title: Markedly elevated nitrate/nitrite levels in the cerebrospinal fluid of children with progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome).
abstract: OBJECTIVE
  To compare the levels of brain nitric oxide production in patients with PEHO or PEHO-like syndrome and in controls with other neurologic disease.
  
  
  METHODS
  Nitric oxide metabolites, nitrates, and nitrites (NNx), were measured in the cerebrospinal fluid (CSF) of children with PEHO syndrome or PEHO-like syndrome, and in controls with other neurologic diseases.
  
  
  RESULTS
  The NNx levels were markedly higher in both PEHO (mean, 48 microM; p < 0.001) and PEHO-like (22 microM; p < 0.003) patients as compared with the controls (6 microM), but did not correlate with age or with brain atrophy or CSF levels of insulin-like growth factor-1 (IGF-1).
  
  
  CONCLUSIONS
  Our findings suggest that in PEHO syndrome, production of nitric oxide is markedly increased, suggesting that nitric oxide is involved in the pathologic phenomena (i.e., seizures and neurodegeneration) of the disease.
authors.affiliation: Department of Child Neurology, Children's Hospital, University of Kuopio, Kuopio, Finland. svanhata@helsinki.fi
authors.forename: S
authors.initials: S
authors.lastname: Vanhatalo
authors.forename: R
authors.initials: R
authors.lastname: Riikonen
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001284:Atrophy; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D019636:Neurodegenerative Diseases / Q000134:cerebrospinal fluid* / Q000378:metabolism / Q000473:pathology; D009566:Nitrates / Q000134:cerebrospinal fluid*; D009569:Nitric Oxide / Q000378:metabolism*; D009573:Nitrites / Q000134:cerebrospinal fluid*; D009896:Optic Atrophy / Q000134:cerebrospinal fluid / Q000378:metabolism; D013036:Spasms, Infantile / Q000134:cerebrospinal fluid / Q000378:metabolism; D013577:Syndrome
chemical_list: D009566:Nitrates; D009573:Nitrites; D009569:Nitric Oxide