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Subacute Sclerosing Panencephalitis HELP
Based on 268 articles published since 2010
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These are the 268 published articles about Subacute Sclerosing Panencephalitis that originated from Worldwide during 2010-2020.
 
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6 · 7 · 8 · 9 · 10 · 11
1 Editorial Subacute Sclerosing Panencephalitis in Older Adulthood. 2019

Garg, Ravindra Kumar / Sharma, Praveen Kumar / Kumar, Neeraj / Pandey, Shweta. ·Department of Neurology, King George Medical University, Uttar Pradesh, Lucknow, IN. ·Tremor Other Hyperkinet Mov (N Y) · Pubmed #31566624.

ABSTRACT: -- No abstract --

2 Editorial "Melanie's measles" is deadly and causes permanent neurologic impairment. 2018

Hotez, Peter J. ·Texas Children's Hospital Center for Vaccine Development, National School of Tropical Medicine, Baylor College of Medicine, Houston, TX, USA. Electronic address: hotez@bcm.edu. ·Microbes Infect · Pubmed #29126876.

ABSTRACT: -- No abstract --

3 Editorial Subacute onset encephalopathy: a rare clinical syndrome with many aetiologies. 2013

Davies, Nicholas W S. · ·Br J Hosp Med (Lond) · Pubmed #23665779.

ABSTRACT: -- No abstract --

4 Review Global trends in measles publications. 2020

Kornbluh, Rachel / Davis, Robert. ·Yale University, New Haven, Connecticut, USA. · American Red Cross, Nairobi, Kenya. ·Pan Afr Med J · Pubmed #32373265.

ABSTRACT: Introduction: Beginning with the 1960s, this review analyzes trends in publications on measles indexed by the National Library of Medicine from January 1960 to mid-2018. It notes both the growth in numbers of published papers, and the increasing number and proportion of publications, in the current century, of articles on such items as costing, measles elimination, and determinants of coverage. Methods: A two-person team extracted from the National Library of Medicine (NLM) homepage all citations on measles beginning in 1960 and continuing through mid-2018. These were then classified both by overall number and by subject matter, with tabular summaries of both by decade and by subject matter. The tabular presentation forms the basis for a discussion of the ten most frequently cited subjects, and publication trends, with a special emphasis on the current century. Results: As in the past, the most often currently published items have been on coverage and its determinants, measles elimination, outbreak reports, SSPE, and SIAs. The putative relationship between vaccination and autism saw a spurt of articles in the 1990s, rapidly declining after the IOM report rejecting the causative hypothesis. Conclusion: There is a discussion on the sequencing of polio and measles eradication, the former unlikely before 2022, and an examination of likely research priorities as the world moves from measles control to measles eradication. There is a key role for social science in combatting vaccination reticence. The role of technical innovations, such as micropatch vaccination, is discussed.

5 Review Neurological Complications of Measles (Rubeola). 2020

Patterson, Marc C. ·Mayo Clinic Children's Center, Mayo Clinic College of Medicine and Science, 200 First Street SW, Rochester, MN, 55905, USA. patterson.marc@mayo.edu. ·Curr Neurol Neurosci Rep · Pubmed #32034528.

ABSTRACT: PURPOSE OF REVIEW: Owing to vaccine hesitancy, there has been a resurgence of measles infections in developed countries. Practitioners can expect to see an increase in patients with neurologic complications of measles. These devastating disorders include primary measles encephalitis, acute post measles encephalitis, subacute sclerosing panencephalitis (SSPE), and measles inclusion body encephalitis (MIBE). RECENT FINDINGS: Although there are many unanswered questions regarding the neurologic complications of measles, recent advances have led to better understanding of the mechanism of the spread of measles within the nervous system, particularly the disruption of F protein function, which raises the possibility of treatment with fusion-inhibiting molecules. Measles and its neurological complications are preventable and must be prevented. Neurologists must educate other clinicians and the public regarding the consequences of inadequate herd immunity to measles. More effective treatments for SSPE and MIBE may be available in the near future, but currently these remain lethal diseases.

