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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.

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Treatment options for Myoclonic Atonic Epilepsy

Vagus Nerve Stimulation: By delivering frequent, mild electrical energy pulses to the brain through the vagus nerve, vagus nerve stimulation (VNS) can either prevent or reduce seizures.A device is implanted beneath the skin in the left chest region as part of the therapy. The generating device is connected to an electrode or wire that is positioned beneath the skin. The vagus nerve in the neck is where the wire is fastened or coiled.In the outpatient clinic, the gadget is configured to administer stimulation or pulses at predetermined intervals. This device operates without the user having to do anything.When a VNS device is operating, the user is typically unaware of the stimulation.A person can transmit an additional burst of stimulation to the brain by swiping a magnet over the generator in the left chest area if they are aware of when a seizure occurs. This may help prevent seizures for some people.


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When myoclonic-atonic seizures are observed by family members or medical professionals, EMAS is diagnosed. A physical examination and medical history might give doctors some information regarding the seizures. They might inquire about the types of seizures and the onset of seizures. To find out more, they frequently employ laboratory studies.

Additional indicators of an EMAS diagnosis include:

  • Before seizures, development had been normal or almost normal.
  • Seizures that started between the ages of two and six; normal findings from brain imaging
  • There are no other known causes for these seizures, while GLUT1 syndrome is one possible culprit

Children with MAE who experience prolonged or frequent seizures may require rescue therapy or emergency medical attention. Diazepam rectal gel (Diastat) or another benzodiazepine administered under the tongue or intranasally into the nose are examples of rescue therapy.

For information on seizure crises and choices for home rescue medication, parents of children with MAE should speak with their treating neurologist or other healthcare professional.

Children may require immediate medical attention if rescue medication fails to halt seizures or if they are suspected of having non-convulsive status epilepticus, which is characterized by reduced consciousness, drooling, unsteadiness, and twitching of the arms, legs, or face.

Children with MAE frequently have seizures that are challenging to manage and may not react well to medicine. The types of seizures are used to determine the medications such as:

  • Valproic acid/divalproex, lamotrigine, levetiracetam, topiramate, zonisamide, rufinamide, clobazam, and felbamate are commonly used to treat generalized tonic-clonic, myoclonic, and myoclonic atonic seizures.
  • Lamotrigine, ethosuximide, or valproic acid/divalproex are commonly used to treat absence seizures.

Since some seizure drugs can exacerbate specific seizure types, they should not be administered in children with MAE. These consist of vigabatrin, phenytoin, carbamazepine, and oxcarbazepine.

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Hospitals for Myoclonic Atonic Epilepsy in India

Marengo Asia Hospitals, Gurugram: Top Doctors, and Reviews
Marengo Asia Hospitals, Gurugram

Gurgaon, India

  • Marengo Asia Hospitals, Gurugram, is a 250-bed multi-speciality tertiary care hospital, formerly known as W Pratiksha Hospital.
  • A major medical centre for Delhi NCR and North India, it is situated on Golf Course Extension Road, Sector 56, Gurugram.
  • The hospital's NABH and NABL accreditation ensures compliance with international standards for patient care, safety, and quality.
  • Provides extensive medical care in more than 21 specialities, including as women's health, neurology, orthopaedics, cardiology, and cancer.
  • Advanced robotic-assisted spine surgery tools and neuro-monitoring technology are available at the Marengo Asia International Institute of Neuro & Spine (MAIINS), a Centre of Excellence in Neurosciences.
  • Equipped with cutting-edge equipment, such as automated labs, sophisticated imaging, and contemporary operating rooms.
  • Features specialised ICUs, NICUs, and modular OTs built for complex and minimally invasive procedures.
Jaslok Hospital & Research Center: Top Doctors, and Reviews
Jaslok Hospital & Research Center

