What is SMEB?

for
What is SMEB for Mummies and Daddies

SMEB (Severe Myoclonic Epilepsy of Infancy Borderline)
In Shazza Language

Including conclusions brought about with other parents of kids with Dravet Syndrome, specifically members of the SMEI Yahoo Email Group

Date of Submission 22nd January 2006

What is the difference between SMEB & SMEI?
There is still much speculation as to what the definition is regarding SMEB and the literature changes depending on which source you read, but my primary understanding of the condition is that it is a slight deviation from what we might call the classic SMEI (Severe Myoclonic Epilepsy of Infancy), also known as Dravet's Syndrome.

Most of the clinical features associated with SMEI are present within SMEB patients. I.e. The onset of seizures will occur prior to 12months of age, SMEB children will still present with difficult to control seizures and will also have frequent status episodes.

SMEB patients will present with all the classic symptoms of SMEI, but may also exhibit one or more of the following features:

  • Normal EEG
  • Lack of myoclonic seizures
  • ??There is also some speculation that a child presenting with all the classic signs of SMEI, but with normal, or near normal development may also come under this classification.??

What are Myoclonic Seizures?
Myoclonic seizures are quick jerks, usually singular and can be mistaken for a startled reflex. The myoclonics may occur in clusters (meaning a lot of jerks in a group) sometimes lasting hours. The person experiencing myoclonic seizures are fully awake and aware of what is happening. Myoclonic seizures can be as subtle as a chin twitch and as vicious as to knock a person to the floor.

Treatment
It is assumed that although some of the characteristics of SMEB are different to SMEI, the drugs that are assumed to work well in SMEI patients are also assumed to also work well in SMEB patients. It is also thought that those drugs deemed counterproductive to SMEI patients (in some cases can prove harmful) should also be avoided in SMEB patients when suitable alternatives are available.

In Conclusion
Although there is currently a lot of research into Dravet's Syndrome and SMEB, there are still many questions yet to be answered. Many discoveries have been made I.e. the associated SCN1A mutation often associated with Dravet's Syndrome. But there is a lot more to the story, yet to be discovered. Hopefully in time we'll have a more accurate way of diagnosing these conditions, and diagnosis won't be quite as hit and miss as it seems to be at present.

What is SMEB? In Shazza Language Sister Document
"What is Dravet's Syndrome? In Shazza Language": can be viewed here.

 

Disclaimer: The materials provided within this document are for informational purposes and are not intended for use as a diagnostic or treatment tool, or as a substitute for consulting a licensed medical professional. Please remember I am just a mum and have absolutely no medical expertise what so ever. This site will not be held responsible for any inaccuracy's present.