leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL) presenting a biphasic clinical course characterized by delayed psychomotor development and seizure. High-throughput sequencing revealed a novel compound heterozygous mutation in mitochondrial glutamyl-tRNA synthetase 2 (EARS2), which appears to be causative of disease symptoms.

  8. Detection of multifocal osteonecrosis in an adolescent with dermatomyositis using whole-body MRI

    Energy Technology Data Exchange (ETDEWEB)

    Castro, Tania C.M.; Terreri, Maria Teresa A.; Hilario, Maria Odete E. [Federal University of Sao Paulo, Department of Pediatrics, Division of Allergy, Clinical Immunology and Rheumatology, Sao Paulo, SP (Brazil); Lederman, Henrique [Federal University of Sao Paulo, Image Diagnosis Department, Sao Paulo (Brazil); Kaste, Sue C. [St. Jude Children' s Research Hospital, Department of Radiological Sciences, Division of Diagnostic Imaging, Memphis, TN (United States)

    2010-09-15

    Osteonecrosis is a well-recognized complication of corticosteroid use resulting in significant morbidity, often requiring surgical intervention. Whole-body MRI is a promising method that allows imaging of the whole patient in a reasonable time without the use of ionizing radiation. This technique has the potential for evaluating nonmalignant multifocal skeletal disease like osteonecrosis. This case highlights the value of whole-body MR in an adolescent with dermatomyositis who developed multifocal osteonecrosis. (orig.)

  9. Unusual multifocal granulomatous disease caused by actinomycetous bacteria in a nestling Derbyan parrot (Psittacula derbiana).

    Science.gov (United States)

    Park, F J; Jaensch, S

    2009-01-01

    A nestling Derbyan parrot (Psittacula derbiana) was presented with unusual subcutaneous swellings of the thigh regions, and poor growth. Histological examination revealed actinomycetous bacteria associated with multifocal systemic granulomas. The clinical and pathological findings of the case are presented, and some relevant aspects of actinomycetous bacterial infections in mammals and birds are discussed. Although granulomatous disease is encountered at times in avian species, the actinomycetous bacteria (Nocardia and Actinomyces spp.) have rarely been reported in association with multifocal granulomatous disease in birds.

  10. Multifocal Dysembryoplastic Neuroepithelial Tumour with Intradural Spinal Cord Lipomas: Report of a Case

    Directory of Open Access Journals (Sweden)

    Richard D. White

    2011-01-01

    Full Text Available We report a case of temporal lobe epilepsy and incomplete Brown-Sequard syndrome of the thoracic cord. Computed tomography and magnetic resonance (MR imaging showed multiple supratentorial masses with the classical radiological appearances of multifocal dysembryoplastic neuroepithelial tumour (DNET. Spinal MR imaging revealed intradural lipomas, not previously reported in association with multifocal DNET. Presentation and imaging findings are discussed along with classification and natural history of the tumour.

  11. Multi-focal spherical media and geodesic lenses in geometrical optics

    CERN Document Server

    Sarbort, Martin

    2013-01-01

    This paper presents a general approach to designing the isotropic spherical media with complex spatial structure that provide different types of imaging for different light rays. It is based on equivalence of the spherical medium and the corresponding geodesic lens. We use this approach to design multi-focal gradient- index lenses embedded into an optically homogeneous region and multi-focal absolute instruments that provide perfect imaging of three-dimensional domains.

  12. Multifocal peritoneal splenosis in Tc-99m-labeled heat-denatured red blood cell scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Min Ki; Hwang, Kyung Hoon; Choe, Won Sick [Gachon University Gil Medical Center, Incheon (Korea, Republic of)

    2006-06-15

    A 44-year-old man with a past medical history of splenectomy came to hospital because of epigastric pain abdominopelvic computed tomography(CT) showed a soft tissue mass and multifocal variable-sized nodules as well as finding suggestive of cholecystitis. Subsequently, he underwent Tc-99m-labeled heat- denatured red blood cell(RBC) scintigraphy to evaluate the mass and nodules. The scintigraphy confirmed multifocal peritoneal splenosis in the abdominopelvic cavity.

  13. [Posterior leukoencephalopathy syndrome presenting with bilateral ataxie optique: a case report].

    Science.gov (United States)

    Yamashiro, Kazuo; Kunoki, Makiko; Miura, Yoshiharu; Tomiyama, Junji; Mochizuki, Hideki; Mizuno, Yoshikuni

    2005-01-01

    We report a 45 years old right-handed woman who developed acute respiratory distress syndrome (ARDS) after the operation for rupture of an ovarian cyst. One week after the onset of ARDS, she presented visual disturbance and Gerstmann syndrome. MRI T2-weighted images demonstrated abnormal high intensity lesions involving the gray and white matter of the occipital lobes bilaterally extending to the parietal lobes. Based on these findings, she was diagnosed as having posterior leukoencephalopathy syndrome (PLES). Her neurologic symptoms gradually improved, however, she started to complain of difficulty in grasping a cup placed on her peripheral visual fields. Neurological examination revealed no visual disturbance, weakness or cerebellar ataxia. She could easily reach objects presented in the central visual field, however, she could not grasp objects presented peripheral visual fields, while she was looking straight ahead. This disorder was observed both in the right and left visual field, whether she used the right hand or the left hand. We thought she had ataxie optique of Garcin. She was noted to have bilateral both direct and crossed ataxie optique. Ataxie optique is characterized by disturbance in reaching objects presented in the peripheral visual field. The underlying pathophysiologic mechanism is believed to be disconnection of the fibers between the primary visual area and the angular gyrus at the parietooccipital junction. Crossed ataxie optique consisting of difficulty in reaching objects presented in the contralateral visual field believed to be caused by disconnection of crossed pathways of the corpus callosum. Ataxie optique in our patient can be explained by disconnection of both direct and crossed fibers. Ataxie optique is sometimes unrecognized by the patient. Our patient suggests that ataxie optique may well be a symptom of PLES.

  14. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy resulting in stroke in an 11-year-old male

    DEFF Research Database (Denmark)

    Granild-Jensen, Jakob Bie; Jensen, Uffe Birk; Schwartz, Marianne

    2009-01-01

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the Notch3 gene on chromosome 19. The condition manifests itself clinically typically in the third to fifth decade with migraine and recurrent episodes of stroke or trans......Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the Notch3 gene on chromosome 19. The condition manifests itself clinically typically in the third to fifth decade with migraine and recurrent episodes of stroke...... or transient ischaemic attacks. We report the case of an 11-year-old male with CADASIL resulting in stroke with right hemiparesis and dysphasia. Acute magnetic resonance imaging suggested infarction in the left hemisphere; magnetic resonance angiography revealed calibre variation of the intracerebral arteries....... The patient suffered from common migraine with five to six attacks per month for 3 years 6 months before the stroke. Attacks occurred early in the morning with severe one-sided headache, photophobia, nausea, and vomiting. Antimigraine medications had no effect. The family history revealed more cases...

  15. An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation

    Directory of Open Access Journals (Sweden)

    Rajaie Namas

    2014-01-01

    Full Text Available Case. A 34-year-old African-American female with a history of adult-onset Still’s disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital’s burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD.

  16. Macro creatine kinase type 1: a cause of spuriously elevated serum creatine kinase associated with leukoencephalopathy in a child.

    Science.gov (United States)

    Bodensteiner, John B

    2014-07-01

    Macro creatine kinase type 1 is a complex formed by the creatine kinase isoenzyme BB and monoclonal IgG and occurs in about 1% of patients studied. First identified as a cause of spurious elevation of the total serum creatine kinase in patients suspected of myocardial infarction, the test has been largely replaced by the measurement of troponin levels. We present a child with delayed milestones and persistently elevated total serum creatine kinase measurements (∼ 1000-4000 IU) normal electromyogram and brisk myotatic reflexes. Creatine kinase isoenzymes and brain imaging showed the presence of macro creatine kinase type 1 and extensive signal abnormality of the cerebral white matter. Macro creatine kinase type 1 has been associated with several conditions though it has not been described in association with leukoencephalopathy or in patients this young. Macro creatine kinase type 1 can be a cause of elevated total creatine kinase in patients without primary muscle disease. The significance of the relationship of the macro creatine kinase to the leukoencephalopathy in this patient is unknown.

  17. Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia linked CSF1R mutation: Report of four Korean cases.

    Science.gov (United States)

    Kim, Eun-Joo; Shin, Jin-Hong; Lee, Jeong Hee; Kim, Jong Hun; Na, Duk L; Suh, Yeon-Lim; Hwang, Sun Jae; Lee, Jae-Hyeok; Lee, Young Min; Shin, Myung-Jun; Lee, Myung Jun; Kim, Seong-Jang; Yoon, Uicheul; Park, Do Youn; Jung, Dae Soo; Ahn, Jae Woo; Sung, Suk; Huh, Gi Yeong

    2015-02-15

    We describe detailed clinical, biochemical, neuroimaging and neuropathological features in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), encompassing hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD), linked to colony-stimulating factor 1 receptor (CSF1R) mutations in four Korean cases. Clinical, biochemical, neuroimaging and neuropathological findings were obtained by direct evaluation and from previous medical records. The genetic analysis of the CSF1R gene was done in two autopsy-confirmed ALSP cases and two cases where ALSP was suspected based on the clinical and neuroimaging characteristics. We identified two known mutations: c.2342C>T (p.A781V) in one autopsy-proven HDLS and clinically ALSP-suspected case and c.2345G>A (p.R782H) in another autopsy-proven POLD case. We also found a novel mutation (c.2296A>G; p.M766V) in a patient presenting with hand tremor, stuttering and hesitant speech, and abnormal behavior whose father died from a possible diagnosis of spinocerebellar ataxia. To the best of our knowledge, this is the first documented ALSP-linked CSF1R mutation in Korea and supports the suggestion that HDLS and POLD, with pathological characteristics that are somewhat different but which are caused by CSF1R mutations, are the same spectrum of disease, ALSP. Copyright © 2014 Elsevier B.V. All rights reserved.

  18. [Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS): its clinical concept and the review of the previously reported cases].

    Science.gov (United States)

    Ikeda, Shu-Ichi

    2012-01-01

    Leukoencephalopathy or leukodystrophy is characterized pathologically by extensive degenerative and/or demyelinating lesions in cerebral white matter and produces various clinical manifestations such as behavioral and/or mood changes, dementia, motor impairment and epilepsy. The hereditary form of this disease is rare, but recent advances in diagnostic techniques have made it possible to make a correct pre-mortem diagnosis for some diseases including CADASIL and CARASIL. Recently, another unique form of the disease, hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) has been noted. The clinical picture of HDLS is as follows: age of onset ranging from 8 to 78 years (average: 39 years), autosomal dominant inheritance, and dementia. The presence of numerous neuroaxonal spheroid in cerebral white matter is one of the pathologic hallmarks of HDLS. Another term "familial pigmentary orthochromatic leukodystrophy (POLD)" has been also used for the patients showing similar clinical pictures and pathologic findings of the patients. Both disorders are now regarded as a single disease entity. Rademakers et al have just reported that HDLS is caused by mutation in the colony stimulating factor 1 receptor gene (CSF1R) and HDLS should be included in the differential diagnosis of familial occurrence of pre-senile dementia.

  19. Case report: Multifocal chronic paracoccidioidomycosis in an adult

    Directory of Open Access Journals (Sweden)

    Karen Zapata

    2011-06-01

    Full Text Available Paracoccidioides brasiliensis es el agente causal de una de las micosis sistémicas con mayor prevalencia en Latinoamérica. Existen alrededor de 10 millones de afectados y la mayor incidencia se presenta en Brasil y se han observado casos en Venezuela, Colombia, Ecuador y Argentina. Se presenta el caso de un hombre de 56 años con lesiones de un año de evolución en labios, mucosa oral y pulmón. El estudio histopatológico con hematoxilina-eosina informó la presencia de granulomas y células gigantes multinucleadas. Los estudios micológicos (KOH y tinción de Gomori Grocott mostraron blastoconidias con gemación múltiple. Las pruebas serológicas para paracoccidioidina fueron reactivas. Se diagnosticó un caso de paracoccidioidomicosis multifocal crónica y se inició tratamiento anfotericina B 0.7 mg/kg/día durante 15 días, luego itraconazol 400 mg diarios. Las lesiones cutáneas y pulmonares mejoraron; sin embargo, presentaba una fibrosis residual importante en la región oral y no continuó el seguimiento. Se resalta la importancia de realizar un diagnóstico temprano y disminuir las posibles secuelas que van a repercutir directamente sobre la calidad de vida del paciente.

  20. Lewis-Sumner syndrome and multifocal motor neuropathy.

    Science.gov (United States)

    Verschueren, Annie; Azulay, Jean Philippe; Attarian, Shahram; Boucraut, José; Pellissier, Jean François; Pouget, Jean

    2005-01-01

    We compared the clinical, electrophysiological, laboratory, and pathological features of 13 patients with Lewis-Sumner syndrome (LSS) with those of 20 patients with multifocal motor neuropathy (MMN). LSS and MMN patients have several common clinical features: age at onset, weakness in the distribution of individual peripheral nerves, mild wasting, cramps and fasciculations, partial areflexia, and frequent stepwise disease course. Cerebrospinal fluid protein level was normal or slightly elevated, but always less than 100 mg/dl. Conduction blocks are the electrophysiological hallmarks of these two neuropathies, and no differences in distribution and number of blocks were found. Contrary to MMN, lower-limb involvement at onset was frequent in LSS but extension to the upper limbs was a frequent later feature of the disease. Cranial nerve involvement was noted in 4 LSS patients during relapses and absent in all MMN patients. The major distinguishing features were the clinical and electrophysiological sensory involvement in LSS, and the lack of anti-GM1 antibodies in LSS, whereas IgM anti-GM1 were found in 40% of MMN patients. Some LSS patients responded to steroid therapy, whereas this was ineffective in MMN. From these features, LSS can be considered an entity distinct from MMN, with its own clinical, laboratory, and electrophysiological characteristics, and as an intermediate link between chronic inflammatory demyelinating polyneuropathy and MMN.

  1. Chronic Recurrent Multifocal Osteomyelitis Causing an Acute Scoliosis

    Directory of Open Access Journals (Sweden)

    Alexander Armstrong

    2013-01-01

    Full Text Available Study Design. A Case Report. Objective. We present a 15-year-old girl with an acute atypical scoliosis secondary to chronic recurrent multifocal osteomyelitis (CRMO. Summary of Background Data. CRMO is a rare nonpyogenic inflammatory bone condition with unclear aetiology. CRMO mainly affects the metaphyses of long bones, the pelvis, shoulder girdle, and less commonly the spine. Methods. Our case presented with a 6-month history of worsening thoracic back pain, asymmetry of her shoulders and abnormal posture. Whole spine radiographs revealed a right atypical thoracic scoliosis. Magnetic Resonance Imaging showed abnormal signal on the short TI inversion recovery (STIR sequences in multiple vertebrae. A bone biopsy demonstrated evidence of fibrosis and chronic inflammatory changes. Interval MRI scans revealed new oedematous lesions and disappearance of old lesions. Symptoms improved. Results. It is important to consider CRMO as an acute cause of atypical scoliosis. Malignancy, pyogenic infections and atypical presentations of juvenile arthritis need excluding. Conclusion. This 24-month follow-up describes a rare cause of an atypical scoliosis and fortifies the small amount of the currently available literature. The case highlights the relapsing and remitting nature of CRMO with new lesions developing and older lesions burning out. We advise close radiological surveillance and symptomatic management.

  2. Multifocal atherosclerosis in patient after acute first degree radiation sickness.

    Directory of Open Access Journals (Sweden)

    Metlyaeva N.A.

    2014-12-01

    Full Text Available Purpose: assessment the heavy psychosomatic and all-somatic cardiovascular and cerebrovascular pathology of patient, transferred an acute I degree radiation sickness, from the general evenly gamma-beta radiation. Conclusions. The subdepressive and disturbing-depressive syndrome of patient, transferred an acute radiation sickness (ARS of I degree, from the general evenly gamma-beta radiation, was independent risk factor of development of multifocal atherosclerosis; Features of development of all-somatic and psychosomatic pathology of patient are based on a combination of genetic prerequisites, environment influences (the stress caused by accident on the ChNPP and social factors, influencing on him during a course of life, especially during early socialization. Thus at development of psychosomatic frustration the combination of feature of the mental reaction connected with the personal characteristic and special relationship between mental (stress and physiological (somatic by aspects of reaction which led to metabolism violation, to aging, decrease in adaptation opportunities of an organism and development age — dependent pathology took place.

  3. Software for analysing multifocal visual evoked potential signal latency progression.

    Science.gov (United States)

    de Santiago, L; Klistorner, A; Ortiz, M; Fernández-Rodríguez, A J; Rodríguez Ascariz, J M; Barea, R; Miguel-Jiménez, J M; Boquete, L

    2015-04-01

    This paper describes a new non-commercial software application (mfVEP(2)) developed to process multifocal visual-evoked-potential (mfVEP) signals in latency (monocular and interocular) progression studies. The software performs analysis by cross-correlating signals from the same patients. The criteria applied by the software include best channels, signal window, cross-correlation limits and signal-to-noise ratio (SNR). Software features include signal display comparing different tests and groups of sectors (quadrants, rings and hemispheres). The software's performance and capabilities are demonstrated on the results obtained from a patient with acute optic neuritis who underwent 9 follow-up mfVEP tests. Numerical values and graphics are presented and discussed for this case. The authors present a software application used to study progression in mfVEP signals. It is also useful in research projects designed to improve mfVEP techniques. This software makes it easier for users to manage the signals and allows them to choose various ways of selecting signals and representing results. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Multifocal ERG Guiding Therapy in a Case of Hydroxychloroquine Premaculopathy

    Directory of Open Access Journals (Sweden)

    José Antonio Sáez-Moreno

    2015-01-01

    Full Text Available We report the case of a 28-year-old female treated for systemic lupus erythematosus with hydroxychloroquine (200 mg/day for 11 years. She was visually asymptomatic, with normal fundus appearance, normal colour vision testing findings, 20/20 visual acuity in both eyes, and only mild central bilateral defects on 10-2 perimetry. Multifocal electroretinography (mfERG showed low density values for ring 1 in both eyes. Because the patient had not previously responded to alternative treatments and in consultation with her physician, the hydroxychloroquine dose was reduced to 200 mg four days/week. Four serial mfERGs performed at 4, 18, 25, and 34 months after dose reduction showed a progressive improvement in the definition and density of the responses until they were normalized at the third mfERG (25 months after hydroxychloroquine dose reduction. The fourth and final mfERG at 34 months confirmed the recovery in both eyes. Perimetry defects were mostly normalized. These results demonstrate the importance of mfERG for the safe management of patients under long-term hydroxychloroquine treatment.

  5. Multifocal Toric Intraocular Lens for Traumatic Cataract in a Child

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    Yanfeng Zeng

    2016-10-01

    Full Text Available A child suffering from traumatic cataract and corneal astigmatism of 2.14 D had a phacoemulsification operation and implantation of a ReSTOR Toric intraocular lens (IOL to correct the astigmatism. The primary outcome measurements were the uncorrected distance visual acuity (UDVA, uncorrected near vision at 40 cm, intraocular pressure, spherical equivalent refraction, residual astigmatism, corneal astigmatism, presence of unusual optical phenomena, and use of spectacles. At 7 months postoperatively, UDVA was maintained between 16/20 and 24/20, near vision was between J1 and J3, residual spherical refraction was 0–0.37 D, and residual refractive cylinder was between 0 and 0.67 D. A multifocal toric IOL can provide the possibility of satisfactory vision for both distant and near conditions without the use of spectacles to meet children’s needs when studying and doing sports. Additionally, binocular vision can be reconstructed. This intervention, therefore, seems to be a satisfactory alternative.

  6. Multifocal motor neuropathy: a review of pathogenesis, diagnosis, and treatment

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    Lawson VH

    2014-04-01

    Full Text Available Victoria H Lawson,1 W David Arnold1,2 1Division of Neuromuscular Disorders, Department of Neurology, 2Department of Physical Medicine and Rehabilitation, Wexner Medical Center at The Ohio State University, Columbus, Ohio, USA Abstract: Multifocal motor neuropathy (MMN is an uncommon, purely motor neuropathy associated with asymmetric deficits with predilection for upper limb involvement. Even in the early descriptions of MMN, the associations of anti-GM1 antibodies and robust response to immunomodulatory treatment were recognized. These features highlight the likelihood of an underlying autoimmune etiology of MMN. The clinical presentation of MMN can closely mimic several neurological conditions including those with more malignant prognoses such as motor neuron disease. Therefore early and rapid recognition of MMN is critical. Serological evidence of anti GM-1 antibodies and electrodiagnostic findings of conduction block are helpful diagnostic clues for MMN. Importantly, these diagnostic features are not universally present, and patients lacking these characteristic findings can demonstrate similar robust response to immunodulatory treatment. In the current review, recent research in the areas of diagnosis, pathogenesis, and treatment of MMN and needs for the future are discussed. The characteristic findings of MMN and treatment implications are reviewed and contrasted with other mimicking disorders. Keywords: autoimmune, conduction block, electrodiagnosis, motor neuron, nerve, inflammatory

  7. Multifocal Electroretinography after High Dose Chloroquine Therapy for Malaria

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    Aline Correa de Carvalho

    2013-01-01

    Full Text Available Purpose: To investigate changes in multifocal electroretinography (mfERG parameters associated with high dose chloroquine therapy for treatment of malaria in the Amazonia region of Brazil. Methods: Forty-eight subjects who had received chloroquine treatment for single or multiple malaria infections with a cumulative dose ranging from 1,050 to 27,000mg were included. The control group consisted of 37 healthy aged-matched subjects. Data was collected on amplitude and implicit time of the N1, P1 and N2 waves in the central macular hexagon (R1 and in five concentric rings at different retinal eccentricities (R2-R6. Results: No significant difference was observed in any mfERG parameter between chloroquine treated patients and control subjects. A comparison with previous data obtained from patients with rheumatologic disorders in the same region of Brazil who had received larger cumulative doses of chloroquine and had displayed mfERG changes, indicated that retinal toxicity seems to be dependent on cumulative dose. Conclusion: Lack of mfERG changes in the current study suggests that intensive high dose chloroquine therapy for treatment of malaria is not associated with retinal toxicity.

  8. Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma

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    Susmita Kundu

    2015-11-01

    Full Text Available A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor.

  9. Cutaneous Pythiosis in calves: An epidemiologic, pathologic, serologic and molecular characterization

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    Guilherme Konradt

    2016-12-01

    Full Text Available This study reports the epidemiological, pathological and mycological findings of cutaneous pythiosis in cattle in southern Brazil. 23 calves, that were kept next to a river with extensive marshy regions, presented ulcerated cutaneous lesions in thoracic and pelvic limbs, sometimes extending to the ventral thoracic region. Histopathological examination revealed multifocal pyogranulomas in the superficial and deep dermis. The Grocott-Methenamine silver, immunohistochemistry anti-Pythium insidiosum, ELISA serology and molecular characterization demonstrated the agent P. insidiosum in these cases.

  10. Comparison of visual performance of multifocal intraocular lenses with same material monofocal intraocular lenses.

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    Tomofusa Yamauchi

    Full Text Available PURPOSE: To compare the visual performance of multifocal intraocular lenses (IOLs and monofocal IOLs made of the same material. METHODS: The subjects included patients implanted with either Tecnis® monofocal IOLs (ZA9003 or ZCB00 or Tecnis® multifocal IOLs (ZMA00 or ZMB00 bilaterally. We conducted a retrospective study comparing the two types of IOLs. The multifocal group included 46 patients who were implanted with Tecnis® multifocal IOLs bilaterally. The monofocal group was an age- and sex-matched control group, and included 85 patients who were implanted with Tecnis® monofocal IOLs bilaterally. Lens opacity grading, the radius of corneal curvature, corneal astigmatism, axial length and the refractive status were measured preoperatively. Pupil size, ocular aberrometry, distance, intermediate and near visual acuity, contrast sensitivity with and without glare and the responses to a quality-of-vision questionnaire were evaluated pre- and postoperatively. RESULTS: The uncorrected near visual acuity was significantly better in the multifocal group, whereas both the corrected intermediate and near visual acuity were better in the monofocal group. Contrast sensitivity (with and without glare was significantly better in the monofocal group. The rate of spectacle dependency was significantly lower in the multifocal group. There were no significant differences between the two groups regarding most items of the postoperative quality-of-vision questionnaire (VFQ-25, with the exception that the patients in the monofocal group reported fewer problems with nighttime driving. CONCLUSIONS: The multifocal IOLs used in this study reduced spectacle dependency more so than monofocal IOLs and did not compromise the subjective visual function, with the exception of nighttime driving.

  11. Epidemiological causality.

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    Morabia, Alfredo

    2005-01-01

    Epidemiological methods, which combine population thinking and group comparisons, can primarily identify causes of disease in populations. There is therefore a tension between our intuitive notion of a cause, which we want to be deterministic and invariant at the individual level, and the epidemiological notion of causes, which are invariant only at the population level. Epidemiologists have given heretofore a pragmatic solution to this tension. Causal inference in epidemiology consists in checking the logical coherence of a causality statement and determining whether what has been found grossly contradicts what we think we already know: how strong is the association? Is there a dose-response relationship? Does the cause precede the effect? Is the effect biologically plausible? Etc. This approach to causal inference can be traced back to the English philosophers David Hume and John Stuart Mill. On the other hand, the mode of establishing causality, devised by Jakob Henle and Robert Koch, which has been fruitful in bacteriology, requires that in every instance the effect invariably follows the cause (e.g., inoculation of Koch bacillus and tuberculosis). This is incompatible with epidemiological causality which has to deal with probabilistic effects (e.g., smoking and lung cancer), and is therefore invariant only for the population.

  12. Inner retinal contributions to the multifocal electroretinogram: patients with Leber's hereditary optic neuropathy (LHON). Multifocal ERG in patients with LHON.

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    Kurtenbach, Anne; Leo-Kottler, Beate; Zrenner, Eberhart

    2004-05-01

    In this study we examine the multifocal electroretinogram (mfERG) recorded from patients suffering from Leber's hereditary optic neuropathy (LHON), a degeneration of the ganglion cell and nerve fibre layers of the retina. We compared the mfERGs recorded from 11 patients with LHON, to those from 11 control subjects. The pattern ERG (PERG) was additionally performed with 9 of the patients. MfERGs were recorded and analysed using the VERIS 3.01 system with a stimulus of 103 equal-sized hexagons. For analysis, hexagons were grouped according to distance from the optic nerve head (ONH) and according to distance from the fovea. Two significant differences were found between the waveforms of the two groups: In the first order kernel, the control group showed a component around 34 ms that decreased with distance from the ONH. This component was reduced in the LHON group of subjects. In the second order (first slice) kernel, the patient group was missing features that decrease with distance from the fovea in the control group. PERG amplitudes showed a significant correlation with the amplitude of the second order mfERG kernel. The results show that the damage to ganglion cells and nerve fibres caused by LHON can be detected in mfERG recordings and indicate that activity from the inner retina can contribute significantly to first and second order waveforms.

  13. Comparison of outcomes with multifocal intraocular lenses: a meta-analysis

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    Béatrice Cochener

    2011-01-01

    Full Text Available Béatrice Cochener1, Antoine Lafuma2, Babak Khoshnood2, Laurène Courouve2, Gilles Berdeaux3,41Centre Hospitalier Universitaire Brest, Brest, France; 2Cemka Eval, Bourg la Reine, France; 3Alcon France, Rueil-Malmaison, France; 4Conservatoire National des Arts et Métiers, Paris, FrancePurpose: To compare the clinical outcome of different multifocal intraocular lenses (IOLs based on information reported in the international literature.Methods: All comparative clinical trials that involved implanting at least one multifocal IOL in patients with cataract or presbyopia were extracted from the literature. Clinical outcomes included uncorrected near visual acuity, uncorrected distance visual acuity, visual acuity, spectacle independence, and halos. Random effects meta-analyses were conducted to compare outcomes for the different IOL types.Results: Twenty papers were identified describing 11 monofocal IOLs and 35 multifocal IOLs (19 diffractive, including 12 ReSTOR®, 14 refractive, and two accommodative patient cohorts. Multifocal and monofocal uncorrected distance visual acuity was 0.165 (0.090–0.240 and 0.093 (0.088–0.098, respectively. Compared with monofocal IOLs, multifocal IOLs produced better uncorrected near visual acuity (0.470 [0.322–0.618] versus 0.141 [0.131–0.152]; P < 0.0001, resulting in higher spectacle independence (incidence rate ratio [IRR] 3.62 [2.90–4.52]; P < 0.0001. Compared with refractive multifocal IOLs, diffractive multifocal IOLs produced a similar uncorrected distance visual acuity (0.105 [0.098–0.111] versus 0.085 [0.029–0.140]; P ≤ 0.78, not significant and better uncorrected near visual acuity (0.217 [0.118–0.317] versus 0.082 [0.067–0.098]; P < 0.0001 resulting in higher spectacle independence (IRR 1.75 [1.24–2.48]; P < 0.001. Compared with other multifocal IOLs, ReSTOR produced a better uncorrected distance visual acuity (0.067 [0.059–0.076] versus 0.093 [0.088–0.098]; P < 0.0001 and better

  14. Association between multifocal soft contact lens decentration and visual performance

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    Fedtke C

    2016-06-01

    Full Text Available Cathleen Fedtke,1 Klaus Ehrmann,1,2 Varghese Thomas,1 Ravi C Bakaraju1,2 1The Brien Holden Vision Institute, Clinical Trial Research Centre, 2School of Optometry and Vision Science, The University of New South Wales, Sydney, NSW, Australia Purpose: The aim of this study was to assess the association between decentration of several commercial multifocal soft contact lenses (MFCLs and various objective and subjective visual performance variables in presbyopic and non-presbyopic participants. Materials and methods: All presbyopic (age >40 years, near add ≥+1.25 D and non-presbyopic (age ≥18 years, no near add requirements, spherical equivalent ≤-0.50 D participants were each fitted bilaterally with six and two MFCLs (test lens, respectively, and with one single vision lens (control lens. Lens decentration, ie, the x- and y-differences between the contact lens and pupil centers, was objectively determined. Third-order aberrations were measured and compared. Visual performance (high- and low-contrast acuities and several subjective variables was analyzed for any associations (Pearson’s correlation, r with MFCL decentration. Results: A total of 17 presbyopic (55.1±6.9 years and eight non-presbyopic (31.0±3.3 years participants completed the study. All lenses displayed a temporal–inferior decentration (x=-0.36±0.29 mm, y=-0.28±0.28 mm, mean ± SD. Compared to the control, a significant inferior decentration was found for the Proclear® MFCL Near lens in both groups (ypresbyopic =-0.26 mm, ynon-presbyopic =-0.70 mm and for the Proclear® MFCL Distance lens in the non-presbyopic group (ynon-presbyopic =-0.69 mm. In both groups, lens-induced vertical coma (C(3, -1 was, by at least tenfold, significantly more positive for the Proclear® MFCL Distance lens and significantly more negative for the Proclear® MFCL Near lens. In the presbyopic group, the correlation of total MFCL decentration with vision variables was weak (r<|0

  15. Sensitivity of imaging for multifocal-multicentric breast carcinoma

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    Viale Giuseppe

    2008-09-01

    Full Text Available Abstract Background This retrospective study aims to determine: 1 the sensitivity of preoperative mammography (Mx and ultrasound (US, and re-reviewed Mx to detect multifocal multicentric breast carcinoma (MMBC, defined by pathology on surgical specimens, and 2 to analyze the characteristics of both detected and undetected foci on Mx and US. Methods Three experienced breast radiologists re-reviewed, independently, digital mammography of 97 women with MMBC pathologically diagnosed on surgical specimens. The radiologists were informed of all neoplastic foci, and blinded to the original mammograms and US reports. With regards to Mx, they considered the breast density, number of foci, the Mx characteristics of the lesions and their BI-RADS classification. For US, they considered size of the lesions, BI-RADS classification and US pattern and lesion characteristics. According to the histological size, the lesions were classified as: index cancer, 2nd lesion, 3rd lesion, and 4th lesion. Any pathologically identified malignant foci not previously described in the original imaging reports, were defined as undetected or missed lesions. Sensitivity was calculated for Mx, US and re-reviewed Mx for detecting the presence of the index cancer as well as additional satellite lesions. Results Pathological examination revealed 13 multifocal and 84 multicentric cancers with a total of 303 malignant foci (282 invasive and 21 non invasive. Original Mx and US reports had an overall sensitivity of 45.5% and 52.9%, respectively. Mx detected 83/97 index cancers with a sensitivity of 85.6%. The number of lesions undetected by original Mx was 165/303. The Mx pattern of breasts with undetected lesions were: fatty in 3 (1.8%; scattered fibroglandular density in 40 (24.3%, heterogeneously dense in 91 (55.1% and dense in 31 (18.8% cases. In breasts with an almost entirely fatty pattern, Mx sensitivity was 100%, while in fibroglandular or dense pattern it was reduced to 45

  16. Genome-Wide Investigation of Multifocal and Unifocal Prostate Cancer—Are They Genetically Different?

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    Ibeawuchi, Chinyere; Schmidt, Hartmut; Voss, Reinhard; Titze, Ulf; Abbas, Mahmoud; Neumann, Joerg; Eltze, Elke; Hoogland, Agnes Marije; Jenster, Guido; Brandt, Burkhard; Semjonow, Axel

    2013-01-01

    Prostate cancer is widely observed to be biologically heterogeneous. Its heterogeneity is manifested histologically as multifocal prostate cancer, which is observed more frequently than unifocal prostate cancer. The clinical and prognostic significance of either focal cancer type is not fully established. To investigate prostate cancer heterogeneity, the genetic profiles of multifocal and unifocal prostate cancers were compared. Here, we report observations deduced from tumor-tumor comparison of copy number alteration data of both focal categories. Forty-one fresh frozen prostate cancer foci from 14 multifocal prostate cancers and eight unifocal prostate cancers were subjected to copy number variation analysis with the Affymetrix SNP 6.0 microarray tool. With the investigated cases, tumors obtained from a single prostate exhibited different genetic profiles of variable degrees. Further comparison identified no distinct genetic pattern or signatures specific to multifocal or unifocal prostate cancer. Our findings suggest that samples obtained from multiple sites of a single unifocal prostate cancer show as much genetic heterogeneity and variability as separate tumors obtained from a single multifocal prostate cancer. PMID:23736690

  17. Chronic Recurrent Multifocal Osteomyelitis: A Case Report with Atypical Presentation.

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    Figueiredo, Miguel Pádua; Pato, Marco; Amaral, Fernando

    2017-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory condition. The clinical picture consists of sterile osteomyelitis, typically with multiple-site lesions in the metaphysis of long bones and not uncommonly, symmetrical bone involvement. It is a poorly understood entity, whose prognosis, etiology and ideal treatment are still controversial. The authors report a case of unifocal presentation with an atypical location. A previously healthy 12-year-old Caucasian girl came to our institution due to progressive pain on her left thigh for the previous 3 months. The initial X-ray showed a permeative, diaphyseal lesion of her left femur, with marked periosteal reaction. The differential initially included Ewing's sarcoma, osteosarcoma, subacute osteomyelitis, and Langerhans cell histiocytosis. Needle and open biopsies demonstrated the presence of chronic inflammatory infiltrate, with fibrosis, but no signs of neoplastic disease. Serologic and microbiological studies failed to demonstrate an infectious etiology. The patient was treated with nonsteroid anti-inflammatories, corticosteroids, and bisphosphonates for 6 months. Although no antibiotics were employed, the patient showed clinical and radiological improvement, at 18-month follow-up. CRMO is a rare condition, and the absence of specific features constitutes a diagnostic challenge. A high level of suspicion is paramount to avoid unnecessary biopsies and repeated antibiotic regimens. Unifocal presentation of this disease, atypical locations, and absence of recurrence have all been previously reported, with the evidence pointing to a shared etiological process with no distinction being made between these variants. For this reason, the authors believe that the term "nonbacterial osteomyelitis" might be a more all-embracing designation.

  18. Ultrasound screening of multifocal atherosclerosis: markers for coronary heart disease

    Institute of Scientific and Technical Information of China (English)

    Lachezar Grozdinski; Mario Stankev; Alexander Doganov

    2009-01-01

    Background and Objective The frequency of multifocal atherosclerosis (MFA) in patients with coronary heart disease (CHD) has not been thoroughly studied. The purpose of our study was to perform ultrasound screening for MFA in patients with coronary atherosclerosis and make evaluation of the sensitivity and significance of different atherosclerosis markers. Methods Using Color Dupplex Ultrasound (CDU), we studied 32 clinically healthy persons and 87 patients of the city of B with clinical data for CHD where we also performed coronarography. Results In patients with coronary atherosclerosis we found high frequency of carotid atherosclerosis (93%) and peripheral artery disease (PAD) (81%). We established verifiable thickening of the intima-media (IMT) of the common carotid artery (CCA) and common femoral artery (CFA) in patients with CHD. There is a correlation between the frequency of carotid and femoral stenoses and CHD proven by coronarography. Patients with CHD had a high relative risk to develop carotid (RR = 5) and peripheral atherosclerosis (RR=3.5) and high frequency of asymptomatic stenoses and thromboses of the internal carotid artery (86.9%) and femoral artery (78.3%), as well as aneurisms of the abdominal aorta (8.1%). Markers for CAD with high sensitivity were the atherosclerotic plaques of ICA (0.93) and CFA (0.81) as well as IMT of the CFA (0.84). Conclusions MFA are common among patients with CHD. Ultrasound diagnosis is the method of choice for simultaneous non-invasive screening of carotid, peripheral and MFA and provides sensitive markers for coronary atherosclerosis. The most sensitive and specific markers for CHD are the combination of the IMT and atherosclerotic plaques of CCA, ICA and CFA (100% sensitivity and 0.92 specificity).