Mumbai, India

  • Jaslok Hospital & Research Centre, Mumbai, is a 350-bed super-speciality tertiary care hospital, established in 1973 and among India’s most respected private trust hospitals.
  • Located on Peddar Road in South Mumbai, overlooking the Arabian Sea, it serves as a major referral centre for Mumbai, Maharashtra, and patients from across India and abroad.
  • The hospital offers comprehensive care across 50+ specialities and nearly 20 multidisciplinary speciality clinics, addressing complex and advanced medical conditions.
  • Supported by 300+ senior consultants and 200 resident doctors, ensuring high standards of expertise and round-the-clock patient care.
  • Equipped with 75 ICU beds, advanced critical care units, modular operation theatres, and state-of-the-art diagnostic and imaging facilities.
  • Recognised as a leading centre of excellence in Critical Care, Cardiac Sciences, Neurosciences, Oncosciences, Renal Sciences, Gastroenterology, and Assisted Reproduction.
  • A prominent research and academic institution, conducting clinical trials and recognised by the National Board of Examinations (NBE) in 22 specialities.
  • Strong emphasis on quality, patient safety, and evidence-based care, with continuous monitoring of clinical and operational outcomes to meet international healthcare standards.
Max Super Speciality Hospital, Patparganj: Top Doctors, and Reviews
Max Super Speciality Hospital, Patparganj

Delhi, India

Max Super Speciality Hospital, Patparganj, a 400+ bed NABH-accredited facility under Balaji Medical and Diagnostic Research Centre, offers world-class care across 33+ specialties, including Cardiac Sciences, Oncology, Neurosciences, Orthopaedics, Obstetrics & Gynaecology, and Kidney Transplant. With 116 ICU beds, 14 HDU beds, 11 modular OTs, and an NABL-accredited Max Lab, the hospital ensures advanced, quality-driven care. Staffed by 510+ expert doctors and 770+ nurses, it’s India’s first AACI-accredited super speciality hospital and North & West India’s first QAI-accredited Advanced Brain Stroke Centre. Max Patparganj is a regional leader in complex surgeries, blending cutting-edge technology with compassionate, patient-centric healthcare.

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Why Choose India for Myoclonic Atonic Epilepsy Treatment?

Here are some of the reasons for choosing India:

Because the cost of vagus nerve stimulation therapy is far lower in India than in other countries, people from all over the world pick this country as the leader in medical tourism for this treatment. The finest outcomes are achieved by top neurosurgeons in India who specialize in Vagus Nerve Stimulation procedures and have exceptional surgical abilities.

Frequently Asked Questions

Although there is no known cure for myoclonic atonic seizures, some people can completely control them with medication and vagal nerve stimulation therapy. Epilepsy surgery can occasionally stop myoclonic seizures in some patients who have myoclonus due to focal epilepsy. However, once myoclonic seizures have started, it is impossible to tell if a person will outgrow them. Some patients with myoclonic atonic epilepsy have seizures that are resistant to medication, making treatment difficult. Managing seizures and reducing the risk of developmental delays need early treatment with the right medicine and close observation.

India has shown notable success rates in treating and managing myoclonic atonic epilepsy thanks to its highly qualified pediatric neurosurgeons and state-of-the-art medical equipment. Many patients are guaranteed satisfactory outcomes thanks to the excellent treatment options provided by the country's healthcare facilities.

Indeed, a lot of Indian hospitals treat and manage myoclonic atonic epilepsy with medicine and vagal nerve stimulation therapy. • There are qualified neurosurgeons in India who are capable of vagal nerve stimulation therapy.

Always consider the following factors when selecting Indian hospitals:

  • Hospitals that are accredited (authorised by JCI and NABH).
  • Patient reviews and success rates.
  • Hospitals that provide seamless care to patients from abroad.

The success rate of treating atonic epilepsy in India is great, as are the survival rates for major surgeries such vagal nerve stimulation. Leading hospitals provide state-of-the-art care and specialized knowledge.

EMAS cannot be diagnosed by a single test. Instead, Myoclonic Atonic Epilepsy must be diagnosed by physicians using clinical data, laboratory testing, and tests like electroencephalograms.

This kind of epilepsy is extremely severe and becomes worse over time. It typically involves brain damage or degradation that impacts memory, motor control, cognitive function, and other areas. In some cases, this can lead to death.

The "cure" for Myoclonic Atonic Epilepsy (MAE) cannot be performed before birth.

A helmet that provides protection wherever your child goes can be fitted by your neurologist and/or the hospital. Starting with this medical equipment from your healthcare providers should be your initial move, however some parents have also used off-the-shelf solutions like hockey helmets. Second, parents can buy baby gates to keep their kids from walking upstairs or downstairs without an adult present if their home contains stairs. Third, parents can use their imagination to create safety barriers around fireplaces, furniture edges, and other areas. Finally, to assist prevent injuries during dining, parents can buy safety mats and eating utensils.