Fatal acute encephalopathy in two siblings: A distinct hereditary entity?

Viral infections particularly the influenza infection can be associated with significant central nervous system complications. The encephalopathy associated with viral infection may not be necessarily due to invasion by the microorganism but rather the hypercytokinemic response of the host. Individuals with certain genetic background are postulated to be at risk of this complication. Recent works in this area are aiming to elucidate the molecular mechanism and genetic susceptibility of this condition. Familial cases of infection associated encephalopathy indicate strong hereditary predisposition in some individuals. We describe a family with two siblings who developed fatal acute encephalopathy following respiratory tract infection of likely viral etiology.