6 Review Subacute sclerosing panencephalitis. 2019

Garg, Ravindra Kumar / Mahadevan, Anita / Malhotra, Hardeep Singh / Rizvi, Imran / Kumar, Neeraj / Uniyal, Ravi. ·Department of Neurology, King George Medical University, Lucknow, India. · Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India. ·Rev Med Virol · Pubmed #31237061.

ABSTRACT: Subacute sclerosing panencephalitis (SSPE) is a slowly progressive brain disorder caused by mutant measles virus. SSPE affects younger age groups. SSPE incidence is proportional to that of measles. High-income countries have seen substantial decline in SSPE incidence following universal vaccination against measles. SSPE virus differs from wild measles virus. Measles virus genome recovered from the autopsied brain tissues demonstrates clustered mutations in virus genome particularly in the M gene. These mutations destroy the structure and functioning of the encoded proteins. Complete infectious virus particle has rarely been recovered from the brain. Human neurons lack required receptor for entry of measles virus inside the neurons. Recent in vitro studies suggest that mutations in F protein confer hyperfusogenic properties to measles virus facilitating transneuronal viral spread. The inflammatory response in the brain leads to extensive tissue damage. Clinically, SSPE is characterized by florid panencephalitis. Clinically, SSPE is characterized by cognitive decline, periodic myoclonus, gait abnormalities, vision loss, and ultimately to a vegetative state. Chorioretinitis is a common ocular abnormality. Electroencephalography (EEG) shows characteristic periodic discharges. Neuroimaging demonstrates periventricular white matter signal abnormalities. In advanced stages, there is marked cerebral atrophy. Definitive diagnosis requires demonstration of elevated measles antibody titers in cerebrospinal fluid (CSF). Many drugs have been used to stabilize the course of the disease but without evidence from randomized clinical trials. Six percent of patients may experience prolonged spontaneous remission. Fusion inhibitor peptide may, in the future, be exploited to treat SSPE. A universal vaccination against measles is the only proven way to tackle this menace currently.

7 Review Inosine Pranobex: A Key Player in the Game Against a Wide Range of Viral Infections and Non-Infectious Diseases. 2019

Sliva, Jiri / Pantzartzi, Chrysoula N / Votava, Martin. ·Department of Pharmacology, 3rd Faculty of Medicine, Charles University, Prague, Czech Republic. · Institute of Pharmacovigilance, Prague, Czech Republic. chrysoula.pantzartzi@primevigilance.com. · Institute of Pharmacovigilance, Prague, Czech Republic. ·Adv Ther · Pubmed #31168764.

ABSTRACT: Inosine pranobex (IP), commonly known as inosine acedoben dimepranol, isoprinosine and methisoprinol, has been proven to positively impact the host's immune system, by enhancing T-cell lymphocyte proliferation and activity of natural killer cells, increasing levels of pro-inflammatory cytokines, and thereby restoring deficient responses in immunosuppressed patients. At the same time, it has been shown that it can affect viral RNA levels and hence inhibit growth of several viruses. Due to its immunomodulatory and antiviral properties, and its safety profile, it has been widely used since 1971 against viral infections and diseases, among which subacute sclerosis panencephalitis, herpes simplex virus, human papilloma virus, human immunodeficiency virus, influenza and acute respiratory infections, cytomegalovirus and Epstein-Barr virus infections. Following an analysis of almost five decades of scientific literature since its original approval, we here summarize in vivo and in vitro studies manifesting the means in which IP impacts the host's immune system. We also provide a synopsis of therapeutic trials in the majority of which IP was found to have a beneficial effect. Lastly, positive results from limited studies, suggesting the putative future use of IP in new therapeutic indications are briefly described. In order to support use of IP against viral infections apart from those already approved, and to establish its use in clinical practice, further well-designed and executed trials are warranted.Funding: Ewopharma International.

