Firstly may I thank you in advance for reading and responding to my question.I live in Sydney Australia and have had what a fourth neurologist has now described as "sub-cortical myoclonus".
In June 1998 I started having "spells" where I would fall to the ground unexpectedly. Initially my GP referred me to a cardiologist and a neurologist. I had all the relevant heart investigations and the cardiologist said that basically my heart was perfect. (which was pleasing considering I am overweight).
Neurologist Number 1 did an EEG and CAT scan (which were normal) and also sent me for an MRI. That was also normal. He referred me to a Professor of Neurology (Neurologist No.2)The Professor did a "hyperventilation test" on me, he also did a 24 hour urine test (I can't recall what he was testing for. sorry. AAnd sent me away with some tablets to return in two weeks.
By this stage it was about October/November and I had been house bound since June as I was having up to 15 "seizures" or whatever they are a day. I couldn't shower alone go to the laundry alone etc. When the episodes started in June 1998 I had a 9 month old baby so I am unsure whether any of this could be related to the birth of the baby.
After 2 weeks I revisited The Professor who advised me that the urine test was normal and that what was happening to me was most likely stress or anxiety or psychological. I was adamant that this was not the case as I have always been a cool calm and collected person. Mty only agitation was not getting an answer to my problem. The Professor announced that "He was not God you know" and that "There is nothing physically wrong with you" and sent me home with some more tablets. He told me to ring him in 2 weeks (not even go in to see him) I was seething. Nevertheless, the tablets were Xanax and after reading about them on the internet and potential side effects I didn't take them nor did I go back to see The Professor.
I then went to another GP and got a referral to another neurologist (No.3). He looked at all the results (which were negative anyway) and said that what was happening to me sounded very much like an epileptic episode. As I would fall to the ground, my eyes would flutter and my arms and legs would jerk for a few minutes, and after the episode I would feel very tired as though I wanted to be in a deep sleep. At no time though have I been unconscious. I can hear and even respond too those around me but not control the other functions I have described.
Neuro No 3. put me on Tegretol which didn't help me in the slightest. It was making me drowsy and the episodes did not decrease. My memory is not as good as it was however I think he may have tried me on something else as well. (I know this doesn't help you however my memory is shot). Anyway Neuro3. decided to refer me Neuro 4 who would put me into hospital to do extended telemetry testing.
I was on video surveillance and EEG monitor for four days. I wasn't on any medication. I normally get a weird sensation that warns me of the onset of these attacks, so each time I would buzz the nurse. After watching the video and analysing the result, Neuro 4 concluded that there was no abnormal EEG activity except for the fluttering of the eyes (which is probably irrelevant) however by watching the episodes he concluded that it was "suc-cortical myoclonus". I can't find any info on this at all.
Neuro No.3 when I went back to him concurred with his diagnosis. The hospital stay was in February 1999. Since then I have been a walking medical experiment in order to find medication to control the episodes. I have been on Epilim consistently since Feb 1999. Tegretol and Promanal were not successful. Currently I am on Epilim 700mg 3 times a day, Lamictal 25mg twice a day, and Rivotril 1/4 in morn, 1/4 at lunch and 1/2 at dinner of a 2 mg tablet. I have only been on the Rivotril for about two and a half weeks and I have been unable to work, I am drowsy, I fall over, I lose concentration, I fell overwhelmed by things, I cry at the drop of a hat. Maybe it's not the Rivotril but a culmination of 18 months worth of "stuff".
I have been seeing a counsellor and I also know that Neuro No 4 wrote in his report that the episodes appeared genuine implying that they were not psychological.
I am married with two lovely children, I had an excellent career and all was cruising along fine until this. Basically I am at my wits end. I need help. I need answers, or a need someone to point me in the right direction.
I apologise for the length of this posting. I suppose my questions to you are given all that I have explained:
1. Does this sound like the correct diagnosis
2. Can you recommend other medications which may be more suitable to control this situation
3. If given the symptoms I have described do not sound like "sub-cortical myoclonus" can you suggest another avenue of investigation
4. Please help
I am not sure what to tell you. The normal EEGs are encouraging as one never wants to have abnormal brain activity. I am assuming that you didn't have an episode while being recorded by video and EEG in the hospital. Some of the medications I am not sure what they are as except for lamictal, I am not familiar with the names of Rivotril or Epilim. The term subcortical myoclonus is used for lesions in the subthalmus or the basal ganglia that cause myclonic movements. They are very difficult to control with medications. However, every patient with these have a correlation with MRI changes, usually ischemia (stroke) is the etiology. As you MRI is completely normal, I don't think that you have what I am used to seeing. So, I am not sure what your physician's are seeing to conclude that your myoclonic seizures are "sub-cortical". What I would recommend is to have the video EEG monitoring until they record an event and see if the event is correlated with EEG changes. Myoclonic epilepsy is usually generalized epilepsy and one usually looses consciousness. Since you don't, it is another reason to question what is actually happening. The medication we usually use for myoclonic epilepsy that is generalized is valproic acid or a benzodiazepine for myoclonic movements not associated with seizure activity.
Sorry, I am not sure what to tell you. I really think you need to try and correlate an event to the EEG and see if these events are cortical driven. If they are not, then other things should be investigated such as non-epileptic events, non-cortical myoclonus etc. What do people tell you that your doing in one of these events? Do you have limb movements? Do all you limbs move? Will one side of your body move differently than the other? Do you jerk your extremities? If someone has a video recorder that you may set up to try and capture one of these events, it would greatly help your neurologist to try and treat you.
The good news is that the EEG is normal. The distressing news is your seizure-like activity. If you had a lesion in the subthalmic nucleus, I would understand the myoclonus but with a normal MRI that would be doubtful. Valproic acid and the benzodiazepines are very good medications for myoclonic epilepsy and if they did not have an effect then treatment becomes very problematic. You may want to talk to your epileptologist about something called non-epileptoform seizure-like events. These are seizure-like events that are not driven by an abnormal brain.
I just wanted to respond to some of the questions you ask in your response to my posting.
Whilst being monitored in hospital I did have numerous episodes which were recorded on EEG and on video. I had been taking Tegretol (Carbamazepine) before going into hospital and I stopped taking those prior to going into hospital so that they could make a "true" recording. There was no recordable evidence on the EEG of cortical epileptic myoclonus however the neurologist said that based on his observation on the video of my episodes, he believed I had sub-cortical myoclonus or maybe it's as you say non-cortical myoclonus. Immediately after making the diagnosis he put me on Epilim (Sodium Valproate) and since then Prominal(Methylphenobarbitol? as I can't read the label anymore it's worn away) and now Rivotril (Clonazepam).
What happens during an episode is that if I get a warning (which is usually rapid flickering of my eyelids and/or trembling of my right hand) I immediately lie on the floor because I know if I don't I will fall. When on the floor my eyelids continue to flicker and my right hands shakes then every now and then i get a sudden jerking whereby my arms legs and head jerk up and back down. I have hurt my head in some instances. These sudden jerks continue for a few minutes until my eyes slowly get back to normal and I go into a "short deep sleep" type phase. My neuro has witnessed this on video and live in his rooms recently. The scary part is that's what happens when I get the warning signals, when I don't I just drop to the ground wherever I am and all the other symptoms are the same. This is when I have got many bumps and bruises.
Do you think I should seek another opinion? Have another MRI done? Any other ideas?
Thank you for your time. I will speak to my neurologist about what you have suggested. Might I say that I believe that this forum is remarkable in that given the constraints of time doctors have the doctors at your clinic still respond to such a vast number of queries.
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