Abstract
Aicardi-Goutieres syndrome was first described in 1984 in a series of eight patients. The clinical picture is of infants with familial progressive encephalopathy, basal ganglia calcification, and chronic cerebrospinal fluid pleocytosis (Table 1). It is a very rare syndrome, suspected to be familial with autosomal-recessive inheritance, and occurrence in siblings has been reported. A majority of reported cases have elevated serum levels of α-interferon, which is of unclear pathogenic significance.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Sources
Aicardi-Goutieres Syndrome
Goutieres F, Aicardi J, Barth PG, Lebon P. Aicardi-Goutieres syndrome: an update and results of interferon-alpha studies. Ann Neurol 1998;44:900–907.
Koul R, Chacko A, Joshi S, Sankhla D. Aicardi-Goutieres syndrome in siblings. J Child Neurol 2001;16:759–761.
Early Myoclonic Encephalopathy
Aicardi J. Early myoclonic encephalopathy. In: Roger J, Dravet C, Bureau M, Dreifuss FE, Wolf P, eds. Epileptic Syndromes of Infancy and Adolescence.London: John Libbey, 1985:12–22.
Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30:389–399.
Lombroso CT. Early myoclonic encephalopathy, early infantile epileptic encephalopathy, and benign and severe infantile myoclonic epilepsies: a critical review and personal contributions. J Clin Neurophysiology 1990;7:380–408.
Wang PJ, Lee WT, Hwu WL, et al. The controversy regarding diagnostic criteria for early myoclonic encephalopathy. Brain Dev 1998;20:530–535.
Idiopathic Localization-Related Epilepsies
Negoro T. Diagnosis and treatment of idiopathic focal epilepsies (benign partial epilepsies) in infancy and childhood. Epilepsia 2005;46(Suppl 3):S3–S38.
Lennox-Gastaut Syndrome
Lennox WG, Davis JP. Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 1950;5:626–644.
Medial Temporal Lobe Epilepsy
ILAE commission report. Mesial temporal lobe epilepsy with hippocampal sclerosis. Epilepsia 2004;45:695–714.
Pfander M, Arnold S, Henkel A, et al. Clinical features and EEG findings differentiating mesial from neocortical temporal lobe epilepsy. Epileptic Disord 2002;4:189–195.
Sudden Unexpected Death in Epilepsy
Langan Y, Sander JWAS. Sudden unexpected death in patients with epilepsy. Definition, epidemiology and therapeutic implications. CNS Drugs 2000;13:337–349.
Nashef F. Sudden unexpected death in epilepsy: terminology and definitions. Epilepsia 1997;38:S6–S8.
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2006 Humana Press Inc., Totowa, NJ
About this chapter
Cite this chapter
(2006). Epilepsy. In: Lerner, A.J. (eds) Diagnostic Criteria in Neurology. Current Clinical Neurology. Humana Press. https://doi.org/10.1007/978-1-59745-078-2_6
Download citation
DOI: https://doi.org/10.1007/978-1-59745-078-2_6
Publisher Name: Humana Press
Print ISBN: 978-1-58829-482-1
Online ISBN: 978-1-59745-078-2
eBook Packages: MedicineMedicine (R0)