  19. Effect of eccentricity on pattern-pulse multifocal VEP.

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    Klistorner, Alexander I; Graham, Stuart L

    2005-01-01

    The sparse pattern-pulse stimulation has been suggested to produce better cortical evoked responses compared to pattern reversal stimulation. This study examines varying pattern-pulse states and the effect of eccentricity of the stimulated visual field on the response amplitude and latency. The multifocal visual evoked potential (mfVEP) was recorded using Accumap. 58 close-packed checkerboard segments in a dartboard configuration were used. The best configuration for pattern-pulse stimulation was determined. This optimal stimulus condition was then compared to pattern-reversal stimulation at different eccentricities of visual field.in terms of latency and signal/noise ratio (SNR) of mfVEP amplitude. The maximal response was seen when each element "1" of the binary sequence was represented by two "pattern on" frames followed by two "pattern off" frames while each element "0" of the binary sequence is represented by four "pattern off" frames. There was a significant overall increase of SNR using this pattern-pulse stimulating mode (SNR=15.5+/-3.8) compared with pattern-reversal stimulation (SNR=12.4+/-2.6). However, this was strongly dependant on eccentricity. In rings 1, 2 and 3 SNR improved by 48%, 43% and 26% respectively with ring 4 the effect was marginal and ring 5 was not significantly different. There was also a significant delay (10.1+/-5.3 msec) of the mfVEP response in pattern-pulse stimulation compared to pattern-reversal. The pattern-pulse method offers some advantages for achieving larger mfVEP signals from the central visual field. However, the more peripheral field where it is the most difficult to obtain signals, does not show any benefit.

  20. Reproducibility of multifocal VEP latency using different stimulus presentations.

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    Sriram, Prema; Klistorner, Alexander; Arvind, Hemamalini; Graham, Stuart L

    2012-08-01

    The aims of the article were to study the reproducibility of latency of multifocal visual evoked potential (mfVEP) recorded using different stimulus presentations and to identify the peak with least variability. Ten normal subjects, aged between 22 and 52 years (mean age 32 ± 8.37 years), participated in the study. All subjects underwent mfVEP testing with pattern reversal and pattern pulse stimulus presentations. The stimulus subtends 26° from fixation and includes 24 segments. Only the vertical channel was recorded on all subjects. Testing was repeated after 1-2 weeks. Only the right eye of all subjects was analysed. Segments with low signal-to-noise ratios (SNR < 1.5) were excluded from analysis. The latencies were analysed to confirm values from the same peak for the two tests. The latency values were then analysed for the start of the response, the first peak and the second peak. The waveforms were reproducible throughout the field. Reproducibility of latency at the "start of the response" was significantly lesser than the first and the second peaks studied, while the reproducibility of latency at the first peak was not statistically different from the second peak for either pattern reversal or pattern pulse stimulation. The latency values were not different between the first and the second sessions for either pattern reversal or pattern pulse stimulation for any of the peaks. The pattern reversal stimulus presentation produced less variability in latency. The first peak is the most reproducible among the three measures in both the stimulus presentation.

  1. Effect of fixation tasks on multifocal visual evoked potentials.

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    Martins, Alessandra; Klistorner, Alexander; Graham, Stuart; Billson, Frank

    2005-10-01

    This study investigated the effects of cognitive influence on the multifocal visual evoked potential (mVEP) at different levels of eccentricity. Three different foveal fixation conditions were utilized involving varying levels of task complexity. A more complex visual fixation task has been known to suppress peripheral signals in subjective testing. Twenty normal subjects had monocular mVEPs recorded using the AccuMap objective perimeter. This allowed simultaneous stimulation of 58 segments of the visual field to an eccentricity of 24 degrees. The mVEP was recorded using three different fixation conditions in random order. During task 1 the subject passively viewed the central fixation area. For task 2 alternating numbers were displayed within the fixation area; the subject on viewing the number '3' in the central fixation area indicated recognition by pressing a button. Throughout task 3, numbers were displayed as in task 2. The subject had the cognitive task of summating all the numbers. Analysis revealed that the increased attention and concentration demanded by tasks 2 and 3 in comparison with task 1 resulted in significantly enhanced central amplitudes of 9.41% (Mann-Whitney P = 0.0002) and 13.45% (P = 0.0002), respectively. These amplitudes became reduced in the periphery and approached those of task 1, resulting in no significant difference between the three tasks. Latencies demonstrated no significant difference between each task nor at any eccentricity (P > 0.05). As the complexity of each task increased the amount of alpha rhythm was significantly reduced. Our findings indicate that task 1 required a minimal demand of cognition and was associated with the greatest amount of alpha rhythm. It was also the most difficult to perform because of loss of interest. The other two tasks required a greater demand of higher order cognitive skills resulting in significantly enhanced amplitudes centrally and the attenuation of alpha rhythm. Therefore, amplitudes are

  2. Conservative surgery for multifocal/multicentric breast cancer.

    Science.gov (United States)

    Nijenhuis, Matthijs V; Rutgers, Emiel J Th

    2015-11-01

    Multifocal (MF) and multicentric (MC) breast cancer is regularly considered a relative contraindication for breast-conserving therapy (BCT). There are two reasons for this wide spread notion: However, we concur that if optimal 'cytoreductive surgery' is achieved this will result in good local control (i.e. in-breast relapse breast irradiation and systemic treatments as indicated by primary cancer biology. Careful planning and adaptive application of oncoplastic techniques will result in an optimal cosmetic results. The meticulous work of Roland Holland and coworkers(1) in the early 1980's on whole breast specimen showed invasive foci at more then 2 cm distance from the invasive primary cancer in more then 40% of specimen. Although multiple tumor foci may occur in up to 60% of mastectomy specimens, equivalent survival outcomes were observed in prospective trials comparing BCT and mastectomy for clinically unifocal lesions, suggesting that the majority of these foci are not, or do not become, biologically relevant or clinically significant with appropriate treatment. As diagnostic tools advance, MF and MC tumors are more commonly diagnosed. Cancers that previously would have been classified as unifocal now can be detected as MF or MC. In addition, locoregional treatment modalities have improved significantly over the past decade. More recent studies reflect these advances in diagnosis and treatment. Studies evaluated staging MRI showed that up to 19% of woman with diagnosed breast cancer harbor a second malignant ipsilateral lesion. These findings should only have consequences when additional lesions are proven cancer. Multiple enhancing lesions on MRI are in itself not an indication for a mastectomy. The Z0011 trial and the AMAROS trial demonstrated a similar phenomenon for axillary treatment; less surgery does not necessarily lead to inferior local control or survival outcomes. Recent studies supplement the growing evidence that treatment of patients with MF

  3. Progressive multifocal leucoencephalopathy in HIV/AIDS: Observational study from a tertiary care centre in northern India

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    S K Sharma

    2013-01-01

    Full Text Available Background & objectives: Progressive multifocal leucoencephalopathy (PML is seen mostly in advanced human immunodeficiency virus (HIV infection. Little is known about the epidemiology and disease course of these patients from India. This study was aimed to determine the frequency of PML in patients with HIV/AIDS, and the clinical features and survival of these patients. Methods: The charts of HIV/AIDS patients with PML seen over a period of five years (2006-2011 at the Antiretroviral treatment (ART centre at a tertiary care centre in New Delhi, India, were retrospectively reviewed. Results: Of 1465 patients with HIV/AIDS, 18 (1.2% were diagnosed with PML; four were laboratory confirmed and 14 had consistent clinical and radiological features. PML was the initial presentation of HIV infection in 10 (56% patients, and 16 (89% patients had CD4 count less than 200/μl. Insidious onset focal limb weakness (78% and visual disturbance (28% were common symptoms. Magnetic resonance imaging (MRI of the brain revealed characteristic white matter lesions in all the patients. The estimated median survival was 7.6 months (95% CI, 0-20 months. Interpretation & conclusions: Our results show that the patients present late to access treatment with advanced immunosuppression at presentation. PML is associated with high morbidity and mortality despite institution of highly active antiretroviral therapy (HAART. There is a need to address the lacuna in diagnostic and management services for these patients in India.

  4. A mutation in the canine BHD gene is associated with hereditary multifocal renal cystadenocarcinoma and nodular dermatofibrosis in the German Shepherd dog

    NARCIS (Netherlands)

    Lingaas, F; Comstock, KE; Kirkness, EF; Sorensen, A; Aarskaug, T; Hitte, C; Nickerson, ML; Moe, L; Schmidt, LS; Thomas, R; Breen, M; Galibert, F; Zbar, B; Ostrander, EA

    2003-01-01

    Hereditary multifocal renal cystadenocarcinoma and nodular dermatofibrosis (RCND) is a naturally occurring canine kidney cancer syndrome that was originally described in German Shepherd dogs. The disease is characterized by bilateral, multifocal tumors in the kidneys, uterine leiomyomas and nodules

  5. Multifocal tenosynovial giant cell tumors in a child with Noonan syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Meyers, Arthur B. [Children' s Hospital of Wisconsin, Department of Radiology, Milwaukee, WI (United States); Nemours Children' s Health System/Nemours Children' s Hospital, Department of Radiology, Orlando, FL (United States); Awomolo, Agboola O. [Children' s Hospital of Wisconsin, Department of Radiology, Milwaukee, WI (United States); Szabo, Sara [Medical College of Wisconsin and Children' s Hospital of Wisconsin, Department of Pathology, Milwaukee, WI (United States); Cincinnati Children' s Hospital Medical Center, Division of Pathology and Laboratory Medicine, Cincinnati, OH (United States)

    2017-03-15

    Noonan syndrome is a genetic disorder with variable expression of distinctive facial features, webbed neck, chest deformity, short stature, cryptorchidism and congenital heart disease. The association of Noonan syndrome and giant cell granulomas of the mandible is widely reported. However, Noonan syndrome may also be associated with single or multifocal tenosynovial giant cell tumors, also referred to as pigmented villonodular synovitis. We report a child with Noonan syndrome, giant cell granulomas of the mandible and synovial and tenosynovial giant cell tumors involving multiple joints and tendon sheaths who was initially misdiagnosed with juvenile idiopathic arthritis. It is important for radiologists to be aware of the association of Noonan syndrome and multifocal giant cell lesions, which can range from the more commonly described giant cell granulomas of the mandible to isolated or multifocal intra- or extra-articular tenosynovial giant cell tumors or a combination of all of these lesions. (orig.)

  6. Multifocal visual evoked potential latency analysis: predicting progression to multiple sclerosis.

    Science.gov (United States)

    Fraser, Clare; Klistorner, Alexander; Graham, Stuart; Garrick, Raymond; Billson, Francis; Grigg, John

    2006-06-01

    To monitor the difference in conversion rates to multiple sclerosis (MS) in 46 patients with optic neuritis between patients with multifocal visual evoked potential latency delay and those with normal latency. Prospective case series. Metropolitan neuro-ophthalmology clinic. Forty-six patients with optic neuritis who did not have a diagnosis of MS on enrollment in the study. Conversion to MS according to the McDonald criteria. Analysis revealed that only 22 subjects had multifocal visual evoked potential latency delay. Over 1 year, 36.4% of patients with optic neuritis with latency delays progressed clinically to MS compared with 0% of those with normal latencies (P = .03, chi2). This may indicate that multifocal visual evoked potential latency delay can assist in predicting progression to future MS.

  7. Sporadic bilateral synchronous multicentric papillary renal cell carcinoma masquerading as bilateral multifocal pyelonephritis.

    Science.gov (United States)

    Karthikeyan, V S; Dorairajan, L N; Kumar, S; Vijayakumar, A R; Ramesh, A; Ganesh Rajesh, N; Halanaik, D; Gupta, S

    2014-07-01

    Pyelonephritis is defined as an inflammation of the kidney and renal pelvis. The diagnosis is usually clinical. Acute multifocal bacterial nephritis is a rare form of pyelonephritis that is more severe and sepsis is more common. We report a patient who presented with fever and right-sided abdominal pain associated with right flank tenderness, suggesting right acute pyelonephritis. Bilateral multifocal pyelonephritis was diagnosed on ultrasonography, radionuclide renal scintigraphy and computed tomography. However, owing to non-resolution of symptoms, a biopsy was performed, which showed bilateral papillary renal cell carcinoma (PRCC). PRCC is known to exhibit multicentricity. To our knowledge, a case of bilateral multicentric PRCC masquerading as bilateral multifocal pyelonephritis has not been reported in the English literature. This case highlights the need to be vigilant while treating patients with focal lesions of the kidney as an inflammatory condition lest a malignancy should be missed.

  8. Presumed toxoplasmic central retinal artery occlusion and multifocal retinitis with perivascular sheathing

    Directory of Open Access Journals (Sweden)

    Arai H

    2014-04-01

    Full Text Available Haruka Arai,1 Tsutomu Sakai,1 Kiichiro Okano,1 Ranko Aoyagi,1 Ayano Imai,2 Hiroshi Takase,2 Manabu Mochizuki,2 Hiroshi Tsuneoka11Department of Ophthalmology, Jikei University School of Medicine, Tokyo, Japan; 2Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo, JapanAbstract: Central retinal artery occlusion (CRAO and multifocal retinitis with perivascular sheathing are rare in ocular toxoplasmosis. We report a case of toxoplasmic CRAO and multifocal retinitis with perivascular sheathing. A healthy 83-year-old male developed left panuveitis. Funduscopic examination of the left eye showed a swollen optic disc and sheathing of the retinal artery with a dense vitreous haze and a white retinal lesion. Serum anti-toxoplasma antibodies were positive in a latex agglutination assay. Vitrectomy was performed to improve visualization of the retinal lesions and for examination of causative microorganisms. A postoperative fundus examination revealed CRAO with optic disc involvement and multifocal retinitis with perivascular sheathing. Qualitative multiplex polymerase chain reaction detected the Toxoplasma gondii B1 gene in ocular fluid from both the aqueous and vitreous humor. The presumed diagnosis of ocular toxoplasmosis was made and treatment was started with prednisone and acetylspiramycin with subsequent improvement. Two months later, the patient developed active retinochoroiditis in the left eye. After 6 weeks of anti-toxoplasma therapy, the disease involuted. Retinal vascular occlusions and multifocal retinitis with perivascular sheathing are rare in toxoplasmosis. This is the first case report of toxoplasmic CRAO and multifocal retinitis with perivascular sheathing. The diagnosis of ocular toxoplasmosis should be considered in patients with retinal artery occlusions and multifocal retinitis with perivascular sheathing associated with inflammation.Keywords: ocular toxoplasmosis, toxoplasma retinochoroiditis

  9. Fatal posterior revesible leukoencephalopathy syndrome associated coma induced by bevacizumab in metastatic colorectal cancer and review of literature.

    Science.gov (United States)

    Eryılmaz, Melek Karakurt; Mutlu, Hasan; Salim, Derya Kıvrak; Musri, Fatma Yalçın; Coşkun, Hasan Şenol

    2016-12-01

    Posterior reversible leukoencephalopathy syndrome (PRES) is a syndrome characterized by headache, hypertension, confusion, visual disturbance, and seizures accompanied by subcortical vasogenic edema, predominantly involving the parietal and occipital lobes. The syndrome is usually described in malignant hypertension, eclampsia, renal failure, immunosuppressive, and cytotoxic chemotherapies. Bevacizumab, a monoclonal antibody that binds to the vascular endothelial growth factor (VEGF) has been linked to PRES. We carried out review of reports documenting the occurrence of PRES in patients receiving bevacizumab. This literature review was conducted by utilizing PubMed Database. If early diagnosed, PRES is reversible. We present a case of fatal PRES-associated coma induced by bevacizumab in metastatic colorectal cancer. © The Author(s) 2015.

  10. Multifocal fibrosclerosis: a new case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Oguz, Kader Karli; Oguz, Oguzhan; Cila, Aysenur; Oto, Aytekin [Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara (Turkey); Kiratli, Hayyam [Department of Ophthalmology, Faculty of Medicine, Hacettepe University, Ankara (Turkey); Gokoz, Aytac [Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara (Turkey)

    2002-05-01

    A case of multifocal fibrosclerosis is presented with MR images. Bilateral sclerosing orbital pseudotumor invading cavernous sinuses were the presenting disorder. Magnetic resonance imaging showed involvement of paranasal sinuses bilaterally and multiple supratentorial dural masses. Retroperitoneal fibrosis was associated with the condition. Multifocal involvement should be considered in patients with sclerosing orbital pseudotumor and an imaging approach should be performed on the appropriate clinical condition to document possible coexistence of other disorders including retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, and Riedel's thyroiditis (orig.)

  11. Multifocal gastrointestinal stromal tumor (GIST) of the stomach in an 11-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jin [University of North Carolina Hospitals, Department of Radiology, Chapel Hill, NC (United States); Rubinas, Tara C. [University of North Carolina Hospitals, Department of Pathology, Chapel Hill, NC (United States); Fordham, Lynn A. [University of North Carolina School of Medicine, Department of Radiology, Chapel Hill, NC (United States); Phillips, J.D. [University of North Carolina School of Medicine, Department of Surgery, Division of Pediatric Surgery, Chapel Hill, NC (United States)

    2006-11-15

    A previously healthy 11-year-old girl presented with an 8-month history of anemia and left upper quadrant abdominal pain. US examination demonstrated a 9-cm cystic mass with a fluid-fluid level in the left upper quadrant with unclear organ of origin. Abdominal MR imaging demonstrated a complex cystic mass, likely arising from the stomach. Additional T2 hyperintense submucosal lesions were identified in the gastric wall. Surgical excision confirmed the diagnosis of multifocal gastric gastrointestinal stromal tumor (GIST). MR imaging was helpful in suggesting a gastric origin of the primary mass and in demonstrating multifocal disease within the stomach. (orig.)

  12. Multifocal acquired demyelinating sensory and motor neuropathy presenting as a peripheral nerve tumor.

    Science.gov (United States)

    Allen, David C; Smallman, Clare A; Mills, Kerry R

    2006-09-01

    A man with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), or Lewis-Sumner syndrome, presented with a progressive left lumbosacral plexus lesion resembling a neurofibroma. After 7 years he developed a left ulnar nerve lesion with conduction block in its upper segment. Treatment with intravenous immunoglobulin improved the symptoms and signs of both lesions. We conclude that inflammatory neuropathy must be considered in the differential diagnosis of peripheral nerve tumors, and that unifocal lesions may precede multifocal involvement in MADSAM by several years. In addition, we discuss the clinical features in 9 patients attending a specialist peripheral nerve clinic and review the literature.

  13. Inadvertent implantation of a reversed-optic Tecnis ZM900 multifocal intraocular lens: case report Implante invertido da lente intraocular Tecnis ZM900 multifocal: relato de caso

    Directory of Open Access Journals (Sweden)

    Wilson Takashi Hida

    2012-12-01

    Full Text Available A 51-year-old woman with age-related cataract had an uneventful phacoemulsification and a Tecnis ZM900 multifocal intraocular lens (IOL implantation in both eyes. During IOL implantation in the left eye, the optic was unintentionally reversed in the bag and left that way. The refraction surprise was not significant and six months postoperatively the corrected distance visual acuity in both eyes was 20/20 and neither complained of visual discomfort. In conclusion, we found that a reversed-optic Tecnis multifocal IOL in the present case resulted in good final visual acuity without significant differences in aberrations compared to the other eye, and a conservative management can be taken as a safe option.Paciente de 51 anos, sexo feminino, apresentando catarata foi submetida a cirurgia de facoemulsificação com implante de lente intraocular (LIO multifocal Tecnis ZM900 em ambos os olhos (AO. Durante a implantação da LIO no olho esquerdo, a lente foi inadvertidamente implantada invertida e deixada dessa maneira. Seis meses pós-operatório a surpresa refracional não se mostrou significativa e a acuidade visual corrigida era de 20/20 em AO, sem nenhuma queixa de desconforto visual. Em conclusão, no presente caso, o implante de uma LIO Tecnis multifocal invertida resultou em boa acuidade visual final e sem diferenças significativas na aberrometria em comparação ao olho contralateral, tendo sido possível conduzir o caso de maneira conservadora.

  14. Epidemiology chapter.

    Science.gov (United States)

    Wolfram, J H; Butaev, M K; Duysheev, A; Gabbasova, A R; Khasanov, O S; Kulakov, Yu K; Mkrtchyan, A R; Myrzabekov, A M; Nurgaziev, R Z; Tsirel'son, L E; Willer, R D; Yaraev, R G; Zheludkov, M M

    2010-10-01

    This chapter outlines the epidemiology of brucellosis in the Russian Federation and in five countries bordering Russia. Since the Soviet Union's dissolution, Russia and the newly formed independent republics have failed to maintain policies to control brucellosis and other zoonotic diseases. Many of these republics, due to weak animal control and prevention systems and dangerous food preparation practices, are still burdened with the human cost of brucellosis. The final summary of this section provides an example of the successful transboundary cooperative efforts between Arizona and Mexico, which could be applied to the situation between Russia and the bordering independent republics.

  15. Brief Screening of Vascular Cognitive Impairment in Patients With Cerebral Autosomal-Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Without Dementia

    OpenAIRE

    2016-01-01

    Background and Purpose - Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic form of cerebral small vessel disease leading to early-onset stroke and dementia, with younger patients frequently showing subclinical deficits in cognition. At present, there are no targeted cognitive screening measures for this population. However, the Brief Memory and Executive Test (BMET) and the Montreal Cognitive Assessment (MoCA) have shown utilit...

  16. Late hemodynamic failure following percutaneous transluminal angioplasty for long and multifocal femoropopliteal stenoses

    DEFF Research Database (Denmark)

    Jørgensen, B; Tønnesen, K H; Holstein, P

    1991-01-01

    The outcome of percutaneous transluminal angioplasty (PTA) was evaluated for treatment of 87 limbs with femoropopliteal stenoses, including 55 short stenoses (2.4 +/- 1.6 cm, mean +/- SD), seven single long stenoses (10.4 +/- 2.0 cm), and 25 multifocal stenoses (2.1 +/- 1.6 cm), where two to four...

  17. Effect of Nd:YAG laser capsulotomy on refraction in multifocal apodized diffractive pseudophakia.

    Science.gov (United States)

    Vrijman, Violette; van der Linden, Jan Willem; Nieuwendaal, Carla P; van der Meulen, Ivanka J E; Mourits, Maarten P; Lapid-Gortzak, Ruth

    2012-08-01

    To evaluate the effect on refraction of neodymium:YAG (Nd:YAG) laser posterior capsulotomy for posterior capsule opacification (PCO), and to evaluate the correlation between automated and subjective refraction in multifocal apodized diffractive pseudophakia. A retrospective study of 75 pseudophakic eyes (50 patients) with multifocal apodized diffractive pseudophakia, treated for PCO with Nd:YAG laser posterior capsulotomy, was performed. Pre- and postintervention values of refractive and visual parameters were compared. The outcomes of autorefraction and subjective refraction were also compared. Uncorrected and corrected distance visual acuity improved significantly after Nd:YAG capsulotomy (Prefraction and autorefraction. Spherical equivalent changed significantly in autorefraction (P=.008), but not in subjective refraction. Autorefraction and subjective refraction were highly correlated in spherical equivalent, defocus equivalent, and blurring strength (r(2)>0.59). In approximately 7% of eyes, a change of more than 0.50 diopters in spherical equivalent in subjective refraction occurred. In most cases, Nd:YAG laser capsulotomy in patients with multifocal pseudophakia did not result in a change in refraction. However, 7% of eyes experienced a significant change in subjective refraction. Autorefraction correlated well with subjective refraction in apodized diffractive multifocal IOLs. Copyright 2012, SLACK Incorporated.

  18. Exploring the methods of data analysis in multifocal visual evoked potentials

    DEFF Research Database (Denmark)

    Malmqvist, Lasse; De Santiago, L; Fraser, C

    2016-01-01

    PURPOSE: The multifocal visual evoked potential (mfVEP) provides a topographical assessment of visual function, which has already shown potential for use in patients with glaucoma and multiple sclerosis. However, the variability in mfVEP measurements has limited its broader application. The purpose...

  19. Comparative study of peripheral nerve Mri and ultrasound in multifocal motor neuropathy and amyotrophic lateral sclerosis

    DEFF Research Database (Denmark)

    Jongbloed, Bas A.; Haakma, Wieke; Goedee, H. Stephan

    2017-01-01

    INTRODUCTION: Differentiating multifocal motor neuropathy (MMN) from amyotrophic lateral sclerosis (ALS) is important, as MMN is a difficult, but treatable disorder. METHODS: We studied peripheral nerve imaging techniques in differentiating MMN from ALS by measuring the cross-sectional area (CSA...

  20. Velopharyngeal incompetence in a patient with multifocal eosinophilic granuloma (Hand-Schüller-Christian disease).

    Science.gov (United States)

    Cohn, E R; Garver, K L; Metz, H C; McWilliams, B J; Skolnick, M L; Garrett, W S

    1982-08-01

    The diagnosis and management of velopharyngeal insufficiency in a 16-year-old boy with multifocal eosinophilic granuloma (Hand-Schüller-Christian disease) is described. Medical history, speech and velopharyngeal valving information, and the results of clinical management are presented.

  1. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome.

    Science.gov (United States)

    Saperstein, D S; Amato, A A; Wolfe, G I; Katz, J S; Nations, S P; Jackson, C E; Bryan, W W; Burns, D K; Barohn, R J

    1999-05-01

    We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve conduction studies showing conduction block and other features of demyelination. The clinical, laboratory, and histological features of these patients were contrasted with those of 16 patients with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM1 antibody titers, compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies. Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0). Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical variant. Given their different clinical patterns and responses to treatment, it is important to distinguish between MADSAM neuropathy and MMN.

  2. Unusually high incidence of multifocal epithelial hyperplasia in children of the Nahuatl population of Mexico.

    Science.gov (United States)

    Ledesma-Montes, Constantino; Mendez-Mendoza, Amilcar

    2017-08-09

    Multifocal epithelial hyperplasia is an uncommon disease of the oral mucosa caused by the human papilloma virus. To study the clinical and pathological findings of multifocal epithelial hyperplasia detected during an oral examination of 343 Mexican Nahuatl children from a single primary school in El Paso de Cupilco, Mexico. A thorough oral examination was performed in all children and clinical data (age, gender, location and number of lesions) were documented and analyzed. Multifocal epithelial hyperplasia was diagnosed in 110 of the 343 children (32.3%). The ages of the children varied from 5 to 15 years, and of these, 56.3% were girls. The lesions were asymptomatic, 0.2 to 3.0 cm in diameter, soft, round to oval, smooth surfaced, sessile papulonodules, similar in colour to that of the surrounding mucosa. The lesions were commonly seen on the buccal mucosa and tongue, and most affected children (85%) had less than 5 lesions. Children in the 7 to 10 years age group were most often affected. Human papillomavirus typing was not done owing to a lack of facilities. There is a high incidence of multifocal epithelial hyperplasia in Nahuatl children with a predilection for females.

  3. Genome-wide Investigation of multifocal and unifocal prostate cancer-are they Genetically different?

    NARCIS (Netherlands)

    C. Ibeawuchi (Chinyere); H. Schmidt (Hartmut); R. Voss (Reinhard); A. Titze (Anja); M. Abbas (Mahmoud); J. Neumann (Joerg); E. Eltze (Elke); A.M. Hoogland (Marije); G.W. Jenster (Guido); B. Brandt (Burkhard); A. Semjonow (Axel)

    2013-01-01

    textabstractProstate cancer is widely observed to be biologically heterogeneous. Its heterogeneity is manifested histologically as multifocal prostate cancer, which is observed more frequently than unifocal prostate cancer. The clinical and prognostic significance of either focal cancer type is not

  4. 78 FR 33098 - Prospective Grant of Co-Exclusive Licenses: Multi-Focal Structured Illumination Microscopy...

    Science.gov (United States)

    2013-06-03

    ... HUMAN SERVICES National Institutes of Health Prospective Grant of Co-Exclusive Licenses: Multi-Focal... February 22, 2013 to Andor Technology PLC. having a principle place of business in Belfast, Northern Ireland, and to Vutara, Inc. having a principle place of business in Salt Lake City, Utah. The United...

  5. Clinical developments in Multifocal Intraocular Lens Surgery: The ease of being specs-free

    NARCIS (Netherlands)

    van der Linden, J.W.

    2016-01-01

    In multifocal IOL implantation the aim is to achieve a high level of spectacle independence for all vision distances. Many types of mIOLs have been invented and implemented. Data on the performance of these different types of mIOLs comes from the medical devices industry at the time of launch, and f

  6. Multifocal choroiditis as the first sign of systemic sarcoidosis associated with pembrolizumab

    Directory of Open Access Journals (Sweden)

    Qu-Knafo Lise

    2017-04-01

    Conclusions and importance: Pembrolizumab is an immune checkpoint inhibitor therapy used in the treatment of metastatic melanoma. We report a pembrolizumab-associated sarcoidosis revealed by a panuveitis with multifocal choroiditis. Physicians should be aware of the potential inflammatory and autoimmune disease that may be induced by immunomodulatory therapies.

  7. European Federation of Neurological Societies Peripheral Nerve Society guideline on management of multifocal motor neuropathy

    NARCIS (Netherlands)

    I.N. van Schaik; P. Bouche; I. Illa; J.M. Leger; P. van den Bergh; D.R. Cornblath; E.M.A. Evers; R.D.M. Hadden; R.A.C. Hughes; C.L. Koski; E. Nobile-Orazio; J. Pollard; C. Sommer; P.A. van Doorn

    2006-01-01

    Several diagnostic criteria for multifocal motor neuropathy have been proposed in recent years and a beneficial effect of intravenous immunoglobulin (IVIg) and various other immunomodulatory drugs has been suggested in several trials and uncontrolled studies. The objectives were to prepare consensus

  8. Right ventricular lipomatous mass and biventricular multifocal fat in a young woman: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Bo Rahm; Park, Jae Hyeong; Ahn, Kye Taek; Kim, Song Soo; Jeong, Jin Ok; Choi, Si Wan; Jin, Seon Ah; Lee, Jae Hwan [Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon (Korea, Republic of)

    2015-04-15

    Cardiac lipoma is a rare neoplasm of encapsulated mature adult adipose tissue. It is usually asymptomatic, but it may be related to hemodynamic obstruction depending on its location. We report a typical case of right ventricular lipomatous mass and multifocal fat infiltration of both ventricles, which were detected incidentally in a young woman.

  9. Effect of Nd:YAG laser capsulotomy on refraction in multifocal apodized diffractive pseudophakia.

    NARCIS (Netherlands)

    Vrijman, V.; Linden, J.W.M. van der; Nieuwendaal, C.P.; Meulen, I.J. van der; Mourits, M.P.; Lapid-Gortzak, R.

    2012-01-01

    PURPOSE: To evaluate the effect on refraction of neodymium:YAG (Nd:YAG) laser posterior capsulotomy for posterior capsule opacification (PCO), and to evaluate the correlation between automated and subjective refraction in multifocal apodized diffractive pseudophakia. METHODS: A retrospective study

  10. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy

    NARCIS (Netherlands)

    Cats, E. A.; van der Pol, W. -L.; Piepers, S.; Franssen, H.; Jacobs, B. C.; van den Berg-Vos, R. M.; Kuks, J. B.; van Doorn, P. A.; van Engelen, B. G.; Verschuuren, J. J.; Wokke, J. H.; Veldink, J. H.; van den Berg, L. H.

    2010-01-01

    Objective: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome. Methods: A national cross-sectional descriptive study was performed.

  11. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy

    NARCIS (Netherlands)

    Cats, E.A.; Pol, W.L. van der; Piepers, S.; Franssen, H.; Jacobs, B.C.; Berg-Vos, R.M. van den; Kuks, J.B.M.; Doorn, P.A. van; Engelen, B.G.M. van; Verschuuren, J.J.; Wokke, J.H.J.; Veldink, J.H.; Berg, L.H. van den

    2010-01-01

    OBJECTIVE: Identification and examination of all patients with multifocal motor neuropathy (MMN) in the Netherlands to document the clinical spectrum and response to IV immunoglobulin (IVIg) and to determine correlates of outcome. METHODS: A national cross-sectional descriptive study was performed.

  12. Contactin-1 and Neurofascin-155/-186 Are Not Targets of Auto-Antibodies in Multifocal Motor Neuropathy.

    Directory of Open Access Journals (Sweden)

    Kathrin Doppler

    Full Text Available Multifocal motor neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyneuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal motor neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal motor neuropathy patients' sera. We analyzed sera of 33 patients with well-characterized multifocal motor neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal motor neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal motor neuropathy.

  13. Mathematical epidemiology

    CERN Document Server

    Driessche, Pauline; Wu, Jianhong

    2008-01-01

    Based on lecture notes of two summer schools with a mixed audience from mathematical sciences, epidemiology and public health, this volume offers a comprehensive introduction to basic ideas and techniques in modeling infectious diseases, for the comparison of strategies to plan for an anticipated epidemic or pandemic, and to deal with a disease outbreak in real time. It covers detailed case studies for diseases including pandemic influenza, West Nile virus, and childhood diseases. Models for other diseases including Severe Acute Respiratory Syndrome, fox rabies, and sexually transmitted infections are included as applications. Its chapters are coherent and complementary independent units. In order to accustom students to look at the current literature and to experience different perspectives, no attempt has been made to achieve united writing style or unified notation. Notes on some mathematical background (calculus, matrix algebra, differential equations, and probability) have been prepared and may be downlo...

  14. Short-term visual performance of soft multifocal contact lenses for presbyopia

    Directory of Open Access Journals (Sweden)

    Jennifer Sha

    2016-04-01

    Full Text Available ABSTRACT Purpose: To compare visual acuity (VA, contrast sensitivity, stereopsis, and subjective visual performance of Acuvue® Oasys® for Presbyopia (AOP, Air Optix® Aqua Multifocal (AOMF, and Air Optix® Aqua Single Vision (AOSV lenses in patients with presbyopia. Methods: A single-blinded crossover trial was conducted. Twenty patients with mild presbyopia (add ≤+1.25 D and 22 with moderate/severe presbyopia (add ≥+1.50 D who wore lenses bilaterally for 1 h, with a minimum overnight washout period between the use of each lens. Measurements included high- and low-contrast visual acuity (HCVA and LCVA, respectively at a distance, contrast sensitivity (CS at a distance, HCVA at intermediate (70 cm and near (50 cm & 40 cm distances, stereopsis, and subjective questionnaires regarding vision clarity, ghosting, overall vision satisfaction, and comfort. The test variables were compared among the lens types using repeated-measures ANOVA. Results: Distance variables (HCVA, LCVA, and CS were significantly worse with multifocal lens than with AOSV lens (p≤0.008, except for AOMF lens in the mild presbyopia group in which no significant difference was observed (p>0.05. Multifocal lenses had significantly greater HCVA at 40 cm than AOSV lens (p≤0.026. AOMF lens had greater intermediate HCVA than AOP lens (p0.05. The proportions of patients willing to buy AOSV, AOMF, and AOP lenses were 20%, 40%, and 50%, respectively, in the mild presbyopia group and 14%, 32%, and 23%, respectively, in the moderate/severe presbyopia group; however, these differences were not statistically significant (p≥0.159. Conclusions: Further development of multifocal lenses is required before significant advantages of multifocal lenses over single vision lens are observed in patients with presbyopia.

  15. Wavefront analysis and modulation transfer function of three multifocal intraocular lenses

    Directory of Open Access Journals (Sweden)

    Santhiago Marcony

    2010-01-01

    Full Text Available Purpose: To evaluate wavefront performance and modulation transfer function (MTF in the human eye after the implantation of diffractive or refractive multifocal intraocular lenses (IOLs. Materials and Methods: This was a prospective, interventional, comparative, nonrandomized clinical study. Uncorrected distance and near visual acuity, and wavefront analysis including MTF curves (iTrace aberrometer, Tracey Technologies, Houston, TX, USA were measured in 60 patients after bilateral IOL implantation with 6 months of follow-up. Forty eyes received the diffractive ReSTOR (Alcon, 40 eyes received the refractive ReZoom (Advanced Medical Optics and 40 eyes, the Tecnis ZM900 (Advanced Medical Optics. The comparison of MTF and aberration between the intraocular lenses was performed using analysis of variance (ANOVA, followed by the Dunn test when necessary. Results: The mean uncorrected distance visual acuity was similar in all three groups of multifocal IOLs. The ReSTOR group provided better uncorrected near visual acuity than the ReZoom group ( P < 0.001, but similar to the Tecnis group. Spherical aberration was significantly higher in the ReZoom group ( P = 0.007. Similar MTF curves were found for the aspheric multifocal IOL Tecnis and the spheric multifocal IOL ReSTOR, and both performed better than the multifocal IOL ReZoom in a 5 mm pupil ( P < 0.001 at all spatial frequencies. Conclusions: Diffractive IOLs studied presented similar MTF curves for a 5 mm pupil diameter. Both diffractive IOLs showed similar spherical aberration, which was significantly better with the full-diffractive IOL Tecnis than with the refractive IOL ReZoom.

