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What is Aicardi-Goutieres syndrome?

What is Aicardi-Goutieres syndrome?

  • Aicardi-Goutieres syndrome: A rare inherited progressive disease that affects the brain and immune system.

Aicardi-Goutieres syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Aicardi-Goutieres syndrome, or a subtype of Aicardi-Goutieres syndrome, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Aicardi-Goutieres syndrome as a "rare disease".
Source - Orphanet

Aicardi-Goutieres syndrome: Introduction

Types of Aicardi-Goutieres syndrome:

Broader types of Aicardi-Goutieres syndrome:

How serious is Aicardi-Goutieres syndrome?

Prognosis of Aicardi-Goutieres syndrome: death common early childhood or persistent vegetative state in less severe cases
Complications of Aicardi-Goutieres syndrome: see complications of Aicardi-Goutieres syndrome

What causes Aicardi-Goutieres syndrome?

Causes of Aicardi-Goutieres syndrome: see causes of Aicardi-Goutieres syndrome

What are the symptoms of Aicardi-Goutieres syndrome?

Symptoms of Aicardi-Goutieres syndrome: see symptoms of Aicardi-Goutieres syndrome

Complications of Aicardi-Goutieres syndrome: see complications of Aicardi-Goutieres syndrome

Onset of Aicardi-Goutieres syndrome: symptoms usually appear within six months of birth

Aicardi-Goutieres syndrome: Testing

Diagnostic testing: see tests for Aicardi-Goutieres syndrome.

Misdiagnosis: see misdiagnosis and Aicardi-Goutieres syndrome.

How is it treated?

Doctors and Medical Specialists for Aicardi-Goutieres syndrome: Medical Geneticist ; see also doctors and medical specialists for Aicardi-Goutieres syndrome.
Treatments for Aicardi-Goutieres syndrome: see treatments for Aicardi-Goutieres syndrome
Research for Aicardi-Goutieres syndrome: see research for Aicardi-Goutieres syndrome

Name and Aliases of Aicardi-Goutieres syndrome

Main name of condition: Aicardi-Goutieres syndrome

Other names or spellings for Aicardi-Goutieres syndrome:

encephalopathy infantile [calcification basal ganglia - chronic CSF lymphocytosis], Encephalopathy-basal ganglia-calcification

AGS1, Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Aicardi-Goutieres syndrome: Related Conditions

Research the causes of these diseases that are similar to, or related to, Aicardi-Goutieres syndrome:


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