Alexander disease type I

Alexander disease type I, also known as AxD type I, is a rare neurological condition that affects the brain’s support cells, called astrocytes. It is the most severe and common form of Alexander disease and typically appears before a child reaches 4 years old. This form of the disease is marked by significant early onset symptoms including seizures, an unusually large brain (megalencephaly), and delays in development. Over time, children with this condition experience a progressive decline in their neurological function. The disease leads to a gradual deterioration in many areas of brain function, which contributes to developmental challenges. As it is an astrogliopathy, the problem lies in the abnormal functioning of astrocytes, which play critical roles in maintaining the health and structure of the brain.

People with Alexander disease type I often show signs such as seizures, an enlarged brain (megalencephaly), and developmental delays. As the condition progresses, there is also a gradual deterioration in neurological function.

Information about causes is currently limited for this condition.

Information about treatment is currently limited for this condition.

The condition is characterized by progressive deterioration in development and neurological function, leading to significant challenges as it advances. Detailed information on the long-term outlook is not available.

Information about current research and future directions is currently limited for this condition.