8 Review Subacute sclerosing panencephalitis: clinical phenotype, epidemiology, and preventive interventions. 2019

Mekki, Mohammed / Eley, Brian / Hardie, Diana / Wilmshurst, Jo M. ·Paediatric Neurology Division, Department of Paediatrics and Child Health, Neuroscience Institute, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa. · Paediatric Infectious Diseases Unit, Department of Paediatrics and Child Health, Faculty of Health Sciences, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa. · Division of Medical Virology, Department of Clinical Laboratory Sciences, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa. · National Health Laboratory Service, Cape Town, South Africa. ·Dev Med Child Neurol · Pubmed #30680706.

ABSTRACT: Subacute sclerosing panencephalitis (SSPE) is a preventable condition reported in 6.5 to 11 per 100 000 cases of measles, and highest in children who contracted measles infection when they were less than 5 years of age. Children residing in areas with poor vaccination coverage and high prevalence of human immunodeficiency virus are at increased risk of developing SSPE. SSPE is life-threatening in most affected children. This report documents current data relating to the clinical phenotype, epidemiology, and understanding of SSPE, inclusive of preventive interventions. While improvements in disease progression with immunomodulation may occur, overall there is no cure. Most therapies focus on supportive needs. Seizures and abnormal movements may respond to carbamazepine. Many countries advocate policies to enhance vaccination coverage. Effective preventive health care programmes, assurance of parental perceptions, and crisis support for unprecedented events obstructing effective primary health care are needed. Until measles is eradicated worldwide, children in all regions remain at risk. WHAT THIS PAPER ADDS: Measles contracted under 5 years of age has highest risk of developing subacute sclerosing panencephalitis (SSPE). Children with, or exposed to, human immunodeficiency virus infection, who contract measles may be at increased risk of SSPE.

9 Review New Insights into Measles Virus Brain Infections. 2019

Watanabe, Shumpei / Shirogane, Yuta / Sato, Yuma / Hashiguchi, Takao / Yanagi, Yusuke. ·Department of Virology, Faculty of Medicine, Kyushu University, Fukuoka 812-8582, Japan; Department of Microbiology, Faculty of Veterinary Medicine, Okayama University of Science, Ehime 794-8555, Japan. Electronic address: s-watanabe@vet.ous.ac.jp. · Department of Virology, Faculty of Medicine, Kyushu University, Fukuoka 812-8582, Japan. · Department of Virology, Faculty of Medicine, Kyushu University, Fukuoka 812-8582, Japan. Electronic address: yyanagi@virology.med.kyushu-u.ac.jp. ·Trends Microbiol · Pubmed #30220445.

ABSTRACT: Measles virus (MeV) may persist in the brain, causing fatal neurodegenerative diseases, subacute sclerosing panencephalitis, and measles inclusion-body encephalitis. However, the mechanism of MeV propagation in the brain remains unexplained because human neurons affected by the diseases do not express the known receptors for MeV. Recent studies have revealed that certain changes in the ectodomain of the MeV fusion (F) protein play a key role in MeV spread in the brain. These changes destabilize the prefusion form of the F protein and render it hyperfusogenic, which in turn allows the virus to propagate in neurons. Based on crystal structures of the F protein, effective fusion inhibitors could be developed to treat these diseases.

10 Review Measles: a disease often forgotten but not gone. 2018

Leung, A Kc / Hon, K L / Leong, K F / Sergi, C M. ·Department of Pediatrics, The University of Calgary, Calgary, Alberta, Canada. · Department of Paediatrics, The Chinese University of Hong Kong, Shatin, Hong Kong. · Department of Pediatrics, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia. · Department of Pediatrics and Department of Laboratory Medicine and Pathology, The University of Alberta, Edmonton, Alberta, Canada. ·Hong Kong Med J · Pubmed #30245481.

ABSTRACT: Measles (rubeola) is a highly contagious vaccine-preventable disease caused by the measles virus-a virus of the

11 Review Subacute sclerosing panencephalitis - current perspectives. 2018

Jafri, Sidra K / Kumar, Raman / Ibrahim, Shahnaz H. ·Department of Pediatrics and Child Health, Aga Khan University Hospital, Karachi, Pakistan, shahnaz.ibrahim@aku.edu. ·Pediatric Health Med Ther · Pubmed #29985487.

