Juvenile Myoclonic Epilepsy

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Juvenile Myoclonic Epilepsy

25 Mar, 2:30 PM

[Music] very warm welcome and good evening to all the doctors medical students and other healthcare professionals who have joined in for this talk today which is being hosted on netflix my name is dr avantika chaitanya and i'm a pediatric residency applicant passionate about child health care and the co-founder of the auto research initiative i will be your host and moderator for this enriching talk on the approach to management of juvenile biochronic epilepsy in children tubulin myoclonic epilepsy is one of the many types of epilepsies its most common symptom is repeated seizures known as micronic seizures these cause quick jerking movements people with this form of epilepsy often go on to have very severe seizures often known as grandma seizures in adulthood one of the most interesting aspects of juvenile myocloic epilepsy is that there are two very common factors which are very common which cause stress which are stress and lack of sleep which cause seizures making a correct epilepsy diagnosis is very important the electroencephalogram or the eeg is the most important test in making a diagnosis seizures are very well controlled with medications generally in up to 90 percent of the population and hence early detection and proper management could be life-altering for many children to enlighten us further on this topic we have with us today dr sita jayalakshmi consultant neurologist at kim's hospital's hyderabad dr sita is a distinguished neurologist having served in various capacities at prestigious tertiary care centers she carries with her great expertise and thoroughness in patient care she worked as a professor of neurology at nazam's institute of medical sciences and has nearly 20 years of experience as a neurologist dr sita has special interest in epilepsy and coordinates a comprehensive epilepsy surgery care program at kim's hospitals their group has performed more than 750 epilepsy surgeries dr sita has more than 90 publications in national and international journals and is the associate editor of the international journal of epilepsy she has been awarded the japanese society of child neurology award for her paper on outcome of epilepsy surgery in children with calculations of cortical development after evaluation with non-invasive protocol a very warm welcome to you one and over to you for today's talk yeah good evening good evening and i thank you for giving me this opportunity and asking inviting me to speak at this in this session on a very important and common topic today evening i will be discussing about juvenile myoclonic epilepsy which is one of the most important topic especially among the epilepsies in children adolescents as well as others because this is said to be a lifelong disease and hence most of the people will continue to have the caesars and they go on to the adult life so now what i will do is briefly discuss about what is generalized epilepsy because jme is a generalized epilepsy then what are the clinical features which is very important and core part of juvenile micronic epilepsy which we need to identify in the outpatient department because always in the management of epilepsy it is the right syndrome and right drug which is very important how do we evaluate these people how do you manage and most important is women with epilepsy the management of ideas especially is very important so before we go into jme we know that juvenile micronic epilepsy is a generalized epilepsy so what are generalized epilepsies these are epileptic disorders we generalize cheeses indicating involvement of both the hemispheres simultaneously and the initial intel eeg pattern is bilateral there is no focal onset in the intellectual arthritis and generalized epilepsies account for 40 percent of the epilepsies of which juvenile mycolonic epilepsy is the commonest epilepsy so recently il international league against epilepsy has given this term for generalized epilepsies which defines it as those epilepsies which originate at some point are within a rapidly engaging bilaterally distributed networks they can include cortical and subcortical structures but do not necessarily include the entire cortex the location and lateralization are not consistent from one she said to another and generalized seizures can sometimes be asymmetric because there is a the originating one part of the network resulting in generalizations then actually the classification of epilepsy has changed in 2017 given by international league against epilepsy so most of the times we have to classify them as poker cheeses where there is a clear-cut focal onset or generalized scissors where there is no focal onset or unknown where it is not possible whether it is vocal or generalization so jme comes under generalization which are more generalized very suddenly cessation of the ongoing activity happens so jme has both generalized mortar as well as non-motor sheathers if the water is gtcs and non-motor is absences and after this it is further classified in the generalized into what are the types of fisa jesus and after this it's important that we classify them as what are the caesar types the schizotypes are first ones you say it is generalized onset next it's important to say what is the epilepsy again caesar is generalized and neplex is also generalized and then the epilepsy syndrome the epilepsy syndrome is nothing but which is a combination of the various types of fuses in case of jme it is the combination of juvenile micronic epilepsy com consisting of dtcs absences and myoclonic jerks and the comorbidities sometimes can be present the form of morbidities can be anxiety depression or associated other issues but however till now ilae did not recognize any specific epilepsy syndromes in the new classification and the work is still going on but still juvenile microlens epilepsy comes under generalized epilepsy syndrome and as i already said the ids