  16. Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL): Assessment of the involved white matter tracts by MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kassem, Hassan [Department of Radiology, Benha University (Egypt); Wafaie, Ahmed, E-mail: a_wafaie@yahoo.com [Department of Radiology, Cairo University (Egypt); Abdelfattah, Sherif [Department of Radiology, Cairo University (Egypt); Farid, Tarek [Pediatric Department, Egyptian National Research Center (Egypt)

    2014-01-15

    Background and purpose: Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is a recently identified autosomal recessive disorder with early onset of symptoms and slowly progressive pyramidal, cerebellar and dorsal column dysfunction. LBSL is characterized by distinct white matter abnormalities and selective involvement of brainstem and spinal cord tracts. The purpose of this study is to assess the imaging features of the involved white matter tracts in cases of LBSL by MRI. Patients and methods: We retrospectively reviewed the imaging features of the selectively involved white matter tracts in sixteen genetically proven cases of leukoencephalopathy with brainstem and spinal cord involvement and elevated brain lactate (LBSL). All patients presented with slowly progressive cerebellar sensory ataxia with spasticity and dorsal column dysfunction. MRI of the brain and spine using 1.5 T machine and proton magnetic resonance spectroscopy ({sup 1}H MRS) on the abnormal white matter were done to all patients. The MRI and MRS data sets were analyzed according to lesion location, extent, distribution and signal pattern as well as metabolite values and ratios in MRS. Laboratory examinations ruled out classic leukodystrophies. Results: In all cases, MRI showed high signal intensity in T2-weighted and FLAIR images within the cerebral subcortical, periventricular and deep white matter, posterior limbs of internal capsules, centrum semiovale, medulla oblongata, intraparenchymal trajectory of trigeminal nerves and deep cerebellar white matter. In the spine, the signal intensity of the dorsal column and lateral cortico-spinal tracts were altered in all patients. The subcortical U fibers, globi pallidi, thalami, midbrain and transverse pontine fibers were spared in all cases. In 11 cases (68.8%), the signal changes were inhomogeneous and confluent whereas in 5 patients (31.2%), the signal abnormalities were spotty. MRI also showed variable

  17. Coccidioidomycosis: epidemiology

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    Brown J

    2013-06-01

    Full Text Available Jennifer Brown,1 Kaitlin Benedict,2 Benjamin J Park,2 George R Thompson III1,31Department of Internal Medicine, Division of Infectious Diseases, University of California, Davis Medical Center, Sacramento, CA, USA; 2Mycotic Diseases Branch, Division of Foodborne, Waterborne, and Environmental Diseases, US Centers for Disease Control and Prevention, Atlanta, GA, USA; 3Department of Medical Microbiology and Immunology, One Shields Avenue, Tupper Hall, Coccidioidomycosis Serology Laboratory, University of California, Davis, CA, USAAbstract: Coccidioidomycosis consists of a spectrum of disease, ranging from a mild, self-limited, febrile illness to severe, life-threatening infection. It is caused by the soil-dwelling fungi, Coccidioides immitis and C. posadasii, which are present in diverse endemic areas. Climate changes and environmental factors affect the Coccidioides lifecycle and influence infection rates. The incidence of coccidioidomycosis has risen substantially over the past two decades. The vast majority of Coccidioides infections occur in the endemic zones, such as California, Arizona, Mexico, and Central America. Infections occurring outside those zones appear to be increasingly common, and pose unique clinical and public health challenges. It has long been known that elderly persons, pregnant women, and members of certain ethnic groups are at risk for severe or disseminated coccidioidomycosis. In recent years, it has become evident that persons with immunodeficiency diseases, diabetics, transplant recipients, and prisoners are also particularly vulnerable.Keywords: coccidioidomycosis, Coccidioides, epidemiology, incidence, risk factors, geography

  18. [Eco-epidemiology: towards epidemiology of complexity].

    Science.gov (United States)

    Bizouarn, Philippe

    2016-05-01

    In order to solve public health problems posed by the epidemiology of risk factors centered on the individual and neglecting the causal processes linking the risk factors with the health outcomes, Mervyn Susser proposed a multilevel epidemiology called eco-epidemiology, addressing the interdependence of individuals and their connection with molecular, individual, societal, environmental levels of organization participating in the causal disease processes. The aim of this epidemiology is to integrate more than a level of organization in design, analysis and interpretation of health problems. After presenting the main criticisms of risk-factor epidemiology focused on the individual, we will try to show how eco-epidemiology and its development could help to understand the need for a broader and integrative epidemiology, in which studies designed to identify risk factors would be balanced by studies designed to answer other questions equally vital to public health.

  19. Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), and neuromuscular and vascular hamartoma (NMVH): two sides of the same coin?

    Science.gov (United States)

    Setaffy, Lisa; Osuna, María José Martín; Plieschnegger, Wolfgang; del Pino Florez Rial, María; Geboes, Karel; Langner, Cord

    2015-04-01

    Multifocal stenosing enteritis, not related to Crohn's disease or drug intake, has been described under two different terms: "cryptogenic multifocal ulcerous stenosing enteritis" (CMUSE) and "neuromuscular and vascular hamartoma" (NMVH). We present three new cases of this condition and argue that the two terms reflect the same disease entity. Although etiology and pathogenesis of the disease remain largely unclear, obliterative vascular changes may play an important role.

  20. An unusual case of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy with occipital lobe involvement

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    Bhavesh Trikamji

    2016-01-01

    Full Text Available Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura, psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.

  1. M-CSF receptor mutations in hereditary diffuse leukoencephalopathy with spheroids impair not only kinase activity but also surface expression

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    Hiyoshi, Masateru; Hashimoto, Michihiro; Yukihara, Mamiko; Bhuyan, Farzana; Suzu, Shinya, E-mail: ssuzu06@kumamoto-u.ac.jp

    2013-11-01

    Highlights: •Many mutations were identified in Fms as a putative genetic cause of HDLS. •All of the mutations tested severely impair the kinase activity. •Most of the mutations also impair the trafficking to the cell surface. •These defects further suggest that HDLS is caused by a loss of Fms function. -- Abstract: The tyrosine kinase Fms, the cell surface receptor for M-CSF and IL-34, is critical for microglial proliferation and differentiation in the brain. Recently, a number of mutations have been identified in Fms as a putative genetic cause of hereditary diffuse leukoencephalopathy with spheroids (HDLS), implying an important role of microglial dysfunction in HDLS pathogenesis. In this study, we initially confirmed that 11 mutations, which reside within the ATP-binding or major tyrosine kinase domain, caused a severe impairment of ligand-induced Fms auto-phosphorylation. Intriguingly, we found that 10 of the 11 mutants also showed a weak cell surface expression, which was associated with a concomitant increase in the low molecular weight hypo-N-glycosylated immature gp130Fms-like species. Indeed, the mutant proteins heavily accumulated to the Golgi-like perinuclear regions. These results indicate that all of the Fms mutations tested severely impair the kinase activity and most of the mutations also impair the trafficking to the cell surface, further suggesting that HDLS is caused by the loss of Fms function.

  2. Effects of Lacunar Infarctions on Cognitive Impairment in Patients with Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

    Science.gov (United States)

    Choi, Jay Chol; Kang, Sa-Yoon; Kang, Ji-Hoon; Na, Hae Ri; Park, Ji-Kang

    2011-01-01

    Background and Purpose Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited microangiopathy caused by mutations in the Notch3 gene. Although previous studies have shown an association between lacunar infarction and cognitive impairment, the relationship between MRI parameters and cognition remains unclear. In this study we investigated the influence of MRI parameters on cognitive impairment in CADASIL. Methods We applied a prospective protocol to 40 patients. MRI analysis included the normalized volume of white-matter hyperintensities (nWMHs), number of lacunes, and number of cerebral microbleeds. Cognition was assessed with the aid of psychometric tests [Mini-Mental State Examination (MMSE), Alzheimer's Disease Assessment Scale-cognition (ADAS-cog), Trail-Making Test, and Stroop interference (Stroop IF)]. Results A multivariate regression analysis revealed that the total number of lacunes influenced the performance in the MMSE, ADAS-cog, and Stroop IF, while nWMHs had a strong univariate association with ADAS-cog and Stroop IF scores. However, this association disappeared in the multivariate analysis. Conclusions These findings demonstrate that the number of lacunes is the main predictive factor of cognitive impairment in CADASIL. PMID:22259617

  3. Repeated cyst formation in a patient with leukoencephalopathy, cerebral calcifications, and cysts: effectiveness of stereotactic aspiration with Ommaya reservoir placement.

    Science.gov (United States)

    Ooba, Hiroshi; Abe, Tatsuya; Hisamitsu, Yoshinori; Fujiki, Minoru

    2013-08-01

    The combination of leukoencephalopathy, cerebral calcifications, and cysts (LCC) constitutes a rare cerebral disorder characterized by distinctive neuroradiological and clinical findings. Twenty-eight cases of LCC have been reported. Surgery is usually required to treat progressively expanding cysts, but surgical strategies have been varied. The authors present the case of a patient who underwent 4 surgical procedures for repeated cyst formation. The first operation was performed for the removal of a cyst and to make a histopathological diagnosis. The second and third operations were performed to treat de novo cysts. The fourth operation was performed to treat a recurrence. This is the first reported case of LCC in which it was necessary to perform surgery 4 times, in large part due to de novo cyst formation. It provides evidence that multiple cysts may develop in these patients and that several surgical operations may be needed to treat LCC. Stereotactic aspiration with Ommaya reservoir placement is an appropriate procedure for this condition because it is less invasive and more repeatable than open surgery.

  4. Is radioiodine administration in patients with papillary thyroid multifocal microcarcinoma unnecessary?

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    Eva Krčálová

    2016-05-01

    Full Text Available Radioiodine (RAI has played a crucial role in differentiated thyroid cancer treatment for more than 60years. However, the use of RAI administration in patients with papillary thyroid microcarcinoma (even multifocal is now being widely discussed and often not recommended. In accordance with European consensus, and contrary to the American Thyroid Association (ATA guidelines, we recently performed RAI thyroid remnant ablation in a patient with differentiated papillary multifocal microcarcinoma. The post-therapeutic whole-body scan and SPECT/CT revealed the real and unexpected extent of disease, with metastases to upper mediastinal lymph nodes. This finding led to the patient’s upstaging from stage I to stage IVa according to the American Joint Committee on Cancer/International Union Against Cancer criteria.

  5. Optimizing distance image quality of an aspheric multifocal intraocular lens using a comprehensive statistical design approach.

    Science.gov (United States)

    Hong, Xin; Zhang, Xiaoxiao

    2008-12-01

    The AcrySof ReSTOR intraocular lens (IOL) is a multifocal lens with state-of-the-art apodized diffractive technology, and is indicated for visual correction of aphakia secondary to removal of cataractous lenses in adult patients with/without presbyopia, who desire near, intermediate, and distance vision with increased spectacle independence. The multifocal design results in some optical contrast reduction, which may be improved by reducing spherical aberration. A novel patent-pending approach was undertaken to investigate the optical performance of aspheric lens designs. Simulated eyes using human normal distributions were corrected with different lens designs in a Monte Carlo simulation that allowed for variability in multiple surgical parameters (e.g. positioning error, biometric variation). Monte Carlo optimized results indicated that a lens spherical aberration of -0.10 microm provided optimal distance image quality.

  6. Multifocal oral melanoacanthoma associated with Addison's disease and hyperthyroidism: a case report.

    Science.gov (United States)

    Dantas, Thinali Sousa; Nascimento, Isabelly Vidal do; Verde, Maria Elisa Quezado Lima; Alves, Ana Paula Negreiros Nunes; Sousa, Fabrício Bitu; Mota, Mário Rogério Lima

    2017-01-01

    Oral melanoacanthoma is a mucocutaneous, pigmented, rare, benign, and probably reactive lesion. This paper reports for the first time in the literature a case of multifocal oral melanoacanthoma in a patient diagnosed with Addison's disease and concomitant Graves' disease with hyperthyroidism. The patient presented with oral pigmented lesions, which were hypothesized to be mucosal pigmentation associated with Addison's disease. Due to their unusual clinical pattern, these oral lesions were biopsied and diagnosed as oral melanoacanthoma on histopathology and immunohistochemistry for HMB-45. At the moment of this report, the patient was being treated for her systemic conditions, but the lesions had not regressed. Reactive hyperpigmentation of the skin and mucous membranes may be found in Addison's disease and hyperthyroidism. This case reinforces the hypothesis of a reactive nature for oral melanoacanthoma and highlights the need for investigation of endocrine disorders in patients with multifocal oral melanoacanthoma.

  7. Brucella spondylodiscitis: Multifocal involvement in thoracic and lumbar areas; a rare case

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    Pınar Korkmaz

    2015-09-01

    Full Text Available Brucellosis is a zoonosis that can affect many organs or systems. Musculoskeletal system is the most commonly affected site in brucellosis and the frequency of musculoskeletal system involvement varies between 2% and 53%. Multifocal spinal involvement is rare. Here, it was aimed to present our case with brucellar spondylodiscitis involving the thoracic and lumbar regions simultaneously. MRI is a useful imaging method in the diagnosis of multifocal spinal infection caused by brucellosis. Especially in endemic regions, at least two serological tests should be used in the diagnosis of brucellosis. Brucellosis should be absolutely kept in mind for the differential diagnosis of the lderly patients with complaint of longstanding thoracic and back pain in the regions where brucellosis is endemic. J Microbiol Infect Dis 2015;5(3: 129-132

  8. The diagnostic significance of the multifocal pattern visual evoked potential in glaucoma.

    Science.gov (United States)

    Graham, S L; Klistorner, A

    1999-04-01

    The concept of objective perimetry is an exciting one because it strives to assess glaucoma damage without relying on psychophysical testing. The recent introduction of multifocal stimulus recording has enhanced our ability to examine the human visual field using electrophysiology. A multifocal pattern visual evoked potential can now be recorded, testing up to 60 sites within the central 25 degrees. The test requires only that the subject fixate on a target, while a cortically scaled dartboard pattern stimulus undergoes pseudorandom alternation within each of the test segments. In its present configuration the test requires at least 8 minutes recording time per eye. Modified bipolar electrode positions are required to ensure that adequate signals are detected from all parts of the visual field. In glaucoma patients, pattern visual evoked potential amplitudes have been shown to reflect visual field loss with reduction of signal amplitude in the affected areas. This technique represents the first major step toward objective detection of visual field defects in glaucoma.

  9. An adult multifocal medulloblastoma with diffuse acute postoperative cerebellar swelling: immunohistochemical and molecular genetics analysis.

    Science.gov (United States)

    Balik, Vladimir; Trojanec, Radek; Holzerova, Milena; Tuckova, Lucie; Sulla, Igor; Megova, Magdalena; Vaverka, Miroslav; Hrabalek, Lumir; Ehrmann, Jiri

    2015-01-01

    Medulloblastoma (MB), the most common malignant tumor typically affecting children, occurs only exceptionally in adults. Multifocal presentation of this malignancy in adulthood is even much rarer—only four cases with favorable postoperative course have been reported, so far. The study illustrates a very rare rapid postoperative clinical deterioration due to diffuse cerebellar swelling (DCS) in an adult multifocal MB (MMB). To the best of their knowledge, authors for the first time performed genetic analysis of MMB and demonstrated expression patterns of selected markers that put the patient within the sonic hedgehog (SHH) molecular subgroup and at least partially explain her unsatisfactory clinical course. Herein, authors summarized the relevant literature concerning this issue with the aim to determine features that would facilitate diagnosis and therapy of such a scarce clinical entity.

  10. Multifocal Buruli Ulcer Associated with Secondary Infection in HIV Positive Patient

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    Kassi Komenan

    2013-01-01

    Full Text Available Buruli ulcer is a chronic and infectious skin disease, caused by Mycobacterium ulcerans. It leads to large skin ulceration and sometimes bone infection which is responsible for deformities. Here, we report a case of multifocal form of Buruli ulcer associated with secondary infection in a 46-year-old human immunodeficiency virus (HIV positive woman. The antimycobacterial drugs combined to surgery allowed curing this multifocal case and rose up two relevant issues: the susceptibility of immune reconstitution inflammatory syndrome (IRIS occurrence and Mycobacterium dissemination. The deep immune depression, the underline biological, and clinical disorders of the patient might contribute to IRIS occurrence and Buruli ulcer dissemination. Future investigations have to be conducted on the mechanism of IRIS on set and on Mycobacterium ulcerans dissemination after ARV drugs initiation and the patient related underline clinical or biological disorders.

  11. Inadvertent implantation of a reversed-optic Tecnis ZM900 multifocal intraocular lens: case report

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    Wilson Takashi Hida

    2012-12-01

    Full Text Available A 51-year-old woman with age-related cataract had an uneventful phacoemulsification and a Tecnis ZM900 multifocal intraocular lens (IOL implantation in both eyes. During IOL implantation in the left eye, the optic was unintentionally reversed in the bag and left that way. The refraction surprise was not significant and six months postoperatively the corrected distance visual acuity in both eyes was 20/20 and neither complained of visual discomfort. In conclusion, we found that a reversed-optic Tecnis multifocal IOL in the present case resulted in good final visual acuity without significant differences in aberrations compared to the other eye, and a conservative management can be taken as a safe option.

  12. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood

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    Bhagyalakshmi Atla

    2016-01-01

    Full Text Available Kaposiform hemangioendothelioma (KHE is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach–Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.

  13. Chronic recurrent multifocal osteomyelitis manifested as painful clavicular swelling: a case report

    OpenAIRE

    Bleckwenn, Markus; Sommer, Bernd; Weckbecker, Klaus

    2014-01-01

    Background Chronic recurrent multifocal osteomyelitis is a form of non-bacterial osteomyelitis which occurs primarily in childhood. In some cases painful bone swelling occurs. After a malignancy has been ruled out, antibiotic therapy is often started to treat the osteomyelitis. The course of this benign disease is self-limiting and is not positively affected by the antibiotic therapy. Case presentation A 14-year-old German girl from South Africa came to the surgery with painful swelling in th...

  14. Multifocal choroiditis following simultaneous hepatitis A, typhoid, and yellow fever vaccination

    OpenAIRE

    Escott S; Tarabishy AB; Davidorf FH

    2013-01-01

    Sarah Escott, Ahmad B Tarabishy, Frederick H DavidorfHavener Eye Institute, The Ohio State University, Columbus, OH, USAAbstract: The paper describes the first reported case of multifocal choroiditis following simultaneous hepatitis-A, typhoid, and yellow fever vaccinations. A 33-year-old male developed sudden onset of flashing lights and floaters in his right eye 3 weeks following hepatitis A, typhoid, and yellow fever vaccinations. Fundus examination and angiography confirmed the presence o...

  15. Multi-focal lobular carcinoma in situ arising in benign phylodes tumor: A case report

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    Lee, Taeg Ki; Choi, Chang Hwan; Kim, Youn Jeong; Kim, Mi Young; Lee, Kyung Hee; Cho, Soon Gu [Inha University Hospital, Incheon (Korea, Republic of)

    2015-08-15

    Coexistent breast malignancy arising in phyllodes tumor is extremely rare, and most of them are incidental reports after surgical excision. Coexistent malignancy in phyllodes tumor can vary from in-situ to invasive carcinoma. Lobular neoplasia is separated into atypical lobular hyperplasia and lobular carcinoma in situ (LCIS). LCIS is known to have a higher risk of developing invasive cancer. We reported imaging findings of multifocal LCIS arising in benign phyllodes tumor.

  16. Multifocal meningeal melanocytoma: a new pathological entity or the result of leptomeningeal seeding?

    Science.gov (United States)

    Ali, Youssef; Rahme, Ralph; Moussa, Ronald; Abadjian, Gerard; Menassa-Moussa, Lina; Samaha, Elie

    2009-09-01

    Meningeal melanocytoma is a rare benign CNS tumor derived from the leptomeningeal melanocytes. Although unusual, malignant transformation with leptomeningeal seeding into the brain or spinal cord may occur years after the initial diagnosis. The authors report a unique case of multifocal benign meningeal melanocytoma involving both cerebellopontine angles and the thoracic spinal cord, with associated diffuse leptomeningeal hyperpigmentation. They review the literature relevant to this topic and discuss the radiological and pathological features of this disease as well as its treatment options.

  17. Meningeal Castleman's disease with multifocal involvement: a case report and review of literature.

    Science.gov (United States)

    Coca, Santiago; Salas, Isabel; Martínez, Roberto; Saez, Miguel Angel; Vaquero, Jesús

    2008-05-01

    Castleman's disease is an uncommon lymphoproliferative disorder characterized by lymph node enlargement, hyperplasia of lymphoid follicles surrounded by mature lymphocytes, and capillary proliferation with endothelial hyperplasia. Although intracranial location is very uncommon, it should be considered in the differential diagnosis of meningeal tumors. We describe a new case appearing as two solid extraaxial masses simulating meningiomas. This multifocal meningeal involvement can be considered as an exceptional form of presentation of intracranial Castleman's disease.

  18. Outcomes of excimer laser enhancements in pseudophakic patients with multifocal intraocular lens

    Science.gov (United States)

    Schallhorn, Steven C; Venter, Jan A; Teenan, David; Schallhorn, Julie M; Hettinger, Keith A; Hannan, Stephen J; Pelouskova, Martina

    2016-01-01

    Purpose The aim of this study was to assess visual and refractive outcomes of laser vision correction (LVC) to correct residual refraction after multifocal intraocular lens (IOL) implantation. Patients and methods In this retrospective study, 782 eyes that underwent LVC to correct unintended ametropia after multifocal IOL implantation were evaluated. Of all multifocal lenses implanted during primary procedure, 98.7% were refractive and 1.3% had a diffractive design. All eyes were treated with VISX STAR S4 IR excimer laser using a convectional ablation profile. Refractive outcomes, visual acuities, patient satisfaction, and quality of life were evaluated at the last available visit. Results The mean time between enhancement and last visit was 6.3±4.4 months. Manifest spherical equivalent changed from −0.02±0.83 D (−3.38 D to +2.25 D) pre-enhancement to 0.00±0.34 D (−1.38 D to +1.25 D) post-enhancement. At the last follow-up, the percentage of eyes within 0.50 D and 1.00 D of emmetropia was 90.4% and 99.5%, respectively. Of all eyes, 74.9% achieved monocular uncorrected distance visual acuity 20/20 or better. The mean corrected distance visual acuity remained the same before (−0.04±0.06 logMAR [logarithm of the minimum angle of resolution]) and after LVC procedure (−0.04±0.07 logMAR; P=0.70). There was a slight improvement in visual phenomena (starburst, halo, glare, ghosting/double vision) following the enhancement. No sight-threatening complications related to LVC occurred in this study. Conclusion LVC in pseudophakic patients with multifocal IOL was safe, effective, and predictable in a large cohort of patients. PMID:27175059

  19. Multifocal fibrosing thyroiditis: report of 55 cases of a poorly recognized entity.

    Science.gov (United States)

    Fellegara, Giovanni; Rosai, Juan

    2015-03-01

    During the course of our consultation activity, we have recognized a peculiar form of thyroiditis in which multiple foci of fibrosis, most of which were associated with reactive atypia of the surrounding follicles, are present. We have referred to this condition, both in our consultation reports and in the third series of A.F.I.P. Fascicle on Tumors of the Thyroid Gland, as "multifocal fibrosing thyroiditis" or (less frequently) "multifocal sclerosing thyroiditis," which are descriptive terms that highlight the benign/inflammatory nature of the process, its multiplicity, and its unknown pathogenesis. The aim of this study is to better define the morphologic features of this process and correlate it with some clinical data. With this purpose, the consultation files of one of the authors (J.R.) were searched for cases coded as multifocal fibrosing thyroiditis or multifocal sclerosing thyroiditis in a 20-year period ranging from January 1989 to December 2009. A total of 55 cases were identified that displayed the above-listed features. There were 51 (93%) female and 4 (7%) male patients (F/M=12.75), with ages ranging between 15 and 71 years (mean age, 47.03 y; median age, 44.5 y). Microscopically, multiple foci of fibrosis were identified in all cases, their number ranging from 2 to 51 per case (mean number, 16), with a mean diameter of 3 mm (range: 0.36 to 15.1 mm). Although heterogenous in shape and size, the individual foci were rather similar to each other in composition, being characterized by a fibrotic poorly cellular center that merged with a cellular peripheral zone. Some of the follicular structures present at the periphery of the scar and-to a lesser extent-those entrapped inside it underwent complex reactive and regenerative (atypical) changes that simulated malignancy. We discuss the differential diagnosis with other benign and malignant thyroid conditions and speculate about its pathogenesis and possible relationship with papillary thyroid microcarcinoma.

  20. Usefulness of Implantation of Diffractive Multifocal Intraocular Lens in Eyes with Long Axial Lengths

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    Tomoichiro Ogawa

    2015-01-01

    Full Text Available Purpose. This study retrospectively analyzed the postoperative visual functions of myopic eyes implanted with multifocal intraocular lens (IOL to evaluate the efficacy of multifocal IOL in highly myopic eyes. Methods. We studied 61 patients (96 eyes who were implanted with multifocal IOL ZMA00 or ZMB00 (Abbott Medical Optics. The patients were stratified into two groups by axial length: 26 mm or above (AL≥26 group and below 26 mm (AL<26 group. Postoperative corrected and uncorrected distance (5 m and near (30 cm visual acuity (VA, contrast sensitivity, and depth of focus were compared between two groups. Results. In the AL≥26 group and the AL<26 group, the mean ± standard deviation uncorrected distance logMAR VA at 12-month postoperative follow-up was -0.04±0.11 and -0.01±0.14, respectively; and the corrected distance VA was -0.17±0.08 and -0.14±0.07, with no significant differences between two groups (p=0.558 and 0.101; Mann-Whitney U test. For near VA, the corresponding uncorrected VA was 0.06±0.08 and 0.05±0.09; and distance-corrected VA was 0.01±0.06 and 0.01±0.02, with no significant differences between two groups (p=0.572, and 0.157; Mann-Whitney U test. Conclusion. The present study demonstrates that it is possible to achieve good uncorrected near and distance VA following implantation of multifocal IOL in eyes with long axial lengths.

  1. Usefulness of Implantation of Diffractive Multifocal Intraocular Lens in Eyes with Long Axial Lengths

    Science.gov (United States)

    Ogawa, Tomoichiro; Shiba, Takuya; Tsuneoka, Hiroshi

    2015-01-01

    Purpose. This study retrospectively analyzed the postoperative visual functions of myopic eyes implanted with multifocal intraocular lens (IOL) to evaluate the efficacy of multifocal IOL in highly myopic eyes. Methods. We studied 61 patients (96 eyes) who were implanted with multifocal IOL ZMA00 or ZMB00 (Abbott Medical Optics). The patients were stratified into two groups by axial length: 26 mm or above (AL ≥ 26 group) and below 26 mm (AL < 26 group). Postoperative corrected and uncorrected distance (5 m) and near (30 cm) visual acuity (VA), contrast sensitivity, and depth of focus were compared between two groups. Results. In the AL ≥ 26 group and the AL < 26 group, the mean ± standard deviation uncorrected distance logMAR VA at 12-month postoperative follow-up was −0.04 ± 0.11 and −0.01 ± 0.14, respectively; and the corrected distance VA was −0.17 ± 0.08 and −0.14 ± 0.07, with no significant differences between two groups (p = 0.558 and 0.101; Mann-Whitney U test). For near VA, the corresponding uncorrected VA was 0.06 ± 0.08 and 0.05 ± 0.09; and distance-corrected VA was 0.01 ± 0.06 and 0.01 ± 0.02, with no significant differences between two groups (p = 0.572, and 0.157; Mann-Whitney U test). Conclusion. The present study demonstrates that it is possible to achieve good uncorrected near and distance VA following implantation of multifocal IOL in eyes with long axial lengths. PMID:26609428

  2. Current Understanding of the Pathogenesis and Management of Chronic Recurrent Multifocal Osteomyelitis

    OpenAIRE

    Ferguson, P. J.; M. Sandu

    2012-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory disorder that primarily affects children. Its hallmark is recurring episodes of sterile osteomyelitis. The clinical presentation is insidious onset of bone pain with or without fever. Laboratory studies typically reveal nonspecific evidence of inflammation. Radiologic imaging and histologic appearance resemble those of infectious osteomyelitis. There is a strong association with inflammatory disorders of the skin and intesti...

  3. Multifocal phaeohyphomycosis caused by Exophiala xenobiotica in a kidney transplant recipient.

    Science.gov (United States)

    Palmisano, A; Morio, F; Le Pape, P; Degli Antoni, A M; Ricci, R; Zucchi, A; Vaglio, A; Piotti, G; Antoniotti, R; Cremaschi, E; Buzio, C; Maggiore, U

    2015-04-01

    In recent years, black fungi have been increasingly reported as causing opportunistic infections after solid organ transplantation. Here, we report a case of insidious, relentless, and multifocal Exophiala xenobiotica infection in a kidney transplant recipient that eventually required multiple surgical excisions along with oral and intravenous antifungal combination therapy using liposomal amphotericin B and posaconazole. We compare the present case with all previously reported cases of Exophiala infection after kidney transplantation.

  4. Multifocal VEP (mfVEP) reveals abnormal neuronal delays in diabetes

    OpenAIRE

    2010-01-01

    This pilot study examined the diagnostic role of multifocal visually evoked potentials (mfVEP) in a small number of patients with diabetes. mfVEP, mfERG, and fundus photographs of both eyes of five patients with diabetes, three with nonproliferative diabetic retinopathy (NPDR) and two without NPDR were examined. Thirteen control subjects were also examined. Eighteen zones were constructed from the 60-element mfVEP stimulus array. mfVEP implicit time (IT) and amplitude (SNR) differences were t...

  5. Multifocal Epithelial Hyperplasia of Oral Cavity Expressing HPV 16 Gene: A Rare Entity

    OpenAIRE

    M. P. V. Prabhat; Chintamaneni Raja Lakshmi; Sai Madhavi, N.; Sujana Mulk Bhavana; Gummadapu Sarat; Kodali Ramamohan

    2013-01-01

    Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of...

  6. Autorefraction versus subjective refraction in a radially asymmetric multifocal intraocular lens.

    Science.gov (United States)

    van der Linden, Jan Willem; Vrijman, Violette; Al-Saady, Rana; El-Saady, Rana; van der Meulen, Ivanka J; Mourits, Maarten P; Lapid-Gortzak, Ruth

    2014-12-01

    To evaluate whether the automated refraction (AR) correlates with subjective manifest (MR) refraction in eyes implanted with radially asymmetric multifocal intraocular lens (IOLs). This retrospective study evaluated 52 eyes (52 patients) implanted with a radially asymmetric multifocal IOL (LS-312 MF30, Oculentis, Germany). At 3 months postoperatively, the AR and MR values were compared to determine the correlation between the sphere (S), the spherical equivalent (SE) and the astigmatic components J0 and J45. The difference of mean spherical measurement was +0.98D ± 0.62, with the AR measuring more myopic. The difference of the mean spherical equivalent was +1.11D ± 0.57, again with AR being more myopic. Both these differences were statistically significant (p refractive parameters showed r(2) = 0.067, r(2) = 0.078, r(2) = 0.018 and r(2) = 0.015, respectively, all of which point to a low correlation between the AR and the MR. Autorefraction shows poor correlation to manifest subjective refraction with these radially asymmetric multifocal IOLs. The autorefraction systematically underestimates the spherical and spherical equivalent power, while the correlation between the astigmatic components was also low. Autorefraction seems not a valid starting point for manifest subjective refraction with these types of lenses, unless a corrective factor of about +1 dioptre is used. © 2014 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  7. N-3 Polyunsaturated Fatty Acids of Marine Origin and Multifocality in Human Breast Cancer.

    Directory of Open Access Journals (Sweden)

    Lobna Ouldamer

    Full Text Available The microenvironment of breast epithelial tissue may contribute to the clinical expression of breast cancer. Breast epithelial tissue, whether healthy or tumoral, is directly in contact with fat cells, which in turn could influence tumor multifocality. In this pilot study we investigated whether the fatty acid composition of breast adipose tissue differed according to breast cancer focality.Twenty-three consecutive women presenting with non-metastatic breast cancer underwent breast-imaging procedures including Magnetic Resonance Imaging prior to treatment. Breast adipose tissue specimens were collected during breast surgery. We established a biochemical profile of adipose tissue fatty acids by gas chromatography. We assessed whether there were differences according to breast cancer focality.We found that decreased levels in breast adipose tissue of docosahexaenoic and eicosapentaenoic acids, the two main polyunsaturated n-3 fatty acids of marine origin, were associated with multifocality.These differences in lipid content may contribute to mechanisms through which peritumoral adipose tissue fuels breast cancer multifocality.

  8. Classical Hodgkin lymphoma masquerading as chronic recurrent multifocal osteomyelitis: a case report.

    Science.gov (United States)

    Pham, Michael; Ressler, Steven; Rosenthal, Allison; Kelemen, Katalin

    2017-02-18

    Hodgkin lymphoma is a hematologic malignancy usually confined to lymphatic structures and commonly associated with constitutional symptoms. Bony involvement and musculoskeletal symptoms are uncommon and typically seen in advanced disease. In this case, we report an unusual presentation of classical Hodgkin lymphoma and highlight diagnostic challenges leading to the misdiagnosis and treatment as chronic recurrent multifocal osteomyelitis. A 38-year-old white man presented with lower extremity musculoskeletal pain. Imaging studies revealed multifocal lytic and sclerotic osseous axial lesions. Multiple core needle bone marrow and excisional lymph node biopsies were non-diagnostic. Having met the criteria, a tentative diagnosis of chronic recurrent multifocal osteomyelitis was given. He was treated with non-steroidal anti-inflammatory medications with partial clinical response but had persistent symptoms. A second medical opinion was pursued. An open bone marrow biopsy was performed and yielded a diagnosis of classical Hodgkin lymphoma after 13 months of diagnostic uncertainty. A chemotherapy regimen of doxorubicin, bleomycin, vinblastine, and dacarbazine was instituted with complete symptomatic and radiologic response. This case illustrates diagnostic difficulties of a musculoskeletal presentation of Hodgkin lymphoma, challenges of non-diagnostic bone marrow and lymph node biopsies, and resultant diagnostic delays in delivering a potentially curative therapy. Had the additional open bone marrow biopsy not been performed, the diagnosis and treatment of Hodgkin lymphoma would have been missed.

  9. Chlorogenic acid supplementation improves multifocal electroretinography in patients with retinitis pigmentosa.

    Science.gov (United States)

    Shin, Joo Young; Yu, Hyeong Gon

    2014-01-01

    To evaluate the effect of chlorogenic acid supplementation in patients with retinitis pigmentosa, we evaluated objective change in visual function with multifocal electroretinography, along with visual acuity, visual field, standard electroretinography, and contrast sensitivity. Eighteen patients diagnosed with retinitis pigmentosa were enrolled in this prospective, non-comparative, single-arm study. Multifocal electroretinography, best-corrected visual acuity in Early Treatment Diabetic Retinopathy Study letters, total point score on visual field examination with Humphrey Field Analyzer II, electroretinography, and contrast sensitivity were measured and repeated after 3 months supplementation with chlorogenic acid. The amplitude of ring 5 was significantly higher on multifocal electroretinography after 3 months of chlorogenic acid supplementation (7.2 ± 9.5 vs 8.3 ± 10.8 nV/deg(2), mean ± standard deviation, P = 0.022). There were no significant changes in the best-corrected visual acuity, total point score on Humphrey Field Analyzer, 30 Hz flicker amplitude on standard electroretinography, or contrast sensitivity. Chlorogenic acid may have a beneficial effect on the peripheral area at the margins of retinal degeneration, and should be considered as an anti-oxidant for the management of retinitis pigmentosa.

