title: Communicating hydrocephalus following eosinophilic meningitis is pathogenic for chronic Viliuisk encephalomyelitis in Northeastern Siberia.
abstract: BACKGROUND
  Viliuisk encephalomyelitis (VE) is an endemic neurological disease in Northeast Siberia and generally considered to be a chronic encephalomyelitis of unknown origin actually spreading in the Sakha (Yakutian) Republic.
  
  
  RESULTS
  In search for the pathophysiology and causative agent of VE, we performed a cross-sectional study on clinical, serological and neuroimaging data on chronic VE patients during two medical expeditions to three villages within the Viliuiski river basin in the Republic of Sakha in 2000 and to the capital Yakutsk in 2006. The severity of the core clinical picture with predominant sensory ataxia, gait apraxia, lower limb spasticity, cognitive impairment and bladder dysfunction correlated with the degree of MRI findings showing enlargement of inner ventricular spaces as in communicating hydrocephalus. Laboratory studies revealed transient eosinophilia during the preceding acute meningitis-like phase, but no ongoing inflammatory process in the CSF. We found immune reactions against Toxocara canis in the majority of chronic VE patients but rarely in controls (P = 0.025; Fisher's exact test). Histological analysis of subacute to subchronic VE brain samples showed eosinophilic infiltrations with no signs of persistent Toxocara canis infection.
  
  
  CONCLUSIONS
  Our data showed that pressure by the communicating hydrocephalus as a mechanical factor is the major pathogenic mechanism in chronic VE, most likely triggered by eosinophilic meningitis. There are no signs for an ongoing inflammatory process in chronic VE. The past eosinophilic reaction in VE might be caused by Toxocara ssp. infection and might therefore represent the first hint for an initial cause leading to the development of chronic VE. Our data provide a framework for future studies and potential therapeutic interventions for this enigmatic epidemic neurological disease potentially spreading in Sakha Republic.
authors.affiliation: Division of Neurodegenerative Diseases, Department of Neurology, Dresden University of Technology, Dresden, Germany ; German Center for Neurodegenerative Diseases (DZNE) Dresden, Dresden, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Storch
authors.affiliation: Department of Neurology, University of Ulm, Ulm, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Kassubek
authors.affiliation: Department of Neurology, University of Ulm, Ulm, Germany.
authors.forename: Hayrettin
authors.initials: H
authors.lastname: Tumani
authors.affiliation: North-Eastern Federal University (NEFU), Scientific Research Institute of Health, Yakutsk, Republic of Yakutia, Russian Federation.
authors.forename: Vsevolod A
authors.initials: VA
authors.lastname: Vladimirtsev
authors.affiliation: Division of Neurodegenerative Diseases, Department of Neurology, Dresden University of Technology, Dresden, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Hermann
authors.affiliation: North-Eastern Federal University (NEFU), Scientific Research Institute of Health, Yakutsk, Republic of Yakutia, Russian Federation.
authors.forename: Vladimir L
authors.initials: VL
authors.lastname: Osakovsky
authors.affiliation: Department of Radiology, National Medical Center of Yakutia, Yakutsk, Republic of Yakutia, Russian Federation.
authors.forename: Vladimir A
authors.initials: VA
authors.lastname: Baranov
authors.affiliation: North-Eastern Federal University (NEFU), Scientific Research Institute of Health, Yakutsk, Republic of Yakutia, Russian Federation.
authors.forename: Vadim G
authors.initials: VG
authors.lastname: Krivoshapkin
authors.affiliation: Department of Neurology, University of Ulm, Ulm, Germany.
authors.forename: Albert C
authors.initials: AC
authors.lastname: Ludolph
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D004594:Electrophysiology; D004679:Encephalomyelitis / Q000503:physiopathology*; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000503:physiopathology*; D008297:Male; D008581:Meningitis / Q000503:physiopathology*; D008875:Middle Aged; D012800:Siberia; D055815:Young Adult