account for 30 to 40 percent of all epilepsies and among all the epilepsy juvenile micronic epilepsy accounts for 10 percent of the epilepsies the most important core features of any generalized the so-called genetic generalized reproductives now the term is previously it was idiopathic generalized epilepsy now it is genetic generalized epilepsies these are genetically determined they affect both men and women equally boys and girls equally all the races across the world they start in childhood are adolescents there are three main types of fuses which occur alone in combination as i already said they are generalized on the cloning cheeses my cloning jerks absences and the sheath especially after awakening from sleep and they are precipitated by sleep deprivation and specific precipitating factors like photosensitivity is very specific for yeah generalized genetic epilepsies especially juvenile bicyclonic epilepsy in all generalized epilepsies the eeg is characterized by spike and wave polyspike and wave activity i will show some examples and genetic heterogeneity is common with this we will go to the syndrome of amy so the syndrome of any ig can manifest with three types of fuses as i already said absences my cloning shows us and gtcs in case of giami the most important is every person will not have all the three trigger types but it is a must to have myoclonus because we call it as juvenile micronic epilepsy myclonic users must be present in all the people that is present in hundred percent gtcs are present in eighty-five percent absences in only thirty percent that is only thirty percent of the people will have all the three types of seizures whereas gtcs and micronutrients are present in 85 percent only myclonus is still present in 15 percent and myoclonus mostly disappears by 4th decade though the gtcs continue and we will come to that further details that are later and if somebody has got all three types of cheeses sometimes it is difficult to respond to the anti-seizure medication if there is only myoclonus or absences with myoclonus the response to treatment is excellent sometimes when the child comes in the opd with the childhood absence epilepsy we may think that it is cae but once the child reaches the age of 10 to 12 years he may start having epcs and myclonus so and hence cie can evolve as jme and such cases do have a difficult to control with the anti-fusion medication and sometimes the patients will have practice in nutrition means that with concentration especially while doing mathematics they will develop jerks or gtcs again they are difficult to control so that's why in history taking it's very important to look at all these aspects i'll just briefly show an example of absent cheeser this is a young girl who is 12 years so this is a video eg you see now she is just staring and you can see the mouth there are some brief automatisms she is not responding technologist is asking the name now she is answering so it lasted for few seconds that is a absence user absent she said is nothing but sudden brief cessation of ongoing activity along with eye blinking with or without eye blinking and sometimes they will have some oral automatisms so now i will show an example of micro energetics keep watching the child will develop jerks is a 7 year old child so he gets jerks of the hands so he developed my chlorine gels which are bilateral and simultaneous and these myclinic jerks and generalized strong economic cheeses typically appear on awakening they have a diurnal pattern and this is proven by transcranial magnetic stimulation so most important feature is on awakening they develop microorgs and these are aggravated by sleep deprivation the previous night and diurnal pattern is sometimes absent in practice induced diseases but most of the time in eighty percent of the people with your children with bi juvenile microbial equipment you get it in addition to this the most other important features are there are reflex traits present in jme that is photosensitivity is present in almost 25 to 90 percent in various uh studies photosensitive means the moment you sit on a tube like or the moment there was something like way flashes of light on a tv or that is seen in that is photo sensitivity which can be induced in the lab by doing for pcr eye closure sensitivity means the moment you close the eyes they develop eye blinking praxis induced micronus means especially with concentration or some practice especially while doing maths or concentrating on something are thinking they get the micronus that is seen in almost one third of the children oro facial reflex micronus where there will be twitching and jetting all around the mouth and also in the tongue in 20 around 20 to 25 percent 40 percent of the children will not have any reflex rates but 60 percent can have either photo sensitivity or eye closure sensitivity or praxis or oro facial reflex micronus either in combination or separately so coming to photosensitivity this can be induced in the lab by doing photic stimulation where we get a photo perhaps smell response during which spike and wave discharges do appear and this phenomena depends on the flicker frequency the light illuminations and amount of stimulated retina and this is because of why do you get a photo of photosensitivity or ppr in the lab due to hyperexcitability of the visual cortex with increased gamma synchrony preceding the ppr resulting in upregulation of the occipital to frontal pathways and this is an example of ppr this is yellow line indicates the peripheric was given and the red line below shows the photic frequency here the photic frequency is around 10 hertz so at the 10 hertz we can see that there is a three to three and half hertz spike and wave activity which is seen lasting for about six seconds in a child with juvenile micronic epilepsy the next one is eye closure sensitivity means there is appearance of three hertz or even faster spiken wave activity predominantly occipital but will be generalized which is seen with eye