PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Recognizing involuntary emotional expression disorder. (J Neurosci Nurs. 2007 Aug;39(4):202-7). Authors are Robinson-Smith G and Grill JD from the Villanova University’s College of Nursing, Villanova, PA, USA. Involuntary crying or laughing are symptoms of a condition known as involuntary emotional expression disorder (IEED). This disorder is common among patients with stroke and other neurological disorders, such as multiple sclerosis, amyotrophic lateral sclerosis, and traumatic brain injury.IEED can become disabling for patients who are not accurately diagnosed and treated. When the condition is found to be present, effective management must include education, pharmacological treatment, and teaching of self-care strategies. To access the full abstract of the article, click here.
Publications
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Motor unit number estimation (mune) as a quantitative measure of disease progression and motor unit reorganization in amyotrophic lateral sclerosis (Int J Neurosci. 2007 Sep;117(9):1229-36). Authors are Sartucci F Maritato P Moscato G et al. from the Department of Neuroscience, Clinical Neurophysiology, Pisa University Medical School, Pisa, Italy. Motor Unit Number Estimation (MUNE), a technique allowing to estimate the number of functioning Motor Units (MU) in single muscles, was used to score the disease’s severity and progression rate in a group of 58 patients with Amyotrophic Lateral Sclerosis (ALS). The results confirm that MUNE is a suitable tool to quantify the pathological changes in MU in patients with ALS. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Infectious mononucleosis and Epstein-Barr virus. (Comp Immunol Microbiol Infect Dis. 2007 May;30(3):153-62. Epub 2007 Jan 22). Authors are Vetsika EK and Callan M from the Division of Medicine, Imperial College, Chelsea and Westminster Hospital, 369 Fulham Road, London, SW10 9NH, UK. Epstein-Barr virus (EBV) is a herpesvirus that infects over 90% of the human population worldwide. It is transmitted between individuals in saliva, and establishes replicative infection within the oropharynx as well as life-long latent infection of B cells. Primary EBV infection occurs during childhood and is asymptomatic. If delayed until adolescence, it can be associated with the clinical syndrome of infectious mononucleosis – an illness characterised by fevers, pharyngitis, lymphadenopathy and malaise. EBV infection is also associated with the development of EBV-associated lymphoid or epithelial cell malignancies in a small proportion of individuals. This review focuses on primary EBV infection in individuals suffering from infectious mononucleosis. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled The role of reactive oxygen species in the hearts of dystrophin-deficient mdx mice. ( Am J Physiol Heart Circ Physiol. 2007 Sep;293(3):H1969-77.). Authors are Williams IA and Allen DG from the Bosch Institute, School of Medical Sciences, University of Sydney F13, NSW 2006 Australia. Duchenne muscular dystrophy (DMD) is caused by deficiency of the cytoskeletal protein dystrophin. Oxidative stress is thought to contribute to the skeletal muscle damage in DMD; however, little is known about the role of oxidative damage in the pathogenesis of the heart failure that occurs in DMD patients. The dystrophin-deficient (mdx) mouse is an animal model of DMD that also lacks dystrophin. The current study investigates the role of the antioxidant N-acetylcysteine (NAC) on mdx cardiomyocyte function, Ca(2+) handling, and the cardiac inflammatory response.Therapies designed to reduce oxidative damage might be beneficial to DMD patients with heart failurе. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Defect in glycosylation that causes muscular dystrophy (Methods Enzymol. 2006;417:137-52.). Authors are Endo T Manya H from the Glycobiology Research Group, Tokyo Metropolitan Institute of Gerontology Foundation for Research on Aging and Promotion of Human Welfare, Tokyo, Japan. Muscular dystrophies are a diverse group of inherited disorders characterized by progressive muscle weakness and wasting. The dystrophin-glycoprotein complex is composed of alpha-, beta-dystroglycan (DG), dystrophin and some other molecules.New findings indicate that disrupted glycosylation of alpha-DG results in a loss of ligand binding, giving rise to both progressive muscle degeneration and abnormal neuronal migration in the brain. This article discusses methods, including purification of alpha-DG and glycosyltransferase assays involved in alpha-DG glycosylation. