First time I met her in my clinic; she came with her parents. Quite aloof, lost in herself, shy or dull, I could not figure out. Did not speak a single word in her first visit. Her parents narrated her story in front of her and I did not see any reaction on her face.
So, she was fine till the age of 5 when it all started. She started waking up suddenly from her sleep around midnight and will scream loudly, with abnormal movements of her limbs and will again sleep after 20 min. These incidents are ignored initially as some sleep related problems of kids. But this started happening every day, almost 3-5 times every night, so she was taken to pediatrician. Pediatrician diagnosed this as epilepsy, and prescribed some medicines, and counseled parents about benign epilepsy. Benign epilepsies are common in childhood and they generally respond to single medicine and disappears in around 4-5 years so patients are withdrawn of the medicines in 4-5 years.
Unfortunately, after one month, she started having similar episodes again, which was quite unusual for benign epilepsies. Her pediatrician, acting in her best interest, sent her to pediatric neurologist this time for further management. Pediatric neurologist got her EEG done, which tells us about the electrical currents in our brain and supposed to be abnormal in epilepsy case. To his surprise, EEG was normal and he could not get any clue about this. With her seizures uncontrolled, in couple of months, she was prescribed two more medicines to control, which has now become habitual. It is very unusual for benign epilepsies to not respond to three medicines, so suspecting drug resistant epilepsy, her pediatric neurologist after 10-12 months of trial of different drugs advise qd MRI scan of her brain. Unfortunately, MRI scan of brain is also normal and she was labelled as unusual epilepsy, but told to continue medicines. Now, it’s been a year and half and her seizures are
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continued. Over a period of two years, convulsions which were only evident at night, now started occurring at daytime. So, she was taken to pediatric neurologist, who repeated her EEG. This time EEG showed spiking activity, which is characteristic of epilepsy, coming from her right side of the frontal brain. So at least a diagnosis of epilepsy is made with certainty and now she is labelled as drug resistant epilepsy. Drug resistant epilepsy is the term used when two or more medicines, prescribed by experienced neurologist, consumed by patients without any default, fails to control seizures or epileptic attacks.
As all parents would do, they took her to another pediatric neurologist for opinion and treatment. This doctor after reviewing all the progress so far, changed the medicines and asked her to follow up again. She remained well for another two months and her parents were about to go into happiness phase when seizure episodes recurred again. This is typically called honeymoon period, when addition of new drug will stop the seizures for some time. And the same story continued.
During all these medical events happening, her life changed a lot. When she was put on two drugs, she started getting too much sleepy all the time due to medicine effects. Daytime attacks in front of her friends made her a topic of discussion and clutter in the class, which she could not cope up with. She started staying isolating herself from her friends and became little irritated and angry all the time. Due to excessive sleepiness, she just could not concentrate in class and within no time, once a bright girl of her class could not even pass monthly class tests. Her parents were worried for her too much but every effort went in vain. Now, she had few falls during school time and hence she was hold back by teachers from running, swimming, skating and contacts sports. Cycling, which she used to enjoy most became a forbidden thing for her. Also, she became little dull due to medicines effects and
hence could not play most of these sports anyway. So, a bright, playful, cheerful life came to absolute halt and her parents just could not watch it further.
So, it was in fifth year of illness she came to my clinic and I could sense the despair in parents that probably we also won’t be able to offer much and it would be just another visit. Going through above history, I have already noted that its very unlikely for benign epilepsies to not respond to drugs. There has to be some small structural fault or small cortical malformation in the brain, which is not evident on routine MRI. We just have to find it.
Parents agreed for video EEG test where we record all the seizure episodes in hospital unit equipped with nursing staff and technicians. We recorded exact body movements during seizure along with synchronized EEG of brain, which gave us complete idea of possible area of brain giving rise to seizures. So, right frontal lobe was possible location, all her seizures are comming from. Now, we do a dedicated epilepsy surgery protocol MRI aimed at detecting these small malformations, which is different from routinely performed scans, so also a PET scan. MRI gives idea about the finest structural details of brain and PET depicts the glucose metabolism of brain. Epileptogenic areas are supposed to be having faulty glucose utilization mechanism and hence these are seen as cold areas on PET scan. Thus, if all the three datasets match, then we could have got out of seizures in no time.
To our surprise, the most dedicated sequence of MRI, did not show anything positive and reported negative. We did lot of post processing of MRI scans, adjusting windows, contast, exposure to better it, along with MRI_PET fusion but did not reveal anything, so by now everybody started believing that the girl will have to continue with seizures.
At this juncture, we have to tell them that we could not do anything as of now as MRI is negative and we have to do further test called stereo EEG. In stereo EEG, which is a surgical procedure, based on previous data of seizures, MRI and PET, few electrodes are inserted in brain based on understanding of previous tests . These electrodes directly record electrical signals from brain of the patient at the start of seizures, thus giving the exact details of seizure onset area.
Stereo EEG planning where we have to plan the electrodes we put inside the brain in details so as to avoid any neurological injury
We first insert the screws in the skull of patients after giving him/her an anesthesia. Depending on the previous planning, a perfect trajectory and length electrode was inserted in patients brain. This is routine procedure we perform and its very safe as far as the chances of neurological injury is concerned.
After doing stereo EEG, we do post processing on scans to exactly know the location of every electrode and trace the electrical activity from every contact. In this patient, the circle around the electrode was throwing characteristic electrical discharges and gave us the clue of abnormality.
It took one year for them to decide to go ahead with such procedure but finally better sense prevailed and parents agreed. We inserted the electrodes and recorded her habitual seizures with the electrodes poked inside her head. It gave us a very classic electrical signatures of the abnormality, surely it was small malformation of brain called focal cortical dysplasia.
So, based on those inputs, a small area of abnormality was present in depth of one of the convolutions of the brain called sulcus. It was so small that even after this signals, review of MRI again was not convincing. But stereo EEG being gold standard, we went ahead and performed the resection of the small area of brain. It was very small surgery and very safe compared to the routine brain surgeries we do for tumors. We came out victorious after
resection and patient reported no seizures at all. It was a clear success as the kid who had 30-40 seizures every day did not have a single seizure in first 24 hours of surgery.
She had completely uneventful recovery after surgery and remained seizure free during hospitalization. She went home exactly the same as she came but without any seizures. Histopathological examination of her brain sent for examination turned out to be focal cortical dysplasia type 2a, as expected during surgery. Yesterday, I saw her after 6 months of her surgery, she was so full of cheer and life all around, giggling all the time and narrating her school gathering function where she had participated in some Bollywood dance.
Epilepsy surgeries are highly rewarding in a way, that if we act in time, a normal productive lifespan can be gifted to these otherwise normal patients, who otherwise would have a very difficult life to ahead.