closure along with rhythmic eyelid myoclonus so this is very seen and this is due to involvement of both hospital cortex and supplementary motor area for example if you so during the aegis this is eyes open this is eyes closed the moment the patient closes the eyes we are seeing the high amplitude generalized epilepsy from discharge again next you can see during eye opening there is nothing the moment the child closes the eyes we are seeing the high amplitude generalized epileptic form activity the third feature is praxis induced diseases which may be genetically determined occurring in one thirty fifty percent of the cases more common in uh asian countries like japan and this is characterized by uh patients with the praxis induce represent the most drug resistant subtype and this is due to altered functional connectivity of the anterior supplementary motor cortex then i as already mentioned oro facial reflex myoclonus is nothing but small single arrhythmic myoclonic movements occurring in the perioral and lingual and pharyngeal muscles are usually triggered by talking which is seen in 40 percent of the cases and this is due to hyperactivation of the cerebral subsystem supporting the linguistic communication finally how do we make a diagnosis of juvenile mycolonic epilepsy we knew that there are three important user types and what are the precipitating factors and then so the correct diagnosis depends on three important features one is the history where you find either the combination of two or more than two types of fuses and in few patients only micronus second is the eeg is the most important and during the eeg there are certain important features in the eeg which is mandatory and most of the times eeg is positive in 85 percent of the people with jme so why why do people with jaime develop generalized chronic clonic jesus for this there are many theories one is sentence afflict theory second is cortical subparticle theory other one is cartico reticular theory so in central separate theory it says that ah there is a thalamus is the pacemaker and the stimuli go to the cortex simultaneously resulting in the sheases in the cortical particle it starts in the context in one area this is now most widely accepted and it goes to the other parts of the cortex through bilaterally spread rapid networks other one is the cortico reticular theory where it starts from the cortex and goes to the reticular activating system or the vice versa so these are responsible for the various eeg activities the intellectual eeg in juvenile microbial epilepsy is very characteristic the most important criteria is the background has to be normal as we see in this page and after that there will be high amplitude generalized poly spike and wave discharges if you see here there are two small spikes followed by a wave so this is called poly spike and wave discharges again if you see they have bi-frontal amplitude predominance so this is a fp2 f4 this is fp and f3 these are the frontal leads of the right followed by the left side so that is called frontal predominance again if you see there are generalized discharges with again there is background is normal in between this is called fragmentation of the generalized epilepsy form discharges and the discharges are activated by hyperventilation so this is yellow the green color indicates hyperventilation is going on and during hyperventilation we there will be facilitation of the discharges with occurrence of this i have high amplitude poly spike android discharges and i have shown pcr previously so this is again high amplitude discharges which are generalized with fragmentation so to summarize the eeg in given and molecular epilepsy background will be normal there are spontaneous bursts of generalized by synchronous epileptiform discharges sometimes poly spikes are spike and wave maximum voltage will be in the frontal and central regions the frequency can range anywhere from usually three to four point five but can vary from two point five to five and sometimes if it is fast it can reach even six hertz hyperventilation typically activates photo photo sensitivity that is positive ppr is common in uh forty percent especially girls and it these discharges are triggered by eye closure and excessive theta is seen in poorly controlled cheeses and this usually provoking factor is sleep deprivation that's why we ask the patients to come to sleep deprivation for doing the eeg where you ask them to sleep for only four hours in the preceding 24 hours and that is we request them to sleep at 12 in the night and get up at four in the morning and come to the lab early morning by eight o'clock after having a good breakfast then you do a sleep initially followed by you make the patient awake because the transition from sleep to awake will uh stimulate the discharges so you you you want to record during the transition from sleep to awake during which again you will do an awake recording with photic stimulation and hyperventilation then the next important feature of jme is can focal eeg abnormalities occur in jva the answer is yes which is seen in upto one third of the patients in various series from 20 to 50 percent so the focal abnormalities can be in the form of a symmetry in the amplitude of generalized discharges or vocal spikes or sharp waves intermittent and shifting their location persisting focal abnormalities do occur and but this is one of our publications on juvenile myoclonic epilepsy into 66 patients with jme the focal abnormalities occur in the form of amplitude asymmetry or even independent eeg focal abnormalities and whenever there is focal abnormalities the it is associated with a presence of gtcs and mycologics strong family history of epilepsy and if they are not responding to anti-seizure medication so this is an example in a patient who already has a generalized policy spike and we have discharges we can see independent by frontal spikes so these are generalized and preceding this there are by frontal even parasagittal that is ah