  10. Outcomes of excimer laser enhancements in pseudophakic patients with multifocal intraocular lens

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    Schallhorn SC

    2016-04-01

    Full Text Available Steven C Schallhorn,1–3 Jan A Venter,2 David Teenan,2 Julie M Schallhorn,3 Keith A Hettinger,2 Stephen J Hannan,2 Martina Pelouskova2 1Department of Ophthalmology, University of California, San Francisco, CA, USA; 2Optical Express, Glasgow, UK; 3Department of Ophthalmology, University of Southern California, Los Angeles, CA, USA Purpose: The aim of this study was to assess visual and refractive outcomes of laser vision correction (LVC to correct residual refraction after multifocal intraocular lens (IOL implantation. Patients and methods: In this retrospective study, 782 eyes that underwent LVC to correct unintended ametropia after multifocal IOL implantation were evaluated. Of all multifocal lenses implanted during primary procedure, 98.7% were refractive and 1.3% had a diffractive design. All eyes were treated with VISX STAR S4 IR excimer laser using a convectional ablation profile. Refractive outcomes, visual acuities, patient satisfaction, and quality of life were evaluated at the last available visit. Results: The mean time between enhancement and last visit was 6.3±4.4 months. Manifest spherical equivalent changed from -0.02±0.83 D (-3.38 D to +2.25 D pre-enhancement to 0.00±0.34 D (-1.38 D to +1.25 D post-enhancement. At the last follow-up, the percentage of eyes within 0.50 D and 1.00 D of emmetropia was 90.4% and 99.5%, respectively. Of all eyes, 74.9% achieved monocular uncorrected distance visual acuity 20/20 or better. The mean corrected distance visual acuity remained the same before (-0.04±0.06 logMAR [logarithm of the minimum angle of resolution] and after LVC procedure (-0.04±0.07 logMAR; P=0.70. There was a slight improvement in visual phenomena (starburst, halo, glare, ghosting/double vision following the enhancement. No sight-threatening complications related to LVC occurred in this study. Conclusion: LVC in pseudophakic patients with multifocal IOL was safe, effective, and predictable in a large cohort of

  11. 一种新的儿童脑白质病-巨颅伴皮层下海绵样囊肿性脑白质病%Megalencephalic Leukoencephalopathy with Subcortical Cysts-a New Child Leukoencephalopathy

    Institute of Scientific and Technical Information of China (English)

    袁宝强; Marjo S.van der Knaap

    2004-01-01

    巨颅伴皮层下海绵样囊肿性脑白质病是近年来被认识的一种新的儿童脑白质病.这种脑白质病患者出生时头围增大,神经发育基本正常或接近正常.随后逐渐出现缓慢进展性运动功能恶化如走路不稳、共济失调、肢体痉挛和瘫痪,而同时智能损伤相对较轻.大多数患者可有惊厥发作但对抗惊厥治疗反应良好.除脑电图上可有痫样放电外其他神经电生理检查基本正常.在影像学上所有患儿的脑白质均受累,早期患者MRI上表现出脑组织水肿,特别是额叶以及颞叶和顶叶的前部十分明显,后期脑皮层下出现囊肿样变化.病理学显示这些囊肿样结构主要是由单一五层膜结构所构成的髓鞘板层覆盖的空泡.目前已知的、常见的遗传代谢物筛查在该病患者中无阳性发现.该病是一种常染色体隐性遗传病,位于22qtel上的KIAA0027基因被认为是该脑白质病的致病基因.%Here we review a new variety of leukoencephalopathy with infantile megalencephaly and discrepant clinical course (MLC, MIM: 604004). These children bad megalencephaly in the first year of life, with or without mild delay of motor function and/or seizures. After a few years, motor handicap was slowly progressive with unsteady gait,serious cerebellar ataxia and mild plasticity. Eventually most of patients were confined to a wheelchair. Meanwhile mental development was relatively preserved, although the learning problems was increased from the midway of elementary school. Most of patients had tonic-clonic seizure and some might advance to status epilepticus. Antiepileptic drugs may effectively control seizure. The disorders of known metabolic defects were excluded. Neurophysiological examination showed that EEG had interictal epileptic discharges on the generalized slow wave background in most patients. The cerebral white matter had diffuse abnormality, with swelling of white matter, and cysts in the frontoparietal

  12. Fatores técnicos intervenientes na realização do exame de eletrorretinograma multifocal (ERGmf Technical factors that influence multifocal electroretinogram (mfERG recording

    Directory of Open Access Journals (Sweden)

    Maria Kiyoko Oyamada

    2007-08-01

    Full Text Available OBJETIVO: Descrever os principais fatores intervenientes observados durante a aquisição e análise do exame de eletrorretinograma multifocal (ERGmf, que afetam o registro gráfico das ondas obtidas e portanto sua análise. MÉTODOS: Análise dos fatores de erro observados durante a aquisição de 100 exames seqüenciais de eletrorretinograma multifocal, realizados com o Reti System da Roland Consult, no período de maio a julho de 2005. Os exames de eletrorretinograma multifocal foram realizados conforme recomendações internacionais, respeitando-se os parâmetros preestabelecidos do equipamento, com 61 elementos, e ângulo visual de 30º. Todos os pacientes foram submetidos à avaliação oftalmológica completa, excluindo-se aqueles com transparência inadequada de meios, córneas planas e os incapazes de visualizar as linhas de fixação. RESULTADOS: Os fatores intervenientes, que poderiam causar erros na interpretação dos resultados obtidos, observados e corrigidos durante a realização dos exames foram: fixação excêntrica, movimentação dos olhos, contração do orbicular, contração da musculatura cervical, não-correção adequada, não-transparência adequada do eletrodo corneano, deslocamento do eletrodo em geral para baixo, descentralização do suporte da lente corretora, distância inadequada paciente-monitor em pacientes com alta ametropia, altura inadequada dos olhos em relação ao centro da tela, ruído na freqüência de 60 HZ (rede elétrica e de campo eletromagnético, impedâncias elevadas. No processamento das ondas, a utilização excessiva de filtros para remoção de ruídos dos registros obtidos, pode interferir na análise dos resultados obtidos. CONCLUSÕES: Cuidados simplesmente observacionais durante a aquisição dos sinais são de importância significativa para a obtenção de registros com boa morfologia e baixo nível de ruído. Permitindo, por meio de sua pronta correção, a análise correta e

  13. International Lymphoma Epidemiology Consortium

    Science.gov (United States)

    The InterLymph Consortium, or formally the International Consortium of Investigators Working on Non-Hodgkin's Lymphoma Epidemiologic Studies, is an open scientific forum for epidemiologic research in non-Hodgkin's lymphoma.

  14. Ehrlichiosis: Statistics and Epidemiology

    Science.gov (United States)

    ... a tick Diseases transmitted by ticks Statistics and Epidemiology Recommend on Facebook Tweet Share Compartir On this ... Holman RC, McQuiston JH, Krebs JW, Swerdlow DL. Epidemiology of human ehrlichiosis and anaplasmosis in the United ...

  15. Nephroangiosclerosis in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: is NOTCH3 mutation the common culprit?

    Science.gov (United States)

    Guerrot, Dominique; François, Arnaud; Boffa, Jean-Jacques; Boulos, Nada; Hanoy, Melanie; Legallicier, Bruno; Triquenot-Bagan, Aude; Guyant-Marechal, Lucie; Laquerriere, Annie; Freguin-Bouilland, Caroline; Ronco, Pierre; Godin, Michel

    2008-08-01

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a systemic arterial disease characterized by impairment of vascular smooth muscle cell structure and function related to NOTCH3 mutations. Pathological findings include pathognomonic granular osmiophilic material (GOM) deposition with nonspecific hyalinization within the artery wall in a variety of tissues. The main clinical presentation is iterative strokes in young adults despite the lack of cardiovascular risk factors, leading to early dementia. Although arteriosclerosis and GOM have been found in kidneys from patients with CADASIL, kidney disease has been described only once up to now, in association with immunoglobulin A nephropathy. We report the case of a 61-year-old patient with a medical history of CADASIL and recent mild hypertension. His mother also showed neuropsychiatric symptoms and end-stage renal disease of unknown cause. The patient had a chronic kidney disease defined by means of estimated glomerular filtration rate using the 4-variable Modification of Diet in Renal Disease Study equation of 58 mL/min/1.73 m(2) associated with mild proteinuria and intermittent microscopic hematuria. Renal histological analysis showed severe arteriosclerosis and mild interstitial fibrosis. Glomeruli did not show mesangial immunoglobulin A deposition or focal segmental proliferation. Electron microscopic analysis showed typical GOM deposition in the vicinity of altered vascular smooth muscle cells in interlobular and juxtaglomerular arteries. The nephroangiosclerosis-like lesions were unusually severe in contrast to the recent mild hypertension. The presence of GOM strongly suggests that renal lesions were related to the NOTCH3 mutation. Here, we describe the first case of familial occurrence of kidney disease with decreased kidney function in the absence of coexisting nephropathy in patients with CADASIL. We discuss the role of NOTCH3 mutation in the pathogenesis

  16. Imaging features in conventional MRI, spectroscopy and diffusion weighted images of hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS).

    Science.gov (United States)

    Bender, Benjamin; Klose, Uwe; Lindig, Tobias; Biskup, Saskia; Nägele, Thomas; Schöls, Ludger; Karle, Kathrin N

    2014-12-01

    Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is a rare autosomal dominant disease caused by mutations within the colony stimulating factor 1 receptor (CSF1R) gene. While a small number of reports on imaging findings in routine MRI exist, reported imaging findings in DWI and spectroscopy are scarce, and limited to not genetically proven case reports. We assessed MRI including DWI and MR spectroscopy in six patients with HDLS and two asymptomatic mutation carriers. A total of 13 MRIs were evaluated and a score of the white-matter lesion (WML) load was calculated. The course of MR abnormalities was followed for 6-19 months in four patients and 95 months in one carrier. MRI revealed widespread white-matter lesions of patchy or confluent pattern especially in the frontal and occipital lobe. The pyramidal tract was less affected than the surrounding tissue in all symptomatic patients on conventional T2WI. Three of four cases with DWI showed small dots of diffusion restriction within WML. Spectroscopy showed increased levels of mIns, Cho and lactate while NAA was decreased. Asymptomatic mutation carriers had, for the age of the patients, unusually pronounced unspecific WMLs. No diffusion restriction or alterations in metabolite levels could be detected in asymptomatic mutation carriers. Microbleeds were not found in any patient. Diffusion restriction seems to be a typical imaging pattern visible in patients with active disease progression in HDLS. Spectroscopic findings and the absence of microbleeds differ clearly from reported findings in CADASIL and subcortical arteriosclerotic encephalopathy. While the distribution and character of WMLs in asymptomatic cases remain unspecific they are likely to represent subclinical markers of HDLS.

  17. Severe leukoencephalopathy with fulminant cerebral edema reflecting immune reconstitution inflammatory syndrome during HIV infection: a case report

    Directory of Open Access Journals (Sweden)

    Niederstadt Thomas

    2010-07-01

    Full Text Available Abstract Introduction Immune reconstitution inflammatory syndrome is a well-known complication in HIV-infected patients after initiation of highly active antiretroviral therapy resulting in rapid CD4+ cell count recovery and suppression of viral load. Generally, immune reconstitution inflammatory syndrome is based on opportunistic infections, but rare cases of immune reconstitution inflammatory syndrome inducing demyelinization of the nervous system have also been observed. Case presentation A 37-year-old African woman with HIV infection diagnosed at 13 years of age was admitted to the emergency department after experiencing backache, severe headache, acute aphasia and psychomotor slowing for one week. Nine weeks earlier, highly active antiretroviral therapy in this patient had been changed because of loss of efficacy, and a rapid increase in CD4+ cell count and decrease of HIV viral load were observed. Magnetic resonance imaging of the brain showed extensive white matter lesions, and analysis of cerebrospinal fluid revealed an immunoreactive syndrome. Intensive investigations detected no opportunistic infections. A salvage therapy, including osmotherapy, corticosteroids and treatment of epileptic seizures, was performed, but the patient died from brainstem herniation 48 hours after admission. Neuropathologic examination of the brain revealed diffuse swelling, leptomeningeal infiltration by CD8 cells and enhancement of perivascular spaces by CD8+ cells. Conclusion Immune reconstitution inflammatory syndrome in this form seems to represent a severe autoimmunologic disease of the brain with specific histopathologic findings. This form of immune reconstitution inflammatory syndrome did not respond to therapy, and extremely rapid deterioration led to death within two days. Immune reconstitution inflammatory syndrome may also occur as severe leukoencephalopathy with fulminant cerebral edema during HIV infection with rapid immune reconstitution.

  18. [Dermato-epidemiology].

    Science.gov (United States)

    Apfelbacher, C J; Diepgen, T L; Weisshaar, E

    2011-11-01

    Dermato-epidemiology is an important scientific discipline which investigates skin diseases using epidemiological methods. Epidemiology is the science of the distribution and determinants of disease in specified populations. We describe fundamental terms of dermato-epidemiology (measures of disease occurrence, measures of risk), different study types (observational studies, interventional studies), the selection of statistical tests, bias and confounding as well as the principles of evidence-based dermatology, and give illustrative examples.

  19. Cancer Epidemiology Matters Blog

    Science.gov (United States)

    The Cancer Epidemiology Matters blog helps foster a dialogue between the National Cancer Institute's (NCI) Epidemiology and Genomics Research Program (EGRP), extramural researchers, and other individuals, such as clinicians, community partners, and advocates, who are interested in cancer epidemiology and genomics.

  20. Depósito multifocal de grasa en hígado: Reporte de un caso Liver multifocal fat deposition: Case report

    Directory of Open Access Journals (Sweden)

    Federico Pasardi

    2009-03-01

    Full Text Available Describir los hallazgos radiológicos distintivos en ultrasonido (US, tomografía computada (TC y resonancia magnética (RM del depósito multifocal de grasa en hígado y su importancia en el diagnóstico diferencial en pacientes con antecedente neoplásico. Se reporta el caso de una paciente femenina de 66 años de edad, operada de cáncer de mama 2 años atrás, que en controles ecográficos de rutina muestra múltiples imágenes nodulares hiperecogénicas hepáticas de reciente aparición. La paciente fue estudiada con TC, RM e histo-patológicamente, confirmándose la naturaleza grasa de las imágenes nodulares. La esteatosis hepática se trata de una patología bastante frecuente, bien caracterizada por los métodos de imágenes. Presenta patrones de depósito diferentes, siendo necesario en algunos casos para el diagnóstico de certeza la toma de biopsia del tejido afectado, que, en nuestro caso, fue realizada con guía ecográfica.To describe the distinctive imaging findings in ultrasound (US, computed tomography (CT and magnetic resonance imaging (MRI of liver multifocal fat deposition and its importance in the differential diagnosis in patients with neoplasic antecedents. The authors reported a 66 years old female patient, who had surgery for breast cancer two years ago, and presents in routine US multiple liver nodular hiperecogenic images from recent diagnosis. The patient was studied with CT, MRI and histo-pathology confirmed fatty liver disease. Fatty liver disease is a frequent pathology, well characterized with imaging, that presents different patterns, being necessary biopsy for final diagnosis, that in our patient was performed under US guidance.

  1. Indocyanine Green Angiographic and Multifocal Electroretinographic Features in the Diffuse and Regional Form of Bietti′s Crystalline Retinopathy

    Institute of Scientific and Technical Information of China (English)

    Libin Jiang; Feng Wen; Lezheng Wu; Hong Yan; Shixing Hu

    2002-01-01

    Prpose: To observe different features of indocyanine green angiography(ICGA) andtifocal electroretinography (ERG) in the diffuse and regional type of Bieti′sc stalline retinopathy (BCR). Thods: ICGA and the multifocal ERG were performed in two cases of the diffuse andregional type of BCR respectively. These data were compared with fluoresceinangiography (FA), standard Ganzfeld ERG, and visual field testing. Results: In the regional case, ICGA revealed reduced perfusion of the choroidalcirculation in the early phase and multiple hypofluorescent spots in the posterior pole in the late phase, due to choriocapillaris filling defect; the extent of choroiocapillaris losswas shown in early phase of ICGA and there were multifocal hyperfluorescent dotssurrounding hypofluorescent spots in late phase in the diffuse case. The multifocal ERGshowed that the central responses were markedly depressed, corresponding to the visualfield defects, while the findings of Ganzfeld ERG were normal in the regional BCR;however, both the multifocal ERG and Ganzfeld ERG were severely subnormal in thediffuse case.Conclusions: The features of ICGA and multifocal ERG are different between the diffuseand regional BCR. In the meantime, the two tools are also useful to differentiate the typeand assess the extentof evolution in BCR.

  2. Comparison between OPD-Scan results and visual outcomes of monofocal and multifocal intraocular lenses Comparação dos resultados do OPD-Scan e performance visual das lentes intraoculares monofocal e multifocal

    Directory of Open Access Journals (Sweden)

    Wilson Takashi Hida

    2009-08-01

    Full Text Available PURPOSE: To compare the visual outcome, contrast sensitivity and wavefront analysis of patients that underwent cataract surgery and implantation of AcrySof SN60D3 multifocal intraocular lens with those who received the AcrySof SN60AT monofocal IOL. METHODS: This was a prospective clinical trial of forty eyes that received the multifocal IOL and thirty-two eyes that received the monofocal IOL after phacoemulsification. RESULTS: Values for total and spherical aberrations in the multifocal group were statistically lower than in the monofocal group. In the monofocal group, 75% achieved uncorrected intermediate visual acuities between Jaeger 1 and 6. In the multifocal group, 75% of the eyes achieved more than Jaeger 6. At least 87.5% of the multifocal group and 6.3% of the monofocal group achieved monocular uncorrected near acuity of 20/30 (J2, N5 or better. And 90.0% of the eyes in the multifocal group and 37.5% in the monofocal group achieved an uncorrected near acuity of 20/40 (J3, N6 or better. The mean spherical error was 0.11 D in the multifocal group and -0.18 D in the monofocal group (p=0.0379. The SN60D3 group compared to SN60AT group had low contrast sensitivity (log units with statistically significant differences in 6.0 cpd in photopic conditions (p=0.014 and the SN60D3 group compared to SN60AT group had higher contrast sensitivity (log units under mesopic conditions (p=0.044. CONCLUSION: The multifocal IOLs induced less spherical aberration than monofocal IOLs and predictably good uncorrected distance and uncorrected near acuities. However, contrast sensitivity was lower in the multifocal group.OBJETIVO: Comparar a performance visual, sensibilidade ao contraste e de wavefront com OPD-Scan em pacientes submetidos a cirurgia de facoemulsificação com implante de lente intraocular AcrySof SN60D3 multifocal e AcrySof SA60AT monofocal. MÉTODOS: Quarenta olhos com a lente intraocular multifocal e trinta e dois olhos com a lente intraocular

  3. Multifocal topographic visual evoked potential: improving objective detection of local visual field defects.

    Science.gov (United States)

    Klistorner, A I; Graham, S L; Grigg, J R; Billson, F A

    1998-05-01

    To investigate the relationships between the pattern stimulation of different parts of the visual field (up to 25 degrees of eccentricity), the electrode position, and the cortical response to improve objective detection of local visual field defects. The human visual evoked potential (VEP) was assessed using multifocal pseudorandomly alternated pattern stimuli that were cortically scaled in size. Monopolar and bipolar electrode positions were used. The visual field was investigated up to 26 degrees of eccentricity. Twelve normal subjects and seven subjects with visual field defects of different nature were studied. Although the monopolar response is heavily biased toward the lower hemifield, bipolar leads overlying the active occipital cortex (straddling the inion) demonstrate good signals from all areas of the visual field tested. The amplitude is almost equal for the averaged upper and lower hemifields, but the polarity is opposite, causing partial cancellation of the full-field VEP. The degree of cancellation depends mainly on latency differences between the vertical hemifields. The bipolar VEP corresponded well with Humphrey visual field defects, and it showed a loss of signal in the scotoma area. The multifocal VEP demonstrates good correspondence with the topography of the visual field. Recording with occipital bipolar electrode placement is superior to standard monopolar recording. To avoid a full-field cancellation effect, a separate evaluation of upper and lower hemifields should be used for the best assessment of retinocortical pathways. This technique represents a significant step toward the possible application of the multifocal VEP to objective detection of local defects in the visual field.

  4. Electroencephalogram-based scaling of multifocal visual evoked potentials: effect on intersubject amplitude variability.

    Science.gov (United States)

    Klistorner, A I; Graham, S L

    2001-08-01

    The interindividual variability of the visual evoked potential (VEP) has been recognized as a problem for interpretation of clinical results. This study examines whether VEP variability can be reduced by scaling responses according to underlying electroencephalogram (EEG) activity. A multifocal objective perimeter provided different random check patterns to each of 58 points extending out to 32 degrees nasally. A multichannel VEP was recorded (bipolar occipital cross electrodes, 7 min/eye). One hundred normal subjects (age 58.9 +/- 10.7 years) were tested. The amplitude and inter-eye asymmetry coefficient for each point of the field was calculated. VEP signals were then normalized according to underlying EEG activity recorded using Fourier transform to quantify EEG levels. High alpha-rhythm and electrocardiogram contamination were removed before scaling. High intersubject variability was present in the multifocal VEP, with amplitude in women on average 33% larger than in men. The variability for all left eyes was 42.2% +/- 3.9%, for right eyes 41.7% +/- 4.4% (coefficient of variability [CV]). There was a strong correlation between EEG activity and the amplitude of the VEP (left eye, r = 0.83; P < 0.001; right eye, r = 0.82; P < 0.001). When this was used to normalize VEP results, the CVs dropped to 24.6% +/- 3.1% (P < 0.0001) and 24.0% +/- 3.2% (P < 0.0001), respectively. The gender difference was effectively removed. Using underlying EEG amplitudes to normalize an individual's VEP substantially reduces intersubject variability, including differences between men and women. This renders the use of a normal database more reliable when applying the multifocal VEP in the clinical detection of visual field changes.

  5. Renal Function Outcomes for Multifocal Renal Neoplasms Managed by Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Pushpender, E-mail: pugupta@wakehealth.edu; Allen, Brian C., E-mail: bcallen2@wakehealth.edu; Chen, Michael Y., E-mail: mchen@wakehealth.edu; Childs, David D., E-mail: dchilds@wakehealth.edu; Kota, Gopi, E-mail: gkota@wakehealth.edu; Zagoria, Ronald J., E-mail: rzagoria@wakehealth.edu [Wake Forest University School of Medicine, Department of Radiology (United States)

    2013-10-15

    Purpose: To evaluate renal function changes related to radiofrequency ablation (RFA) for the treatment of multifocal renal neoplasms. Methods: This is an institutional review board-approved, Health Insurance Portability and Accountability Act compliant retrospective study of all patients treated with computed tomography guided RFA for multifocal renal neoplasms at one institution. Fifty-seven subjects, mean age 70 (range 37-88) years, underwent RFA of 169 renal neoplasms (average size 2.0 cm). Subjects had between 2 and 8 (mean 2.96) neoplasms ablated. Estimated glomerular filtration rate (eGFR) was measured before and after RFA. Complications related to RFA were recorded. Results: eGFR decreased on average of 4.4 % per tumor treated and 6.7 % per ablation session (average 1.76 tumors treated per session). For subjects with the largest neoplasm measuring >3 cm, eGFR decreased an average of 14.5 % during the course of their treatment. If the largest neoplasm measured 2-3 cm, eGFR decreased an average of 7.7 %, and if the largest neoplasm measured <2 cm, eGFR decreased an average of 3.8 %. Subjects with reduced baseline renal function were more likely to have a greater decline in eGFR after RFA. There was a minor complication rate of 6.3 % (6 of 96 sessions), none of which required treatment, and a major complication rate of 4.2 % (4 of 96 sessions). Conclusion: RFA for the treatment of multifocal renal neoplasms results in mild decline of renal function.

  6. Imaging of chronic recurrent multifocal osteomyelitis of childhood first presenting with isolated primary spinal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, S.E. [Department of Radiology, University Hospital of Bern, Inselspital, 3010, Bern (Switzerland); Heini, P.; Kalbermatten, D. [Department of Orthopedic Surgery, University Hospital of Bern, Inselspital, Bern (Switzerland); Sauvain, M.J. [Department of Pediatric Rheumatology, University Hospital of Bern, Inselspital, Bern (Switzerland); Stauffer, E. [Department of Pathology, University Hospital of Bern, Inselspital, Bern (Switzerland); Geiger, L. [Section Nuclear Medicine, Department of Radiology, University Hospital of Bern, Inselspital, Bern (Switzerland); Johnston, J.O. [Department of Orthopedic Surgery, University of California at San Francisco, San Francisco, California (United States); Roggo, A. [Department of Surgery, University Hospital of Bern, Inselspital, Bern (Switzerland); Steinbach, L.S. [Department of Radiology, University of California at San Francisco, San Francisco, California (United States)

    2003-06-01

    Initial presentation with primary spinal involvement in chronic recurrent multifocal osteomyelitis of childhood (CRMO) is rare. Our objective was to review the imaging appearances of three patients who had CRMO who initially presented with isolated primary spinal involvement.Design and patients The imaging, clinical, laboratory and histology findings of the three patients were retrospectively reviewed. Imaging included seven spinal MR imaging scans, one computed tomography scan, nine bone scans, two tomograms and 16 radiographs. These were reviewed by two musculoskeletal radiologists and a consensus view is reported. All three patients presented with atraumatic spinal pain and had extensive bone spinal pathology. The patients were aged 11, 13 and 12 years. There were two females and one male.Results and conclusions The initial patient had thoracic T6 and T8 vertebra plana. Bone scan showed additional vertebral body involvement. Follow-up was available over a 3 year period. The second patient had partial collapse of T9 and, 2 years later, of C6. Subsequently extensive multifocal disease ensued and follow-up was available over 8 years. The third patient initially had L3 inferior partial collapse and 1 year later T8 involvement with multifocal disease. Follow-up was available over 3 years. The imaging findings of the three patients include partial and complete vertebra plana with a subchondral line adjacent to endplates associated with bone marrow MR signal alterations. Awareness of the imaging appearances may help the radiologist to include this entity in the differential diagnosis in children who present with spinal pathology and no history of trauma. Histopathological examination excludes tumor and infection but with typical imaging findings may not always be necessary. (orig.)

  7. [RET/PTC rearrangement affects multifocal formation of papillary thyroid carcinoma].

    Science.gov (United States)

    Zhang, X; Su, X; Chen, W C; Li, Y; Yang, Z Y; Deng, W Z; Deng, T C; Yang, A K

    2017-06-07

    Objective:RET/PTC gene rearrangement can lead to aberrant activation of tyrosine kinase receptors, which is a common mutation in papillary thyroid carcinoma (PTC). This study focuses on the association of RET/PTC rearrangements with PTC clinical factors. Methods: From January 2011 to December 2013, a total of 114 patients with PTC were enrolled in this study. Clinicopathological parameters, lifestyle, and thyroid hormone levels were collected. RET/PTC rearrangements were detected by TaqMan PCR and verified by Sanger sequencing.Data were analyzed with SPSS software, including chi-square test, Fisher's exact test, Mann-Whitney U test, Student's t-test, and Logistic regression. Results:RET/PTC rearrangements were not found in all paracancerous normal thyroid tissues, and were detected in 23.68% (27/114) of PTC. Further analysis revealed no correlation between RET/PTC rearrangement and thyroid function, clinicopathologic parameters, and lifestyle in the total PTC group or in the subgroup of patients with concomitant diseases (including Hashimoto's thyroiditis and nodular goiter). But in the subgroup of PTC without concomitant disease, RET/PTC rearrangement was associated with tumor multifocal (P=0.018), and RET/PTC-positive PTC patients had an increased risk of tumor multifocal (OR=5.57, 95% CI 1.39-22.33). It was also found that RET/PTC rearrangement was associated with an abnormal increase in TSH level of one month after surgery (P= 0.037). Conclusion: Nodular goiter and Hashimoto 's thyroiditis may be a confounding factor in PTC. RET/PTC rearrangement may play an important role in the occurrence of thyroid carcinoma multifocal after exclusion of this confounding factor.

  8. Synchronous multifocal osteosarcoma with small cell histological variant: A double rarity

    Directory of Open Access Journals (Sweden)

    Sonia Gon

    2016-01-01

    Full Text Available Multifocal osteosarcoma (MFOS, osteosarcoma involving multiple sites, is a rare variant of osteosarcoma. When the lesions appear within 6 months of initial presentation of the tumor, it is known as synchronous MFOS. Synchronous MFOS has an incidence of 1%–3% only. Moreover, the histological variant of small cell osteosarcoma is even rarer. A case of 14-year-old male with synchronous MFOS of small cell type involving frontal and mandibular bone simultaneously is being reported here. It poses a dilemma to both the clinician and the pathologist to diagnose whether it represents multiple primary tumor or metastatic disease.

  9. Multifocal, recurrent malignant chondroid syringoma with visceral metastases: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Ann Meredith Garcia

    2016-03-01

    Full Text Available We present the unusual case of malignant chondroid syringoma (MCS in a 64-year-old male with recurrent nodular skin lesions and visceral metastases. The patient underwent repeated excisions, with eventual development of widespread nodules and multiple pulmonary and hepatic nodules. Systemic chemotherapy was planned. MCS is a very rare skin adnexal tumor of the sweat glands with only around 50 cases reported worldwide. To the authors’ knowledge, this is the first reported case of MCS with a multifocal presentation and mucosal involvement.

  10. Progressive multifocal leucoencephalopathy in a patient with idiopathic CD4+ lymphocytopenia.

    LENUS (Irish Health Repository)

    Moloney, F

    2012-03-01

    Progressive multifocal leucoencephalopathy (PML) is an opportunistic, demyelinating neurological disease caused by reactivation of the JC polyomavirus. PML occurs almost exclusively in immunosuppressed individuals, with only isolated case reports of PML occurring in patients without apparent immunosuppression. Idiopathic CD4+ lymohocytopenia (ICL) is a syndrome defined by the Centre for Disease Control and Prevention as a CD4+ count <300 cells\\/uL or <20% of total T cell count on >1 occasion, with no evidence of human immunodeficiency virus (HIV) infection, and the absence of other known immunodeficiency or therapy associated with lymphocytopenia. We describe a case of PML occurring in a patient with idiopathic CD4+ lymphocytopenia.

  11. Multifocal periostitis as a complication of chronic use of voriconazole in a lung transplant recipient.

    Science.gov (United States)

    Tedja, R; El-Sherief, A; Olbrych, T; Gordon, S

    2013-08-01

    Fungal infections are common in solid organ transplantation. An increasing number of transplant recipients receive antifungal therapy for prolonged duration owing to invasive fungal infections. Herein, we describe a diagnosis of periostitis as a complication of chronic use of voriconazole in a lung transplant recipient. The patient was diagnosed with probable pulmonary aspergillosis and was treated with oral voriconazole for a total of 9 months. Evidence of multifocal periostitis was observed in the axial and appendicular skeleton. Early recognition of this phenomenon is important to prevent unnecessary tests and procedures. Prompt discontinuation of voriconazole should result in improvement of symptoms.

  12. MRI shows thickening and altered diffusion in the median and ulnar nerves in multifocal motor neuropathy

    DEFF Research Database (Denmark)

    Haakma, Wieke; Jongbloed, Bas A.; Froeling, Martijn

    2016-01-01

    Objectives To study disease mechanisms in multifocal motor neuropathy (MMN) with magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) of the median and ulnar nerves. Methods We enrolled ten MMN patients, ten patients with amyotrophic lateral sclerosis (ALS) and ten healthy controls...... (HCs). Patients underwent MRI (in a prone position) and nerve conduction studies. DTI and fat-suppressed T2-weighted scans of the forearms were performed on a 3.0T MRI scanner. Fibre tractography of the median and ulnar nerves was performed to extract diffusion parameters: fractional anisotropy (FA...... nerves. CSA was significantly larger in MMN patients compared to ALS patients and HCs (p nerves...

  13. [Wave front aberrations -- practical conclusions in eye with Restor 3+ difractive multifocal lens].

    Science.gov (United States)

    Staicu, Corina; Moraru, Ozana; Moraru, Cristian

    2014-01-01

    Implantation of multifocal intraocular lenses has become a rutine nowadays, but achieving good visual results requires a perfect intraoperative technique and also an adequate preoperative selection of the patients. We analysed the wave front aberrations (spherical aberations, coma and astigmatism) in the eyes implanted with ReStor + 3 IOL, and we realized some clinical correlations of these aberations with the pupil diameter in scotopic and fotopic conditions, kappa angle, IOL centration, residual refraction errors postoperatively. Taking into account the causes of postoperative high order aberration will allow the surgeon to make a good selection of the patiens and to a higher degree of satisfaction of both sides.

  14. Noncontiguous multifocal brucellar spondylitis involving the cervical, thoracic, and lumbar spine: A case report

    Directory of Open Access Journals (Sweden)

    Rong Wang

    2017-06-01

    Full Text Available Brucellosis is a zoonosis infectious disease, brucellar spondylitis primarily infects the lumbar, the cervical is uncommon. Multiple-level involvement is extremely rare. This report describes a 46-year-old man with noncontiguous multifocal brucellar spondylitis involving the cervical, thoracic, and lumbar spine. Brucellar spondylitis is difficult to diagnose. Routine laboratory findings for the disease have little diagnostic value. Manifestations and radiological features are usually nonspecific. Radiological features of brucellar spondylitis are similar to tuberculous spondylitis and pyogenic spondylitis. MRI findings have diagnostic value. Suspicious patients with unexplained fever, musculoskeletal complaints at risk of infection should be considered.

  15. Arbitrary continuous nano-marks generated by multifocal spot arrays for controllable laser printing

    Science.gov (United States)

    Wen, Jing; Feng, Hui; Liu, Shiliang; Zhang, Dawei

    2017-04-01

    Phase-only modulations derived from the Debye approximation of the Richards–Wolf vectorial integral are used to produce a multifocal spot array. An analytical solution, which controls the position of each focal spot, can generate phase modulation images which are loaded into spatial light modulators. The calculated field distributions and the experimental images of the spot array on the back aperture of the objective are both demonstrated and validated. By overlapping the discrete focal points, continuous nano-structures of arbitrary marks can be achieved conveniently and easily. The above-proposed approach offers opportunities for flexible laser printing and creative micro-manipulation in the future.

  16. Multifocal hemangioendothelioma of the lumbar spine and response to surgical resection and radiation.

    Science.gov (United States)

    Kelahan, Linda C; Sandhu, Faheem A; Sayah, Anousheh

    2015-11-01

    Epithelioid hemangioendothelioma rarely occurs in the lumbosacral spine, with very few case reports of spinal hemangioendothelioma in the literature. There is variability in aggressiveness of these lesions without established treatment guidelines. The aim was to present a case of epithelioid hemangioendothelioma in the lumbar spine, including magnetic resonance imaging (MRI) findings, which rapidly progressed over a 2-month period as regional multifocal lumbosacral spinal lesions with epidural extension causing severe spinal canal stenosis. This was a case report in a university hospital setting. The sample included an otherwise healthy adult male with low back pain. Multimodality imaging was performed to help with diagnosis and management including computed tomography, MRI, and positron emission tomography (PET). The patient was treated by embolization, L5 corpectomy and L4-S1 stabilization, and radiation therapy. The diagnosis was confirmed by tissue biopsy. The patient initially presented with severe back and leg pain after a vertebroplasty for an L5 compression fracture at an outside hospital where biopsy was negative for malignancy. Magnetic resonance imaging showed diffuse abnormality of L5 with several smaller lesions in the sacrum. Due to progressive pain 2 weeks after the vertebroplasty, the patient underwent an L5 laminectomy, L4-S1 instrumented posterior fusion, and attempted partial corpectomy for stenosis. At this surgery, the L5 corpectomy was aborted owing to profound bleeding. Pathology was again negative for malignancy. Presumed to be an atypical hemangioma, the lesion was embolized before repeat surgery where the thecal sac was decompressed by partial L5 corpectomy. Biopsy at this time revealed a vascular neoplasm, with hemangioendothelioma not excluded. Approximately 2 months after the stabilization procedure, the patient had increasing pain and bilateral lower extremity weakness. Magnetic resonance imaging was performed and demonstrated marked

  17. Multifocal renal cell carcinoma of different histological subtypes in autosomal dominant polycystic kidney disease.

    Science.gov (United States)

    Na, Ki Yong; Kim, Hyun-Soo; Park, Yong-Koo; Chang, Sung-Goo; Kim, Youn Wha

    2012-08-01

    Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney (ADPKD) is rare. To date, 54 cases of RCC in ADPKD have been reported. Among these, only 2 cases have different histologic types of RCC. Here we describe a 45-year-old man who received radical nephrectomy for multifocal RCC with synchronous papillary and clear cell histology in ADPKD and chronic renal failure under regular hemodialysis. The case reported herein is another example of the rare pathological finding of RCC arising in a patient with ADPKD.

  18. Multifocal Epithelial Hyperplasia of Oral Cavity Expressing HPV 16 Gene: A Rare Entity

    Science.gov (United States)

    Prabhat, M. P. V.; Raja Lakshmi, Chintamaneni; Sai Madhavi, N.; Bhavana, Sujana Mulk; Sarat, Gummadapu; Ramamohan, Kodali

    2013-01-01

    Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of HPV 16 expression, as detected by polymerase chain reaction. The present report illustrates the need for careful examination and prompt diagnosis of the disease, as it might be associated with high risk genotypes such as HPV 16 and 18. PMID:24455323

  19. Peripheral refraction with dominant design multifocal contact lenses in young myopes

    Science.gov (United States)

    Lopes-Ferreira, Daniela; Ribeiro, Cláudia; Neves, Helena; Faria-Ribeiro, Miguel; Queirós, António; Villa-Collar, César; Jorge, Jorge; González-Méijome, José Manuel

    2013-01-01

    Purpose The purpose of this study was to show the potential of a commercial center-distance multifocal soft contact lens to induce relative peripheral myopic defocus in myopic eyes. Methods Twenty-eight myopic right eyes from 28 patients (mean age: 22.0 ± 2.0 years) were evaluated. The measurements of axial and off-axis refraction were made using a Grand-Seiko WAM-5500 open-field autorefractometer without lens and with multifocal contact lenses (Proclear Multifocal D® Design) of +2.00 D and +3.00 D add power applied randomly. Central mean spherical equivalent refraction was −2.24 ± 1.33 D. Ocular refraction was measured at center and at eccentricities between 35° nasal and 35° temporal (in 5° steps). Results Baseline relative peripheral refractive error (RPRE) as spherical equivalent (M) was −0.69 ± 1.14 D and −0.46 ± 1.38 D at 35° in the nasal and temporal degrees of visual field, respectively. Both add powers increased the relative peripheral myopic defocus up to −0.82 ± 1.23 D (p = 0.002) and −1.42 ± 1.45 D (p < 0.001) at 35° in the nasal field; and −0.87 ± 1.42 D (p = 0.003) and −2.00 ± 1.48 D (p < 0.001) at 35° in the temporal retina with +2.00 D and +3.00 D add lenses, respectively. Differences between +2.00 and +3.00 D add lenses were statistically significant beyond 20° in the nasal visual field and 10° in the temporal visual field. Conclusion It is possible to induce significant changes in the pattern of relative peripheral refraction in the myopic direction with commercially available dominant design multifocal contact lenses. The higher add (+3.00 D) induced an significantly higher effect than the +2.00 D add lens, although an increase of 1 D in add power does not correspond to the same amount of increase in RPRE.

  20. Autopsy case of acute multiple sclerosis with multifocal low density areas in the cerebral white matter on CT scans

    Energy Technology Data Exchange (ETDEWEB)

    Kamikura, Isao; Mizutani, Tomohiko; Sakamaki, Shuji; Takasu, Toshiaki; Kawamura, Toshiaki

    1988-01-01

    A 34-year-old woman presented with urination difficulty and consciousness disturbance, followed by persistent neurologic findings, such as semicomatose mental status and bilateral optic neuritis, and monophasic clinical course. Cranial CT showed multifocal low density areas in cerebral white matter. The patient was clinically diagnosed as having acute disseminated encephalomyelitis. She died of sepsis four months later. Autopsy revealed multifocal large demyelinating lesions confined to the cerebral white matter, shown as low density areas on CT scans, and demyelinating plaques scattered in the optic nerves and chiasm, and cerebral peduncle. The final diagnosis was acute multiple sclerosis. The CT appearance of multifocal low density areas was most likely due to demyelinating lesions causing edema and tissue necrosis. (Namekawa, K.).

