Viliuisk or Vilyuisk encephalitis
(VE) / bokhoror / bokhoroor
Epidemiology : it strikes exclusively
in eastern Siberia in the Republic of Sakha (Yakutia / Yakutija) in Vilyui
river basin (mainly in Vilyuisk and Kobyaisk regions) among natives Yukat
(among which also SCA1
is much common; more they have fewer T cells than Russian), Evenks and
Evens; it is known there since the middle of the last century. The Vilyuy
region is a diamond province where a complex of chemical pollutants from
diamond mining, products of wood decay in places of flooding of the water
reservoir for the Vilyuisk power station, highly mineralised underground
waters and consequences of underground explosions have caused a substantial
negative effect on the environment and people.
Aetiology : previously considered caused
murine encephalomyelitis virus (TMEV), but it was just a contaminant.
Hypothetical role for an Herpesvirus
or autoimmune disorder similar to neuro-Behçet
: anyway infective aetiology hypothesis is supported by infection of Ludmila
Alekseeva, a Russian lab technician that tried to kill herself by injecting
into her hand CSF from a man who died from VE.
Pathogenesis : multiple micronecrotic
foci surrounded by inflammatory infiltrates are observed throughout the
cerebral cortex and other gray matter areas
=> the incubation time could be possibly 14 years or more.
rapid (subacute) form : severe subacute meningo-encephalitis progressing
to a more prolonged pan-encephalitic syndrome, stiff gait, slurred speech,
rigid, spastic muscles, encephalitis lethargica (sleeping sickness), die
slowly progressive form : recover from the acute onset, recurrences
followed by remissions
chronic form : lack inflammatory cells, in people who never
have had an acute attack
Laboratory examinations : intrathecal synthesis
of oligoclonal IgG.
Prognosis : fatal outcome within 1 to
10 years. Some patients survive to a steady state of global dementia and
severe spasticity that may last for over 20 years