Partial seizures

Partial seizures in 4 years old girl
Short Summary

A 4 year old girl with normal birth history and development with the exception of an episode of maternal hemiplegia during pregnancy. There is no significant family history of any neurological issues. Her medical history is unremarkable except for celiac disease which is treated with a gluten-free diet.
An half a year ago she developed scarlet fever and began to have episodes of right hand tremor lasting 6 seconds with no alteration of consciousness. One month later she had more events and was taken to the local ED where an EEG was performed.
The EEG showed a normal background with left centrotemporal spikes. A repeat EEG the next day showed similar findings but recorded sleep and sleep activation of the discharges.
Due to these findings, she was placed on Zarontin. An MRI of the brain was performed and was normal except for "slight signal alteration involving the posterior periventricular white substance of both sides of a non-specific nature". There was also opacification of the sinuses and otomastoiditis.
There were no further episodes from but some "restlessness of her limbs" was noticed in sleep.
She then had 2 larger events this past month. She was then placed on Depakote at 160 mg bid.

Patient's Questions

1) What do you think is the possible triggering event of this epilepsy? Is it possible a causal relationship with the abnormalities detected by the MRN: otomastoiditis and sinuses opacification?
2) Do you recommend any further medical tests in addition to those carried out till now?
3) Do you agree with the therapy set out?
4) What, according to your opinion, is the diagnosis?
 

Medical Background

4 years old female.
Family history:
Parents not related by blood.
Mother with previous transitory left hemiplegia during pregnancy. Father and 2 years old brother apparently in good health.
No familiarity with epilepsy or mental retardation.

Medical background:

Born from normal spontaneous delivery at 39th week + 2.
No pre-, peri- and prenatal suffering reported. Good suction. Maternal breastfeeding until six months then regular weaning.
Psychomotor development within normal limits: Seated posture when 4 1/2 months, standing up when 11 months, first words when 6 months.
Alvus and diuresis within normal limits, feeding lacking in gluten due to coeliac disease, sleep-wake rhythm within normal limits. No allergies reported. Patient suffering from coeliac disease.

5 months ago episode of scarlet fever treated with antibiotic therapy. One month later, having a temperature and under antibiotic therapy, the child started to show short episodes characterised by right hand tremors, lasting approximately 6 seconds each, without any concomitant consciousness alterations.
Her parents report that these episodes started on 7 months ago and they were occasional. This time she showed a similar episode of tremors again and, therefore, her parents sent her to the emergency room.

The young patient was, therefore, hospitalized in a pediatric neurology ward where a diagnosis of “Focal epilepsy of myoclonic nature in patient with normal psychomotor development” was placed. During hospitalization the following diagnostic medical tests were carried out:
- Blood tests: within normal limits apart from Hb(Hemoglobin) 11.5 g/dl (v.n. 12-14) MCV 68.8 fl (v.n. 75-85), Platelets 605 (v.n. 130-400), iron in the blood 45 mcg/dl (v.n. 50-80).
- EEG: “Left centrotemporal paroxysmal abnormalities with tendency to omo and controlateral spreading in tracing with normal background rhythm.”
- EEG during sleeping: 2 left centrotemporal paroxysmal abnormalities with tendency to spreading and with activation when sleeping.”
Therefore, at discharge the patient underwent therapy with:
Zarontin 80 mg x 2 a day at 8 a.m. and 8 p.m. for 1 week
Zarontin 80 mg + 160 mg at 8 a.m. and 8 p.m. for 1 week
Zarontin 160 mg x 2 a day at 8 a.m. and 8 p.m. then continues.

Brain MRN carried out under sedation resulted within normal limits apart from a “slight signal alteration involving the posterior peri-ventricular white substance of both sides, of non-specific nature, and bilateral otomastoiditis, opacification of maxillary sinuses, of ethmoid cells and of sphenoidal sinuses bilaterally. Asymmetry of temporal-basal superficial vessels, due to ectasia of a vessel on the left side, is observed."

Her parents noticed, when she was sleeping, a kind of limbs restlessness (arms and legs) sometimes, after a few hours and early in the morning. Moreover, it often happened, and still happens, also in the evening (7 p.m. – 8 p.m.) and, sometimes, also when going to bed (between 9:30 p.m. – 10:30 p.m.) that the child complains she is cold, repeating this several times.

In the current month she had her first two epileptic seizures.
The first one occurred while she was sleeping in the afternoon (she woke up catatonic, staring, with little response to questions and sometimes, mainly her right side was a bit rigid) and ended after about ten minutes when she fell asleep: when she woke up, the patient took about 2 hours to recover either her mood or her right eye and her stomach soreness.
The second one occurred in the morning, when she was in clinique to check ethosuximide dosage and to carry out a specialist follow-up visit. On awakening a 30 seconds long crisis occurred. With remarkable tremors to arms, legs, eyes and twisted and munching mouth.

