title: Challenges in diagnosing SSPE.
abstract: OBJECTIVE
  The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties.
  
  
  METHODS
  We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study.
  
  
  RESULTS
  Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria.
  
  
  CONCLUSIONS
  We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization.
authors: Department of Neurology, Cerrahpasa Faculty of Medicine, Istanbul University, 34098 Fatih, Istanbul, Turkey.
authors: Ozdem
authors: O
authors: Erturk
authors: Beyhan
authors: B
authors: Karslıgil
authors: Ozlem
authors: O
authors: Cokar
authors: Zuhal
authors: Z
authors: Yapici
authors: Veysi
authors: V
authors: Demirbilek
authors: Candan
authors: C
authors: Gurses
authors: Cengiz
authors: C
authors: Yalcinkaya
authors: Aysen
authors: A
authors: Gokyigit
authors: Guher Saruhan
authors: GS
authors: Direskeneli
authors: Sibel
authors: S
authors: Yentur
authors: Emel
authors: E
authors: Onal
authors: Gulden
authors: G
authors: Yilmaz
authors: Aysin
authors: A
authors: Dervent
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology*; D004569:Electroencephalography / Q000379:methods; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000134:cerebrospinal fluid; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012640:Seizures / Q000209:etiology*; D013344:Subacute Sclerosing Panencephalitis / Q000134:cerebrospinal fluid / Q000150:complications / Q000175:diagnosis*; D014760:Viral Fusion Proteins / Q000134:cerebrospinal fluid; D014786:Vision Disorders / Q000209:etiology
chemical_list: D007074:Immunoglobulin G; D014760:Viral Fusion Proteins