  1. Electrode position and the multi-focal visual-evoked potential: role in objective visual field assessment.

    Science.gov (United States)

    Klistorner, A I; Graham, S L; Grigg, J R; Billson, F A

    1998-05-01

    To improve the performance of visual-evoked potentials (VEP) in the assessment of the human visual field, the multi-focal cortically scaled pattern VEP was recorded up to 250 of eccentricity in normal subjects. Monopolar and varying bipolar electrode positions were used. The monopolar response was strongly biased towards the lower hemifield. Bipolar leads straddling the inion (2 cm above and below) achieved approximately equal signals from the upper and lower visual field. Division into sectors of similar wave-form augments the analysis compared with summed full-field responses. With this technique, the multi-focal VEP can be used to objectively assess the visual field.

  2. Osteoblastomatosis of bone. A benign, multifocal osteoblastic lesion, distinct from osteoid osteoma and osteoblastoma, radiologically simulating a vascular tumor

    Energy Technology Data Exchange (ETDEWEB)

    Kyriakos, Michael [Washington University School of Medicine, Division of Surgical Pathology, Campus Box 8118, St. Louis, MO (United States); El-Khoury, Georges Y. [University of Iowa, Department of Radiology, Roy J. and Lucille A. Carver School of Medicine, Iowa City, IA (United States); McDonald, Douglas J. [Washington University School of Medicine, Department of Orthopaedic Surgery, St. Louis, MO (United States); Buckwalter, Joseph A. [University of Iowa, Department of Orthopaedics, Roy J. and Lucille A. Carver School of Medicine, Iowa City, IA (United States); Sundaram, Murali [Cleveland Clinic Foundation, Department of Radiology, Cleveland, OH (United States); DeYoung, Barry [University of Iowa, Department of Pathology, School of Medicine, Iowa City, IA (United States); O' Brien, Michael P. [University of Wisconsin Hospital, Department of Radiology, Madison, WI (United States)

    2007-03-15

    Two adult patients are described with multifocal osteolytic lesions radiologically simulating a vascular tumor. One patient had multiple bones involved. Histologically, the individual lesions had the features of the nidus of osteoid osteoma/osteoblastoma. A review of the English language medical literature yielded only one other reported case with similar features. The process is designated as osteoblastomatosis to indicate its bone-forming character, prominent osteoblast proliferation, and multiplicity. The cases are distinguished from multifocal/multicentric osteoid osteoma and osteoblastoma, and from benign and malignant vascular tumors. (orig.)

  3. The brain triuno and the ethical intelligence: fundamental counterfoil of the multifocal intelligence

    Directory of Open Access Journals (Sweden)

    C. Seijo

    2013-10-01

    Full Text Available This study has for aim offer an analysis as for the brain triuno and the ethical intelligence: fundamental Counterfoil of the multifocal intelligence, taking in tells one of the theories that it sustains her like they are the different types of multiple intelligences established by Beauport and Cury (2004. The theoretical sustenance, it is based on the contents of Martin (2005, Belohlavek (2007, Galicians (2002, Beauport and Cury (2004, between others, being realized under a symbolic interpretive approach, across a qualitative methodology, type descriptive and not experimental design, by means of a documentary analysis. In this regard, it is found that the ethical intelligence is a mental mechanism that constructs the structural preconceptos and the rules of game with which an individual approaches the reality, that is to say, it is the capacity of the general formation, predicting the behavior for the achievement of aims organizacionales. As for the final considerations they focused in obtaining the most wide knowledge inside the organizations, allowing to reflect before the weaknesses that they present thinking about the brain triuno applying the multifocal intelligence, fundamental counterfoil of the ethical intelligence and of what way the rationing visualizes the strengths, nevertheless of the weaknesses that they present. 

  4. Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options.

    Science.gov (United States)

    Meuth, Sven G; Kleinschnitz, Christoph

    2010-01-01

    Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Motor neuron disease, other chronic dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy and the Lewis-Sumner syndrome (MADSAM neuropathy), are important differential diagnoses. While corticosteroids and plasma exchange are largely ineffective, high-dose intravenous immunoglobulins are regarded as first-line treatment. In spite of significant success in elucidating the underlying disease mechanisms in MMN during the past few years, important pathophysiological issues and the optimum long-term therapy remain to be clarified. The present review summarizes the clinical picture and current pathophysiological concepts of MMN with a special focus on the molecular and electrophysiological basis of CBs and highlights established therapies as well as possible novel treatment options.

  5. Visual outcomes and optical quality after implantation of a diffractive multifocal toric intraocular lens

    Science.gov (United States)

    Chen, Xiangfei; Zhao, Ming; Shi, Yuhua; Yang, Liping; Lu, Yan; Huang, Zhenping

    2016-01-01

    Background: This study evaluated the visual function after implantation of a multifocal toric intraocular lenses (IOLs). Materials and Methods: This study involved 10 eyes from eight cataract patients with corneal astigmatism of 1.0 diopter (D) or higher who had received phacoemulsification with implantation of an AcrySof IQ ReSTOR Toric IOL. Six-month evaluations included visual acuity, spherical equivalent (SE), defocus curve, residual astigmatism, IOL rotation, contrast sensitivity (CS), wavefront aberrations, modulation transfer function (MTF), and patient satisfaction assessments. Results: At 6 months postoperatively, uncorrected distance visual acuity (logarithm of the minimum angle of resolution) was 0.09 ± 0.04, corrected distance visual acuity was 0.02 ± 0.11, and uncorrected near visual acuity was 0.12 ± 0.07. The mean SE was −0.095 ± 0.394 D (±0.50 D in 90%). Refractive astigmatism at the 6-month follow-up visit was significantly reduced to 0.35 ± 0.32 D from 1.50 ± 0.41 D presurgery (P 0.05). There was an increase in MTF results between preoperative and postoperative evaluations at all spatial frequencies. Conclusions: The diffractive multifocal toric IOL is able to provide a predictable astigmatic correction with apparently outstanding levels of optical quality after implantation. PMID:27221680

  6. Evaluation of Different Power of Near Addition in Two Different Multifocal Intraocular Lenses

    Directory of Open Access Journals (Sweden)

    Ugur Unsal

    2016-01-01

    Full Text Available Purpose. To compare near, intermediate, and distance vision and quality of vision, when refractive rotational multifocal intraocular lenses with 3.0 diopters or diffractive multifocal intraocular lenses with 2.5 diopters near addition are implanted. Methods. 41 eyes of 41 patients in whom rotational +3.0 diopters near addition IOLs were implanted and 30 eyes of 30 patients in whom diffractive +2.5 diopters near addition IOLs were implanted after cataract surgery were reviewed. Uncorrected and corrected distance visual acuity, intermediate visual acuity, near visual acuity, and patient satisfaction were evaluated 6 months later. Results. The corrected and uncorrected distance visual acuity were the same between both groups (p=0.50 and p=0.509, resp.. The uncorrected intermediate and corrected intermediate and near vision acuities were better in the +2.5 near vision added intraocular lens implanted group (p=0.049, p=0.005, and p=0.001, resp. and the uncorrected near vision acuity was better in the +3.0 near vision added intraocular lens implanted group (p=0.001. The patient satisfactions of both groups were similar. Conclusion. The +2.5 diopters near addition could be a better choice in younger patients with more distance and intermediate visual requirements (driving, outdoor activities, whereas the + 3.0 diopters should be considered for patients with more near vision correction (reading.

  7. An unusual case of bilateral multifocal retinal pigment epithelial detachment with methanol-induced optic neuritis.

    Science.gov (United States)

    Ranjan, Ratnesh; Kushwaha, Rajnath; Gupta, Ramesh Chandra; Khan, Perwez

    2014-03-01

    To describe an unusual case of methanol-induced optic neuritis with bilateral multifocal extrafoveal serous retinal pigment epithelial (RPE) detachment. Single case report. A 40-year-old male presented with acute bilateral loss of vision and history of consumption of adulterated alcohol. On examination, his vision was perception of light in the right eye and finger counting at 1-ft distance in the left eye. Pupillary reactions were sluggish. The optic discs were normal. An elevated lesion with subretinal serous fluid was present over macula adjacent to superior major vessel arcade in the right eye, which was confirmed as a large extrafoveal RPE detachment on fluorescein angiography. There were two more small RPE detachments in the right eye as well as in the left eye. All RPE detachments were extrafoveal in location. The patient was managed medically with intravenous methylprednisolone (1 g) in 500 ml of ringer lactate for three consecutive days. After three doses, visual acuity of both eyes was recorded as 20/20. We herein report an unusual case of bilateral multifocal extrafoveal serous RPE detachment in a patient of methanol-induced optic neuritis. RPE detachments may be due to the toxic effect of methanol metabolites.

  8. [Multifocal periventricular leukomalacia in children up to 1 year of life].

    Science.gov (United States)

    Sobaniec-Lotowska, M; Sobaniec, W; Ostapiuk, H; Sulkowski, S; Kemona, A; Sulik, M

    The authors present an analysis of the multifocal leucoencephalomalacia in children under 1 year of life based on the material obtained from 2.398 autopsies. It was found that 32 autopsied children suffered from this disease (1.33%). Twenty four of these children were premature. The disease was more frequent in the infants (23 cases) than in neonates (9 cases), and in boys (20 cases) than in girls (12 cases). Multifocal leucoenephalomalacia was manifested mainly as spastic infantile paralysis in the majority of the infants (14 cases). Morphological lesions to the brain most frequently embraced white substance adjacent to the cerebral ventricles (foci of leucoencephalomalacia of 1 cm in diameter) and cerebral vessels. Widening of the ventricular system of the brain was seen in 15 cases. Pathological reaction resulted from the brain anoxia and ischemia in perinatal period (20 cases) or septicaemia (12 cases) while predisposing factors included: prematurity, respiratory failure with hyaline membranes in the lungs and congenital abnormalities of the heart.

  9. The clinical research of central serous chorioretinopathy by multifocal electro- retinography

    Directory of Open Access Journals (Sweden)

    Qin Ye

    2013-07-01

    Full Text Available AIM: To elevate the diagnostic accuracy and guide the clinical treatment of central serous chorioretinopathy(CSCRby means of multifocal electroretinogram(mf-ERGat presentation and after resolution of the acute phase. METHODS: A comparative observational case series group and a normal sample group were set. Thirty-one eyes of 31 patients with unilateral CSCR were examined. Both eyes underwent complete ophthalmological examination, which included measurement of best-corrected visual acuity, fluorescein angiography, OCT and mf-ERG recording. The results were compared with the corresponding findings of 30 normal volunteers of the same age.RESULTS: At presentation, the averaged mean retinal response density of the mf-ERG of the affected eyes in area 1 was 60.54±18.20 nV/degree2, 45.43% lower compared to the normal controls(P2, 37.42% lower compared to the normal controls(P2, 15.53% lower compared to normal controls(P2, 16.01% lower compared to the normal controls(P2. In the remaining 23 eyes, the mf-ERG values were within normal limits.CONCLUSION: Multifocal electroretinography is favorable in the clinical treatment and the following observation of central serous chorioretinopathy. It may play an important role in the diagnosis, pathogenic condition evaluation and prognosis of CSC.

  10. Multifocal intraductal papillary mucinous neoplasm of the pancreas-A case report

    Institute of Scientific and Technical Information of China (English)

    Kun-Chun Chiang; Jun Te Hsu; Huang-Yang Chen; Shyh Chuan Jwo; Tsann-Long Hwang; Yi-Yin Jan; Chun-Nan Yeh

    2009-01-01

    Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas,papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas (IPMNs). IPMN was first described in 1982.It has been most commonly described in 60 to 70 years old males, and represents a relatively "new" but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head (75%) while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-yearold male diagnosed with IPMN (carcinoma in situ) in the pancreatic head and a branch duct type IPMN (duct atypia) in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.

  11. Cochear implantion for prelingually deafened children with leukoencephalopathy%脑白质病语前聋儿童人工耳蜗植入疗效观察

    Institute of Scientific and Technical Information of China (English)

    吴莎; 华清泉; 曹永茂; 杨琨; 张志敏

    2014-01-01

    目的:通过对脑白质病语前聋儿童人工耳蜗植入术疗效的观察,探讨人工耳蜗植入术在脑白质病语前聋儿童中的临床应用。方法经术前常规听力学、影像学、智力、心理等评估,筛选30例脑白质病语前聋儿童(脑白质病组)实施人工耳蜗植入术,并与同期30例接受人工耳蜗植入未伴脑白质病等中枢神经系统疾病的语前聋儿童(对照组)进行术后听觉语言康复情况疗效比较。结果(1)术后半年行声场测试,脑白质病组声场听阈为39.67±5.28dBHL,对照组为37.67±3.84dBHL,差异无统计学意义(P>0.05);(2)术后1年行听觉语言能力评估,听觉能力评估显示脑白质病组言语识别率为83.48±7.63%,对照组为85.03±9.43%;语言能力评估显示脑白质病组语言年龄为2.63±0.69岁,对照组语言年龄为2.71±0.74岁,两组患儿听觉能力及语言能力均无统计学差异(P>0.05)。结论脑白质病语前聋儿童在经过术前充分评估及观察筛选后实施人工耳蜗植入术与单纯语前聋儿童疗效相当,说明脑白质病聋儿实施人工耳蜗植入术是可行的。%Objective To observe the effects of cochear implantion for prelingually deafened children with leukoence-phalopathy. Methods 30 prelingually deafend children with leukoencephalopathy were screened in this study as leukoencephalopathy group by preoperative audiology, imaging data, intelligence and psychology assessment , and another 30 prelingually deafend children without central nervous diseases and accepted cochlear implantation at the same period were chosen as control group. The postoperative outcomes of the two groups were compared. Results (1)Half a year after cochlear implication, the field hearing threshold of leukoencephalopathy group was 39.67±5.28dBHL, the control group was 37.67±3.84dBHL, there was no significant difference in the two groups(P>0.05);(2)All children were

  12. 多焦点多光子显微技术及其研究进展%Multifocal Multiphoton Microscopy and Its Research Progress

    Institute of Scientific and Technical Information of China (English)

    刘立新

    2011-01-01

    Multifocal multiphoton microscopy ( MMM) can greatly improve the utilization of the excitation light and imaging speed, and it can also acquire three-dimensional fluorescence microscopic images by multiphoton excitation with the advantages of high speed, reduced photobleaching and photodamage, enhanced penetration depth and high signal to noise ratio. The realization and research progresses of multifocal multiphoton microscopy are discussed in detail, which include simultaneous time- and spectrum-resolved multifocal multiphoton microscopy(STSR-MMM) , multifocal multiphoton microscopy based on time-correlated single photon counting( TCSPC-MMM ) , multifocal multiphoton microscopy using a field of view zoom scanning protocol and stochastic scanning multifocal multiphoton microscopy (SS-MMM) , etc.%多焦点多光子显微技术(multifocal multiphoton microscopy,MMM)提高了激发光能的利用率和成像速度,可以实现样品的三维快速多光子激发荧光显微成像,并具有对活体样品损伤小,成像深度大,图像信噪比高等优点.详细阐述了MMM的实现方法及其研究进展,包括同时时间和光谱分辨的MMM(simultaneous time- and spectrum-resolved multifocal multiphoton microscopy,STSR-MMM)、基于时间相关单光子计数技术的MMM( multifocal multiphoton microscopy based on time-correlated single photon counting,TCSPC-MMM)、基于随机扫描的MMM(stochastic scanning multifocal multiphoton microscopy,SS-MMM)、基于固定光路系统的变视场扫描的MMM等技术.

  13. Multifocal sparganosis mimicking lymphoma involvement: Multimodal imaging findings of ultrasonography, CT, MRI, and position emission tomography-computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Heo, So Young; Park, Ji Yeon; Park, Noh Hyuck; Park, Chan Sub; Kim, Tae Jung [Myongji Hospital, Seonam University College of Medicine, Goyang (Korea, Republic of); Yi, Seong Yoon [Dev. of Hematology-Omcology, Dept. of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang (Korea, Republic of); Jun, Hyun Jung [Dev. of Hematology-Omcology, Dept. of Internal Medicine, Seoul Medical Center, Seoul (Korea, Republic of)

    2016-01-15

    Sparganosis is a rare parasitic disease caused by the migrating plerocercoid larva of Spirometra species tapeworms. The most frequent clinical manifestation is a subcutaneous nodule resembling a neoplasm. In this study, we presented multimodal findings of ultrasonography, computed tomography, magnetic resonance imaging, positron emission tomography-computed tomography and follow-up imagings on multifocal sparganosis, mimicking lymphoma involvement in a patient with lymphoma.

  14. In-the-bag decentration of a hydrophilic radially asymmetric multifocal intraocular lens secondary to capsule contraction.

    NARCIS (Netherlands)

    Linden, J.W.M. van der; Meulen, I.J. van der; Mourits, M.P.; Lapid-Gortzak, R.

    2013-01-01

    We report a case of in-the-bag decentration and tilt of a hydrophilic rotationally asymmetric multifocal intraocular lens (IOL) of the M Plus type secondary to capsule contraction. After uneventful surgery and follow-up for 3 months, progressive decentering and tilting of the IOL secondary to capsul

  15. Surgical treatment of multifocal giant cell tumor of carpal bones with preservation of wrist function: case report.

    Science.gov (United States)

    Tarng, Yih-Wen; Yang, Shan-Wei; Hsu, Chien-Jen

    2009-02-01

    We report a rare case of multifocal giant cell tumor of bone involving the trapezium, trapezoid, capitate, and scaphoid with soft tissue extension. Following intralesional resection, an autogenous corticocancellous iliac crest bone graft was used to fill the resultant defect and preserve carpal height and radiocarpal motion. Successful union with no recurrence was noted at 1-year follow-up.

  16. LEUCOENCEFALOPATÍA QUÍSTICA NO MEGALENCEFÁLICA VS. ENCEFALOPATÍA HIPOXICOISQUÉMICA: DIFICULTADES EN EL ASESORAMIENTO GENÉTICO Non-megalencephalic cystic leukoencephalopathy compared to hypoxic ischemic encephalopathy: difficulties in geneticassessment

    Directory of Open Access Journals (Sweden)

    Deisy Rincón

    2012-09-01

    Full Text Available Existen varias enfermedades que alteran la conformación de la mielina. Éstas se clasifican en Leucoencefalopatías primarias y secundarias, algunas de ellas con características clínicas, radiológicas, bioquímicas y moleculares que permiten realizar una aproximación diagnóstica. Pese a ello, muchas de éstas tienen cuadros clínicos similares y ya que no se cuenta con ayudas diagnósticas para todas estas patologías, se hace complejo realizar un diagnóstico diferencial. Se presenta el análisis del asesoramiento genético de una pareja con antecedentes de una hija con cuadro de leucoencefalopatía en quien se sospecha una Leucoencefalopatía Quística No Megalencefalica (Leucoencefalopatía primaria versus una LeucoencefalopatíaHipóxicoIsquemica (Leucoencefalopatía secundaria, donde no se logró llegar a un diagnóstico confirmado por la ausencia de herramientas diagnósticas, sobretodo de índole molecular. Desde esta perspectiva, el asesoramiento genético en esta paciente se constituye en un gran reto para el grupo multidisciplinario que maneja este tipo de casos.Several diseases alter myelin formation; they are classified into primary and secondary leukoencephalopathies, some of them having clinical, radiological, biochemical and molecular characteristics enabling a diagnostic approach. In spite of this, making a differential diagnosis becomes complicated as many of them have similar clinical pictures and there is a lack of diagnostic aids for all such pathologies. This article analyses the genetic counselling provided for a pair whose background consisted of a daughter having a picture of leukoencephalopathy in whom non-megalencephalic cystic leukoencephalopathy (primary leukoencephalopathy was suspected as opposed to hypoxic ischemic leukoencephalopathy (secondary leukoencephalopathy, as a confirmed diagnosis could not be reached due to a lack of diagnostic tools, particularly molecular ones. Genetic counselling for this

  17. Sporadic myopathy, myoclonus, leukoencephalopathy, neurosensory deafness, hypertrophic cardiomyopathy and insulin resistance associated with the mitochondrial 8306 T>C MTTK mutation.

    Science.gov (United States)

    Cardaioli, Elena; Malfatti, Edoardo; Battisti, Carla; Da Pozzo, Paola; Rubegni, Anna; Gallus, Gian Nicola; Malandrini, Alessandro; Federico, Antonio

    2012-10-15

    We report a new T8306C transition in the D-stem of the MTTK gene of a 67-year-old man who manifested severe adult onset myopathy, myoclonus, leukoencephalopathy, neurosensory hypoacusis, hypertrophic cardiomyopathy and insulin resistance. No other family member was affected, suggesting that our patient was a sporadic case. The T8306C mutation was heteroplasmic in several tissues of the proband, while it was absent from his asymptomatic siblings. Single fibre analysis confirmed the segregation of higher mutational load in cytochrome c oxidase-deficient fibres. The mutation T8306C is predicted to disrupt a highly conserved base pair and was not found in more than 120 controls. This finding broadens the phenotypic and molecular spectrum of mitochondrial tRNA(Lys) associated disorders.

  18. Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease.

    Science.gov (United States)

    Henderson, Jessica N; Noetzel, Michael J; McKinstry, Robert C; White, Desiree A; Armstrong, Melissa; DeBaun, Michael R

    2003-01-15

    Patients with severe acute chest syndrome (ACS) requiring endotracheal intubation and erythrocytopheresis are at increased risk for neurologic morbidity. This study examines patients with sickle cell disease who developed severe episodes of ACS, leading to endotracheal intubation, ventilatory support for respiratory failure, and erythrocytapheresis. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) studies, a neurologic examination by a pediatric neurologist, and cognitive testing were done in all patients. Five consecutive patients, aged 3 to 9 years, were identified with severe ACS. All patients developed neurologic complications resulting from ACS episodes, including seizures (n = 2), silent cerebral infarcts (n = 3), cerebral hemorrhage (n = 2), and reversible posterior leukoencephalopathy syndrome (n = 3). Children with severe ACS should have a magnetic resonance image of the brain, neurologic examination by a neurologist, and cognitive testing to detect the presence of neurologic morbidity.

  19. Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia Caused by a Novel R782G Mutation in CSF1R.

    Science.gov (United States)

    Foulds, Nicola; Pengelly, Reuben J; Hammans, Simon R; Nicoll, James A R; Ellison, David W; Ditchfield, Adam; Beck, Sarah; Ennis, Sarah

    2015-05-15

    We report a new family with autosomal dominant inheritance of a late onset rapidly progressive leukodystrophy in which exome sequencing has revealed a novel mutation p.R782G in the Colony-Stimulating Factor 1 Receptor gene (CSF1R). Neuropathology of two affected family members showed cerebral white matter degeneration with axonal swellings and pigmented macrophages. The few recently reported families with CSF1R mutations had been previously labelled "hereditary diffuse leukencephalopathy with axonal spheroids" (HDLS) and "pigmentary orthochromatic leukodystrophy" (POLD), disorders which now appear to form a disease continuum. The term "adult-onset leukoencephalopathy with axonal spheroids and pigmented glia" (ALSP) has been proposed to encompass this spectrum. As CSF1R regulates microglia this mutation implies that dysregulation of microglia is the primary cause of the disease.

  20. [Epidemiology and public policies].

    Science.gov (United States)

    Barata, Rita Barradas

    2013-03-01

    The present essay deals with the relation between epidemiology and public policies, highlighting the epidemiology position in the public health field, analyzing the impact of public policies over epidemiological profile and contributions from epidemiology to the lay down, implementation and evaluation of public health policies. In the first title, the essay debates the links between the epidemiology and public health field, the social determinants and political action framework proposed by the WHO's Commission on Social Determinants of Health, and different approaches of health policies. In the second title the essay analyses the reduction of child stunting in Brazil as an example of public policies that impact epidemiological profile. The third title presents three strategic topics for the application of public health policies: reduction of social inequalities in health, health promotion and regulation of products and services that have impact over health. The fourth title discusses the possibilities and difficulties to combine the epidemiological knowledge in the lay down, implementation and evaluation of public policies and, finally, material examples of such relation between epidemiology and public policies are presented.

  1. Adult onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) and Nasu-Hakola disease: lesion staging and dynamic changes of axons and microglial subsets.

    Science.gov (United States)

    Oyanagi, Kiyomitsu; Kinoshita, Michiaki; Suzuki-Kouyama, Emi; Inoue, Teruhiko; Nakahara, Asa; Tokiwai, Mika; Arai, Nobutaka; Satoh, Jun-Ichi; Aoki, Naoya; Jinnai, Kenji; Yazawa, Ikuru; Arai, Kimihito; Ishihara, Kenji; Kawamura, Mitsuru; Ishizawa, Keisuke; Hasegawa, Kazuko; Yagisita, Saburo; Amano, Naoji; Yoshida, Kunihiro; Terada, Seishi; Yoshida, Mari; Akiyama, Haruhiko; Mitsuyama, Yoshio; Ikeda, Shu-Ichi

    2016-09-08

    The brains of 10 Japanese patients with adult onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) encompassing hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD) and eight Japanese patients with Nasu-Hakola disease (N-HD) and five age-matched Japanese controls were examined neuropathologically with special reference to lesion staging and dynamic changes of microglial subsets. In both diseases, the pathognomonic neuropathological features included spherically swollen axons (spheroids and globules), axon loss and changes of microglia in the white matter. In ALSP, four lesion stages based on the degree of axon loss were discernible: Stage I, patchy axon loss in the cerebral white matter without atrophy; Stage II, large patchy areas of axon loss with slight atrophy of the cerebral white matter and slight dilatation of the lateral ventricles; Stage III, extensive axon loss in the cerebral white matter and dilatation of the lateral and third ventricles without remarkable axon loss in the brainstem and cerebellum; Stage IV, devastated cerebral white matter with marked dilatation of the ventricles and axon loss in the brainstem and/or cerebellum. Internal capsule and pontine base were relatively well preserved in the N-HD, even at Stage IV, and the swollen axons were larger with a higher density in the ALSP. Microglial cells immunopositive for CD68, CD163 or CD204 were far more obvious in ALSP, than in N-HD, and the shape and density of the cells changed in each stage. With progression of the stage, clinical symptoms became worse to apathetic state, and epilepsy was frequently observed in patients at Stages III and IV in both diseases. From these findings, it is concluded that (i) shape, density and subsets of microglia change dynamically along the passage of stages and (ii) increase of IBA-1-, CD68-, CD163- and CD204-immunopositive cells precedes loss of axons in ALSP. © 2016

  2. 新型多焦点人工晶状体临床应用的研究进展%Clinical application of new types of multifocal intraocular lens

    Institute of Scientific and Technical Information of China (English)

    张景尚; 万修华

    2015-01-01

    近年来新型多焦点人工晶状体(IOL)如非球面多焦点IOL、Toric多焦点IOL和非球面Toric多焦点IOL等的不断问世及临床应用,更好地改善了白内障患者术后的生活视力、工作视力以及生存质量.对于新型多焦点IOL的适应证应严格掌握,以使患者术后获得最佳效果.%In recent years new types of multifocal intraocular lens have been developed,such as aspheric multifocal intraocular lens,Toric multifocal intraocular lens and aspheric Toric multifocal intraocular lens and so on.The vision in life and work and the quality of life in patients with cataract were significantly improved after phacoemulsification with the new types of multifocal intraocular lens.At the same time there should be a strict indications for the new types of multifocal intraocular lens,so as to make the cataract patients implanted multifocal intraocular lens getting the best visual quality.

  3. The future of epidemiology.

    Science.gov (United States)

    Ness, Roberta B; Andrews, Elizabeth B; Gaudino, James A; Newman, Anne B; Soskolne, Colin L; Stürmer, Til; Wartenberg, Daniel E; Weiss, Stanley H

    2009-11-01

    In this article, the authors discuss current challenges and opportunities in epidemiology that will affect the field's future. Epidemiology is commonly considered the methodologic backbone for the fields of public health and outcomes research because its practitioners describe patterns of disease occurrence, identify risk factors and etiologic determinants, and demonstrate the usefulness of interventions. Like most aspects of science, epidemiology is in rapid flux. Several factors that are influencing and will continue to influence epidemiology and the health of the public include factors fundamental to framing the discipline of epidemiology (i.e., its means of communication, its methodologies, its access to data, its values, its population perspective), factors relating to scientific advances (e.g., genomics, comparative effectiveness in therapeutics), and factors shaping human health (e.g., increasing globalism, the environment, disease and lifestyle, demographics, infectious disease).

  4. MRI characteristics are predictive for CDMS in monofocal, but not in multifocal patients with a clinically isolated syndrome

    Directory of Open Access Journals (Sweden)

    Nielsen Jessica M

    2009-05-01

    Full Text Available Abstract Background To diagnose multiple sclerosis (MS, evidence for dissemination in space and time is required. There is no clear definition on how symptoms and signs of a patient indicate clinical dissemination in space. To provide a uniform approach on this subject, a clinical classification system was described recently differentiating patients with mono- and multifocal clinical presentation. Here we assess the predictive value of clinically defined dissemination in space at first presentation for time to clinically definite MS (CDMS. Methods Four hundred and sixty-eight patients with a first episode suggestive of MS were classified as clinically mono- or multifocal by two neurologists blinded to magnetic resonance imaging (MRI results. These patients were part of the BENEFIT study in which 292 patients were randomized to interferon beta-1b (IFNB-1b and 176 to placebo. By using Kaplan-Meier statistics the risk for CDMS was studied in mono- and multifocal patients of the placebo group, both with and without taking into account MRI measures of potential prognostic relevance. Results Time to CDMS was similar in monofocal and multifocal patients. In monofocal patients, the risk for CDMS over 2 years was significantly higher when ≥ 9 T2 lesions or at least one Gd-enhancing lesion were present at the first event or 3 or 6 months after the first event. In patients with multifocal presentation, these MRI measures had no significant added value in predicting time to CDMS. Conclusion These data indicate that a carefully performed neurological assessment of symptoms and signs, combined with lesions on MRI, is important for defining the risk of conversion to CDMS. Trial Registration The Benefit trial has been registered under NCT00185211 http://www.clinicaltrials.gov

  5. Denaturing high-performance liquid chromatography to diagnose cerebral autosomal dominant arteriopathy in Chinese patients with subcortical infarcts and leukoencephalopathy

    Institute of Scientific and Technical Information of China (English)

    Xiaomei Tang; Biao Chen

    2008-01-01

    BACKGROUND: Notch3 mutations are the molecular genetic foundation for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Of all currently available detection methods, direct sequencing or restriction enzymes are frequently used, but the cost is relatively high, because the Notch3 gene is composed of many exons and mutational sites are widely distributed. Denaturing high-performance liquid chromatography (DHPLC) exhibits high efficiency and specificity and has been applied to gene detection. To date, there has no report regarding DHPLC in gene detection of large-scale CADASIL families in China. OBJECTIVE: To explore the application and value of DHPLC in the diagnosis of CADASIL by a mutation screening for Notch3 gene in CADASIL probands and their family members. DESIGN, TIME AND SETTING: A comparative observation was performed at the Genetic Diagnosis Laboratory of Institute of Geriatrics, Xuanwu Hospital of Capital Medical University and the Key Laboratory for Neurodegenerative Disease of the Ministry of Education between August 2003 and May 2004. PARTICIPANTS: Fourteen CADASIL patients and their family members, comprising eight males and six females, aged 38-62 years, were included. Their key features included recurrent sub-cortical ischemic events and vascular dementia. In addition, 100 healthy physical examinees were selected as controls, including 52 males and 48 females, aged 56-72 years, who had no neurodegenerative disease or psychosis, and no history or high risk for cerebrovascular disease. METHODS: DNA was extracted from white blood cells. Ten hotspots of the Notch3 gene for sequence variation were first amplified by PCR, and the products were detected using DHPLC. Exons exhibiting a variant in the DHPLC profile underwent another PCR amplification, followed by DNA sequencing to identify the mutation type. In addition, patients with normal DHPLC peak profiles underwent PCR amplification for the remaining

  6. High Resolution Depth-Resolved Imaging From Multi-Focal Images for Medical Ultrasound

    DEFF Research Database (Denmark)

    Diamantis, Konstantinos; Dalgarno, Paul A.; Greenaway, Alan H.

    2015-01-01

    An ultrasound imaging technique providing subdiffraction limit axial resolution for point sources is proposed. It is based on simultaneously acquired multi-focal images of the same object, and on the image metric of sharpness. The sharpness is extracted by image data and presents higher values...... for in-focus images. The technique is derived from biological microscopy and is validated here with simulated ultrasound data. A linear array probe is used to scan a point scatterer phantom that moves in depth with a controlled step. From the beamformed responses of each scatterer position the image...... calibration curves combined with the use of a maximum-likelihood algorithm is then able to estimate, with high precision, the depth location of any emitter fron each single image. Estimated values are compared with the ground truth demonstrating that an accuracy of 28.6 µm (0.13λ) is achieved for a 4 mm depth...

  7. TBC1D24 Mutations in a Sibship with Multifocal Polymyoclonus

    Science.gov (United States)

    Ngoh, Adeline; Bras, Jose; Guerreiro, Rita; McTague, Amy; Ng, Joanne; Meyer, Esther; Chong, W. Kling; Boyd, Stewart; MacLellan, Linda; Kirkpatrick, Martin; Kurian, Manju A.

    2017-01-01

    Background Advances in molecular genetic technologies have improved our understanding of genetic causes of rare neurological disorders with features of myoclonus. Case Report A family with two affected siblings, presenting with multifocal polymyoclonus and neurodevelopmental delay, was recruited for whole-exome sequencing following unyielding diagnostic neurometabolic investigations. Compound heterozygous mutations in TBC1D24, a gene previously associated with various epilepsy phenotypes and hearing loss, were identified in both siblings. The mutations included a missense change c.457G>A (p.Glu157Lys), and a novel frameshift mutation c.545del (p.Thr182Serfs*6). Discussion We propose that TBC1D24-related diseases should be in the differential diagnosis for children with polymyoclonus. PMID:28428906

  8. A rare case of extra nodal Rosai-Dorfman disease with isolated multifocal osseous manifestation

    Directory of Open Access Journals (Sweden)

    Maharshi H Patel

    2015-01-01

    Full Text Available Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease is a non-neoplastic condition which typically presents as massive, bilateral cervical lymphadenopathy and can involve multiple extranodal organ systems such as skin, eyes, and upper respiratory tract in about 28% cases. Bone lesions in association with nodal disease are seen in less than 10% cases. Isolated bone involvement as the only manifestation of SHML is extremely rare, with less than 50 cases reported in the literature. We report a very uncommon case of Rosai-Dorfman disease with isolated multifocal osseous involvement as the only presenting feature, involving about 10 different sites with no lymphadenopathy or other organ system involvement.

  9. [Multifocal demyelinating polyneuropathy with persistent conduction block (Lewis-Sumner syndrome)].

    Science.gov (United States)

    Mezaki, T; Kaji, R; Hamano, T; Kimura, J; Kameyama, M

    1990-11-01

    Multifocal demyelinating neuropathy with persistent conduction block (Lewis-Sumner syndrome) is a variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which often clinically simulates a motor neuron disease (MND). We report here three patients initially suspected to have MND, who later were diagnosed as a Lewis-Sumner syndrome. One of them showed a remarkable clinical improvement after immunoglobulin therapy. The definitive diagnosis of this syndrome rests upon nerve conduction studies, uncovering multiple sites of persistent conduction block. Technically, it is important to exclude insufficient stimulus which may lead to an erroneous impression of conduction block. Magnetic stimulation, as compared to electric current, elicited larger responses possibly because of deeper current penetration. We found this mode of stimulation useful especially in testing focal demyelination requiring full activation of a diseased nerve at a most proximal segment.

  10. Chronic Recurrent Multifocal Osteomyelitis in a 9-year-old Boy

    Directory of Open Access Journals (Sweden)

    Abdolreza Malek

    2017-05-01

    Full Text Available Chronic recurrent multifocal osteomyelitis (CRMO is a rare aseptic, auto-inflammatory bone disorder. CRMO presents with bone pain with or without fever. The diagnosis of CRMO is a diagnosis of exclusion and should be included in the differential diagnosis of chronic inflammatory bone lesions in children. Cultures of the bone are typically sterile, antibiotic therapy does not result in clinical improvement whereas anti-inflammatory drugs improve the condition. Furthermore, biopsy should be considered in chronic and relapsing bone pain and swelling unresponsive to treatment. Herein, we present a nine-year-old boy complaining of recurrent pain in his upper and lower extremities. On examination he had mild fever and cervical lymphadenopathy. He also had experienced bone pain and weight loss in the recent month. Based on biopsy and bone scan he was finally diagnosed with CRMO. Naproxen and Pamidronate was prescribed and he was getting better and returned to normal life and activity without need to corticosteroids.

  11. Development of a Normative Database for Multifocal Electroretinography in the Context of a Multicenter Clinical Trial

    DEFF Research Database (Denmark)

    Simão, Sílvia; Costa, Miguel Ângelo; Sun, Jennifer K

    2017-01-01

    PURPOSE: The aim of this study is to present the largest normative database using multifocal electroretinography (mfERG) in the context of a multicenter clinical trial. METHODS: This investigational study included 156 eyes from 78 Caucasian subjects aged 45-70 years without known ophthalmic disease...... specialized training and certification. The main variables that could have influenced the results were considered in the analyses. RESULTS: The normative database was based on 111 eyes. The overall mean P1-implicit time (IT) was 33.94 ± 1.70 ms, and the mean P1 amplitude was 30.58 ± 5.20 nV/deg2. Age....... CONCLUSIONS: This normative database can be used as a comparative index of expected normal values in the clinical setting and for examining the effect of studies testing neuroprotective agents....

