title: A Multinational Survey on Actual Diagnostics and Treatment of Subacute Sclerosing Panencephalitis.
abstract: Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system caused by the measles virus (MV). Its prevalence remains high in resource poor countries and is likely to increase in the Northern Europe as vaccination rates decrease. Clinical knowledge of this devastating condition, however, is limited. We therefore conducted this multinational survey summarizing experience obtained from more than 500 patients treated by 24 physicians in seven countries. SSPE should be considered in all patients presenting with otherwise unexplained acquired neurological symptoms. In most patients, the diagnosis will be established by the combination of typical clinical symptoms (characteristic repetitive myoclonic jerks), a strong intrathecal synthesis of antibodies to MV and typical electroencephalogram findings (Radermecker complexes). Whereas the therapeutic use of different antiviral (amantadine, ribavirin) and immunomodulatory drugs (isoprinosine, interferons) and of immunoglobulins has been reported repeatedly, optimum application regimen of these drugs has not been established. This is partly due to the absence of common diagnostic and clinical standards focusing on neurological and psychosocial aspects. Carbamazepine, levetiracetam, and clobazam are the drugs most frequently used to control myoclonic jerks. We have established a consensus on essential laboratory and clinical parameters that should facilitate collaborative studies. Those are urgently needed to improve outcome.
authors.affiliation: Division of Pediatric Neurology and Social Pediatrics, Department of Pediatrics, University Hospital RWTH Aachen, Aachen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Häusler
authors.affiliation: Clinics of Pediatric Neurology, Dr. Sami Ulus Research and Training Hospital of Women's and Children's Health and Diseases, Ankara, Turkey.
authors.forename: Ayse
authors.initials: A
authors.lastname: Aksoy
authors.affiliation: Department of Neuropaediatrics, University Children's Hospital, Tübingen, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Alber
authors.affiliation: Department of Pediatric Neurology, Cukurova University, Faculty of Medicine, Saricam/Adana, Turkey.
authors.forename: Sakir
authors.initials: S
authors.lastname: Altunbasak
authors.affiliation: Private Practice, Göztepe, Istanbul, Turkey.
authors.forename: Aydan
authors.initials: A
authors.lastname: Angay
authors.affiliation: Pediatric Neurology Clinic, Professor Doctor Alexandru Obregia Clinical Hospital, Bucharest, Romania.
authors.forename: Oana Tarta
authors.initials: OT
authors.lastname: Arsene
authors.affiliation: Pediatric Neurology Clinic, Professor Doctor Alexandru Obregia Clinical Hospital, Bucharest, Romania.
authors.forename: Dana
authors.initials: D
authors.lastname: Craiu
authors.affiliation: Department of Pediatrics, Hannover Medical School, Hannover, Germany.
authors.forename: Hans
authors.initials: H
authors.lastname: Hartmann
authors.affiliation: Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.
authors.forename: Semra
authors.initials: S
authors.lastname: Hiz-Kurul
authors.affiliation: Department of Pediatrics, Tsudumigaura Handicapped Children's Hospital, Yamaguchi, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ichiyama
authors.affiliation: Pediatric Neurology Clinic, Professor Doctor Alexandru Obregia Clinical Hospital, Bucharest, Romania.
authors.forename: Catrinel
authors.initials: C
authors.lastname: Iliescu
authors.affiliation: Department of Pediatric Neurology, Clinical Center Nis, Pediatric Clinic, Nis, Serbia.
authors.forename: Bosanka
authors.initials: B
authors.lastname: Jocic-Jakubi
authors.affiliation: Division of Neuropediatrics and Muscular Diseases, Department of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Freiburg, Germany.
authors.forename: Rudolf
authors.initials: R
authors.lastname: Korinthenberg
authors.affiliation: Department of Child Neurology, Health Ministery Şişli Hamidiye Etfal Education and Research Hospital, Istanbul, Turkey.
authors.forename: Gülsen
authors.initials: G
authors.lastname: Köse
authors.affiliation: Manila Department of Pediatrics and Neurosciences, University of the Philippines College of Medicine, Manila, Philippines.
authors.forename: Marissa B
authors.initials: MB
authors.lastname: Lukban
authors.affiliation: Clinics of Pediatric Neurology, Dr. Sami Ulus Research and Training Hospital of Women's and Children's Health and Diseases, Ankara, Turkey.
authors.forename: Mehpare
authors.initials: M
authors.lastname: Ozkan
authors.affiliation: Department of Pediatrics and Medical Genetics, Medical University-Plovdiv, Plovdiv, Bulgaria.
authors.forename: Iliyana
authors.initials: I
authors.lastname: Patcheva
authors.affiliation: Department of Neuropediatrics, University Children's Hospital Zurich, Zürich, Switzerland.
authors.forename: Jens
authors.initials: J
authors.lastname: Teichler
authors.affiliation: Department of Neuroscience, Faculty of medicine, University of Medicine and Pharmacy, Cluj Napoca, Romania.
authors.forename: Mihaela
authors.initials: M
authors.lastname: Vintan
authors.affiliation: Pediatric Neurology Department, Dicle University, Diyarbakır, Turkey.
authors.forename: Ahmet
authors.initials: A
authors.lastname: Yaramis
authors.affiliation: Child Neurology Unit, Eskisehir Osmangazi University, Eskisehir, Turkey.
authors.forename: Coskun
authors.initials: C
authors.lastname: Yarar
authors.affiliation: Division of Child Neurology, Department of Pediatrics, Dokuz Eylül University Faculty of Medicine, İzmir, Turkey.
authors.forename: Uluc
authors.initials: U
authors.lastname: Yis
authors.affiliation: Clinics of Pediatric Neurology, Dr. Sami Ulus Research and Training Hospital of Women's and Children's Health and Diseases, Ankara, Turkey.
authors.forename: Deniz
authors.initials: D
authors.lastname: Yuksel
authors.affiliation: Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: Banu
authors.initials: B
authors.lastname: Anlar
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use*; D001208:Asia; D002220:Carbamazepine / Q000627:therapeutic use; D004569:Electroencephalography; D005060:Europe; D006801:Humans; D007542:Inosine Pranobex / Q000627:therapeutic use*; D007372:Interferons / Q000627:therapeutic use*; D008459:Measles virus / Q000302:isolation & purification; D009207:Myoclonus / Q000188:drug therapy / Q000209:etiology; D013344:Subacute Sclerosing Panencephalitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D011795:Surveys and Questionnaires
chemical_list: D000927:Anticonvulsants; D000998:Antiviral Agents; D002220:Carbamazepine; D007372:Interferons; D007542:Inosine Pranobex