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Epilepsy Syndromes

by Carlo Raj, MD

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      Slides 09 Seizures Neuropathology I.pdf
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    00:00 Let's talk about epilepsy syndromes, benign rolandic epilepsy. Age of onset: Childhood.

    00:08 Seizure types: You can differentiate between focal onset, aware, and with motor onset; possible frontoparietal opercular features are hemifacial clonic movements. Aphasia, hypersalivation, and chewing movements, automatisms. Seizures typically last less than 5 minutes, most less than 10 minutes. There is also the possibility of secondary generalization.

    00:34 Associated findings: Nocturnal preponderance of seizures, nocturnal, at night. EEG finding: We have temporal spikes or centrotemporal spikes. Treatment: Frequently no treatment is needed as seizures are nocturnal and self-limited, maybe perhaps you're thinking about a sodium channel blocker known as carbamazepine. We'll get into treatment further, but at this point benign rolandic epilepsy luckily most of the time with this being a nocturnal issue, self-limited in nature. Centrotemporal spikes is the clinical pearl. Here, it's called Lennox-Gastaut syndrome. Interesting enough. There has been this huge push, well not just push but let's just say cannabis has taken on quite a bit of media attention. Hasn't it? CNN, all of these and you know about Dr. Gupta and his studies and his investigations on children that have had particular epileptic issues such as Lennox-Gastaut syndrome. Let's begin. Age of onset: Childhood, early adolescence. We're going to be very deliberate here because this condition is no joke. Seizure type: Tonic, clonic, myoclonic, generalized. All over the place, huh. All over the place. What else? Mental retardation association. EEG findings and you should know this, slow 1-2 hertz paroxysmal fast activity, multifocal spikes. Now, what you need to know at this point for management is not going to be cannabis. That is not going to be an answer, please don't choose that. Any other point is we know that marijuana and cannabis has been receiving quite a bit of attention because in certain types of epilepsies especially in children in which the occurrence of such seizures are so severe that maybe perhaps marijuana has a place, but as far as you're concerned right now on your boards and words and current clinical practice, this is what you need to know. Almost all required EEG type of work-up, now we'll go into the drug soon enough. We have valproic acid or valproate, lamotrigine, felbamate are most broad spectrum type of drugs that you're thinking about with a particular childhood epileptic syndrome known as Lennox-Gastaut syndrome. You've heard of Charlotte's web.

    03:23 Here we have absence epilepsy and this is the epilepsy or seizure that I had demonstrated at the beginning of our topic. Once again, childhood and adolescence would be most common.

    03:34 Absence meaning to say that you have these blank stares that may last for seconds.

    03:40 Associated findings: Hyperventilation could be a trigger but usually you have these children that will grow out of these seizures with time, thank goodness. I'll get into the treatment soon enough. You must know for absence here we have 3 hertz spike in wave. Clinical pearl: 3 hertz for absence epilepsy. The drug of choice is going to be ethosuximide. Sure, valproic could be here too, but your focus should be on ethosuximide and I'll tell you right now focus on letter T please in ethosuximide. And those of you that are well versed with the antiepileptic drugs you know exactly why I'm having you focus on them, I'm just giving you introduction. Let's talk about juvenile myoclonic epilepsy. Adolescence as the name implies, young adult. Myoclonic. What does that mean? Absence could be generalized tonic-clonic as well. In terms of your jerky movements, you must know the associated findings. Here we have early morning preponderance please. Clinical pearl: Early morning, young adult, no doubt juvenile myoclonic. Triggers include sleep deprivation and perhaps alcohol. Here we have 4-6 hertz. Treatment here would be valproate, maybe lamotrigine and topiramate.

    05:06 Usually here are the medications required, however, for lifelong with juvenile myoclonic unfortunately many of your patients are not going to outgrow the issue. Whereas in absence, luckily many of your children in fact will outgrow the issue.


    About the Lecture

    The lecture Epilepsy Syndromes by Carlo Raj, MD is from the course Seizures. It contains the following chapters:

    • Benign Rolandic Epilepsy
    • Lennox-Gastaut Syndrome
    • Absence Epilepsy
    • Juvenile Myoclonic Epilepsy

    Included Quiz Questions

    1. 3 Hz spike-and-wave pattern on EEG
    2. Nocturnal seizures
    3. Tonic-clonic movements
    4. Chewing and automatisms
    5. Hemifacial clonic movements
    1. Benign rolandic epilepsy
    2. Absence seizure
    3. Lennox-Gastaut syndrome
    4. Juvenile myoclonic epilepsy
    5. Complex partial seizure
    1. Benign rolandic epilepsy reveals centrotemporal spikes on EEG.
    2. Absence seizure reveals centrotemporal spikes on EEG.
    3. Lennox-Gastaut syndrome reveals 3 Hz spike-and-wave pattern on EEG.
    4. Complex partial seizure reveals 3 Hz-spike-and-wave pattern on EEG.
    5. Juvenile myoclonic epilepsy reveals 3 Hz-spike-and-wave pattern on EEG.

    Author of lecture Epilepsy Syndromes

     Carlo Raj, MD

    Carlo Raj, MD


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