  12. Nerve sonography in multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy

    Directory of Open Access Journals (Sweden)

    D. S. Druzhinin

    2016-01-01

    Full Text Available The quantitative ultrasound characteristics (USC of the median, ulnar nerve at different levels and the spinal nerves in patients with multifocal motor neuropathy (MMN; n=13; 40,4 ± 12,6 years old and chronic inflammatory demyelinating polyneuropathy (CIDP; n = 7; 47,3 ± 11,2 year old did not reveal statistical difference in cross sectional area (CSA between analyzed groups. Patients with MMN have more pronounced asymmetry of CSA in comparison with CIDP patients which have a symmetrical pattern of diffuse nerve involvement. Quantitative USC has shown to be not informative enough in differentiation of MMN and CIDP. The qualitative analysis (QA according to 3 described types of nerve changes has shown that CIDP is characterized by the prevalence of type 3 pattern (85.8 % while MMN – by type 2 (69.2 %. The sensitivity and specificity of proposed QA patterns in nerve USC need to be analyzed in additional investigations. 

  13. Skull involvement in a pediatric case of chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Watanabe, Toru; Ono, Hiroyuki; Morimoto, Yoshitaka; Otsuki, Yoshiro; Shirai, Masami; Endoh, Akira; Naito, Masaaki; Inoue, Yoshiya; Hongo, Teruaki

    2015-08-01

    An 11-year-old boy was diagnosed with chronic recurrent multifocal osteomyelitis (CRMO) and presented with right sacro-femoral and occipital lesions. Initially, a tumor was suspected. However, the bone biopsy showed osteomyelitis with a negative bacterial culture. Bone scintigraphy revealed inflammatory changes on multiple bone lesions. The slight elevation in inflammatory markers such as C-reactive protein was of little clinical value. He was diagnosed with CRMO by sacral biopsy, and the clinical course progressed, with the presence of a new occipital lesion observed after the 1-year follow-up. The administration of non-steroidal anti-inflammatory drugs successfully improved his clinical symptoms. The presence of a skull lesion in the occipital bone of a pediatric patient with CRMO has not been previously reported.

  14. Effective treatment with interferon-alpha in chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Andersson, R

    1995-10-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disease of unknown etiology characterized by multiple osteomyelitic changes in the predominantly metaphysial regions of long bones. It was first described by Giedon et al. in 1972. Cultures for all known microorganisms are negative. Pain is the most common symptom, and sometimes soft tissue swelling is present. Patients are usually treated with nonsteroidal antiinflammatory drugs (NSAIDs) or corticosteroids and respond, at least partly, to these treatments. CRMO is most commonly seen in children and is in the majority of cases self-limiting but has a protracted course of several years. Some patients have a more prolonged disease period, as in the patient reported here. Treatment with corticosteroids in children has the risk of causing growth retardation as a potential adverse effect, and alternative treatments are of great interest. In the actual paper, a successful treatment with interferon-alpha 2b in a 34-year-old man with CRMO is presented.

  15. Current understanding of the pathogenesis and management of chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Ferguson, Polly J; Sandu, Monica

    2012-04-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory disorder that primarily affects children. Its hallmark is recurring episodes of sterile osteomyelitis. The clinical presentation is insidious onset of bone pain with or without fever. Laboratory studies typically reveal nonspecific evidence of inflammation. Radiologic imaging and histologic appearance resemble those of infectious osteomyelitis. There is a strong association with inflammatory disorders of the skin and intestinal tract in affected individuals and their close relatives, suggesting a shared pathophysiology and supporting a genetic component to disease susceptibility. Two genetic syndromes have CRMO as a prominent phenotype-Majeed syndrome and deficiency of the interleukin-1 receptor antagonist-and suggest that interleukin-1 may be a key cytokine in disease pathogenesis. This review briefly summarizes the main clinical and radiologic aspects of the disease and then focuses on genetics and pathophysiology and provides an update on treatment.

  16. Improvement of the classification accuracy in discriminating diabetic retinopathy by multifocal electroretinogram analysis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    The multifocal electroretinogram (mfERG) is a newly developed electrophysiological technique. In this paper, a classification method is proposed for early diagnosis of the diabetic retinopathy using mfERG data. MfERG records were obtained from eyes of healthy individuals and patients with diabetes at different stages. For each mfERG record, 103 local responses were extracted. Amplitude value of each point on all the mfERG local responses was looked as one potential feature to classify the experimental subjects. Feature subsets were selected from the feature space by comparing the inter-intra distance. Based on the selected feature subset, Fisher's linear classifiers were trained. And the final classification decision of the record was made by voting all the classifiers' outputs. Applying the method to classify all experimental subjects, very low error rates were achieved. Some crucial properties of the diabetic retinopathy classification method are also discussed.

  17. Multifocal epithelial hyperplasia: a case report of a family of Somalian descent living in Australia.

    Science.gov (United States)

    Hall, Charmaine; McCullough, Michael; Angel, Chris; Manton, David

    2010-01-01

    Multifocal epithelial hyperplasia (MEH) presents as painless, single or multiple nodular or papular lesions with a sessile base, ranging in diameter from 0.1 cm to 1.0 cm, frequently coalescing. Lesion color varies from red to white, depending on the extent of keratinization. The condition appears mainly in children and adolescents and regresses spontaneously. The etiological factors and pathogenesis of MEH are speculative. A 13-year-old boy presented to the Royal Dental Hospital of Melbourne for dental examination revealing several asymptomatic, exophytic, pink, sessile, smooth-surfaced nodules on the lateral borders of the tongue and lingual surface of the lower lip. The subject is of Somalian background and one of 11 siblings aged 2 to 22 years old living with his parents in an overcrowded residence. Examination of other family members revealed the presence of similar lesions in 2 younger sisters. An incisional biopsy confirmed the diagnosis of MEH histopathologically.

  18. Media richness and social information processing: rationale for multifocal continuing medical education activities.

    Science.gov (United States)

    Gilman, S C; Turner, J W

    2001-01-01

    Academic business communication has studied the results of media selection in organizations. Little of this work has been discussed in the context of continuing medical education (CME); however, it may apply to improving the design of educational activities. This article reviews literature on media richness and social information processing theories. The concept of media richness suggests that media choice results from a match between the objective characteristics of the medium and the content requirements of a message. In this context, media include face-to-face conversation and print and electronic media. Social information processing theory suggests that media selection is also based on participants' social norms for how information is communicated in their environment and the participants' familiarity with specific media types. Appraisal of CME with respect to these theories suggests that the complex relationship of CME content and CME participant environments invites the most effective strategies of multiple media experienced over time in what might be called multifocal continuing medical education.

  19. Time-Dependent Decline in Multifocal Electroretinogram Requires Faster Recording Procedures in Anesthetized Pigs

    DEFF Research Database (Denmark)

    Sørensen, Nina Buus; Christiansen, Anders Tolstrup; Kjær, Troels Wesenberg

    2017-01-01

    PURPOSE: The time-dependent effect of anesthetics on the retinal function is debated. We hypothesize that in anesthetized animals there is a time-dependent decline that requires optimized multifocal electroretinogram (mfERG) recording procedures. METHODS: Conventional and four-frame global-flash mf...... injection of tetrodotoxin (TTX) was given and the mfERG was rerecorded as described above. Data were analyzed using mixed models in SAS statistical software. RESULTS: Propofol significantly decreases the conventional and global-flash amplitudes over time. The only significant effect of isoflurane...... is sufficient in anesthetized pigs. Recordings should be obtained immediately after the induction of anesthesia. The effect of TTX is significant 15 minutes after injection, but is contaminated by the effect of anesthesia 90 minutes after injection. Therefore, the quality of mfERG recordings can be further...

  20. Multifocal Epithelial Hyperplasia of Oral Cavity Expressing HPV 16 Gene: A Rare Entity

    Directory of Open Access Journals (Sweden)

    M. P. V. Prabhat

    2013-01-01

    Full Text Available Focal epithelial hyperplasia is a rare contagious disease caused by human papilloma virus. Usually HPV involves either cutaneous or mucosal surfaces, whereas concomitant mucocutaneous involvement is extremely rare. We report such a unique case of multifocal epithelial hyperplasia involving multiple sites of oral cavity along with skin lesions in a 65-year-old female. We also discuss the probable multifactorial etiology and variable clinical presentations of the lesions, including evidence of HPV 16 expression, as detected by polymerase chain reaction. The present report illustrates the need for careful examination and prompt diagnosis of the disease, as it might be associated with high risk genotypes such as HPV 16 and 18.

  1. Multifocal Epithelioid Hemangioendothelioma Derived from the Spine Region: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    G. Kerry

    2012-02-01

    Full Text Available Background: Epithelioid hemangioendothelioma (EHE is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations. Case Report: We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found. Results: To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks. Conclusion: Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.

  2. Office immunotherapy in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy.

    Science.gov (United States)

    Dyck, Peter J; Taylor, Bruce V; Davies, Jenny L; Mauermann, Michelle L; Litchy, William J; Klein, Christopher J; Dyck, P James B

    2015-10-01

    Intravenous immunoglobulin [IVIg], plasma exchange [PE], and corticosteroids are efficacious treatment in chronic inflammatory demyelinating polyneuropathy [CIDP]. IVIg is effective in multifocal motor neuropathy [MMN]. NIS, NIS-weakness, sum scores of raw amplitudes of motor fiber (CMAPs) amplitudes, and Dyck/Rankin score provided reliable measures to detect and scale abnormality and reflect change; they are therefore ideal for office management of response-based immunotherapy (R-IRx) of CIDP. Using efficacious R-IRx, a large early and late therapeutic response (≥ one-fourth were in remission or had recovered) was demonstrated in CIDP. In MMN only an early improvement with late non-significant worsening was observed. The difference in immunotherapy response supports a fundamental difference between CIDP (immune attack on Schwann cells and myelin) and MMN (attack on nodes of Ranvier and axons).

  3. Multifocal lateral periodontal cysts: a report of 4 cases and review of the literature.

    Science.gov (United States)

    Siponen, Maria; Neville, Brad W; Damm, Douglas D; Allen, Carl M

    2011-02-01

    Lateral periodontal cyst (LPC) is a developmental jaw cyst of odontogenic origin. It has characteristic histopathologic features that are identical to those seen in the peripherally occurring gingival cyst of adults (GCA). The polycystic variant of LPC is termed the botryoid odontogenic cyst (BOC). The histogenetic origin of LPC is probably the rests of dental lamina in the alveolar bone. In the case of BOC, it might be that several adjacent epithelial rests simultaneously undergo cystic change and eventually form a polycystic lesion. Few previous examples of multifocal occurrence of LPC can be found in the literature. We report an additional 4 patients with this rare presentation of multiple, separate LPCs, and review the literature on this topic. Copyright © 2011 Mosby, Inc. All rights reserved.

  4. A Novel Segment Classification for Multifocal and Multicentric Breast Cancer to Facilitate Breast-Conservation Treatment.

    Science.gov (United States)

    Tan, Mona P

    2015-01-01

    Breast conservation treatment (BCT) is an appropriate alternative to mastectomy for the treatment of unifocal breast cancer. Multifocal and multicentric breast cancers (MFMCBC) challenge conventional indications for BCT and are often treated with mastectomy. Following progress in treatment strategies for unifocal tumors, there was a movement to evaluate the use of BCT for MFMCBC. Now a growing body of evidence from retrospective data has emerged, demonstrating acceptable local control and overall survival rates with BCT for MFMCBC. Prospective studies are needed to confirm these findings. One of the possible barriers to such trials is the absence of a standardized classification and nomenclature for MFMCBC at this point in time. A novel segment classification is presented in this article in an endeavor to overcome this deficiency and allow future work on this issue.

  5. Chronic recurrent multifocal osteomyelitis with an atypical presentation in an adult man

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Cheng William [University of California San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States); Cleveland Clinic, Lerner College of Medicine, Cleveland, OH (United States); Hsiao, Edward C. [University of California San Francisco, Division of Endocrinology and Metabolism, and the Institute for Human Genetics, Department of Medicine, San Francisco, CA (United States); Horvai, Andrew E. [University of California San Francisco, Department of Pathology, San Francisco, CA (United States); Link, Thomas M. [University of California San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States)

    2015-09-15

    We present the case of a 33-year-old man with no significant medical history who developed right scapular pain, left-sided sacroiliac joint pain, and lower back pain, and was eventually diagnosed with chronic recurrent multifocal osteomyelitis (CRMO). Imaging demonstrated multiple scattered T2-hyperintense lesions on MRI at the spine and the left SI joint, some of which progressed and one regressed in size on follow-up. Histopathology demonstrated only non-specific chronic inflammation compatible with CRMO. No evidence of infectious organisms or neoplastic processes was found. The pain was relapsing and remitting in nature. Laboratory investigations were notable for no evidence of hematologic malignancy or infection, but only a mild increase in alkaline phosphatase. This case highlights that CRMO, despite being thought of as a childhood-onset disease, can present in adults as well, and also provides illustrative examples of imaging and histological findings. (orig.)

  6. Rare lung manifestation of multifocal micronodular pneumocyte hyperplasia in a teenage girl with tuberous sclerosis complex

    Energy Technology Data Exchange (ETDEWEB)

    Ha, Seung Mi; Yoon, Hye Kyung; Lee, Seung Koo [Kangwon National University Hospital, Chuncheon (Korea, Republic of)

    2016-08-15

    Multifocal micronodular pneumocyte hyperplasia (MMPH) is a relatively rare pulmonary disorder that can be associated with tuberous sclerosis complex (TSC). It has been rarely reported in children or adolescents. MMPH is a hamartomatous process of the lung with multiple small nodules, composed of type II pneumocytes. Plain radiography and chest CT in MMPH may demonstrate numerous small nodules measuring 1-10 mm in diameters, distributed randomly throughout both lungs. If MMPH is an initial presentation of TSC, and unless we are familiar with this lung manifestation of TSC, radiologic findings can mimic miliary tuberculosis or metastatic disease. We report a teenage girl with TSC and histologically confirmed MMPH which mimicked miliary tuberculosis at the initial presentation.

  7. Welcome to epidemiology and health.

    Science.gov (United States)

    Choi, Bo Youl

    2009-10-29

    The Korean Society of Epidemiology publishes a scholarly journal titled 'Korean Journal of Epidemiology', which announces and discusses the results of epidemiological studies from the past 30 yr. Since its first publication in 1979, the journal has contributed to the advancement of epidemiology as well as the prevention and control of disease, and the promotion of health in Korea.In 2009, the editorial board has decided to publish the journal in English to contribute internationally, and change the journal's name. The new name of the journal is 'Epidemiology and Health'.The abstract and full text of articles will be published as an open access online journal, which will be posted onto the homepage (http://www.e-epih.org/) in real time for anyone in the world to access free of charge. Our editorial policy is that 'Epidemiology and Health' is open to every researcher in fields related to epidemiology, regardless of membership, his or her major and nationality.Editorials, lectures, review papers, original articles, epidemic and case investigations, brief communications and letters will be published to generate active discussion through the journal along with the publication of the papers.'Epidemiology and Health' welcomes articles from various fields of epidemiology, such as 1) infectious diseases epidemiology, 2) chronic diseases epidemiology, 3) nutritional epidemiology, 4) clinical epidemiology, 5) pharmacoepidemiology, 6) genetic or molecular epidemiology, 7) social epidemiology, 8) environmental or occupational epidemiology, 9) epidemiological methods and biostatistics, 10) disease prevention and control, 11) health promotion and, 12) all other fields related to epidemiology.

  8. A missense mutation in pstpip2 is associated with the murine autoinflammatory disorder chronic multifocal osteomyelitis.

    Science.gov (United States)

    Ferguson, Polly J; Bing, Xinyu; Vasef, Mohammed A; Ochoa, Luis A; Mahgoub, Amar; Waldschmidt, Thomas J; Tygrett, Lorraine T; Schlueter, Annette J; El-Shanti, Hatem

    2006-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder that primarily affects bone but is often accompanied by inflammation of the skin and/or gastrointestinal tract. The etiology is unknown but evidence suggests a genetic component to disease susceptibility. Although most cases of CRMO are sporadic, there is an autosomal recessive syndromic form of the disease, called Majeed syndrome, which is due to homozygous mutations in LPIN2. In addition, there is a phenotypically similar mouse, called cmo (chronic multifocal osteomyelitis) in which the disease is inherited as an autosomal recessive disorder. The cmo locus has been mapped to murine chromosome 18. In this report, we describe phenotypic abnormalities in the cmo mouse that include bone, cartilage and skin inflammation. Utilizing a backcross breeding strategy, we refined the cmo locus to a 1.3 Mb region on murine chromosome 18. Within the refined region was the gene pstpip2, which shares significant sequence homology to the PSTPIP1. Mutations in PSTPIP1 have been shown to cause the autoinflammatory disorder PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum and acne). Mutation analysis, utilizing direct sequencing, revealed a single base pair change c.293T --> C in the pstpip2 gene resulting in a highly conserved leucine at amino acid 98 being replaced by a proline (L98P). No other mutations were found in the coding sequence of the remaining genes in the refined interval, although a 50 kb gap remains unexplored. These data suggest that mutations in pstpip2 may be the genetic explanation for the autoinflammatory phenotype seen in the cmo mouse.

  9. Minus Piggyback Lens Overlaying ReSTOR® Multifocal Lens in High Myopia

    Directory of Open Access Journals (Sweden)

    Isha Gupta

    2013-06-01

    Full Text Available Background: We report the case of a 40-year-old female patient treated with implantation of the Acrysof® IQ ReSTOR® lens (Alcon, Fort Worth, Tex., USA with overlaying Acrysof EXpand® minus piggyback lens (Alcon. Methods: The patient had high myopia and was diagnosed with presbyopia and bilateral posterior subcapsular cataract. She desired to be spectacle-free and opted to undergo bilateral placement of the ReSTOR multifocal lens. The necessary intraocular lens (IOL power was +3.5 in the right eye and +4.0 in the left eye, though the range of commercially available ReSTOR lenses is +6.0 to +34.0 D. In order to achieve emmetropia in this case of high myopia, it was determined that an EXpand minus piggyback lens would be necessary. Results: Implantation of the ReSTOR lens with overlaying EXpand minus piggyback lens was performed successfully and without complication. At 5 months postoperatively, the patient had 20/20 uncorrected visual acuity in both eyes. She reported a high level of satisfaction and was able to return to her daily activities including reading and driving without spectacles. Conclusion: We report successful primary implantation of AcrySof EXpand minus piggyback lenses overlying the AcrySof IQ ReSTOR lens in a patient with high myopia. Long-term follow-up and further evaluation is necessary to establish piggyback IOL implantation with multifocal IOL as an accepted treatment for high myopia with presbyopia.

  10. The surgery and early postoperative radicular pain in cases with multifocal lumbar disc herniation

    Science.gov (United States)

    Ulutaş, Murat; Çınar, Kadir; Seçer, Mehmet

    2017-01-01

    Abstract Persistence of postoperative radicular pain after surgery for multifocal disc herniation (MFDH) is a clinical problem. This study aims to evaluate the effects of a combined treatment approach compared with unilateral stabilization on early postoperative radicular pain in patients with MFDH. Age, sex, level of operation, clinical findings, and radicular pain visual analogue scale (VAS) scores before surgery in the early postoperative period and at 3 months after surgery were retrospectively reviewed for 20 cases of multifocal lumbar disc herniation. The combined approach (translaminar and far lateral) was used for 13 cases. Seven cases underwent transforaminal lumbar interbody fusion (TLIF) and unilateral transpedicular stabilization following total facetectomy. The mean age of the sample was 49.4 ± 10.1 years and the female-to-male ratio was 8:12. The mean VAS scores for radicular pain in cases treated with the combined approach were 8.2, 4.07, and 2.3 in the preoperative and early postoperative periods and 3 months after surgery, respectively. The mean score for radicular pain improved by 50.4% in the early postoperative period and by 72% in the late postoperative period. The mean VAS scores for radicular pain in cases who underwent TLIF and unilateral stabilization after facetectomy were 8.4, 2.1, and 1.4 in the preoperative and early postoperative periods and 3 months after surgery, respectively. The mean VAS score for radicular pain improved by 75% in the early postoperative period and by 83.3% in the late postoperative period. The combined approach is an effective alternative in cases with MFDH. TLIF and unilateral segmental stabilization provide substantial decompression and eliminate mechanical compression by conserving the height of the intervertebral foramen in the event that sufficient decompression is unable to obtain. We suggest that elimination of chemical mediators, particularly those causing pain in the dorsal ganglion, contributes to

  11. Correlation between full-field and multifocal VEPs in optic neuritis.

    Science.gov (United States)

    Klistorner, Alexander; Fraser, Clare; Garrick, Raymond; Graham, Stuart; Arvind, Hemamalini

    2008-01-01

    To compare performance of multi-focal and full-field Visual Evoked Potentials (VEP) in patients with optic neuritis (ON). 26 patients with unilateral ON were enrolled. Multi-focal VEP (MF VEP) was recorded using AccuMaptrade mark system. Four bipolar channels were analysed. Full-field VEP (FF VEP) was performed according to ISCEV standard using ESPION with frontal-occipital electrode placement. Pattern-reversal protocol was implemented with check size of 60' and field of view of 30 degrees . For both tests amplitude and latency of affected eye were statistically different from non-affected eye. The asymmetry of amplitude and latency between two eyes was also very similar for both tests. Averaged Relative Asymmetry Coefficient of amplitude (RAC) for the FF VEP was 0.10 +/- 0.15 and for the MF VEP was 0.12 +/- 0.12 (P = 0.21, paired t-test). Averaged latency difference between affected and non-affected eyes was 13.0 +/- 12 ms for FF and 14.1 +/- 11.1 ms for MF VEPs (P = 0.14, paired t-test). Coefficient of correlation (r) of p100 component of the FF VEP and averaged MF VEP was 0.60 (P < 0.0001) for amplitude and 0.79 (P < 0.0001) for latency. Correlation improved when amplitude and latency asymmetry between two eyes was analysed (r = 0.81 and r = 0.92 respectively). Overall 73% of affected eyes were identified as abnormal by amplitude and/or latency of the FF VEP and 89% was considered abnormal when MF VEP was used. Analysis of individual cases revealed superior performance of MF VEP in detecting small or peripheral defects.

  12. Multifocal blue-on-yellow visual evoked potentials in early glaucoma.

    Science.gov (United States)

    Klistorner, Alexander; Graham, Stuart L; Martins, Alessandra; Grigg, John R; Arvind, Hemamalini; Kumar, Rajesh S; James, Andrew C; Billson, Francis A

    2007-09-01

    To determine the sensitivity and specificity of blue-on-yellow multifocal visual evoked potentials (mfVEPs) in early glaucoma. Cross-sectional study. Fifty patients with a confirmed diagnosis of early glaucoma and 60 normal participants. Black-and-white mfVEPs and blue-on-yellow mfVEPs were recorded using the Accumap version 2.0 (ObjectiVision Pty. Ltd., Sydney, Australia). All patients also underwent achromatic standard automated perimetry (SAP). Multifocal VEP amplitude and latency values in glaucoma patients were analyzed and compared with those of the normal controls. Based on the definition of visual field defect, in the group of glaucomatous eyes with SAP defects, amplitude of blue-on-yellow mfVEP was abnormal in all 64 cases (100% sensitivity), whereas black-and-white mfVEP missed 5 cases (92.2% sensitivity). Generally, larger scotomata were noted on blue-on-yellow mfVEP compared with black-and-white mfVEP for the same eyes. There was high topographic correspondence between SAP and amplitude of blue-on-yellow mfVEP and significant (P<0.0001) correlation between them (correlation coefficient, 0.73). Abnormal amplitude was detected in 3 of 60 eyes of control subjects (95% specificity). There was, however, no correlation between visual field defect and latency delay in glaucoma patients. Although there was a significant difference between averaged latency of control and glaucoma eyes, values considerably overlapped. The blue-on-yellow mfVEP is a sensitive and specific tool for detecting early glaucoma based on amplitude analysis.

  13. Clinical application of objective perimetry using multifocal visual evoked potentials in glaucoma practice.

    Science.gov (United States)

    Graham, Stuart L; Klistorner, Alexander I; Goldberg, Ivan

    2005-06-01

    To evaluate the role of objective perimetry using multifocal visual evoked potentials (mVEPs) in glaucoma practice, and to assess its utility in patients with inconclusive standard automated perimetry findings. Method A retrospective case series of 436 consecutive subjects referred for glaucoma investigation who underwent testing with the AccuMap V1.3 mVEP system (ObjectiVision Pty, Ltd, Sydney, New South Wales, Australia) within a defined 12-month period. Sensitivity was determined by comparing this testing with that of standard automated perimetry and that used in a subgroup in whom masked stereoscopic optic disc photographs were used as an alternative reference standard. Overall clinical diagnostic outcomes were assessed. The mVEP changes were correlated with the stage of disease and Humphrey mean deviation (r = 0.78). The overall sensitivity for detecting glaucoma with established subjective field loss was 97.5% (early glaucoma, 95.0%), whereas 92.2% of low-risk suspects had normal mVEPs. When masked disc assessment alone was used for diagnosis of abnormality, sensitivity of mVEP (80.6%) and Humphrey visual field results (81.9%) were similar, but mVEP specificity was better (89.2% vs 79.5%). The mVEP was particularly useful in assessing excessive subjective field loss (45 eyes) by showing a much closer correlation with the clinical picture. Multifocal VEP is an effective method for detecting visual field loss in glaucoma. It provides a valuable aid to the clinician in categorizing patients with unreliable, variable, unconfirmed, or excessive subjective field loss.

  14. Sensory loss in multifocal motor neuropathy: a clinical and electrophysiological study.

    Science.gov (United States)

    Lambrecq, Virginie; Krim, Elsa; Rouanet-Larrivière, Marie; Lagueny, Alain

    2009-02-01

    Some patients fulfilling the criteria for the diagnosis of multifocal motor neuropathy with conduction block (MMN-CB) at the onset of disease may subsequently develop a sensory loss associated with electrophysiological sensory abnormalities. The latter could represent an overlap between MMN-CB and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy. The objective was to specify the features of MMN-CB with sensory loss (MMN-CB-Se). Five patients in a series of 11 consecutive patients who fulfilled the criteria of the American Association of Neuromuscular and Electrodiagnostic Medicine for MMN-CB at the first examination and were treated periodically with intravenous immunoglobulin (IVIg) developed sensory loss in the course of the disease. In these five patients we compared the clinical, laboratory, and electrophysiological features found after the development of sensory loss with those at the first examination. The mean time to appearance of objective sensory signs was 7.2 years. In three of the five patients the sensory loss was preceded by intermittent paresthesias in the same nerve territories as the motor involvement. The most frequent electrophysiological abnormality was amplitude reduction of sensory nerve action potentials. There were no bilateral or symmetrical clinical and electrophysiological sensory abnormalities. Anti-GM1 IgM antibodies were positive in four patients. MMN-CB-Se could be an overlap between MMN-CB and MADSAM. It shares the distribution of the sensory disorders encountered in MADSAM, but it is closer to MMN-CB on clinical and therapeutic levels. Study of more patients would be useful to classify this subgroup more accurately.

  15. Visual outcomes after implantation of a novel refractive toric multifocal intraocular lens

    Directory of Open Access Journals (Sweden)

    Talita Shimoda

    2014-04-01

    Full Text Available Purpose: To assess the postoperative outcomes of a novel toric multifocal in traocular lens (IOL in patients with cataract and corneal astigmatism. Methods: This prospective nonrandomized study included patients with cataract, corneal astigmatism, and a motivation for spectacle independence. In all patients, a Rayner M-flex® T toric IOL was implanted in the capsular bag. Three months after surgery, the distance, intermediate, and near visual acuities; spherical equivalent; residual refractive astigmatism; defocus curve; and contrast sensitivity were evaluated. A patient satisfaction and visual phenomena questionnaire was administered to all patients. Results: Thirty-four eyes of 18 patients were included in this study. Three months after surgery, the mean corrected distance visual acuity (logMAR was 0.00 ± 0.08 at 6 m, 0.20 ± 0.09 at 70 cm, and 0.08 ± 0.11 at 40 cm. Uncorrected distance vision acuity was 20/40 or better in 100% eyes. The preoperative mean refractive cylinder (RC was -2.19 (SD: ± 0.53. After a 3-month follow-up, the average RC was -0.44 D (SD: ± 0.27; p<0.001. Contrast sensitivity levels were high. At the last follow-up, 87.5% patients were spectacle-independent for near, intermediate, and distance vision, and approximately 44% patients reported halos and glare. Conclusion: Toric multifocal IOL implantation in patients with cataract and corneal astigmatism using the Rayner M-flex® T toric IOL was a simple, safe, and accurate option. This technology provides surgeons with a feasible option for meeting patient expectations of an enhanced lifestyle resulting from decreased spectacle dependence.

  16. Epidemiology: Then and Now.

    Science.gov (United States)

    Kuller, Lewis H

    2016-03-01

    Twenty-five years ago, on the 75th anniversary of the Johns Hopkins Bloomberg School of Public Health, I noted that epidemiologic research was moving away from the traditional approaches used to investigate "epidemics" and their close relationship with preventive medicine. Twenty-five years later, the role of epidemiology as an important contribution to human population research, preventive medicine, and public health is under substantial pressure because of the emphasis on "big data," phenomenology, and personalized medical therapies. Epidemiology is the study of epidemics. The primary role of epidemiology is to identify the epidemics and parameters of interest of host, agent, and environment and to generate and test hypotheses in search of causal pathways. Almost all diseases have a specific distribution in relation to time, place, and person and specific "causes" with high effect sizes. Epidemiology then uses such information to develop interventions and test (through clinical trials and natural experiments) their efficacy and effectiveness. Epidemiology is dependent on new technologies to evaluate improved measurements of host (genomics), epigenetics, identification of agents (metabolomics, proteomics), new technology to evaluate both physical and social environment, and modern methods of data collection. Epidemiology does poorly in studying anything other than epidemics and collections of numerators and denominators without specific hypotheses even with improved statistical methodologies.

  17. Desempenho visual após implante de uma lente intraocular asférica multifocal difrativa Visual performance after implantation of an aspheric multifocal diffractive intraocular lens

    Directory of Open Access Journals (Sweden)

    Leonardo Akaishi

    2010-08-01

    Full Text Available OBJETIVO: Avaliar a acuidade visual a distancia, intermediária e perto após o implante de uma lente intraocular (LIO asférica multifocal difrativa AcrySof® ReSTOR® SN6AD1. MÉTODOS: Estudo prospectivo de 50 pacientes com catarata submetidos à facoemulsificação e implante de LIO AcrySof® ReSTOR®. Foram avaliados acuidade visual sem correção (AVSC, acuidade visual com correção (AVCC para longe, acuidade visual para perto corrigida para longe (AVPC e acuidade visual intermediária corrigida para longe (AVIC. Um questionário de satisfação e fenômenos visuais foi administrado ao final do estudo. RESULTADO: Após três meses de cirurgia, a média das acuidades (logMAR era: AVSC, 0,05 ± 0,07; AVCC, 0,00 ± 0,01; AVPC, 0,00 ± 0,0, e AVIC, 0,15 ± 0,05. A AVCC e AVPC era de 20/25 ou melhor em todos os pacientes, sendo a AVIC J3 ou melhor em 83% dos pacientes. Pacientes relataram excelente desempenho visual nas questões relacionadas a atividades para distância, intermediário, e perto com pouca ou nenhuma dificuldade. Nenhum paciente relatou halos ou 'glare' severos, sendo os mesmos caracterizados entre nenhum a moderado. CONCLUSÃO: A LIO asférica AcrySof® ReSTOR® SN6AD1 mostrou excelentes resultados na visão para longe, perto e intermediário, além de apresentar uma baixa incidência de fenômenos visuais.PURPOSE: To evaluate distance, intermediate, and near vision after aspheric multifocal diffractive AcrySof® ReSTOR® SN6AD1 intraocular lens (IOL implantation. METHODS: Prospective study of 50 patients with cataract that had phacoemulsification and AcrySof® ReSTOR® IOL implantation. Uncorrected distance vision acuity (UDVA, corrected distance visual acuity (CDVA, distance-corrected near visual acuity (DCNVA, and distance-corrected intermediate visual acuity (DCIVA were measured postoperatively. A patient-satisfaction and visual phenomena questionnaire was administered at the end of the study. RESULTS: Three months

  18. Evolution and social epidemiology.

    Science.gov (United States)

    Nishi, Akihiro

    2015-11-01

    Evolutionary biology, which aims to explain the dynamic process of shaping the diversity of life, has not yet significantly affected thinking in social epidemiology. Current challenges in social epidemiology include understanding how social exposures can affect our biology, explaining the dynamics of society and health, and designing better interventions that are mindful of the impact of exposures during critical periods. I review how evolutionary concepts and tools, such as fitness gradient in cultural evolution, evolutionary game theory, and contemporary evolution in cancer, can provide helpful insights regarding social epidemiology.

  19. Cancer Epidemiology Cohorts

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    Cohort studies are fundamental for epidemiological research by helping researchers better understand the etiology of cancer and provide insights into the key determinants of this disease and its outcomes.

  20. Epidemiology & Genomics Research Program

    Science.gov (United States)

    The Epidemiology and Genomics Research Program, in the National Cancer Institute's Division of Cancer Control and Population Sciences, funds research in human populations to understand the determinants of cancer occurrence and outcomes.

  1. Occupational cancer epidemiology.

    Science.gov (United States)

    Boffetta, Paolo

    2011-01-01

    Occupational cancer epidemiology has led to the identification of more than 40 agents, groups of agents, and exposure circumstances which cause cancer in humans. This evidence has been followed by preventive and control measures. There are four areas where occupational cancer epidemiology may contribute important results in the future: surveillance of workers exposed to carcinogens, identification of new carcinogens and target organs, study of interactions, and research on special exposure circumstances.

  2. Contribution of Computed Tomography Enema and Magnetic Resonance Imaging to Diagnose Multifocal and Multicentric Bowel Lesions in Patients With Colorectal Endometriosis.

    Science.gov (United States)

    Belghiti, Jeremie; Thomassin-Naggara, Isabelle; Zacharopoulou, Chrysoula; Zilberman, Sonia; Jarboui, Lamia; Bazot, Marc; Ballester, Marcos; Daraï, Emile

    2015-01-01

    To evaluate the diagnostic contribution of the computed tomography (CT) enema and magnetic resonance imaging (MRI) for multifocal (multiple lesions affecting the same segment) and multicentric (multiple lesions affecting several digestive segments) bowel endometriosis. Prospective cohort study (Canadian Task Force classification II-2). Eighty-five patients. Tenon University Hospital, Paris, France. All patients received a preoperative CT enema and underwent MRI interpreted by 2 radiologists. Patients underwent colorectal resection for colorectal endometriosis from February 2009 to November 2012. Nineteen patients (22%) had multifocal lesions, and 11 patients (13%) had multicentric lesions. Six patients (7%) had both multifocal and multicentric lesions. The sensitivity, specificity, and positive and negative likelihood ratios (LRs) of MRI for the diagnosis of multifocal lesions were 0.58, 0.84, 3.55, and 0.5, respectively. The sensitivity, specificity, and positive and negative LRs of the CT enema for the diagnosis of multifocal lesions were 0.64, 0.86, 4.56, and 0.4, respectively. The sensitivity, specificity, and positive LR of MRI for the diagnosis of multicentric lesions were 1, 0.88, and 8.4, respectively. The sensitivity, specificity, and positive and negative LRs of MRI for the diagnosis of multicentric lesions were 0.46, 0.92, 5.6, and 0.59, respectively. No difference was observed between MRI and the CT enema for the diagnosis of multifocal and multicentric colorectal endometriosis. The interobserver agreement was good for MRI and the CT enema (κ = 0.45 and 0.45) for multifocality, and it was poor for both MRI and the CT enema (κ = 0.32 and 0.34) for multicentricity. Both MRI and the CT enema were able to diagnose multifocal and multicentric bowel endometriosis with similar accuracy. Copyright © 2015. Published by Elsevier Inc.

  3. Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I

    Directory of Open Access Journals (Sweden)

    Rachna Rath

    2016-01-01

    Full Text Available Neurofibromatosis type 1 (NF1 is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO and extraosseous (EO neurofibromas is rare. The present case highlights the importance of imaging findings in the diagnosis and management of multifocal jaw, infratemporal, and parotid neurofibromas with muscular hypoplasia in an eight-year-old girl with NF1. Apart from orthopantomograms (OPG, three-dimensional computed tomography (3D CT and cross-sectional reformations were valuable in delineating the extent of the lytic lesion and identifying additional bony deformities of the mandible. Magnetic resonance imaging (MRI helped to identify the solid nature of the lesion and true extent of the soft tissue mass.

  4. Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I.

    Science.gov (United States)

    Rath, Rachna; Kaur, Sheetal; Baig, Shadab Ali; Pati, Punyashlok; Sahoo, Sonalisa

    2016-01-01

    Neurofibromatosis type 1 (NF1) is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO) and extraosseous (EO) neurofibromas is rare. The present case highlights the importance of imaging findings in the diagnosis and management of multifocal jaw, infratemporal, and parotid neurofibromas with muscular hypoplasia in an eight-year-old girl with NF1. Apart from orthopantomograms (OPG), three-dimensional computed tomography (3D CT) and cross-sectional reformations were valuable in delineating the extent of the lytic lesion and identifying additional bony deformities of the mandible. Magnetic resonance imaging (MRI) helped to identify the solid nature of the lesion and true extent of the soft tissue mass.