ABSTRACT: Subacute sclerosing panencephalitis is a progressive neurodegenerative disease. It usually occurs 7-10 years after measles infection. The clinical course is characterized by progressive cognitive decline and behavior changes followed by focal or generalized seizures as well as myoclonus, ataxia, visual disturbance, and later vegetative state, eventually leading to death. It is diagnosed on the basis of Dyken's criteria. There is no known cure for subacute sclerosing panencephalitis to date, but it is preventable by ensuring that an effective vaccine program for measles is made compulsory for all children younger than 5 years in endemic countries.

12 Review Primary Psychiatric Manifestations of Subacute Sclerosing Panencephalitis: A Case Report and Literature Review. 2018

Reddy, Balaswamy / Das, Soumitra / Guruprasad, Srinivas. ·Department of Psychiatry, NIMHANS, Bengaluru, Karnataka, India. Electronic address: gbalambbs7819@gmail.com. · Department of Psychiatry, NIMHANS, Bengaluru, Karnataka, India. · Department of Psychiatry, G K Reddy Hospital, Kadapa, AP, India. ·Psychosomatics · Pubmed #29402453.

ABSTRACT: -- No abstract --

13 Review Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity. 2017

Upadhyayula, Pavan S / Yang, Jason / Yue, John K / Ciacci, Joseph D. ·Department of Neurological Surgery, University of California, San Diego, 200 West Arbor Drive #8893, La Jolla, CA 92103, USA. pavan8632@gmail.com. · Department of Neurological Surgery, University of California, San Diego, 200 West Arbor Drive #8893, La Jolla, CA 92103, USA. jasonyangbd@gmail.com. · Department of Neurological Surgery, University of California, San Francisco, 400 Parnassus Avenue, San Francisco, CA 94122, USA. yuej@neurosurg.ucsf.edu. · Department of Neurological Surgery, University of California, San Diego, 200 West Arbor Drive #8893, La Jolla, CA 92103, USA. jciacci@ucsd.edu. ·Med Sci (Basel) · Pubmed #29112137.

ABSTRACT: Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords "subacute sclerosing panencephalitis" and "brainstem" using the National Library of Medicine PubMed database (March 1981-September 2017). Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV). However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.

14 Review A novel mutation in TREM2 gene causing Nasu-Hakola disease and review of the literature. 2017

Dardiotis, Efthimios / Siokas, Vasileios / Pantazi, Eva / Dardioti, Maria / Rikos, Dimitrios / Xiromerisiou, Georgia / Markou, Aikaterini / Papadimitriou, Dimitra / Speletas, Matthaios / Hadjigeorgiou, Georgios M. ·Department of Neurology, University of Thessaly, University Hospital of Larissa, Larissa, Greece; Laboratory of Neurogenetics, University of Thessaly, University Hospital of Larissa, Larissa, Greece. · Laboratory of Neurogenetics, University of Thessaly, University Hospital of Larissa, Larissa, Greece. · Department of Neurology, University of Thessaly, University Hospital of Larissa, Larissa, Greece. · Department of Immunology and Histocompatibility, School of Health Sciences, Faculty of Medicine, University of Thessaly, Biopolis, Larissa, Greece. · Department of Neurology, University of Thessaly, University Hospital of Larissa, Larissa, Greece; Laboratory of Neurogenetics, University of Thessaly, University Hospital of Larissa, Larissa, Greece. Electronic address: gmhadji@med.uth.gr. ·Neurobiol Aging · Pubmed #28214109.

ABSTRACT: Nasu-hakola disease (NHD) is a rare disease characterized by bone cysts and fractures, frontal lobe syndrome, and progressive presenile dementia. NHD may be the prototype of primary microglial disorders of the CNS or, as they have been coined, "microgliopathies". Mutations in TREM2 and TYROBP genes are known to cause NHD. Interestingly, recent evidence-associated rare genetic variants of TREM2 gene with increased risk of Alzheimer's disease, frontotemporal dementia, amyotrophic lateral sclerosis, and Parkinson's disease. Here, we report a 33-year-old Greek female with phenotype suggestive of NHD. Full gene sequencing of the TREM2 and TYROBP genes revealed a novel mutation in exon 2 of TREM2 gene, namely c.244G>T (p.W50C) and heterozygosity in the parents and her brother. This report extends the range of TREM2 mutations that cause NHD phenotype. In addition, we provide a comprehensive review of all reported in the literature TREM2 gene mutations and the respective wide spectrum of clinical manifestations that highlights the importance of considering TREM2 gene mutations in a variety of neurodegenerative phenotypes.

