Dravet syndrome is a genetic disorder characterized by seizures accompanied by high fevers. Most Dravet syndrome patients begin having symptoms in their first year of life.
The seizures experienced by Dravet syndrome patients can be either generalized or partial.
A generalized seizure, also called a tonic-clonic seizure, is characterized by convulsions and loss of consciousness. A partial seizure (sometimes called a focal seizure) affects only a specific area of the brain.
Types of partial seizures
Partial seizures can be simple, in which patients remain conscious, or complex, in which consciousness is impaired or lost. A simple partial seizure may trigger a complex partial seizure also known as focal impaired-awareness seizure, or a generalized seizure.
Simple partial seizures
Simple partial seizures are further subdivided into different categories: autonomic, emotional, motor, and sensory. Because many Dravet syndrome patients are very young when symptoms start, simple partial seizures can be difficult to diagnose.
Autonomic seizures are accompanied by symptoms such as nausea or abdominal discomfort, stomach pain, or vomiting. This is sometimes referred to as abdominal epilepsy. Other symptoms may include flushing or pallor, and altered heart rate or breathing.
Emotional seizures can be very odd. Patients may see things that are not there, or feel emotions like fear, sadness, anger, or joy for no apparent reason.
Motor seizures cause jerking or convulsive movements. These can begin in one area of the body, such as the face, arm, or leg, and then spread to other parts of the body. Unlike in generalized seizures or complex partial seizures, patients do not lose consciousness and usually remember the seizure afterward.
Sensory seizures involve a sensory experience, like seeing lights, hearing a buzzing noise, or feelings of tingling or numbness.
Focal impaired-awareness seizures
Focal impaired-awareness seizures affect a larger area of the brain than simple partial seizures and also affect consciousness, though patients may not lose consciousness completely. Some may appear to be conscious because they remain standing with eyes open and moving about, but it will be an altered consciousness — a dreamlike, almost trancelike state.
Some patients may be able to speak during a focal impaired-awareness seizure, but the words may not make sense. Patients may engage in a repetitive motion like picking at a button, or other simple gestures. Actions and movements are typically unorganized, confused, and unfocused during a focal impaired-awareness seizure.
Other information
Partial seizures generally last only a few minutes. In the case of simple partial seizures, the patient may be completely aware of everything that has happened during the seizure. In focal impaired-awareness seizures, as in generalized seizures, the patient generally does not remember what happened and may be confused or shaky afterward. As with generalized seizures, careful monitoring is required during partial seizures.
It is important to keep track of how long the seizures last as longer seizures may require emergency medical treatment.
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