is seen very clearly similarly there is amplitude asymmetry the first four is the right side next four is left again this is right and left we can see the discharge amplitude is more on the left hemisphere that also can occur and in addition sometimes if you see this is a generalized discharge but just before at 0.2 seconds there is a vocal uh wave onset suggesting of the early focal abnormalities before a generalized discharge these are all acceptable this is another case where you have central spikes in a patient with generalized epilepsy form abnormalities then these atypical aeg abnormalities have been well described even in other papers where it can be similar to what i have said the discharges can have atypical morphology amplitude asymmetry focal discharges or focal onset of peroxism but most important is though focal occasionally are there you must have generalized spike and wave poly spike in your discharges to say that we are dealing with juvenile vital epilepsy the next important feature after the clinical features eeg is mri mri is always and should always be normal in patients with juvenile micronic epilepsy then what are the comorbidities which can be associated with your jme so juvenile microbes epilepsy is associated with psychiatric comorbidities in almost up to 40 percent of the especially adolescent age that is 12 to 18 years of age they do have specific neuropsychological profile suggestive of some frontal dysfunction especially executive dysfunction will be there and they have poor social uh behaviors and they have in addition to this they have a combination of praxis in huge issues because that is adolescence where they have a lot of studies and stress in addition to this they will have eye closure sensitivity and also it has been shown that patients where the jme does not respond to anti-seizure medication they do have associated psychiatric morbidity which is in the form of severe anxiety and the patients poorly perform on naming ability and also switching from one task to another faster and then what are the genetics of jme there are many genetics of jme described and mostly jme runs in families you will have a strong family history especially in 60 percent of the people with juvenile medical epilepsy and many genes have been mapped to that ah in all over the world it's on chromosome 6 and in india even we were part of that study where it has been mapped to chromosome 15. so finally the next is diagnosis and management which is very crucial so can we diagnose id and aab accurately the answer is yes it's a syndromic diagnosis for example when the patient comes for the first time with a single gtcs you may not be able to diagnose it but over the time the diagnosis becomes apparent especially when the patient starts having myclonic jerks and your eegs are positive showing generalized polystyrene discharges whenever it's not possible to identify this eeg in one hour it's important to do a video eeg study especially if you do overnight eegs where the micronus becomes more clear and also you see more frequent poly spike android discharges on the aeg and mri brain usually as i said most of the time will be normal but sometimes because all these ise's there is a overlap as i already said childhood absence epilepsies we diagnosed in a school going use but 30 percent of them can develop start date developing my clonus gtcs at a later date so the ca is evolving into game similarly there is juvenile absence epilepsy where the patients will have only absences to start with later develop may develop mycolonists and gtcs initially we may think it's a pure juvenile absence epilepsy but it can again develop into july my clinic epilepsy similarly some patients will have only gtcs epilepsy gtcs that is the syndrome vehicle and it can evolve into juvenile and mycolonic epilepsy and there is a small percentage of patients who develop after the age of 20 years the gtcs microelectrics we call it as late onset amy or ibe so one should be aware of all these overlaps whenever we are making a diagnosis and before going into the management it is important that whenever jme starts the micronus gtcs can also be present in patients who have progressive mycolonic epilepsies that is we call it as symptomatic generalized epilepsies so the symptomatic generalized epilepsies are also genetic and they progress and they don't respond to the anti-fusion medication so it's how do we differentiate by various features so the physiotypes in case of jme and iv are absence micron is tonic learning whereas in case of symptomatic you will have a tonic and tonic along with chronic lonic absence and myclonic atomic means the patient suddenly falls onto the ground without any uh premonition so it doesn't evolve then usually ig's onset is in the childhood whereas symptomatic at any age it has multiple etiologies and response to the aed is excellent with idiopathic but not with symptomatic prognosis is favorable in case of jme but not in case of symptomatic epilepsies the brain is normal on mri but however in symptomatic though initially it is normal over the time they will show diffuse cerebral atrophy and some white matter changes may also be positive and when we look at the eeg usually usually in the precipitation okay before the aeg the frustrating factors of sleep deprivation stress and emotion for ideas there are no specific respirating factors in patients with symptomatic epilepsies the most important is the ee in case of ide the er gaming the eeg is normal background with as i already said analyze spike and wave poly spike and wave activity with photosynthetic occurring at faster frequencies but in contrast in case of general symptomatic generalized epilepsies the intellectual eeg will show bilateral less rhythmic if the background will be slow that is most important and spike which is usually more than we said uh four hertz activity here it will be low frequency that is two to three years activity and the generalized issues uh it can show independent focal spikes