  5. Treatment of chronic immune-mediated neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and the Lewis-Sumner syndrome.

    Science.gov (United States)

    Sederholm, Benson H

    2010-09-01

    Current treatment approaches for the management of chronic immune-mediated peripheral neuropathies are reviewed, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), and the Lewis-Sumner syndrome (LSS). A summary of existing evidence for commonly used treatment modalities, such as corticosteroids, intravenous immune globulin (IVIG), and plasma exchange is provided. Evidence for the use of additional immunosuppressant and immunomodulatory agents is also reviewed.

  6. Advancing multifocal nonlinear microscopy: development and application of a novel multibeam Yb:KGd(WO4)2 oscillator.

    Science.gov (United States)

    Sheetz, Kraig E; Hoover, Erich E; Carriles, Ramón; Kleinfeld, David; Squier, Jeff A

    2008-10-27

    We present a novel Yb:KGd(WO(4))(2) oscillator design that generates six beams of temporally delayed, 253 fs, 11 nJ pulses. This allows multifocal nonlinear microscopy to be performed without the need for complicated optical multiplexers. We demonstrate our design with twelve simultaneously acquired two-photon, second-harmonic and/or third-harmonic images generated from six laterally separated foci.

  7. High TPOAb Levels (>1300 IU/mL) Indicate Multifocal PTC in Hashimoto's Thyroiditis Patients and Support Total Thyroidectomy.

    Science.gov (United States)

    Dong, Shuai; Xia, Qing; Wu, Yi-Jun

    2015-07-01

    We aimed to identify whether thyroid peroxidase antibodies (TPOAb) are indicative of multifocal papillary thyroid cancer (PTC) in Hashimoto's thyroiditis (HT) patients and may help to determine necessity for total thyroidectomy. Retrospective cohort study. Teaching hospital. A total of 808 consecutive patients with HT alone or with HT and unifocal or multifocal PTC were included. Preoperative thyroid function tests, TPOAb determination, preoperative ultrasonography, intraoperative frozen biopsy, and postoperative routine pathologic examination to confirm thyroid nodules were performed for all patients. Patients with nodules or malignancy potential on ultrasound and fine-needle aspiration cytology were included. Patients with hyperthyroidism, concomitant chronic disease, a history of other malignant tumors, or history of major diseases were excluded. All patients underwent surgery, and HT and PTC were confirmed by postoperative pathologic results. No significant differences were found in age and sex between groups (P > .05). TPOAb ≤1300 IU/mL were more prevalent in the HT + unifocal PTC group than in the other groups (99.57% vs 15.52% and 60.75%, P 1300 IU/mL were more prevalent in the HT + multifocal PTC group than in the other groups (84.48% vs 0.43% and 39.25%; P PTC group had higher percentages of patients with elevated thyroid-stimulating hormone and positive central lymph node (LN) metastasis (elevated thyroid-stimulating hormone: 8.7% vs 3.2% and 6.5%, P = .008; positive central LN metastasis: 74.57% vs 67.38% and 0%, P 1300 IU/mL) are definitive indicators of multifocal PTC in HT patients, which may support surgical treatment with total thyroidectomy. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  8. Multifocal visual evoked potential analysis of inflammatory or demyelinating optic neuritis.

    Science.gov (United States)

    Fraser, Clare L; Klistorner, Alexander; Graham, Stuart L; Garrick, Raymond; Billson, Francis A; Grigg, John R

    2006-02-01

    To determine the sensitivity of multifocal visual evoked potentials (mVEP) in optic neuritis of an inflammatory or demyelinating nature. Cross-sectional study. Sixty-four patients participated who had a confirmed diagnosis of optic neuritis (ON) (past and acute). Based on the McDonald multiple sclerosis (MS) criteria, 25 patients (27 eyes with ON) were deemed to have isolated optic neuritis and thus not have MS (i.e., the not-MS group), and 19 patients (24 eyes with ON) had a diagnosis of MS (i.e., the MS group). The remaining 20 patients (25 eyes with ON) were at a high risk of MS, but diagnostic evaluation was equivocal, and thus were classified as the possible MS group. A control group of 20 normal patients was enrolled. The mVEP test was performed using the Accumap. All ON patients had recent magnetic resonance imaging scans of the brain and spinal cord. Multifocal visual evoked potentials amplitude and latency values were analyzed within each group and were compared with the normal controls. No abnormality was recorded on mVEP in the control group. Of all the ON eyes, 74 (97.3%) were abnormal on mVEP testing. Amplitude values were abnormal in 92.6% of not-MS eyes, 92.0% of possible MS eyes, and 100% of those with MS, and latency was abnormal in 33.3%, 76.0%, and 100%, respectively. There was a significant difference in the mVEP latency z-scores among all ON groups (P<0.01; Kruskal-Wallis test). Although distribution graphs of latency z-scores in the not-MS and MS groups had single peaks and were clearly separate from each other, the latency z-score distribution within the possible MS group in postacute patients was bimodal, with each peak corresponding to the distribution of the not-MS and MS group, respectively. The mVEP latency z-scores had a sensitivity and specificity of 100% in detecting patients with ON due to MS when compared with normal patients. The mVEP test is a sensitive and specific tool for detecting optic neuritis. There was a significant

  9. Effect of check size and stimulation rate on blue-yellow multifocal visual evoked potentials.

    Science.gov (United States)

    Martins, Alessandra; Klistorner, Alexander; Graham, Stuart; Billson, Frank

    2004-06-01

    To determine the effect of different stimulus frame rates and check sizes on blue-yellow multifocal visual evoked potentials (mVEP). Subjects were examined at the Save Sight Institute at the University Sydney. Experiment 1 involved five adult subjects who underwent binocular stimulation by the Accumap multifocal objective perimeter. The eyes were stimulated with a cortically scaled dartboard pattern consisting of isoluminant blue and yellow checks. These were arranged in three concentric rings extending to an eccentricity of 26 degrees in the visual field. The stimulus pattern was driven by binary sequences resulting in pseudorandom binary exchange of two opposite checkerboard patterns at each of the 32 sites in the visual field. The mVEP were recorded at two different rates of display of the pattern stimulus. In experiment 2, mVEP were tested on 10 normal subjects. Each of the 36 stimulation sites contained a checkerboard pattern of 20, 30, 42 or 56 checks/site, the stimulation pattern was displayed at the optimum rate found in experiment 1. The size of the checks was inversely proportional to the number of checks per site. In experiment 1, the slow frame rate significantly increased the average amplitude throughout the field tested by 50 +/- 10.1% (P = 0.001). Latency was significantly shortened by 6.3% (P < 0.01). In experiment 2, the average amplitude peaked at 30 checks per segment; however, this was only calculated to be significantly different from the smallest check size (F(crit range 4,27) = 0.09 P < 0.05, anova, Tukey's T method). A similar difference was found in ring 1 (F(crit range 4,27) = 0.09, P < 0.05, anova, Tukey's T method). In ring 2, however, there was also a significant difference between 56 checks and 20, 30 and 42 (F(crit range 4,27) = 0.09, anova, P < 0.05). Altering the check sizes did not significantly affect the amplitudes in ring 3. The latencies were not significantly modified by altering check size at any eccentricity. These findings

  10. Resection And Reconstructive-plastic Surgery In Treatment Of Extended And Multifocal Cicatrical Tracheal Stenosis

    Directory of Open Access Journals (Sweden)

    Otabek Eshonkhodjaev

    2015-12-01

    Full Text Available Background. Main issues of treatment of patients with cicatricial stenosis of trachea (CST are to increase its efficacy and safety, as well as to determine indications and contraindications for circular resection and reconstructive interventions on the trachea in patients with extended lesions of trachea, with stenoses involving subvocal cords part of larynx and well as multifocal narrowing of the trachea which could improve treatment results and quality of life of patients, to gain recovery and reduce disability and mortality.Material and methods. One hundred two patients aged from 11 to 62 years with CST were surgically treated. Stenotic site length in patients varied from 0,3 to 7 cm. Most frequently (64,2%, CST length of more than 2 cm was observed. In critical and decompensated stenosis with diameter of CST up to 5 mm with the threat of asphyxiation by the first stage we used endoscopic laser-and electric destruction of constriction followed by restricted zone bouging. If long-term rehabilitation was necessary and in case of absence of the conditions to the implementation of circular tracheal resection, we used tracheal stents. Circular resection of the trachea was performed in 24 patients. In case of contraindications, 28 patients underwent reconstructive plastic surgery with dissection the stenosis, excision of scar tissue and formation of trachea lumen in T-tube. After removing T-tube plasty of anterior tracheal wall and of soft tissues of the neck defects was performed using local tissues and grafts with microsurgical techniques.Results. Long-term results of treatment evaluated and studied in a period of 6 months to 5 years in 89 (87.3% patients. 13 patients continues to one of the stages of complex treatment. Еhe patients are under dynamic endoscopic control. Good results were achieved in the treatment of 71 patients (79.8%, satisfactory - in 12 (13.4%, unsatisfactory results in 6 (6.7% patients.Conclusions. 1. Patients with CST

  11. Implantation of multifocal intraocular lenses using a magnification strategy in cataractous eyes with age-related macular degeneration.

    Science.gov (United States)

    Gayton, Johnny L; Mackool, Richard J; Ernest, Paul H; Seabolt, ReBecca A; Dumont, Susan

    2012-03-01

    To examine visual function after targeting -2.0 diopter (D) spherical equivalent (SE) when implanting a multifocal intraocular lens (IOL) in eyes with cataract and age-related macular degeneration (AMD). Three private practices. Case series. Lenses of cataractous eyes with AMD were replaced with the Acrysof Restor SN60D3 multifocal IOL, targeting an SE of -2.0 D, which yielded +5.2 D near addition. Near and distance visual acuities were examined. Patients completed a visual function questionnaire preoperatively and 6 months postoperatively. At 6 months, 13 patients with 20 eligible eyes were examined. The uncorrected near visual acuity improved in 18 eyes (90%) and was unchanged in 2 eyes. The corrected distance visual acuity improved in 14 eyes (70%), was unchanged in 4 eyes (20%), and decreased (≤ 3 lines) in 2 eyes (10%). All vision-related questionnaire items improved. For cataractous eyes with AMD, replacing the crystalline lens with this myopia-targeted multifocal IOL improved or maintained near vision without severely compromising distance vision. Drs. Mackool and Ernest are consultants to Alcon. Dr. Mackool is an inventor in the patent of the IOLs related to this strategy. No other author has a financial or proprietary interest in any material or method mentioned. Copyright © 2012 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.

  12. Patients' satisfaction and spectacle independence after cataract surgery with multifocal intraocular lens implantation in a tertiary care hospital.

    Science.gov (United States)

    Baig, Rashid; A Chaudhry, Tanveer; Kukreja, Saajan; Shakil, Sidra; Ahmad, Khabir

    2016-06-01

    This single group cohort study, undertaken at Aga Khan University Hospital, Karachi, from Jan 2012 to September 2013, assessed patients' satisfaction, spectacle independence and visual disturbance after implantation of multifocal IOLs. A total of 39 patients who underwent bilateral cataract surgery with multifocal IOL implantation were included for telephonic interviews. The mean age of participants was 55.5 ± 8.1 years. The mean follow up time was 9.60 ± 3.19 months. The overall satisfaction rate after surgery was found to be 84.6% and there was no statistically significant association of satisfaction with gender, age or education. In all 84.6%, 94.9% and 84.6% people had spectacle independence for far, intermediate and near, respectively. More than half of participants (59%) had some degree of visual disturbance. In this study, multifocal IOL implantation provided high levels of satisfaction and spectacle independence despite notable risk of halo or glare symptoms at night. Therefore, careful patient selection and preoperative counselling regarding possible risks and benefits is recommended.

  13. Visual and optical performance of diffractive multifocal intraocular lenses with different haptic designs: 6 month follow-up.

    Science.gov (United States)

    Wang, Mengmeng; Corpuz, Christine Carole C; Fujiwara, Megumi; Tomita, Minoru

    2014-01-01

    To evaluate and compare the visual acuity outcomes and optical performances of eyes implanted with two diffractive multifocal intraocular lens (IOL) models with either a plate haptic design or a modified-C design. This retrospective study comprised cataract patients who were implanted with either a plate haptic multifocal IOL model (Acriva(UD) Reviol BB MFM 611 [VSY Biotechnology, Amsterdam, the Netherlands], group 1) or a modified-C haptic multifocal IOL model (Acriva(UD) Reviol BB MF 613 [VSY Biotechnology, Amsterdam, the Netherlands], group 2) between June 2012 and May 2013. The 6 month postoperative visual acuity, refraction, defocus curve, contrast sensitivity, and wave-front aberration were evaluated and compared between these eyes, using different IOL models. One hundred fifty-eight eyes of 107 patients were included in this study. Significant improvement in visual acuities and refraction was found in both groups after cataract surgery (P0.05). However, the ocular higher-order aberrations in group 2 were significantly greater than in group 1 (Phaptic design resulted in better optical performances than that with the modified-C haptic design.

  14. Choroidal neovascularisation on optical coherence tomography angiography in punctate inner choroidopathy and multifocal choroiditis.

    Science.gov (United States)

    Levison, Ashleigh L; Baynes, Kimberly M; Lowder, Careen Y; Kaiser, Peter K; Srivastava, Sunil K

    2017-05-01

    To describe the findings seen on optical coherence tomography angiography (OCTA) in patients with punctate inner choroidopathy (PIC) and multifocal choroiditis and panuveitis (MCP) complicated by choroidal neovascular membranes. This was an Institutional Review Board-approved prospective, descriptive case series. 12 patients with PIC and MCP complicated by choroidal neovascularisation (CNV) were included. Each patient underwent slit-lamp examination by a uveitis specialist followed by conventional spectral domain OCT imaging of the macula. OCTA images of the macula were then obtained. 12 patients were enrolled in the study, out of which 9 patients were followed longitudinally. CNV was identified in 11 of the 12 patients. In all patients where fluorescein angiography (FA) was inconclusive for presence of CNV, OCTA identified CNV. Various lesions on OCT suggestive of activity correlated with changes in the vascular structure of OCTA to confirm suspicion of clinical activity. In patients with PIC and MCP complicated by CNV, OCTA successfully identified underlying CNV. Given the difficulty of differentiating inflammatory lesions from early CNV on OCT and FA, OCTA may provide a valuable method of monitoring patients with posterior uveitis highly correlated with development of CNV. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  15. Comprehensive treatment of a functional pancreatic neuroendocrine tumor with multifocal liver metastases.

    Science.gov (United States)

    Wang, Wei; Seeruttun, Sharvesh Raj; Fang, Cheng; Zhou, Zhiwei

    2014-08-01

    A 64-year-old man was admitted to the Sun Yat-Sen University Cancer Center with chief complaints of recurrent abdominal pain and diarrhea for about 3 years and with a history of surgical repair for intestinal perforation owing to stress ulcer. Positron emission tomography (PET)/computed tomography (CT) demonstrated a primary tumor on the pancreatic tail with multifocal liver metastases. Pathological and immunohistochemistry staining revealed the lesion to be a pancreatic neuroendocrine tumor (pNET). According to the latest World Health Organization (WHO, 2013) classification, the tumor was classified as stage IV functional G1 pNET. After referral to the multidisciplinary treatment board (MDT), the patient was started on periodic dose of omeprazole, somatostatin analogues and Interferon α (IFNα) and had scanning follow-ups. Based upon the imaging results, CT-guided radioactive iodine-125 ((125)I) seeds implantation therapy, radiofrequency ablation therapy (RFA) or microwave ablation technique were chosen for the treatment of the primary tumor. Transarterial chemoembolization (TACE), RFA and microwave ablation techniques were decided upon for liver metastases. The patient showed beneficial response to the treatment with clinically manageable low-grade side effects and attained partial remission (RECIST criteria) with a good quality of life.

  16. Diffuse and multifocal nephrogenic adenoma with Familial Mediterranean Fever: a case report with molecular study.

    Science.gov (United States)

    Ishikawa, Noriyoshi; Amano, Chika; Taketani, Takeshi; Kumori, Koji; Harada, Yuji; Hiraiwa, Hisayuki; Itamura, Kayoko; Maruyama, Riruke

    2015-07-16

    Nephrogenic adenoma, also referred to nephrogenic metaplasia, is a benign proliferative lesion of urothelium, usually associated with chronic physical stimuli or inflammation. Familial Mediterranean fever is an inherited autosomal recessive disease characterized by recurrent short episodes of fever. The site of mutation is found in MEFV gene which controls inflammatory responses. We have experienced a case of nephrogenic adenoma in a 16-year-old girl with Familial Mediterranean Fever, showing proliferative lesions diffusely in the urinary bladder and multifocally in the other parts of urinary tract. These lesions disappeared after colchicine treatment. We searched for MEFV gene mutation using the specimen from the resected urinary bladder and detected heterozygous mutation of E148Q. There is a possibility that control of inflammation caused by the surgery for vesicoureteral reflux in the local site didn't work well on the background of heterozygous mutation of MEFV gene, and as a result, nephrogenic adenoma appeared. This is the first report of a combination of two rare diseases. We have to be aware that nephrogenic adenoma can occur in association with Familial Mediterranean Fever, and the former condition should be taken into consideration when rendering a correct pathological diagnosis.

  17. Acute Posterior Multifocal Placoid Pigment Epitheliopathy as the Initial Manifestation of Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Adil Darugar

    2011-01-01

    Full Text Available Purpose: To report an undiagnosed case of systemic sarcoidosis manifesting with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE. Case Report: A 26-year-old Caucasian man was referred for management of unilateral visual loss together with a paracentral scotoma developing 2 weeks after a flu-like syndrome. Clinical signs and ancillary diagnostic investigations suggested APMPPE. Laboratory tests demonstrated elevated serum angiotensin converting enzyme and lysozyme levels. Chest CT-scan disclosed moderate hilar lymph node calcifications but QuantiFERON-TB gold test was negative and bronchoalveolar lavage and biopsies were unremarkable. Accessory salivary gland biopsy disclosed epithelioid and gigantocellular granuloma formation without caseum, confirming a diagnosis of sarcoidosis. The fellow eye was involved a few days later and the patient complained of dyspnea. Echocardiography disclosed severe granulomatous myocardial infiltration and high dose corticosteroids and intravenous cyclophosphamide were initiated. Systemic treatment controlled both cardiac and ocular lesions, and was tapered accordingly. Conclusion: The constellation of "white dot syndromes" and systemic symptoms necessitates a general work-up to exclude granulomatous disorders such as sarcoidosis or tuberculosis. Delayed diagnosis of cardiac sarcoidosis may have life-threatening consequences and the ophthalmologist may be the first physician to diagnose the condition.

  18. LGI1-antibody encephalitis is characterised by frequent, multifocal clinical and subclinical seizures.

    Science.gov (United States)

    Aurangzeb, Sidra; Symmonds, Mkael; Knight, Ravi K; Kennett, Robin; Wehner, Tim; Irani, Sarosh R

    2017-08-01

    To describe clinical and electrographic characteristics of seizures LGI1-antibody encephalitis, and their correlations with two-year outcomes. Video-electroencephalography recordings were performed on a cohort of 16 consecutive patients with LGI1-antibodies from two UK neuroscience-centers over five-years. From 14 of 16 patients (13 males; age-range 53-92years), 86 faciobrachial dystonic seizures were recorded at a median frequency of 0.4 per hour (range 0.1-9.8), and ictal EEG changes accompanied 5/86 events. In addition, 11/16 patients showed 53 other seizures - subclinical (n=18), motor (n=16), or sensory (n=19) - at a median of 0.1 per hour (range 0.1-2) associated with temporal and frontal discharges. The sensory events were most commonly thermal sensations or body-shuddering, and the motor events were frequently automatisms or vocalisations. Furthermore, multifocal interictal epileptiform discharges, from temporal, frontal and parietal regions, and interictal slow-wave activity were observed in 25% and 69% of patients, respectively. Higher observed seizure frequency correlated with poorer functional recovery at two-years (p=0.001). Multiple frequent seizure semiologies, in addition to numerous subclinical seizures and interictal epileptiform discharges, are hallmarks of LGI1-antibody encephalitis. High overall seizure frequency may predict more limited long-term recovery. These observations should encourage closer monitoring and proactive treatment of seizure activity in these patients. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  19. Sustained multifocal attentional enhancement of stimulus processing in early visual areas predicts tracking performance.

    Science.gov (United States)

    Störmer, Viola S; Winther, Gesche N; Li, Shu-Chen; Andersen, Søren K

    2013-03-20

    Keeping track of multiple moving objects is an essential ability of visual perception. However, the mechanisms underlying this ability are not well understood. We instructed human observers to track five or seven independent randomly moving target objects amid identical nontargets and recorded steady-state visual evoked potentials (SSVEPs) elicited by these stimuli. Visual processing of moving targets, as assessed by SSVEP amplitudes, was continuously facilitated relative to the processing of identical but irrelevant nontargets. The cortical sources of this enhancement were located to areas including early visual cortex V1-V3 and motion-sensitive area MT, suggesting that the sustained multifocal attentional enhancement during multiple object tracking already operates at hierarchically early stages of visual processing. Consistent with this interpretation, the magnitude of attentional facilitation during tracking in a single trial predicted the speed of target identification at the end of the trial. Together, these findings demonstrate that attention can flexibly and dynamically facilitate the processing of multiple independent object locations in early visual areas and thereby allow for tracking of these objects.

  20. Aspherical Lens Design Using Genetic Algorithm for Reducing Aberrations in Multifocal Artificial Intraocular Lens

    Directory of Open Access Journals (Sweden)

    Chih-Ta Yen

    2015-09-01

    Full Text Available A complex intraocular lens (IOL design involving numerous uncertain variables is proposed. We integrated a genetic algorithm (GA with the commercial optical design software of (CODE V to design a multifocal IOL for the human eye. We mainly used an aspherical lens in the initial state to the crystalline type; therefore, we used the internal human eye model in the software. The proposed optimized algorithm employs a GA method for optimally simulating the focusing function of the human eye; in this method, the thickness and curvature of the anterior lens and the posterior part of the IOL were varied. A comparison of the proposed GA-designed IOLs and those designed using a CODE V built-in optimal algorithm for 550 degrees myopia and 175 degrees astigmatism conditions of the human eye for pupil size 6 mm showed that the proposed IOL design improved the spot size of root mean square (RMS, tangential coma (TCO and modulation transfer function (MTF at a spatial frequency of 30 with a pupil size of 6 mm by approximately 17%, 43% and 35%, respectively. However, the worst performance of spherical aberration (SA was lower than 46%, because the optical design involves a tradeoff between all aberrations. Compared with the traditional CODE V built-in optimal scheme, the proposed IOL design can efficiently improve the critical parameters, namely TCO, RMS, and MTF.

  1. Multifocal fibrosing thyroiditis and its association with papillary thyroid carcinoma using BRAF pyrosequencing.

    Science.gov (United States)

    Frank, Renee; Baloch, Zubair W; Gentile, Caren; Watt, Christopher D; LiVolsi, Virginia A

    2014-09-01

    Multifocal fibrosing thyroiditis (MFT) is characterized by numerous foci of fibrosis in a stellate configuration with fibroelastotic and fibroblastic centers entrapping epithelial structures. MFT has been proposed as a risk factor for papillary thyroid carcinoma (PTC) development. We attempted to identify whether MFT showed such molecular changes and could possibly be related to PTC. We identified seven cases of PTC with MFT in our institutional pathology database and personal consult service of one of the authors (VAL) for the years 1999 to 2012. Areas of PTC, MFT, and normal tissue were selected for BRAF analysis. Macro-dissection, DNA extraction and PCR amplification, and pyrosequencing were performed to detect BRAF mutations in codon 600. All of the MFT lesions and normal thyroid tissue were negative for BRAF mutations. Of the seven PTCs analyzed, five (71 %) were negative for BRAF mutations, while two cases were positive. In our study, none of the MFT lesions harbored BRAF mutations, whereas 29 % (two of seven) PTCs in the same gland were positive. Hence, in this small study, we found no evidence that the MFT lesion is a direct precursor to PTC. It is likely an incidental bystander in the process and a reflection of the background thyroiditis.

  2. Multifocal array with controllable orbital angular momentum modes by tight focusing

    Science.gov (United States)

    Deng, Duo; Li, Yan; Han, Yanhua; Ye, Jingfu; Guo, Zhongyi; Qu, Shiliang

    2017-01-01

    Multifocal spots arrays with controllable three-dimensional (3D) position, orbital angular momentum (OAM) and the number of focal planes along longitudinal direction have been proposed and investigated by using a special designed phase-only multi-zone plate at the back aperture of a high numerical aperture (NA) objective. By using Debye diffraction integral, the focusing field could be rewritten as a fast Fourier transform (FFT), which is the theory and principle for generating the controllable OAM-modes focusing spots. According to this theory, a hybrid multi-zone plate at the back aperture of a high NA objective is designed for the illuminations of the radially polarized Bessel-Gaussian beam. Each focusing spot in the focal plane is generated by independent parts, so the multiplexing OAM-modes could be realized by focusing two or more spots at the same position with different orthogonal eigenstates. It is a practical and flexible OAM multi-focus technique with dynamically controllable 3D position, number of focal planes, corresponding topological charges and focal lengths to meet different requirements, such as 3D optical data storage, air-core fiber coupler and parallel optical manipulation.

  3. Multifocal hepatoblastoma in a 6-month-old girl with trisomy 18: a case report

    Directory of Open Access Journals (Sweden)

    Kitanovski Lidija

    2009-06-01

    Full Text Available Abstract Introduction Edward's syndrome (trisomy 18 is a rare entity with a reported incidence of 1/3000 to 1/7000 births. Less than 10% of patients survive beyond the first year of life, which may influence the fact that malignant tumors are rarely reported in association with this syndrome. Case presentation The authors report a rare case of a 6-month-old girl with trisomy 18 and multifocal hepatoblastoma. The course of the disease, autopsy results and review of the literature are presented. Conclusion Our case represents the seventh published case of hepatoblastoma in a patient with trisomy 18. All of the seven published cases were women, possibly due to the high preponderance of females among the children with Edward's syndrome and longer survival of females with trisomy 18 compared to males. Since both trisomy 18 and hepatoblastoma are rare conditions, the probability that a child with trisomy 18 will independently develop a hepatoblastoma is very low. Therefore, we believe that the existence of these cases in children with trisomy 18 indicates a significant association. It can be assumed that trisomy 18 potentiates the development of hepatoblastoma. Careful clinical and post-mortem studies are needed to recognize the real frequency of hepatoblastoma in children with trisomy 18, who might die from different causes with unrecognizable hepatoblastoma.

  4. Successful treatment of chronic recurrent multifocal osteomyelitis with indomethacin: a preliminary report of five cases.

    Science.gov (United States)

    Abril, Juan Carlos; Ramirez, Ana

    2007-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is a disease of children and young adults. Clinically, the disease is characterized by the insidious onset of local pain and swelling in affected bones. Its course is one of intermittent periods of exacerbation and remission with successive bones affected. The pathogenesis of CRMO remains unknown, although an autoinflammatory disorder may be the cause, with inflammation of bone. This lesion is radiologically characterized as multiple lucencies surrounded by defined zones of patchy but dense sclerosis, cortical thickening from periosteal new bone formation, and increased bone size with different bones involved. Multiple therapeutic regimens had shown only a partial or temporary response. Because indomethacin has been successfully applied in inhibition of ossification and inflammatory processes, we initiated therapy with indomethacin in patients with CRMO. We report on the cases of 5 patients who responded dramatically to treatment with indomethacin. All underwent progressive clinical improvement (mean, 2.8 months). Radiological lesions disappeared after a mean period of 10.5 months. In 1 case where treatment was started late, small osteolytic zones persisted but with no clinical consequences. There were no additional recurrences or new bones affected during follow-up period (mean, 4 years). Our observation indicates that indomethacin may be an effective treatment for CRMO.

  5. Perspectives of Cosmesis following Breast Conservation for Multifocal and Multicentric Breast Cancers

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    Mona P. Tan

    2015-01-01

    Full Text Available Background. Contemporary data suggest that breast conservation treatment (BCT for multifocal and multicentric breast cancer (MFMCBC may be appropriate with noninferior local control rates. However, there is a paucity of data to evaluate patient’s satisfaction with cosmetic outcomes after BCT for MFMCBC. This study was performed to bridge this information gap. Methods. All patients treated at the authors’ healthcare facility were included in the study. Patients with MFMCBC who were assessed to be eligible for BCT underwent tumour resection using standard surgical techniques with direct parenchymal closure through a single incision. After at least three years of follow-up, they were invited to participate in a survey regarding their cosmetic outcomes. Results. Of a total of 160 patients, 40 had MFMCBC, of whom 34 (85% underwent successful BCT. Five-year cancer-specific survival and disease-free survival were 95.7%. Twenty of the 34 patients responded to the survey. No patient rated her cosmetic outcome as “poor.” Analysis indicated low agreement between patients’ self-assessment and clinician-directed evaluation of aesthetic results. Conclusion. BCT for MFMCBC is feasible with acceptable survival and cosmetic outcomes. However, there appears to be a disparity between patient and clinician-directed evaluation of cosmetic results which warrant further research.

  6. Multifocal neuropathic arthropathy in patient with undiagnosed neurosyphilis: A case report.

    Science.gov (United States)

    Kim, Chang-Wan; Lee, Chang-Rack

    2017-06-01

    Although the prevalence of syphilis has decreased significantly, syphilis is still a common cause of neuropathic arthropathy. Tabetic arthropathy means progressive painless joint destruction that is related to neurosensory deficits caused by syphilis. In general, lower limb joints are involved and gradual swelling and instability of the involved joints are observed. Diagnosis of tabetic arthropathy is difficult as its clinical presentation is not specific and differential diagnosis is wide ranging. Hence, diagnosis of tabetic arthropathy requires clinical suspicion and an appropriate serological test. Laboratory tests for the diagnosis of syphilis include nontreponemal tests and treponemal tests. Conventionally, a nontreponemal test such as the Venereal Disease Research Laboratory test or the Rapid Plasma Reagin test is performed first as a screening test for syphilis, followed by a treponemal test to confirm the positive response found in the screening test. However, the sensitivity and specificity of the serum nontreponemal and treponemal tests for the diagnosis of syphilis are different based on the test type and the syphilis stages. We herein report a case of multifocal neuropathic arthropathy found in a patient whose syphilis was not diagnosed due to a nonreactive screening test result.

  7. [Acute posterior multifocal placoid pigment epitheliopathy. A rare cause of ischaemic stroke].

    Science.gov (United States)

    del Saz-Saucedo, Pablo; Alfaya-Muñoz, Laura Blanca; Recio-Bermejo, Marta; Lara-Medina, Francisco Javier; García-Chiclano, Amalia; Ortega-León, Teresa; Rueda-Medina, Ignacio; Domínguez-Fernández, María José; Madrid-Muñiz, Carmen; Franco-Huerta, María

    2013-06-01

    Introduccion. La epiteliopatia pigmentaria placoide multifocal posterior aguda (EPPMPA) es una enfermedad inflamatoria rara, generalmente de etiologia indeterminada, de la coriocapilar, el epitelio pigmentario y la retina externa. Afecta predominantemente a pacientes jovenes y en algunos casos puede involucrar al sistema nervioso central en forma de ictus o de meningoencefalitis. Presentamos el caso clinico de una mujer joven con EPPMPA complicada con ictus e hipertension intracraneal. Caso clinico. Mujer de 16 anos que comienza con cefalea intensa sugestiva de hipertension intracraneal, asi como con un deficit agudo hemisferico izquierdo. La resonancia magnetica craneal ponia de manifiesto lesiones embolicas o vasculiticas en diferentes territorios. No se evidenciaron datos de meningoencefalitis en el estudio del liquido cefalorraquideo, pero si de hipertension intracraneal asociada. La presencia de lesiones muy especificas en el polo ocular posterior permitio el diagnostico de EPPMPA complicada con ictus isquemico, probablemente por mecanismo vasculitico. Un amplio estudio etiologico fue negativo para identificar un factor desencadenante claro del proceso. Se inicio tratamiento corticoideo con buena evolucion clinica y radiologica. Conclusiones. La EPPMPA es una entidad rara que generalmente entrana buen pronostico; sin embargo, en algunos casos puede complicarse con afectacion del sistema nervioso central, y el ictus isquemico secundario a vasculitis es la complicacion mas grave. Ante un paciente joven con ictus que presente sintomatologia visual y lesiones coriorretinianas, debe considerarse la EPPMPA en su diagnostico etiologico.

  8. Chronic Multifocal Inflammation of the Alveolar Bone Mimicking Malignancy: A Case Report

    Directory of Open Access Journals (Sweden)

    Shahidi Sh.

    2012-03-01

    Full Text Available ronic inflammation of the alveolar bone is a great clinical and radiologic mimic, which merits recognition by the clinician and pathologist. The patient can thus be reassured of the proper early treatment and a favorable prognosis. Occasionally, it is difficult to differentiate inflammatory lesions from malign-ant tumors. The aim of this report is to present a case with an inflammatory lesion mimicking malignant condition.We report a 19-year-old male complaining of rapid onset gingival swelling of the right side of both jaws and looseness of the right upper molar teeth in 20 days. Based on the acute onset of the gingival hyperplasia, severe looseness of the affected teeth especially in the maxilla, and the patient's age, multifocal rapid growing malignant condition was not ruled out. The lesion was misdiagnosed as a malignant condition by clinical and radiographic examination. The whole body bone scan showed no significant increased uptake in the right oral cavity compatible with no active bony pathology. The surgical pathology findings of the lesion showed severe chronic inflammation with surface epithelial hyperplasia.The initial diagnosis of the lesion was malignant condition but it was ruled out by bone scan and histological appearance.

  9. Multifocal VEP and OCT in optic neuritis: a topographical study of the structure-function relationship.

    Science.gov (United States)

    Klistorner, Alexander; Arvind, H; Nguyen, T; Garrick, R; Paine, M; Graham, S; O'Day, J; Yiannikas, C

    2009-04-01

    To investigate topographical relationship between amplitude of multifocal visual evoked potentials (mfVEP) and retinal nerve fibre layer (RNFL) thickness following acute optic neuritis (ON). Fifty patients with a clinical diagnosis of acute unilateral ON between 6 and 36 months prior to the study and 25 age-matched controls underwent mfVEP testing (Accumap V 2.1, ObjectiVision Pty Ltd, Sydney, Australia) and OCT imaging (fast RNFL protocol, Stratus, software version 3.0, Carl Zeiss Meditec, Inc., Dublin, CA). RNFL thickness and mfVEP amplitude were measured for upper, temporal and lower retinal sectors and corresponding areas of the visual field in affected eyes of ON patients and control eyes. Inter-eye asymmetry coefficients for both RNFL thickness and mfVEP amplitude were calculated for each zone, and corresponding coefficients were correlated between each other. There was highly significant reduction of RNFL thickness and mean mfVEP amplitude in all three retinal sectors of the affected eye. Largest reduction of RNFL thickness was noticed in temporal sector and of mfVEP amplitude in corresponding central part of the visual field. RNFL thickness correlated highly with amplitude of the mfVEP derived from corresponding areas of the visual field in all three zones. We demonstrated strong topographical associations between structural and functional measures of optic nerve integrity in patients with ON.

  10. Multifocal brain damage due to lacquer sniffing: the first case report of Thailand.

    Science.gov (United States)

    Poungvarin, N

    1991-07-01

    The first patient of multifocal brain damage due to lacquer sniffing was reported in Thailand. He was a 24-year-old man who had a 5 year history of lacquer sniffing (toluene abuse). He had difficulty in walking and had tremor for 1 year which became progressively worse and he was finally confined to bed. Examination revealed marked cerebellar signs of both upper and lower extremities with scanning speech. Five of his friends who were lacquer abusers also had similar symptoms and were confined to bed. Investigations showed marked atrophy of both cerebellar hemispheres, vermis, brainstem and less atrophy of both cerebral hemispheres in both computerized brain scanning and magnetic resonance imaging. Diffuse toxic demyelination of white matter and excess iron deposition over both thalami, basal ganglia and cerebral cortex were demonstrated on magnetic resonance imaging. Brainstem evoked response showed abnormal response of both sides. Nerve conduction velocity, electromyographic study and electroencephalogram were normal. Psychometric tests revealed dull normal or below average IQ-test of 82. He was admitted for 2 months with gradual recovery of neurological deficits. After six months of abstinence from lacquer and daily physical rehabilitation. This report of toluene abuse is not only public health problem but also reflects the socioeconomic status as well as political unawareness of this condition in the Thai community.

  11. Clonality Assessment in a Case of Multifocal Adamantinoma and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Paul Borbas

    2012-01-01

    Full Text Available Adamantinoma is a low-grade, malignant biphasic bone tumour predominantly located in the tibia. In up to 50% of all cases this is combined with one or more lesions in the ipsilateral fibula. Whether these lesions represent regional metastases or arise de novo is not yet exactly known. In order to address this question, we extracted DNA from the respective fresh frozen tumour tissues in a case of a young woman with a multifocal adamantinoma of both the tibia and ipsilateral fibula. Afterwards the X inactivation pattern was studied by means of methylation-sensitive polymerase chain reaction and primers that target the polymorphic CGG trinucleotide repeat of FMR1 gene and the polymorphic CAG repeat, on exon 1 of the human androgen receptor gene (AR. The analysis of the AR was homozygous and not informative. Studying the FMR1 gene, we detected a 100% skewing of the X inactivation pattern of both locations and found that the same allele was methylated. Even if the fibula lesion arose de novo there would have been a 50 : 50 chance that the same allele was methylated. As this methylation pattern was found we cannot provide a valid explanation for the origin of the fibula lesion. Analysis of X inactivation patterns in future cases of polyfocal adamantinoma might provide further evidence for one of the two theories.

