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Encephalitis among cree children in northern Quebec
Author(s) -
Black Deborah N.,
Watters Gordon V.,
Andermann Eva,
Dumont Charles,
Kabay Michel E.,
Kaplan Paige,
MeagherVillemure Kathleen,
Michaud Jean,
O'gorman Gus,
Reece Elena,
Tsoukas Christos,
Wainberg Marchk A.
Publication year - 1988
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410240402
Subject(s) - consanguinity , incidence (geometry) , disease , encephalitis , medicine , pediatrics , white matter , immunology , pathology , virus , physics , optics , magnetic resonance imaging , radiology
We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases and the high degree of parental consanguinity suggest a genetic contribution. We propose that this entity may be caused by an unusual viral infection in a genetically vulnerable host.