occurring bilaterally in the hemisphere especially in the oximeter loops in symptomatic generalized epilepsies so once we have confirmed that it is jme how do we treat these patients the guidelines for the management of jme are similar to any idiopathic generalized epilepsy they have an excellent response to treatment with 80 to 90 percent becoming she's afraid all anti-seizure medications are not effective in iv because there are some drugs which will aggravate diseases so one should be that's why it's very important to identify the syndrome when we are treating so sodium valve plate is the most effective drug and seventy to eighty percent become shizu free on monotherapy with sodium alpha but all of us know that ralph rate is associated with significant side effects especially weight gain in women it is associated women usually do not accept this drug because of menstrual disturbances polycystic ovarian disease and significant alopecia and tremors also very common so if these occur either in men or women they do not accept the drug and what are the drugs which are always useful in iger in addition to sodium vaporite lamotrosin is an excellent drug to be used in women with epilepsy to permit can be used levitra setup is also one good drug so the issue of sheaser aggravation will not happen but by any chance if somebody uses drugs which are used in focal epilepsies like carbohydrate phenitine or even ox carbohydrate if they are used in patients with idiopathic analyzed epilepsies like immunomyclinic epilepsy then here the dtcs may respond but however the micronesis gets aggravated absences get aggravated so the patients may come to the emergency with a myoclonic status where they will develop continuous jerking of the limbs and they get admitted with a status of the myclones so one has to be extremely careful with the use of sodium channel blockers drugs which should not be used in amd and then the treatment is demanding because the drugs which are used for vocal epilepsies can be deleterious in jme and also there will be seizure aggravation so once we decide what is the drug most of the time we use sodium vitamins 300 milligrams twice a day especially in uh all the cases without who are not of childbearing age but if somebody is of childbearing age one should use drugs like levitra saturn or lemontrosine in addition to the management one should talk about lifestyle that is very important if the sleep is less they will get jesus especially in adolescence in children we have to explain that even if they have to every day the sleep cycle has to be correct and they should sleep should be good up six to eight hours especially before the exams they should not have sleep deprivation they should study in advance and keep it so that during the exam there is a risk of having a seizure if they are sleep deprived and most important is the treatment is continuous means almost life long except in a small percentage of cases where there is a chance of stopping the medication and then children and women need specific approach as i already said with monotherapy she's a freedom occurs in almost more than 90 percent levitra saturn is also equally efficacious in up to 80 to 90 percent lamontagne is effective as monotherapy or if there is drug resistance along with valparaiso so what about women we cannot give alperin especially in the child varying age and also in adolescent women because of pcod so what are our options levitra saturn or lemontrazen levitar setup we can go up to a maximum of two grams per day or even more lemon resin up to three to five milligrams per kg that is we give up to 400 to 500 milligrams per day and if their fuses are not controlled adding globus of a small dose definitely will get a good cheese of freedom then most important is in a woman who wants to have children one should know about the aed risk in pregnancy so valve rate is associated with highest risk of major malformations and phenobarbitum dopamine sometimes previously used nowadays we don't use it in jme it is associated with intermediaries so the only two drugs which are associated with lowest fetal malformations are lametrazine and liver system and they should be the drugs of choice during adolescence and also during the child wearing age and also sometimes what happens is the patients women who are in the child wearing yes do not respond to either levitra's atom or even lamotrosin so in such cases we nee we may use a small dose of sodium valve rate after explaining all the risks but we should never go beyond a dose of 800 milligrams usually adding 200 or even 400 milligrams of alpine will make them shizu free along with either lamont resin or even with elevator system and also it has been shown that whenever we are using lamotrosin in this woman if they are using contraceptives the levels of nomatrosine goes down and they may develop cheeses so one has to be the woman should be counseled about this especially when they are starting the oral contraceptives and the dose of lamentation needs to be increased and it and we know that entire epilepsy now levitos atom is the queen of anti-seizure medications and most of the times libertas atom is being used even when woman with epilepsy and if you see in this in the uk pregnancy registry it is associated with lowest malformations that is point seven percent when compared to other drugs carbon jp nine point four percent valkyrie and six point nine percent kimotris in also one point five so the least scientific profile on the fetus heterogeneity is associated with clever symptom so if you look at the tetragenic risk profiles of the various anti-epileptic ducts followed by carbohydrate followed by valprade so we should not use wall create in this group of patients then lastly about drug resistance do patients with jme do not respond to a anti-scissor medication though there is an excellent response it has been reported in about uh 25 percent of the cases but more before we say there is drug resistance one should make sure that whether