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Paternal mosaicism and hereditary angioedema in a Taiwanese family. (Ann Allergy Asthma Immunol. 2007 Oct;99(4):375-9.). Authors are Yu TC Shyur SD Huang LH et al., from the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, Republic of China. Hereditary angioedema (HAE) is a rare disorder characterized by recurrent attacks of localized subcutaneous or submucosal edema. It is inherited in an autosomal dominant fashion and caused by a deficiency of C1 inhibitor (C1 INH). Most patients with HAE have an absolute deficiency of C1 INH (type I HAE), whereas the rest (approximately 15%) synthesize a dysfunctional C1 INH protein (type II HAE). Mosaicism is rare in HAE.Parental mosaicism is a possible explanation for normal C1 INH plasma concentrations in both parents despite clinically apparent HAE in the children. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Thrombotic thrombocytopenic purpura and Graves disease (Am J Med Sci. 2007 Aug;334(2):133-5). Authors are Chaar BT Kudva GC Olsen TJ et al., from the Department of Internal Medicine, Saint Louis University School of Medicine, Saint Louis, Missouri 63110, USA. Patients with an autoimmune disease have a propensity for development of a second autoimmune disease. We report the first instance of a patient with both idiopathic thrombotic thrombocytopenic purpura (TTP) and Graves disease. The TTP remitted with a combination of plasmapheresis and prednisone. Methimazole led to sustained remission of the hyperthyroid state within 6 weeks. Although hyperthyroidism may induce immune imbalance causing autoimmunity, it is unclear if this influenced the development of TTP in our patient and if treatment of hyperthyroidism alone could have resulted in the cure of both diseases. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Botulinum toxin B ultrasound-guided injections for sialorrhea in amyotrophic lateral sclerosis and Parkinson’s disease. (Parkinsonism Relat Disord. 2007 Jul;13(5):299-303. ). Authors are Contarino MF Pompili M Tittoto P et al., from the Istituto di Neurologia, Università Cattolica del Sacro Cuore, Rome, Italy. Sialorrhea is frequent and invalidating in patients with amyotrophic lateral sclerosis (ALS) or Parkinson’s disease (PD). Botulinum toxin (BTX) emerged as an alternative to traditional treatments. We evaluated efficacy and tolerability of ultrasound-guided BTX-B injections in parotids and submandibular glands in 18 patients with ALS or PD.BTX-B seems efficacious in reducing sialorrhea in ALS and PD but the risk-benefit ratio might differ between these two conditions. This might have implications for clinical practice. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine and corticosteroids in a patient with systemic lupus erythematosus and antibodies to ADAMTS13. (Intern Med. 2007;46(13):1033-7). Authors are Enami T Suzuki T Ito S et al., from the Clinical Immunology, Advanced Biomedical Applications, Graduate School of Comprehensive Human Sciences, University of Tsukuba. A 46-year-old woman with systemic lupus erythematosus was hospitalized for purpura, hematochezia and hematuria. One week after admission, she developed grand mal seizures and coma and was diagnosed with thrombotic thrombocytopenic purpura (TTP) when fragmented red cells were found on the peripheral blood smear. She was refractory to methylprednisolone pulse therapy and plasma exchange, but administration of cyclosporine induced remission without adverse effects. We propose that cyclosporine may be an effective treatment for cases of refractory TTP. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled Mazabraud’s Syndrome Coexisting with a Uterine Tumor Resembling an Ovarian Sex Cord Tumor (UTROSCT): a Case Report. ( Korean J Radiol. 2007 Sep-Oct;8(5):438-42.). Authors are Calisir C Inan U Yavas US et al., from the Department of Radiology, Eskisehir Osmangazi University. The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud’s syndrome. We present a case of Mazabraud’s syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature. To access the full abstract of the article, click here.