15 Review Subacute sclerosing panencephalitis in pregnancy. 2016

Chiu, Michael H / Meatherall, Bonnie / Nikolic, Ana / Cannon, Kristine / Fonseca, Kevin / Joseph, Jeffrey T / MacDonald, Judy / Pabbaraju, Kanti / Tellier, Raymond / Wong, Sallene / Koch, Marcus W. ·Department of Medicine, Division of Internal Medicine, University of Calgary, Calgary, AB, Canada. · Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, AB, Canada. · Infection Prevention and Control Program, Alberta Health Services, Calgary, AB, Canada. · Department of Microbiology, Immunology and Infectious Diseases, University of Calgary, Calgary, AB, Canada; Provincial Laboratory for Public Health, Alberta Health Services, Calgary, AB, Canada. · Department of Community Health Sciences, Faculty of Medicine, University of Calgary, Calgary, AB, Canada. · Provincial Laboratory for Public Health, Alberta Health Services, Calgary, AB, Canada. · Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, AB, Canada; Department of Microbiology, Immunology and Infectious Diseases, University of Calgary, Calgary, AB, Canada. · Department of Community Health Sciences, Faculty of Medicine, University of Calgary, Calgary, AB, Canada; Department of Clinical Neurosciences, University of Calgary, Calgary, AB, Canada. Electronic address: mwkoch@ucalgary.ca. ·Lancet Infect Dis · Pubmed #26809815.

ABSTRACT: We present a case of subacute sclerosing panencephalitis that developed in a previously healthy 29-year-old pregnant woman who had returned from a trip to rural India shortly before the onset of symptoms. She was admitted to hospital at 27 weeks' gestation with a history of cognitive decline and difficulty completing simple tasks. She had no clinical signs of infection. The working diagnosis was autoimmune encephalitis, although extensive investigations did not lead to a final classifying diagnosis. The patient became comatose and developed hypertension, and an emergency caesarean section was done at 31 weeks to deliver the child, who seemed healthy. The patient died about 6 weeks after the onset of symptoms. The patient was found to have had subacute sclerosing panencephalitis at autopsy. In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case.

16 Review The Triggering Receptor Expressed on Myeloid Cells 2: A Molecular Link of Neuroinflammation and Neurodegenerative Diseases. 2016

Walter, Jochen. ·From the Department of Neurology, University of Bonn, 53127 Bonn, Germany jochen.walter@ukb.uni-bonn.de. ·J Biol Chem · Pubmed #26694609.

ABSTRACT: The triggering receptor expressed on myeloid cells (TREM) 2 is a member of the immunoglobulin superfamily of receptors and mediates signaling in immune cells via engagement of its co-receptor DNAX-activating protein of 12 kDa (DAP12). Homozygous mutations in TREM2 or DAP12 cause Nasu-Hakola disease, which is characterized by bone abnormalities and dementia. Recently, a variant of TREM2 has also been associated with an increased risk for Alzheimer disease. The selective expression of TREM2 on immune cells and its association with different forms of dementia indicate a contribution of this receptor in common pathways of neurodegeneration.

17 Review Measles virus. 2015

Naim, Hussein Y. ·a Life Sciences and Vaccines Consultant; Bern, Switzerland. ·Hum Vaccin Immunother · Pubmed #25483511.