did we make focal epilepsy into gamey one has to be careful that we call it as pseudo refractory epilepsy sometimes you have ideas imitating focal epilepsies symptomatic epilepsy is imitating ideas and but however still 20 are true drug resistant diabetes and also as i already shown if somebody has absences mycolonosct it's difficult to treat when you go up the pyramid and this is one of our publications that lack of response to valeric acid that is drug resistance is associated with associated psychiatric comorbidity and focal aeg abnormalities and hence it's important that diagnosis of psychiatric disorders should be made whenever somebody is not responding we should send to a psychologist for a detailed assessment for anxiety or depression and that needs to be addressed either by us or by a psychiatrist the predictors of drug resistance in juvenile micronic epilepsy include presence of all the three shesa types that is gtcs mycolonous absences juvenile microbial equipment say preceded by childhood absence epilepsy psychiatric comorbidity if they are personality disorders like ocd and discharges are freezers occurring at the baseline energy every is always showing poly spike and view discharges sensitivity to praxis that is praxis induced refuses younger agent onset is also associated with drug resistance the as i already said how long should we treat the patients with jme if you see here the risk of relapse in iv is relaxed is less than six to ten percent in childhood absence fifty percent in human life but in jme it is hundred percent relaxed and hence they need a almost lifelong treatment and what is the time to relapse after the anti-epileptic drugs are stopped within one year within two years most of them do relapse that is almost nearly 95 percent do relapse and the predictors as i already showed um eed continuously abnormal especially before withdrawal and those who have gtcs with absences and micronic jokes so with this i would like to conclude by saying that juvenile mycolonic epilepsy is a syndrome which may not be apparent at the first visit in the opd but with close follow close history and close clinical and eeg follow-up one will make a diagnosis of jme because the appropriate selection of the anti-seizure medication that is levitra saturn and lamontagne women valparaise in men as well as women of non-child varying age it is the best drug and one should advise in addition to this on lifestyle identification of specific triggers like sleep deprivation stress photosensitivity should be told and response to single anti-seizure medication is excellent in up to 80 to 90 percent and drug resistance is seen in 20 to 30 percent of the iges in spite of an excellent response and one should evaluate them with a video aeg imaging and detailed psychiatric evaluation for associated psychiatric disorders and one should address them with all these issues thank you thank you very much dr sita for the comprehensively exhausted talk on diagnosis and management of juvenile myoclonic epilepsy we learned today that it is imperative to learn how to identify the difference between ij and gma and also the importance of early intervention for the best prognosis once again thank you so much ma'am for this wonderfully enriching talk uh we can now open up the questions so dr sachin is asking role of victims fact in jail spect has no role in case of juvenile migrant epilepsy respect as a role only in patients with focal epilepsy who are drug resistance when we are doing a pre-surgical evaluation okay thank you man um dr konkum is asking about what is a safe drug in pregnancy safe drugs whatever be the epilepsy syndrome the safe drugs are levitar system and lemon processing so only these two should be used we don't have information about any new drugs so elevator setup but liver system all of us know will cause lot of behavioral side effects like anger irritability so in such cases one should go for lemon dressing lemonade is more positive but the disadvantage with clamoring is you have to titrate very slowly we have to start with 25 milligrams half a tablet twice a day and update rate over two months to reach a dose of 200 milligrams twice a day so possibly that is one of the disadvantages but however we can have an overlap of limiter cetera methylmetals in and slowly taper of levitra's atom and continue lemon resin especially if there are significant behavioral side effects with electricity many women with the jme are on these two drugs across the world okay thank you man the next question is by dr deepu what is the initial drug of choice in a patient with ongoing seizures in an emergency uh always we should choose a drug which is easy to use will not cause any side effects that's how a uterus atom has become like i said the queen of anti-seizure medications so it is the drug of choice to give an emergency because phenytoin can be used which we are using over the past two decades but because of the means you have to be very careful while using your tin juice very very slowly or to there is a risk of cardiac arrhythmias i had one child who had especially in children sudden cardiac arrest you are giving so we need to really take 30 minutes to resist it and get him back because it's only a cardiac ancestrally it's not that so that's why liberal system has no side effects so that's so all the ears use limiter okay thank you ma'am uh the next question is by dr ashok what is infanta infectile spasm infantile spasm is otherwise known as now the new terminology says it's an epileptic spasm in which there is a sudden bending of the head and trunk forwards along with flexion of the hands and the legs so certainly you become entire body moves forwards that is flexion uh type of spasm if it suddenly goes backwards you call it as extension type of spiralism these spasms infantile spasms are very important feature rest syndrome which occurs below the age of two years okay thank you ma'am the next question is uh by dr deepa again how long should monotherapy be continued in