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Aicardi-Goutieres syndrome

rare

Aicardi-Goutieres syndrome (AGS) is an inherited, subacute encephalopathy characterized by the association of basal ganglia calcification, leukodystrophy and cerebrospinal fluid (CSF) lymphocytosis.

Identifiers
MONDO:0018866 Orphanet:51
Part of
Autosomal recessive diseaseHereditary disorder of connective tissueInborn error of immunityLeukodystrophyType 1 interferonopathy of childhood
Also known as
AGS · Aicardi Goutieres syndrome · Aicardi-Goutières Syndrome · Cree encephalitis · encephalopathy with basal ganglia calcification

Subtypes

All diseases Rare only
Disease Subtypes
Aicardi-Goutieres syndrome 1 1
Aicardi-Goutieres syndrome 2
Aicardi-Goutieres syndrome 3
Aicardi-Goutieres syndrome 4
Aicardi-Goutieres syndrome 5
Aicardi-Goutieres syndrome 6
Aicardi-Goutieres syndrome 7
Aicardi-Goutieres syndrome 8
Aicardi-Goutieres syndrome 9
Basal ganglia calcification, idiopathic, childhood-onset

Rare disease

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