Myoclonic Atonic Epilepsy Treatment in Thailand

What is Myoclonic Atonic Epilepsy?

Myoclonic-atonic seizures (EMAS) are a rare type of epilepsy that affects children. Seizures are triggered by aberrant electrical firings in the brain. Epilepsy is characterized by recurrent seizures.

Previously known as Doose syndrome, epilepsy with myoclonic atonic seizures is a syndrome marked by the typically sudden development of several generalized seizure types, including myoclonic-atonic seizures in early childhood. The period of active seizures is usually when developmental standstill or regression occurs. Two-thirds of children who have epilepsy attain remission despite having seizures that are initially resistant to medication.

What can I expect if my child has Myoclonic Atonic Epilepsy syndrome?

In EMAS, the initial seizure often happens between the ages of two and six.

The first seizure happens during a fever in 1 in 4 children. EMAS is more common in boys than in girls. The majority of seizures in children with EMAS are generalized seizures, or seizures that impact the entire brain. Focal seizures are uncommon in EMAS and only impact one area of the brain. Multiple seizure types can be included in EMAS, including:

Seizures in EMAS can occasionally alter a child's state of consciousness. This may occur in the absence of overt seizure symptoms. For example, there might not be any jerking. This is known as nonconvulsive status epilepticus when it happens and persists for a long time.

What are the Common Symptoms of Myoclonic Atonic Epilepsy Syndrome?

Seizures that are myoclonic-atonic are the main sign of EMAS. However, developmental setbacks might also be a sign. As EMAS develops, they are observed. A child's development is often normal or just slightly delayed when seizures start. Around the same time as the seizures start, developmental impairments start. Although there may be another cause for both, many researchers think that the seizures are a contributing factor to the developmental deficits. Children with EMAS may have parents who report:

1. Regression (loss of significant events)
2. Develop mental plateau (the inability to acquire new abilities)
3. The Stormy Phase the majority of kids go through an active phase, during which they may have a lot of different kinds of seizures. The "stormy phase" refers to both the seizures and the setbacks.

Causes, Risk Factors and Complications Myoclonic Atonic Epilepsy syndrome

Causes

• At this time, the cause of EMAS is unknown. It appears that genetics is involved. But it doesn't appear to be caused by a single genetic defect.
• EMAS has been associated with a number of genetic variations. For example, it is believed that the SYNGAP1 and SLC6A1 genes have a significant role in EMAS. Additional genes of importance might be found in future research.
• A family history of seizures, especially seizures brought on by fever, is present in one out of every three EMAS cases.

Risk Factors

In addition to seizures, people with epilepsy may also have other health issues. These issues may be caused by the epilepsy itself, or they may be related to the seizures. For instance, they might have mood swings, adjustments to the timing of seizures, or adverse drug reactions. Others could have trouble sleeping, thinking and remembering things, or interacting with people. Rarely, SUDEP (Sudden Unexpected Death in Epilepsy) or early death may transpire.

Complications

EMAS complications are, rarely, people with epilepsy may pass away unexpectedly. While some people may have seizures that are simple to manage, others may have more severe seizures that may require emergency care.

Latest Research and Technologies in the Treatment of Myoclonic Atonic Epilepsy in Thailand

Thailand is using state-of-the-art research and technologies to treat myoclonic atonic epilepsy. To effectively control seizures, hospitals are using neuromodulation techniques such as Vagus Nerve Stimulation (VNS) and Responsive Neurostimulation (RNS), ketogenic diet therapy, and next-generation anti-epileptic medications (AEDs). Precision medicine techniques and genetic testing aid in patient-specific treatment customization.