seizure management and when do we start paper effect yeah so this is that's why it's very important this is a very pertinent question very important to know what syndrome we are treating for example juvenile mycolonic epilepsy the treatment is almost continuous that is lifelong but none of the patients will agree when you say it so always we try to down titrate the medication minimum of two to three years now the recommendation all over is in according to american academy of neurology or the international league against epilepsy or the nice guidelines which are from uk after two years we can reduce and stop the medication if somebody she's a free answering guilt if somebody is having it difficult to treat epilepsy with frequency is a synthetic medication there is no question somebody she's a free and monotherapy you can down that rate after two years okay any childhood absence epilepsy you can stop the medication for example if you take temporal of epilepsy they are drug resistant so it depends on what syndrome you are treating with based on that you will take addiction about down titration of the medication but otherwise as a general uh statement two years she's a freedom okay actually there is a question uh about normal energy versus videoing yeah normally normally whenever we are doing the eeg it's very important to increase the yield of eeg for that we ask the patients to do some activation that is sleep deprivation as i already said then second thing is we ask the patient to sleep during the while doing the sleep making them to sleep itself is an activation procedure in addition to that we do hyperventilation and photic stimulation during the eeg in spite of this your aegis will be normal in up to 40 percent of the cases in spite of doing all this that is because you are recording the eeg only for a period of 30 to 40 minutes whereas if the video easy means you have a video along with an eeg and the idea of having a video age is to record any event if it happens it's possible to record especially in absences even in that one hour but sometimes we do video ed recordings for 12 hours to one day so that you have more time and the chances of identification of the epileptic form discharges either as vocal or generalized or very high when you do prolonged video aed recordings and record all the events described by the patient sometimes they turn as non-epileptic ones sometimes you can classify them clearly as vocal or generalized so if somebody has suddenly a sensation of ongoing activity it can be a temporal of epilepsy or it can be an absenteeism if you have a temporal spikes then you will make it as a temporal of epilepsy and start carbohydrate whereas if it is an accent cheeser you will get generalized discharges then you will start even val period or ethos oximeter so to categorize them into focal length generalized epilepsies the video aeg is definitely a better tool than the eeg what are the drugs for childhood absent epilepsy like the recommended drugs yeah the actually the ideal means recommended drug for a childhood absence epilepsy is etho succimite ethosimide is available recently for the past two years in our country it's an excellent drug when it is not available we were using sodium alpha so the drugs of choice are ito slash sodium alpha okay now the next question mark uh what is the treatment recommended for infantile spasms for infantile spasms uh it's important to identify what is the etiology tuberous sclerosis is associated with infantile spasms very frequently if tuberous sclerosis is ideology one should directly start using megabyte treatment if there is mri is normal when you don't know first we should start with icth acth is asteroid which you should use uh 20 units per day subcutaneously given as an injection for a period of at least two to three weeks the spasms are mainly because of immaturity of the neuronal cells the steroids will help in the maturity of the neurons and hence there is resolution along with this we can use drugs like sodium vaporite levitras saturn and even two pyramid and also clonazepam so in the management even lamontracine in the management but resistance drug resistance is very common sixty percent of the infant spasms do not respond to the medication but early treatment is associated with good outcomes so we have to identify early because parents sometimes themselves may not identify that they are something abnormal okay thank you mom uh the next question is by dr shrira uh is it true that phenobarbitone is no longer indicated in the treatment of epilepsy no all rights there is a place for all the drugs we call all these as fastlane anti-convulsive drugs the second line being levitracitum recently the third line has become rivera saturn parampenal as well as lacosamite so phenobarbitum is a first line aed it has its own place it is a very good drug to be used especially in drug resistant epilepsy in india we have a lot of pharmacoeconomics whenever we treat the patients if somebody every time when we whenever we start the medication we we but it i always tell my patients this medication will cost you 500 rupees per month and 2 000 rupees per month any effort obviously we have all socioeconomic strata so if somebody cannot afford uh phenobarbiton along with phenitane is an excellent combination with a different mechanism fraction so we still use phenobarbiton uh in neonatal physios below the age of two years also it was one of the good drugs but now replaced by electricity so but still i will say the cost difference between the two drugs is more than 10 times so based on the need of the patient we should use the drugs that's what i do and i feel so okay thank you mark uh the next question is by dr deepu in all the eeg study should patient come with uh for every eeg study should patients come with sleep deprivation having only four hours sleep is that unnecessary as far as possible we have to look at so many aspects somebody coming from outstation traveled already in the train got up early in the morning