  12. Multifocal electroretinogram in normal emmetropic subjects: Correlation with optical coherence tomography

    Directory of Open Access Journals (Sweden)

    Rajvardhan Azad

    2012-01-01

    Full Text Available Aim of the Study: To establish the normative database for multifocal electroretinogram (mfERG parameters in a normal emmetropic population. To correlate the data so obtained with the central macular thickness obtained using the optical coherence tomography (OCT scan. Materials and Methods: mfERG data were obtained from 222 eyes of 111 emmetropic subjects. The amplitude (nv/deg 2 and implicit times (ms of the first-order kernel mfERG responses (N1, P1, and N2 waves were obtained and grouped into five rings (Ring 1: Central 2°, Ring 2: 2-5°, Ring 3: 5-10°, Ring 4: 10-15°, Ring 5: >15°. The central macular thickness (CMT was obtained using the macular thickness scan protocol of the OCT. Results: The mfERG data obtained were used to create a normative database. The amplitudes of the mfERG waves were maximum in the fovea and progressively decreased with increasing eccentricity (P = 0.0001. The latencies of the P1 and N2 waves were longest in the central ring and progressively shortened with eccentricity (P = 0.0001. No statistically significant correlations were observed between central ring 1 parameters and the CMT. Conclusion: This study establishes normative database for mfERG parameters in an emmetropic population. No statistically significant correlation was noted between CMT and mfERG parameters.

  13. Chronic recurrent multifocal osteomyelitis: typical patterns of bone involvement in whole-body bone scintigraphy.

    Science.gov (United States)

    Acikgoz, Gunsel; Averill, Lauren W

    2014-08-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease of unknown etiology. It affects children and adolescents predominantly and occurs mostly in the female population. It is characterized by the insidious onset of pain and swelling, with a fluctuating clinical course of relapses and remissions. Typically, several bones are affected, either synchronously or metachronously, and bilateral involvement is common. CRMO most commonly affects the metaphysis of long bones, especially the tibia, femur, and clavicle. The spine, pelvis, ribs, sternum, and mandible may also be affected. Although lesions are mostly multiple, patients may present with a single symptomatic focus. Radiographic findings may be negative early in the course of the disease. Bone scintigraphy is useful in determining the presence of abnormality and the extent of disease. The imaging and clinical features of CRMO overlap with those of infectious osteomyelitis, bone malignancy, and inflammatory arthritis. Nonetheless, CRMO can be confidently diagnosed with the recognition of typical imaging patterns in the appropriate clinical setting. This article reviews imaging findings with special emphasis on bone scintigraphy and specific disease sites.

  14. Non-descanned multifocal multiphoton microscopy with a multianode photomultiplier tube

    Energy Technology Data Exchange (ETDEWEB)

    Cha, Jae Won; Yew, Elijah Y. S. [Department of Mechanical Engineering, Massachusetts Institute of Technology, Cambridge, MA (United States); Kim, Daekeun [Department of Mechanical Engineering, Dankook University (Korea, Republic of); Subramanian, Jaichandar [Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Cambridge, MA (United States); Nedivi, Elly [Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Cambridge, MA (United States); Departments of Brain and Cognitive Sciences, and Biology, Massachusetts Institute of Technology, Cambridge, MA (United States); So, Peter T. C. [Department of Mechanical Engineering, Massachusetts Institute of Technology, Cambridge, MA (United States); Department of Biological Engineering, Massachusetts Institute of Technology, Cambridge, MA (United States)

    2015-08-15

    Multifocal multiphoton microscopy (MMM) improves imaging speed over a point scanning approach by parallelizing the excitation process. Early versions of MMM relied on imaging detectors to record emission signals from multiple foci simultaneously. For many turbid biological specimens, the scattering of emission photons results in blurred images and degrades the signal-to-noise ratio (SNR). We have recently demonstrated that a multianode photomultiplier tube (MAPMT) placed in a descanned configuration can effectively collect scattered emission photons from each focus into their corresponding anodes significantly improving image SNR for highly scattering specimens. Unfortunately, a descanned MMM has a longer detection path resulting in substantial emission photon loss. Optical design constraints in a descanned geometry further results in significant optical aberrations especially for large field-of-view (FOV), high NA objectives. Here, we introduce a non-descanned MMM based on MAPMT that substantially overcomes most of these drawbacks. We show that we improve signal efficiency up to fourfold with limited image SNR degradation due to scattered emission photons. The excitation foci can also be spaced wider to cover the full FOV of the objective with minimal aberrations. The performance of this system is demonstrated by imaging interneuron morphological structures deep in the brains of living mice.

  15. Visual evoked potential (VEP) and multifocal electroretinogram (mfERG) in ocular syphilitic posterior segment inflammation.

    Science.gov (United States)

    Alexander, Philip; Wen, Yaqin; Baxter, Julia M; Tint, Naing L; Browning, Andrew C; Amoaku, Winfried M

    2012-10-01

    The aim of this study is to correlate multifocal electroretinogram (mfERG) and visual evoked potential (VEP) changes with visual acuity and clinical features in patients with posterior segment inflammation secondary to syphilis. A retrospective interventional case series of 4 patients with visual loss secondary to syphilitic uveitis is reported. The mfERG (P1) showed diminished amplitudes and prolonged latency in 7 affected eyes. Visual acuity rapidly improved 2 weeks after initiation of therapy. OCT demonstrated anatomical recovery at 1 month. In three patients, visual acuity was restored to 6/6 at 6-9 months but mfERG responses remained significantly reduced and delayed for 12-15 months before recovery to normal levels. One patient developed a retinal detachment, but achieved 6/9 vision at 30 months. VEP changes, interpreted in combination with mfERG responses, showed evidence of optic nerve involvement in 6 eyes. Ocular findings, including posterior placoid chorioretinitis, are important diagnostic features of secondary and tertiary syphilis. Visual acuity and clinical recovery occur early with appropriate diagnosis and treatment, and precede full electrophysiological recovery of the outer retina-RPE complex. Ophthalmologists have the opportunity to play a key role in undetected or missed diagnoses of syphilis, and with appropriate treatment the visual prognosis is excellent.

  16. Mitoxantrone Therapy for Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis

    Directory of Open Access Journals (Sweden)

    Hélène Massé

    2009-01-01

    Full Text Available Purpose. To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. Design. Single case report. Method. A 22-year-old man presented with acute isolated bilateral loss of vision revealing APMPPE. Corticosteroid therapy was initiated and visual acuity gradually improved. Seventeen days later, visual function deteriorated again, associated with flu-like syndrome and severe headaches. A relapse of APMPPE was diagnosed, complicated with lymphocytic meningitis and cerebral ischemia. Intravenous therapy with mitoxantrone was performed in combination with methylprednisolone. Results. Headaches disappeared in a few days whereas visual acuity gradually improved and stabilized at 20/40 in the right eye and 20/32 in the left eye. No adverse event was observed. Clinical improvement was confirmed by magnetic resonance imaging. Conclusion. Cerebral vasculitis is the most severe complication of the extraocular manifestations of APMPEE. This diagnosis should be evoked when severe headaches or behavior disorder are associated with APMPEE.

  17. Natalizumab-associated progressive multifocal leucoencephalopathy: a practical approach to risk profiling and monitoring.

    Science.gov (United States)

    Hunt, David; Giovannoni, Gavin

    2012-02-01

    Natalizumab reduces relapse frequency, delays onset of disease progression and improves disease outcomes in relapsing-remitting multiple sclerosis (MS) and is a cost-effective treatment for rapidly evolving severe relapsing-remitting MS. However, it is associated with the development of progressive multifocal leucoencephalopathy (PML), a serious opportunistic brain infection caused by a neurotropic strain of the JC virus (JCV). Until May 2011, 83 300 patients had received natalizumab for MS. One hundred and twenty-four patients had developed PML, of whom 23 (19%) died. In order to maximise the benefit-risk ratio of natalizumab for MS patients it is important to develop a strategy for risk profiling and monitoring for PML. Central to this is an understanding of the biology of the JCV and the emerging clinical picture of natalizumab-associated PML. This paper reviews the evidence for managing the risk of PML in natalizumab-treated patients and the authors propose an algorithm for risk profiling and risk management. Key features of this algorithm include risk stratification based on emerging risk factors, heightened clinical vigilance for the clinical features of natalizumab-associated PML and considerations for temporary and permanent cessation of natalizumab dosing.

  18. New concentrator multifocal Fresnel lens for improved uniformity: design and characterization

    Science.gov (United States)

    Vázquez-Moliní, Daniel; Fernández-Balbuena, Antonio Álvarez; Bernabeu, Eusebio; Muñoz de Luna Clemente, Javier; Domingo-Marique, Alfonso; García-Botella, Ángel

    2009-08-01

    The emergence of high efficiency photovoltaic cells is leading the industry into using solar concentrators in order to reduce costs by decreasing the number of cells used. In this paper Optics department of Universidad Complutense de Madrid has designed a multifocal Fresnel lens of PMMA and has studied the main parameters that have influence on its final function. This has been done by taking into account its manufacturing tolerances. The lens is square shaped with sides measuring 270 mm and it is composed of three different zones based on three different criteria: The central zone has been designed by using paraxial formulation, the intermediate one has been designed based on Fresnel classical formula while the marginal zone's purpose is to deflect the light by total internal reflection on prism faces. All three zones have different focal areas and different optical axis so the energy distribution will be more uniform whilst avoiding cell damage caused by hot spots. The design stage is feedback through simulations using a ray tracer software. In order to characterize the lens operation a measure of optical concentration was first taken on different lens areas using an integrating sphere. Finally, the lens performance in terms of concentration and in terms of uniformity at the focal spot was studied by processing the images taken with a CCD camera on a screen placed at the focal plane of the lens.

  19. Recurrent multifocal chronic osteitis in children; Osteite chronique multifocale recurrente de l`enfant

    Energy Technology Data Exchange (ETDEWEB)

    Quelquejay, C.; Hamidou, A.; Benosman, A.; Adamsbaum, C. [Hopital Saint-Vincent-de-Paul, 75 - Paris (France); Job-Deslandre, Ch. [Hopital Cochin, 75 - Paris (France)

    1997-09-01

    We have studied retrospectively a series of 10 children presenting with chronic multifocal osteomyelitis (8 girls, 2 boys, 7 to 16 years). All patients had plain films, bone scintigraphies and histological studies. Three had CT scan and/or MRI. Compared with literature data, we observed only one case of palmo-plantar pustulosis and only 2 cases of lysis of the medial extremity of the clavicle; in addition, we report one case of lateral extremity of the clavicle and 2 vertebral locations. The radiological pattern was typical: at the beginning of the disease, plain films showed lytic areas which became progressively osteosclerotic with enlargement of the bone. In all the cases, bone scintigraphy revealed high uptake areas which were often infra-clinical. The diagnosis was delayed from 3 months to 3 years. This emphasizes the difficulty of the diagnosis which relies on the association of clinical, biological and radiological elements. Biopsies are required to rule out an infectious bacterial osteomyelitis or a tumoral process. The pathogenesis of OCMR remains unknown, but the relation with the SAPHO (synovitis, acne, pustulosis, Hyperostosis, osteitis) syndrome is general accepted because of the similar features of the osteitis. The long term follow up appears to be uncertain: 6 of our patients are still symptomatic after five years despite anti inflammatory treatment. (authors). 22 refs.

  20. Multifocal Breast Cancer in Young Women with Prolonged Contact between Their Breasts and Their Cellular Phones.

    Science.gov (United States)

    West, John G; Kapoor, Nimmi S; Liao, Shu-Yuan; Chen, June W; Bailey, Lisa; Nagourney, Robert A

    2013-01-01

    Breast cancer occurring in women under the age of 40 is uncommon in the absence of family history or genetic predisposition, and prompts the exploration of other possible exposures or environmental risks. We report a case series of four young women-ages from 21 to 39-with multifocal invasive breast cancer that raises the concern of a possible association with nonionizing radiation of electromagnetic field exposures from cellular phones. All patients regularly carried their smartphones directly against their breasts in their brassieres for up to 10 hours a day, for several years, and developed tumors in areas of their breasts immediately underlying the phones. All patients had no family history of breast cancer, tested negative for BRCA1 and BRCA2, and had no other known breast cancer risks. Their breast imaging is reviewed, showing clustering of multiple tumor foci in the breast directly under the area of phone contact. Pathology of all four cases shows striking similarity; all tumors are hormone-positive, low-intermediate grade, having an extensive intraductal component, and all tumors have near identical morphology. These cases raise awareness to the lack of safety data of prolonged direct contact with cellular phones.

  1. Multifocal Breast Cancer in Young Women with Prolonged Contact between Their Breasts and Their Cellular Phones

    Directory of Open Access Journals (Sweden)

    John G. West

    2013-01-01

    Full Text Available Breast cancer occurring in women under the age of 40 is uncommon in the absence of family history or genetic predisposition, and prompts the exploration of other possible exposures or environmental risks. We report a case series of four young women—ages from 21 to 39—with multifocal invasive breast cancer that raises the concern of a possible association with nonionizing radiation of electromagnetic field exposures from cellular phones. All patients regularly carried their smartphones directly against their breasts in their brassieres for up to 10 hours a day, for several years, and developed tumors in areas of their breasts immediately underlying the phones. All patients had no family history of breast cancer, tested negative for BRCA1 and BRCA2, and had no other known breast cancer risks. Their breast imaging is reviewed, showing clustering of multiple tumor foci in the breast directly under the area of phone contact. Pathology of all four cases shows striking similarity; all tumors are hormone-positive, low-intermediate grade, having an extensive intraductal component, and all tumors have near identical morphology. These cases raise awareness to the lack of safety data of prolonged direct contact with cellular phones.

  2. [Development of syndrome of inappropriate secretion of ADH and reversible posterior leukoencephalopathy during initial rituximab-CHOP therapy in a patient with diffuse large B-cell lymphoma].

    Science.gov (United States)

    Mizutani, Minoru; Nakamori, Yoshiki; Sakaguchi, Haruna; Kageyama, Yuki; Oya, Eiko; Ino, Kazuko; Suzuki, Kei; Sekine, Takao

    2013-03-01

    A 61-year-old woman presented with a right mandibular tumor and was diagnosed with DLBCL clinical stage IIIA from the biopsy results of the tumor and CT examination. An initial rituximab was administrated a week after the first CHOP treatment. During the infusion of rituximab, she exhibited disorientation, seizure, and consciousness disturbance. Hyponatremia due to SIADH and hypertension were coincidentally observed. MRI revealed T2 and FLAIR hyperintense signals involving the bilateral occipital, parietal, frontal lobes and the cerebellum that were consistent with reversible posterior leukoencephalopathy syndrome (RPLS). Her consciousness level recovered in parallel with corrections in serum sodium levels and blood pressure. Although she presented with transient cortical blindness, all neurological abnormalities disappeared 40 hours after the occurrence of seizure. She received a further 7 cycles of CHOP followed by 7 cycles of rituximab treatment with no relapse of RPLS. After irradiation for a residual abdominal tumor, she has maintained complete remission for 2 years. Although RPLS is a rare complication of rituximab-CHOP chemotherapy, it should be considered in patients with DLBCL who present with acute neurological deterioration.

  3. Ophthalmic epidemiology in Europe

    DEFF Research Database (Denmark)

    Delcourt, Cécile; Korobelnik, Jean-François; Buitendijk, Gabriëlle H S

    2016-01-01

    The European Eye Epidemiology (E3) consortium is a recently formed consortium of 29 groups from 12 European countries. It already comprises 21 population-based studies and 20 other studies (case-control, cases only, randomized trials), providing ophthalmological data on approximately 170,000 Euro...

  4. Inflammatory bowel disease epidemiology

    DEFF Research Database (Denmark)

    Burisch, Johan; Munkholm, Pia

    2013-01-01

    The occurrence of inflammatory bowel disease (IBD) is increasing worldwide, yet the reasons remain unknown. New therapeutic approaches have been introduced in medical IBD therapy, but their impact on the natural history of IBD remains uncertain. This review will summarize the recent findings in t...... in the epidemiology of IBD....

  5. Phylogenetically resolving epidemiologic linkage.

    Science.gov (United States)

    Romero-Severson, Ethan O; Bulla, Ingo; Leitner, Thomas

    2016-03-08

    Although the use of phylogenetic trees in epidemiological investigations has become commonplace, their epidemiological interpretation has not been systematically evaluated. Here, we use an HIV-1 within-host coalescent model to probabilistically evaluate transmission histories of two epidemiologically linked hosts. Previous critique of phylogenetic reconstruction has claimed that direction of transmission is difficult to infer, and that the existence of unsampled intermediary links or common sources can never be excluded. The phylogenetic relationship between the HIV populations of epidemiologically linked hosts can be classified into six types of trees, based on cladistic relationships and whether the reconstruction is consistent with the true transmission history or not. We show that the direction of transmission and whether unsampled intermediary links or common sources existed make very different predictions about expected phylogenetic relationships: (i) Direction of transmission can often be established when paraphyly exists, (ii) intermediary links can be excluded when multiple lineages were transmitted, and (iii) when the sampled individuals' HIV populations both are monophyletic a common source was likely the origin. Inconsistent results, suggesting the wrong transmission direction, were generally rare. In addition, the expected tree topology also depends on the number of transmitted lineages, the sample size, the time of the sample relative to transmission, and how fast the diversity increases after infection. Typically, 20 or more sequences per subject give robust results. We confirm our theoretical evaluations with analyses of real transmission histories and discuss how our findings should aid in interpreting phylogenetic results.

  6. [Toxoplasmosis: Epidemiology, Diagnosis, Treatment].

    Science.gov (United States)

    Khryanin, A A; Reshetnikov, O V; Kuvshinova, I N

    2015-01-01

    The up-to-date literature and original data on the epidemiology, diagnosis and treatment of toxoplasmosis are presented. Particular attention is paid to the parasite infection during pregnancy. Spiramycin is the drug of choice for acute toxoplasmosis in pregnant women.

  7. Molecular epidemiology of ascariasis

    DEFF Research Database (Denmark)

    Betson, Martha; Halstead, Fennella; Nejsum, Peter

      We are using molecular epidemiology techniques to study the population structure of Ascaris obtained from humans and pigs. Worms were obtained from human hosts on Zanzibar and in Uganda, Bangladesh, Guatemala and Nepal and Ascaris from pigs were collected from in Uganda, Tanzania, Denmark...

  8. Epidemiology of Down Syndrome

    Science.gov (United States)

    Sherman, Stephanie L.; Allen, Emily G.; Bean, Lora H.; Freeman, Sallie B.

    2007-01-01

    Down syndrome (DS) is the most commonly identified genetic form of mental retardation and the leading cause of specific birth defects and medical conditions. Traditional epidemiological studies to determine the prevalence, cause, and clinical significance of the syndrome have been conducted over the last 100 years. DS has been estimated to occur…

  9. Triangulation in aetiological epidemiology.

    Science.gov (United States)

    Lawlor, Debbie A; Tilling, Kate; Davey Smith, George

    2016-12-01

    Triangulation is the practice of obtaining more reliable answers to research questions through integrating results from several different approaches, where each approach has different key sources of potential bias that are unrelated to each other. With respect to causal questions in aetiological epidemiology, if the results of different approaches all point to the same conclusion, this strengthens confidence in the finding. This is particularly the case when the key sources of bias of some of the approaches would predict that findings would point in opposite directions if they were due to such biases. Where there are inconsistencies, understanding the key sources of bias of each approach can help to identify what further research is required to address the causal question. The aim of this paper is to illustrate how triangulation might be used to improve causal inference in aetiological epidemiology. We propose a minimum set of criteria for use in triangulation in aetiological epidemiology, summarize the key sources of bias of several approaches and describe how these might be integrated within a triangulation framework. We emphasize the importance of being explicit about the expected direction of bias within each approach, whenever this is possible, and seeking to identify approaches that would be expected to bias the true causal effect in different directions. We also note the importance, when comparing results, of taking account of differences in the duration and timing of exposures. We provide three examples to illustrate these points. © The Author 2017. Published by Oxford University Press on behalf of the International Epidemiological Association.

  10. The Epidemiology of Pheochromocytoma

    DEFF Research Database (Denmark)

    Ladefoged Ebbehøj, Andreas

    2017-01-01

    Pheochromocytoma and catecholamine secreting paraganglioma (PPGL) are exceedingly rare endocrine tumours, but remain a frequent diagnostic dilemma due to their potential life-threatening nature. Reliable data on the epidemiology of PPGL is lacking and no time trends in incidence rates (IR) have...

  11. Epidemiology of burns

    NARCIS (Netherlands)

    Dokter, Jan

    2016-01-01

    The aim of this thesis is to understand the epidemiology, treatment and outcomes of specialized burn care in The Netherlands. This thesis is mainly based on historical data of the burn centre in Rotterdam from 1986, combined with historical data from the burn centres in Groningen and Beverwijk from

  12. Heterogeneous echogenicity of the thyroid parenchyma does not influence the detection of multi-focality in papillary thyroid carcinoma on preoperative ultrasound staging.

    Science.gov (United States)

    Herh, Sun Jin; Kim, Eun-Kyung; Sung, Ji Min; Yoon, Jung Hyun; Moon, Hee Jung; Kwak, Jin Young

    2014-05-01

    Heterogeneous echogenicity and micro-nodulations of diffuse thyroid disease on ultrasonography (US) might influence the diagnostic performance of pre-operative US staging, especially the detection of multi-focality. This study was designed to determine whether heterogeneous echogenicity of the thyroid parenchyma influences the diagnostic performance of US in the detection of multi-focality in papillary thyroid carcinoma. Between December 2010 and April 2011, 811 patients underwent pre-operative staging US for papillary thyroid carcinoma and surgery. Twelve radiologists performed the pre-operative US for T and N staging. Underlying parenchymal echogenicity and unilateral and bilateral multi-focality of the thyroid nodules were also evaluated. Patients were divided into two groups on the basis of the underlying echogenicity of the thyroid gland. To evaluate the diagnostic accuracy of US with respect to underlying echogenicity, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy were calculated and compared between the two groups. Among the 811 patients included, US revealed underlying heterogeneous echogenicity of the thyroid parenchyma in 204 (25.2%) and underlying homogeneous echogenicity of the thyroid parenchyma in 607 (74.8%). There were no significant differences between the two groups in the diagnostic performance of pre-operative staging US in predicting unilateral multi-focality and bilaterality. Underlying heterogeneous echogenicity in a thyroid gland with Hashimoto's thyroiditis does not significantly influence the detection of multi-focality in papillary thyroid cancer on pre-operative US staging.

  13. What monitor can replace the cathode-ray tube for visual stimulation to elicit multifocal electroretinograms?

    Science.gov (United States)

    Matsumoto, Celso Soiti; Shinoda, Kei; Matsumoto, Harue; Seki, Keisuke; Nagasaka, Eiichiro; Iwata, Takeshi; Mizota, Atsushi

    2014-08-05

    To compare a conventional cathode-ray tube (CRT) screen to organic light-emitting diode (OLED) and liquid crystal display (LCD) screens as visual stimulators to elicit multifocal electroretinograms (mfERGs), mfERGs were recorded from seven eyes of seven healthy volunteers (21 ± 2 years). The mfERGs elicited by a conventional CRT screen (S710, Compaq Computer Co.) were compared to those elicited by a studio-grade master OLED monitor (PVM-1741, Sony, Japan) and a conventional LCD (S1721, Flexscan, Eizo Nanao Corp., Japan). The luminance changes of each monitor were measured with a photodiode. CRT, OLED, and LCD screens with a frame frequency of 60 Hz were studied. A hexagonal stimulus array with 61 stimulus elements was created on each monitor. The serial white stimuli of the OLED screen at 60 Hz did not fuse, and that of the LCD screens fused. The amplitudes of P1 and P2 of the first-order kernels of the mfERGs were not significantly different from those elicited by the CRT and OLED screens, and the P1 amplitude of the first-order kernel elicited by the LCD stimuli was significantly smaller than that elicited by the CRT in all the groups of the averaged hexagonal elements. The implicit times were approximately 10 ms longer in almost all components elicited by the LCD screen compared to those elicited by the CRT screen. The mfERGs elicited by monitors other than the CRT should be carefully interpreted, especially those elicited by LCD screens. The OLED had good performance, and we conclude that it can replace the CRT as a stimulator for mfERGs; however, a collection of normative data is recommended. © 2014 ARVO.

  14. What is the best pre-therapeutic dosimetry for successful radioiodine therapy of multifocal autonomy?

    Energy Technology Data Exchange (ETDEWEB)

    Gotthardt, M. [Radboud Univ. Nijmegen Medical Center, Nijmegen (Netherlands). Dept. of Nuclear Medicine; Philipps Univ., Marburg (Germany). Dept. of Nuclear Medicine; Rubner, C. [Philipps Univ., Marburg (Germany). Dept. of Nuclear Medicine; Bauhofer, A. [Philipps Univ., Marburg (DE). Inst. of Theoretical Surgery] (and others)

    2006-07-01

    Purpose: Dose calculation for radioiodine therapy (RIT) of multifocal autonomies (MFA) is a problem as therapeutic outcome may be worse than in other kinds of autonomies. We compared different dosimetric concepts in our patients. Patients, methods: Data from 187 patients who had undergone RIT for MFA (Marinelli algorithm, volumetric compromise) were included in the study. For calculation, either a standard or a measured half-life had been used and the dosimetric compromise (150 Gy, total thyroid volume). Therapeutic activities were calculated by 2 alternative concepts and compared to therapeutic success achieved (concept of TcTUs-based calculation of autonomous volume with 300 Gy and TcTUs-based adaptation of target dose on total thyroid volume). Results: If a standard half-life is used, therapeutic success was achieved in 90.2% (hypothyroidism 23,1%, n=143). If a measured half-life was used the success rate was 93.1% (13,6% hypothyroidism, n=44). These differences were statistically not significant, neither for all patients together nor for subgroups eu-, hypo-, or hyperthyroid after therapy (ANOVA, all p>0.05). The alternative dosimetric concepts would have resulted either in significantly lower organ doses (TcTUs-based calculation of autonomous volume; 80.76{+-}80.6 Gy versus 125.6{+-}46.3 Gy; p<0.0001) or in systematic over-treatment with significantly higher doses (TcTUs-adapted concept; 164.2{+-}101.7 Gy versus 125.6{+-}46.3 Gy; p=0.0097). Conclusions: TcTUs-based determination of the autonomous volume should not be performed, the TcTUs-based adaptation of the target dose will only increase the rate of hypothyroidism. A standard half-life may be used in pre-therapeutic dosimetry for RIT of MFA. If so, individual therapeutic activities may be calculated based on thyroid size corrected to the 24h ITUs without using Marinelli's algorithm. (orig.)

  15. Losing focus: how lens position and viewing angle affect the function of multifocal lenses in fishes.

    Science.gov (United States)

    Gagnon, Yakir Luc; Wilby, David; Temple, Shelby Eric

    2016-09-01

    Light rays of different wavelengths are focused at different distances when they pass through a lens (longitudinal chromatic aberration [LCA]). For animals with color vision this can pose a serious problem, because in order to perceive a sharp image the rays must be focused at the shallow plane of the photoreceptor's outer segments in the retina. A variety of fish and tetrapods have been found to possess multifocal lenses, which correct for LCA by assigning concentric zones to correctly focus specific wavelengths. Each zone receives light from a specific beam entrance position (BEP) (the lateral distance between incoming light and the center of the lens). Any occlusion of incoming light at specific BEPs changes the composition of the wavelengths that are correctly focused on the retina. Here, we calculated the effect of lens position relative to the plane of the iris and light entering the eye at oblique angles on how much of the lens was involved in focusing the image on the retina (measured as the availability of BEPs). We used rotational photography of fish eyes and mathematical modeling to quantify the degree of lens occlusion. We found that, at most lens positions and viewing angles, there was a decrease of BEP availability and in some cases complete absence of some BEPs. Given the implications of these effects on image quality, we postulate that three morphological features (aphakic spaces, curvature of the iris, and intraretinal variability in spectral sensitivity) may, in part, be adaptations to mitigate the loss of spectral image quality in the periphery of the eyes of fishes.

  16. Spinal involvement in chronic recurrent multifocal osteomyelitis (CRMO) in childhood and effect of pamidronate.

    Science.gov (United States)

    Hospach, Toni; Langendoerfer, Micha; von Kalle, Tekla; Maier, Jan; Dannecker, Guenther E

    2010-09-01

    There are only a few studies that address the frequency and type of spinal involvement in patients with chronic recurrent multifocal osteomyelitis (CRMO) as well as the outcome of these patients treated with pamidronate (PAM). We performed a retrospective study on patients with CRMO and analyzed clinical and pain assessments as well as regional and whole body MRI findings and compared with posttreatment findings. Of 102 children and adolescents with CRMO, 27 (26%) had involvement of the spine. Vertebral deformities were seen in 14 of these 27 patients, scoliosis or kyphosis in 6. After routine whole body MRI, 19 complained of back pain, whereas eight were asymptomatic with spinal lesions detected incidentally. A total of 72 spinal lesions were detected, thoracic vertebrae being the most commonly affected. Seven patients were treated with PAM; all of whom had vertebral deformities and ongoing back pain. Pain resolution was achieved within 3 months of PAM treatment in every case. One patient subsequently developed a pain amplification syndrome. Repeat MRI performed at a mean interval of 13 months revealed partial or complete resolution of vertebral hyperintensities in every patient. Improvement of vertebral height was seen in a total of three vertebrae in two patients. Severe side effects were not observed. In conclusion, we demonstrated that spinal involvement and associated vertebral deformities with or without kyphoscoliosis are not rare in CRMO, and PAM appears to be an effective and safe treatment for this condition. Although controlled studies are urgently needed, the use of PAM for refractory CRMO with extended spinal involvement (vertebral deformities, kyphosis, and scoliosis) should be considered, especially after failing of conventional therapy.

  17. MRI shows thickening and altered diffusion in the median and ulnar nerves in multifocal motor neuropathy

    Energy Technology Data Exchange (ETDEWEB)

    Haakma, Wieke [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Aarhus University, Department of Forensic Medicine and Comparative Medicine Lab, Aarhus (Denmark); Jongbloed, Bas A.; Goedee, H.S.; Berg, Leonard H. van den; Pol, W.L. van der [University Medical Center Utrecht, Brain Centre Rudolf Magnus, Department of Neurology and Neurosurgery, Utrecht (Netherlands); Froeling, Martijn; Bos, Clemens; Hendrikse, Jeroen [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Leemans, Alexander [University Medical Center Utrecht, Image Sciences Institute, Utrecht (Netherlands)

    2017-05-15

    To study disease mechanisms in multifocal motor neuropathy (MMN) with magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) of the median and ulnar nerves. We enrolled ten MMN patients, ten patients with amyotrophic lateral sclerosis (ALS) and ten healthy controls (HCs). Patients underwent MRI (in a prone position) and nerve conduction studies. DTI and fat-suppressed T2-weighted scans of the forearms were performed on a 3.0T MRI scanner. Fibre tractography of the median and ulnar nerves was performed to extract diffusion parameters: fractional anisotropy (FA), mean (MD), axial (AD) and radial (RD) diffusivity. Cross-sectional areas (CSA) were measured on T2-weighted scans. Forty-five out of 60 arms were included in the analysis. AD was significantly lower in MMN patients (2.20 ± 0.12 x 10{sup -3} mm{sup 2}/s) compared to ALS patients (2.31 ± 0.17 x 10{sup -3} mm{sup 2}/s; p < 0.05) and HCs (2.31± 0.17 x 10{sup -3} mm{sup 2}/s; p < 0.05). Segmental analysis showed significant restriction of AD, RD and MD (p < 0.005) in the proximal third of the nerves. CSA was significantly larger in MMN patients compared to ALS patients and HCs (p < 0.01). Thickening of nerves is compatible with changes in the myelin sheath structure, whereas lowered AD values suggest axonal dysfunction. These findings suggest that myelin and axons are diffusely involved in MMN pathogenesis. (orig.)

  18. Multifocal visual evoked potentials are influenced by variable contrast stimulation in MS

    Science.gov (United States)

    Frohman, Audrey R.; Schnurman, Zane; Conger, Amy; Conger, Darrel; Beh, Shin; Greenberg, Benjamin; Sutter, Erich; Calabresi, Peter A.; Balcer, Laura J.; Frohman, Teresa C.

    2012-01-01

    Objective: To test the hypothesis that patients with multiple sclerosis (MS) with intereye asymmetry on low contrast letter acuity, and thickness of the retinal nerve fiber layer (RNFL), would exhibit corresponding changes in cortical timing and amplitude responses on pattern reversal multifocal visual evoked potentials (mfVEP), contingent upon variable stimulus contrast. Methods: In a cross-sectional study, we investigated a cohort of 11 normal subjects and 40 patients with MS, 21 of whom had a history of acute optic neuritis (MS-AON) with an intereye asymmetry with respect to RNFL thickness, and on low contrast letter acuity performance. Pattern reversal mfVEP was performed at high (100%), low (33.3%), and very low (14.2%) Michelson-contrast levels. Results: Compared to baseline measures at 100% contrast, the mean amplitude of the mfVEP was reduced in MS-AON eyes, upon pattern-reversal stimulation at the 2 lower contrast levels (p < 0.0001). With respect to changes in timing responses, the intereye asymmetry was increased in the MS-AON patients upon lower contrast pattern-reversal stimulation (p < 0.0001 for 33.3% compared to 100%, and p < 0.001 for 14.2% compared to 100%). The fellow eye in 12 (57%; p < 0.001) of the patients with an abnormal eye, and a history of AON, revealed abnormal amplitude and timing responses upon low contrast stimulation (signifying unmasking of occult damage). Conclusions: Our findings support the hypothesis that mfVEP metric abnormalities are contingent upon contrast magnitude during pattern reversal stimulation. Further, this paradigm was capable of unmasking occult abnormalities in a significant number of apparently unaffected eyes. PMID:22815550

  19. Dichoptic stimulation improves detection of glaucoma with multifocal visual evoked potentials.

    Science.gov (United States)

    Arvind, Hemamalini; Klistorner, Alexander; Graham, Stuart; Grigg, John; Goldberg, Ivan; Klistorner, Asya; Billson, Frank A

    2007-10-01

    To determine whether simultaneous binocular (dichoptic) stimulation for multifocal visual evoked potentials (mfVEP) detects glaucomatous defects and decreases intereye variability. Twenty-eight patients with glaucoma and 30 healthy subjects underwent mfVEP on monocular and dichoptic stimulation. Dichoptic stimulation was presented with the use of virtual reality goggles (recording time, 7 minutes). Monocular mfVEPs were recorded sequentially for each eye (recording time, 10 minutes). Comparison of mean relative asymmetry coefficient (RAC; calculated as difference in amplitudes between eyes/sum of amplitudes of both eyes at each segment) on monocular and dichoptic mfVEP revealed significantly lower RAC on dichoptic (0.003 +/- 0.03) compared with monocular testing (-0.02 +/- 0.04; P = 0.002). In all 28 patients, dichoptic mfVEP identified defects with excellent topographic correspondence. Of 56 hemifields (28 eyes), 33 had Humphrey visual field (HFA) scotomas, all of which were detected by dichoptic mfVEP. Among 23 hemifields with normal HFA, two were abnormal on monocular and dichoptic mfVEP. Five hemifields (five patients) normal on HFA and monocular mfVEP were abnormal on dichoptic mfVEP. In all five patients, corresponding rim changes were observed on disc photographs. Mean RAC of glaucomatous eyes was significantly higher on dichoptic (0.283 +/- 0.18) compared with monocular (0.199 +/- 0.12) tests (P = 0.0006). Dichoptic mfVEP not only detects HFA losses, it may identify early defects in areas unaffected on HFA and monocular mfVEP while reducing testing time by 30%. Asymmetry was tighter among healthy subjects but wider in patients with glaucoma on simultaneous binocular stimulation, which is potentially a new tool in the early detection of glaucoma.

  20. Separate magnocellular and parvocellular contributions from temporal analysis of the multifocal VEP.

    Science.gov (United States)

    Klistorner, A; Crewther, D P; Crewther, S G

    1997-08-01

    Temporal analysis of the multifocal cortical visual evoked potential (VEP) was studied using pseudo-random (m-sequence) achromatic stimulation. The effects of variation of luminance contrast on the first-order response were complex. At low to mid contrasts (< 60%), a wave doublet (P100-N115) predominated. A second wave complex (N100-P120-N160) dominated at high contrasts. The second-order responses, however, showed an extremely simple variation with luminance contrast. Intrinsic differences in the adaptation time of the generators of these two components caused a distinct separation in the slices of the second-order response. A rapidly adapting nonlinearity saturating at low contrasts was only observable when measuring the responses from two consecutive flashes. Its latency coincided with the contrast saturating first-order response component. By comparison, the nonlinearity derived from the responses to the stimuli with longer interstimulus intervals (second and third slices) yielded a much more linear contrast response function with lower contrast gain and latencies, which clearly corresponded to the longer latency component of the first-order response. Thus, the second-order responses show a first slice which is predominantly driven by neural elements that have a latency and contrast function that mimic those of the magnocellular neurons of the primate LGN and a second slice which is dominated by a generator whose properties resemble primate parvocellular function. This division into magno and parvocellular contribution to the VEP is based on function (interaction time) as distinct from other currently available analyses, with potential for neural analysis of visual disease.