DEEs are a group of rare and ultra-rare epilepsy syndromes that can be severe
They are characterized by seizures, electroencephalogram (EEG) abnormalities, and encephalopathy, meaning significant developmental delay or even loss of developmental skills.
Beyond these clinical features, DEEs can also appear as:
Examples of DEEs include Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS).
As in epilepsy, rare epilepsy syndromes can be traced to several causes, but some people have no identifiable cause.
The body’s immune system, which usually protects the body from illness, starts attacking the body instead.
A mutation or variation in a gene that causes disease can be new (arising in a person with no family history of it) or inherited from parents.
Seizures can be brought on by fever (febrile seizures) or infections in the brain.
Unusual formations in the brain that can be seen with imaging, such as MRI scans. Formations can be present from birth or occur later in life, such as a head injury.
An inability to break down food or problems generating energy to sustain the body.