Following these events, her physician changed the therapy introducing the following outline:
Depakin oral suspension 80 mg at 8 a.m. and 80 mg at 8 p.m. for 7 days
Depakin oral suspension 120 mg at 8 a.m. and 120 mg at 8 p.m. for 7 days
Depakin oral suspension 160 mg at 8 a.m. and 160 mg at 8 p.m. as maintenance.
After 1 week of minimum dosage no real crisis occurred; when sleeping, the child unceasingly turns, she wriggles her lower and upper limbs, either at about 2 a.m. (in a more intense way also for a half hour at intervals until she goes back to sleep) or at about 4/5 a.m. and on waking up for several minutes.

Expert's Opinion

There was a 4-page medical history provided. This contained some medical history as well as summaries of an MRI and EEG's .
The patient is a 4 year old girl with normal birth history and development with the exception of an episode of maternal hemiplegia during pregnancy. There is no significant family history of any neurological issues. Her medical history is unremarkable except for celiac disease which is treated with a gluten-free diet.
An half a year ago she developed scarlet fever and began to have episodes of right hand tremor lasting 6 seconds with no alteration of consciousness. In retrospect she may have had these 3 months ago. One month later she had more events and was taken to the local ED where an EEG was performed.
The EEG showed a normal background with left centrotemporal spikes. A repeat EEG the next day showed similar findings but recorded sleep and sleep activation of the discharges.
Due to these findings, she was placed on Zarontin and increased to a dose of 160 mg bid.
An MRI of the brain was performed was normal except for "slight signal alteration involving the posterior periventricular white substance of both sides of a non-specific nature". There was also opacification of the sinuses and otomastoiditis.
There were no further episodes from but some "restlessness of her limbs" was noticed in sleep.
She then had 2 larger events this past month. The first arose from sleep and consisted of staring with inability to speak and her "right side rigid". The second was also upon awakening with tremors of her arms and legs with chewing movements. She was then placed on Depakote at 160 mg bid.

QUESTIONS:

1. (#4 on the list) What is your diagnosis?
So there are 2 potential diagnoses:
1) Focal-onset epilepsy.
It is possible the patient has seizures that begin on the left side of her brain (which would cause symptoms on the right side of the body like shaking of her right hand and rigidity of her right side). These can potentially spread to the whole brain causing the whole body to shake as was the case with her second seizure. The most important test is the MRI which showed no cause for the seizure (such as a tumor, stroke, or malformation). This is a good sign as there is a much better chance to outgrow seizures if the MRI is normal. The findings in the sinuses and mastoid wouldn't cause these seizures. They are incidental findings but should evaluated by an Ears/Nose/Throat doctor.
2) Benign Rolandic Epilepsy.
This is the most common epilepsy syndrome in childhood (not counting febrile seizures) and begins anywhere from age 1-15 (but most commonly by age 7). The seizures are similar to the partial seizures described above but can occur on either side and typically occur just after falling asleep or upon waking. The EEG is diagnostic and shows centrotemporal spikes which are more frequent in sleep (similar to patient's). These can be only on one side or alternate between the sides on any given EEG. If they are only on one side then subsequent EEG's typically show discharges on the opposite side. All children outgrow this type of epilepsy at puberty or before. The restlessness in sleep is not epileptic and is common in children with epilepsy. They often have restless sleep or even jerks of the extremities known as sleep myoclonus and this is benign.

2. Do you recommend any further tests?

I would recommend repeat EEG testing - possibly a 24-hour EEG if that is available. If the EEG shows centrotemporal spikes on the right at all then that would be diagnostic for benign rolandic epilepsy. Should the abnormalities still be only on the left then it is more likely to be focal-onset epilepsy. The most important test is the MRI which was already done. Her normal development is very reassuring and can be seen with either type of epilepsy. This should continue but some children with these types of seizures can have some problems with attention/focus/memory as they get older but this is usually mild and gets better when the EEG normalizes.

3. What is the possible trigger for the epilepsy?
As stated above - I do not think the MRI findings are responsible for the epilepsy and the white matter changes sound non-specific and likely benign. For Rolandic epilepsy it is a genetic epilepsy and begins around this age. Focal epilepsy can start at any age but the preschool years are a very common time for seizures to begin. There is no specific trigger for these seizures and the celiac is most likely unrelated.

4. Do you agree with the therapy set out?
Yes. Depakote is very effective for these types of seizures where Zarontin is not very effective and could be stopped if she is still taking it. Other medications which may be helpful if Depakote has side effects or stops working include: levetiracetam, lamotrigine, rufinamide, carbamazepine, oxcarbazepine, topiramate and zonisamide.
Benign rolandic epilepsy does not always require treatment since it will be outgrown at some point. Treatment is based on how frequent and severe the seizures are and if there are any learning issues. With these last 2 seizures, I would treat with medication for at least 1 year then re-evaluate. Treatment can be stopped in either seizure type if the EEG is normal after 1 year seizure-free.
 

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