Doose Syndrome

Doose syndrome, also known as Myoclonic-Astatic Epilepsy (MAE), is a rare childhood epilepsy syndrome characterized by a variety of seizure types.

The Epilepsy Institute of New Jersey is a team of board-certified neurologists and neurosurgeons who provide expert care for a variety of pediatric neurological conditions, including Doose syndrome. Our team uses advanced diagnostic tools and innovative treatment techniques to ensure the best outcomes for your child.

The Physical Impact

Doose syndrome, also known as Myoclonic-Astatic Epilepsy (MAE), is a rare childhood epilepsy syndrome characterized by a variety of seizure types, including myoclonic (brief, shock-like muscle jerks) and atonic (sudden loss of muscle tone) seizures. These seizures can significantly affect a child's quality of life, impacting their ability to perform daily activities, learn, and interact socially. Early diagnosis and effective management are key to reducing seizure frequency and improving overall development.

How Common Is This Condition?

Doose syndrome is relatively rare, accounting for a small percentage of childhood epilepsy cases. It typically begins in early childhood, often between the ages of 2 and 5. While the exact cause is not always known, genetic factors are believed to play a role in many cases.

Risk Factors:

  • Family history of epilepsy or neurological disorders
  • Genetic mutations or inherited predispositions
  • Early age of seizure onset

Signs and Symptoms

Children with Doose syndrome may experience a range of seizure types and neurological symptoms, including:

  • Myoclonic seizures (brief muscle jerks)
  • Atonic (drop) seizures, causing sudden loss of muscle tone and falls
  • Generalized tonic-clonic seizures (full-body convulsions)
  • Absence seizures (brief staring spells)
  • Delays in speech, motor skills, or cognitive development due to frequent seizures

How We Diagnose

At the Epilepsy Institute of New Jersey, diagnosing Doose syndrome involves a thorough evaluation of your child's medical history and seizure patterns. We use advanced diagnostic techniques to identify the nature of the seizures and guide a personalized treatment plan. Key diagnostic steps include:

  • EEG (Electroencephalogram): MRecords the brain's electrical activity to identify characteristic patterns associated with Doose syndrome.
  • MRI (Magnetic Resonance Imaging): Helps rule out structural abnormalities in the brain.
  • Genetic Testing: May be recommended if a hereditary cause is suspected.
  • Neurodevelopmental Assessments: Evaluates motor skills, cognitive function, and developmental milestones.

This comprehensive diagnostic approach ensures we understand your child's condition and can create an effective treatment plan.

Treatment Options

Managing Doose syndrome focuses on reducing the frequency and intensity of seizures, as well as supporting healthy development. Treatment options may include:

  • Medications: Anti-seizure drugs tailored to your child's seizure types.
  • Dietary Therapy: The ketogenic or modified Atkins diet, which can be effective in controlling hard-to-treat seizures.
  • Vagus Nerve Stimulation (VNS): An implantable device that sends electrical impulses to help prevent seizures.
  • Behavioral and Developmental Support: Coordination with therapists and educators to help support your child's cognitive and social development.

The Epilepsy Institute of New Jersey takes a multidisciplinary approach, working closely with families to find the right combination of treatments that improve quality of life for children with Doose syndrome.

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Our Commitment to Your Child's Health

“I am neurosurgeon living in New Jersey, board-certified by both the American Board of Neurosurgery (ABNS) and Pediatric Neurosurgery (ABPNS). I specialize in the surgical treatment of complex epilepsy and serve as the Director of the Epilepsy Institute of New Jersey, Chief of Pediatric Epilepsy Surgery at Sanzari Children's Hospital, and Chief of Pediatric Neurosurgery at Children's Hospital of NJ, where I provide exceptional care for children with complex neurological conditions. Your child's health and well-being are our top priorities.”

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Dr. Luke Tomycz
@epilepsyinstnj

Schedule Consultation

If your child has been diagnosed with Doose syndrome or is experiencing challenging seizures, our team at the Epilepsy Institute of New Jersey is here to help. Contact us to schedule a consultation. We'll guide you through every step of the process, offering compassionate and personalized care tailored to your child's needs.

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