ABSTRACT: Measles was an inevitable infection during the human development with substantial degree of morbidity and mortality. The severity of measles virus (MV) infection was largely contained by the development of a live attenuated vaccine that was introduced into the vaccination programs. However, all efforts to eradicate the disease failed and continued to annually result in significant deaths. The development of molecular biology techniques allowed the rescue of MV from cDNA that enabled important insights into a variety of aspects of the biology of the virus and its pathogenesis. Subsequently these technologies facilitated the development of novel vaccine candidates that induce immunity against measles and other pathogens. Based on the promising prospective, the use of MV as a recombinant vaccine and a therapeutic vector is addressed.

18 Review Measles-induced encephalitis. 2015

Fisher, D L / Defres, S / Solomon, T. ·From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK Ha09113@qmul.ac.uk. · From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK. · From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK From the Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Institute of Infection and Global Health, University of Liverpool, Royal Liverpool University and Broadgreen NHS Trust, NIHR Health Protection Research Unit in Emerging and Zoonotic Infections and The Walton Centre Neurology NHS Foundation Trust, Liverpool, L9 7LJ UK. ·QJM · Pubmed #24865261.

ABSTRACT: Encephalitis is the most frequent neurological complication of measles virus infection. This review examines the pathophysiology of measles infection and the presentations, diagnosis and treatment of the four types of measles-induced encephalitis including primary measles encephalitis, acute post-measles encephalitis, measles inclusion body encephalitis and subacute sclerosing panencephalitis. The early symptoms of encephalitis may be non-specific and can be mistakenly attributed to a systemic infection leading to a delay in diagnosis. This review provides a summary of the symptoms that should cause health care workers to suspect measles-induced encephalitis.

19 Review [Neurodegenerative disease and TREM2]. 2014

Takahashi, Kazuya. ·Department of Neurology, National Hospital Organization Iou Hospital. ·Rinsho Shinkeigaku · Pubmed #25519966.

ABSTRACT: The functional loss of triggering receptor expressed on myeloid cells-2 (TREM2) leads to a chronic neurodegenerative disease, named Nasu-Hakola disease. A murine in vitro study revealed that the absence of TREM2 expression on microglia impaired phagocytosis for tissue debris clearance and increased pro-inflammatory cytokine production; these are necessary to maintain the microenvironmental homeostasis of the central nervous system. On the other hand, the function of TREM2 is still unclear, especially in the fields of human pathology and morphology. In 2013, it was reported that non-synonymous variants of TREM2 were associated with Alzheimer's disease in Caucasians; however, supplementary studies in Asian populations, such as the Japanese, could not confirm this result. In this review, I discuss how TREM2 can contribute to neurodegenerative disease.

20 Review Neuropathology of viral infections. 2014

Hatanpaa, Kimmo J / Kim, Jung H. ·Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA. Electronic address: Kimmo.Hatanpaa@UTSouthwestern.edu. · Department of Pathology, Yale University School of Medicine, New Haven, CT, USA. ·Handb Clin Neurol · Pubmed #25015486.

ABSTRACT: -- No abstract --

21 Review Nasu-Hakola disease as suspected cause for bone disease and dementia. 2014

Sahebari, Maryam / Abbasi, Bita / Akhondpour Manteghi, Ali / Abdollahi, Nafiseh. ·From the *Rheumatic Diseases Research Center (RDRC), School of Medicine, †Radiology Department, Ghaem Hospital, School of Medicine, and ‡Avicenna Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Islamic Republic of Iran. ·J Clin Rheumatol · Pubmed #24662559.

ABSTRACT: Progressive dementia in conjunction with multiple bone fractures in a previously healthy young man led to the investigation of the underlying cause. The differential diagnoses (most importantly hypoparathyroidism) were limited given basal ganglia calcifications on the brain computed tomographic scan. Electronic search of the key words basal ganglia calcification, osteoporosis, and dementia revealed a rare condition termed Nasu-Hakola disease or polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy. This very rare and potentially fatal genetic disease is characterized by pathological fractures, multiple lytic bone lesions, and presenile dementia. We report an Iranian patient with this disease and a review of the literature.

22 Review Measles, mumps, rubella, and human parvovirus B19 infections and neurologic disease. 2014

Bale, James F. ·Departments of Pediatrics and Neurology, University of Utah School of Medicine, Salt Lake City, UT, USA. Electronic address: james.bale@hsc.utah.edu. ·Handb Clin Neurol · Pubmed #24365423.