to see the doctor so already sleep deprived so you can do an immediate eeg and for example if the eeg you have done there is no yield then but especially you are not sure what type of epilepsy you are dealing with patient is not responding and your eegs are normal not responding to a disease or medication you need a good eeg to differentiate focal versus generalized then i will definitely do it again okay uh dr arus arus asking about the best book or you would recommend to learn in learn ecg sorry yeah yeah eeg there are many books one is called ever soul [Music] means there are basic eeg books which are available in india also so but eeg is mostly learnt with the machine but along with the bedside rather than with the discussions rather than books it's very difficult to learn from books directly yeah there is one book called john pattern also so so they can listen any of these are available ever soul is one book i will say i recommend that yeah okay uh dr zarathust is asking a young girl called epilepsy and it affected her breathing is that aces to your heart going to a fibrillated state and does epilepsy cause a paralysis effect uh epilepsy if first coming to the question of paralysis if somebody is having a focal epilepsy which is coming from the motor cortex so he will develop suddenly get king of the hand and leg and after some time there is neural exhaustion so postal thoughts paralysis means following the sheath that they have a weakness of hand and leg which usually requires in half an hour to one hour and sometimes up to 24 hours and rarely up to one week so we don't call it as a stroke related paralysis but you call it as poster towards paralysis which recovers on its own then the next question is a sisterly are any cardiac disturbances which can occur during the seasons the answer is yes temporal of epilepsy scissors coming from cingulate cortex or from the insulin are associated with cardiac rhythm disturbances including a sisterly it is the rivers also if somebody has a cardiac disturbance recently we had a young girl who keeps falling getting the gtcs so suddenly in the opd she had a scissor and during the season twice was not palpable so we had to do a cold blue and later we found that she has a prolonged qt syndrome so even cardiac following a cardiac arrhythmia people can develop a scissor so it is vice versa that's a very important that you identify the history very carefully and always whenever we are doing an eeg we keep an easy delete so it's a must record an ecg during an eeg so that you identify whether it is sometimes very it's not so common but still you don't miss a cardiac cause okay thank you mom the next question is by dr suresh how do you approach a refractory seizure in a young patient okay this itself is a attack maybe we can have some time but normally whenever we are having a refractory epilepsy means uncontrolled if somebody is having usually to diagnose refractory ila definition is failure of two appropriately chosen drugs used for an appropriate period of time so usually we follow the rule of thumb of two we have used two drugs for a period of more than two years and getting attention that is bedside i will say so if they are not responding then we call it as so-called drug resistant or refractory epilepsy in that the best way is to use a rational combination therapy where you combine drugs with different mechanisms of action you don't combine carbohydrate along with pheniterin because both are swollen channel blockers so combine carbohydrate with a drug with multiple mechanisms of action like either sodium valve rate or levitra system you don't combine carbon dioxide because both are sodium channel blockers so like that you use different combinations of drugs to achieve issues of freedom and it did not respond always one should go for in children it is six months in adults it is two years if somebody don't keep on having schizos in spite of this duration of treatment one should go for a very good advanced mri that is the so called three tesla mri look for any focal lesions we do a lot of epilepsy surgeries our center has done more than thousand epilepsy surgeries by now so you identify a surgically remediable epilepsy hippocampal sclerosis they don't respond to medication you need to go for it temporal lobectomy similarly focal lesions in the brain like low-grade tumor or a cavernoma or a scar that is gliosis they need to be resected to make the patient fuser-free at least on medication or if there is a chance you can reduce and stop the medication so that is how you approach a drug resistant appears okay uh thank you mom the last question is a doctor sayeth uh is a symptom of a three-year-old girl having tremors while holding an object is that something you would suspect for jme no no jamie after spontaneously the micron is spontaneous but sometimes they present with only subtle tremulousness and clumsiness early in the morning so but every time if it is happening while holding an object we should look for a cell bill or like a intention tremor or something if it occurs on and off then possibly you should have a doubt so only that alone will not make a diagnosis of you need to have a clear-cut micronus without without gtcs okay yeah all right uh i think that's the end of the questions for now yeah so i don't think we have any more yeah thank you dr this was indeed an enriching session and we are truly grateful for this

Description

Clinically, irregular jerks of the shoulders and arms are manifestations of juvenile myoclonic epilepsy (JME). The onset age for this syndrome, which affects 4–6% of all epilepsies, is typically between 12 and 18 years of age. The condition usually starts with solitary jerks and then progresses to widespread tonic-clonic seizures (TCS). Valproate or primidone can effectively control JME. Dr. Sita Jayalakshmi, a Consultant Neurologist at KIMS Hospital, discusses the diagnosis and successful management of JME only on Medflix!

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