ABSTRACT: While the systemic disorders associated with measles, mumps, and rubella viruses and human parvovirus B19 tend to be mild, each virus can produce potentially life-threatening neurologic disease in human hosts, especially when these viruses infect young children. Two of the viruses, rubella and parvovirus B19, can be vertically transmitted to fetuses during maternal infection and cause congenital infection. Neurologic complications are common after intrauterine infection with the rubella virus, a condition known as the congenital rubella syndrome. Two, measles and rubella viruses, can induce "slow viral" infections, serious, disorders that can occur several years after the initial exposure to the virus and typically have fatal outcomes.

23 Review Novel compound heterozygous mutation in TREM2 found in a Turkish frontotemporal dementia-like family. 2013

Guerreiro, Rita / Bilgic, Basar / Guven, Gamze / Brás, José / Rohrer, Jonathan / Lohmann, Ebba / Hanagasi, Hasmet / Gurvit, Hakan / Emre, Murat. ·Department of Molecular Neuroscience, Institute of Neurology, University College London, London, UK. Electronic address: r.guerreiro@ucl.ac.uk. ·Neurobiol Aging · Pubmed #23870839.

ABSTRACT: Triggering receptor expressed on myeloid cells 2 (TREM2) homozygous mutations cause Nasu-Hakola disease, an early-onset recessive form of dementia preceded by bone cysts and fractures. The same type of mutations has recently been shown to cause frontotemporal dementia (FTD) without the presence of any bone phenotype. Here, we further confirm the association of TREM2 mutations with FTD-like phenotypes by reporting the first compound heterozygous mutation in a Turkish family.

24 Review Subacute sclerosing panencephalitis and chronic viral encephalitis. 2013

Anlar, Banu. ·Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey. Electronic address: banlar@hacettepe.edu.tr. ·Handb Clin Neurol · Pubmed #23622327.

ABSTRACT: Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system associated with the presence of mutant measles virus in the brain. It presents as a progressive, usually fatal disease. The diagnosis is based on clinical criteria and an elevated titer of measles antibodies in the cerebrospinal fluid (CSF). Electroencephalography and imaging studies provide supportive laboratory data. A brain biopsy is indicated only when CSF serology is negative or equivocal in a suspected case to assess the presence of inclusion bodies, measles virus antigens, or viral RNA. Among many drugs and methods tried in the treatment, the highest rate of stabilization or improvement was obtained with intraventricular human lymphoblastoid interferon-α and oral inosiplex. Further research for more available and efficient therapeutic regimens is warranted. Measles and SSPE are preventable by maintenance of high rates of immunization in the population.

25 Review Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy (PLOSL): a new report of an Italian woman and review of the literature. 2013

Bock, Veronika / Botturi, Andrea / Gaviani, Paola / Lamperti, Elena / Maccagnano, Carmelo / Piccio, Laura / Silvani, Antonio / Salmaggi, Andrea. ·Neuro-Oncology Unit, Fondazione IRCCS Istituto Neurologico C. Besta, Via Celoria 11, 20133 Milan, Italy. VeronikaBock@gmx.de ·J Neurol Sci · Pubmed #23399524.

ABSTRACT: We report the clinical case of a 43year old Italian woman and her family with Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy (PLOSL), also known as Nasu-Hakola disease. PLOSL is a unique disease clinically characterized by a progressive presenile frontal-lobe dementia and multiple cystic bone lesions, typically leading to fractures of the limbs in the third decade of life. This rare recessively inherited disease is caused by mutations in one of two genes encoding different subunits of a receptor signalling complex, TYROBP and TREM2. In the present case fractures after microtrauma were not diagnosed, despite a radiological demonstration of the characteristic bone lesions in PLOSL. Further investigation led to the same diagnosis in her brother, with similar clinical presentation and the same mutation. Therefore a diagnosis of PLOSL should be considered in cases of presenile frontal-lobe dementia, even if the hallmark of pathological fractures is absent.

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