Epilepsy in children

Epilepsy is the most common childhood brain disorder in the United States. Nearly 3 million people have been diagnosed with this disease, while 450,000 of them are under the age of 17.[1] Fortunately, two thirds of the child population will overcome the side effects, most notably, seizures, through treatment during adolescence.[1] Epilepsy affects all ages groups. But for children, a variety of issues exist that can affect one's childhood.

Seizures are defined as 'a transient occurrence of signs and symptoms due to the abnormal, excessive, or synchronous neuronal activity in the brain characterized by abrupt and involuntary skeletal muscles activity.'[2] A doctor will most often diagnose a child with epilepsy, also known as seizure disorder, if the child has one or more seizures, if the doctor thinks they could have another one, and if their seizures aren't caused by another medical condition.[1]

Some epilepsy ends after childhood. Some forms of epilepsy are associated only with conditions of childhood that cease once a child grows up.[3]:35 Approximately 70% of children who have epilepsy during their childhood eventually outgrow it.[4]:6 There are also some seizures, such as febrile seizures, that are one-time occurrences during childhood, and they do not result in permanent epilepsy.[3]:36

Pediatric epilepsy may cause changes in the development of the brain. For this reason, epilepsy in children is vastly distinct from epilepsy in adults and they must be considered differently in most regards.

Presentation

Diagnosis

In 2014 the International League Against Epilepsy (ILAE) Task Force proposed the operational (practical) clinical definition of epilepsy, intended as a disease of the brain defined by any of the following conditions:[2]

  1. At least two unprovoked (or reflex) seizures occurring >24 h apart.[2]
  2. One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years.[2]
  3. Diagnosis of an epilepsy syndrome.[2]

It is important that as soon as someone is diagnosed with SE, they immediately get medical treatment that usually involves the use of medication. The diagnosis of Epilepsy and a Seizure disorder can eventually be considered resolved if the subject has not experienced a seizure in over 10 years and has also not been on anti-seizure medication for five years.[2]

The most common causes of SE in children are fever and infections of the CNS, or Central Nervous System. Other causes can be genetic and metabolic disorders, abnormalities of the CNS, ingestion of toxic elements, and Hyponatremia.[2]

Education

Epilepsy can affect a child's education, thereby leading to trouble learning and lower grades. While many children are capable of functioning in a normal classroom environment, many end up in special education.[4]:247–48

The child may be forced to miss a lot of school due to seizures. The seizures can impair a child's ability to memorize learning materials.

Tonic-clonic seizures can have a serious impact on education due to the memory loss they cause, and the time needed to recover following the seizure-causing there to be missed time in school.[3]:32

Absence seizures can have a high negative impact on a child's education. As they are less obvious than tonic-clonic seizures, they can occur many times within a single day, thereby resulting in the child's ability to learn being impaired, and leading to low grades.[3]:31 Often, these educational deficits lead to the investigation of neurological conditions and result in the diagnosis of this seizure subtype. Children may appear to be 'zoning out' or day-dreaming during classes when in actuality they are experiencing uncontrolled absent seizures. Once treatment begins, these children often exhibit improved attention and their grades improve.

When seizures are controlled by medication, many anticonvulsants have side effects that include drowsiness, thereby also impacting a child's education.

The high school graduation rate has been reported at 64%, compared with an overall national average of 82%.[5]

Social

It is very difficult for a child to struggle through the constraints of epilepsy. They cannot live the same carefree life that they may watch their friends living, but that does not mean their quality of life is any less. The diagnosis of SE is not a fatal or life-ending one, especially for a child. A child with Epilepsy must be much more wary of their surroundings at all time as well as being in communication with their own physical well-being. The social stigma of epilepsy may stand in the way, as the child is more prone to bullying.[6]:42 But as the child learns to manage the diagnosis, it may become a more conventionally normal life for them.

Many children with epilepsy are overprotected by their parents, who put restrictions on them in the name of safety, requiring more adult supervision than other children, and not allowing them to participate in certain activities normal to the age group, such as sports. It is a subject of debate if a child with controlled seizures needs additional protection or restrictions, or if the benefits outweigh the losses a child would face.[6]:44[7]:104

Language

In cases of chronic pediatric epilepsy there is often an association with reduced language skills. The classically understood language areas of the brain are Broca’s area and Wernicke’s area. Realistically, language is significantly more complex and involves several cortical areas beyond these regions.[8]

Language deficits may present with a wide variety of symptoms ranging from odd patterns of speech to complete aphasia of speech. Unfortunately there is not a significant amount of data that parses out how an epileptic firing patterns will cause a resulting language deficit. The correlation of epileptic activity and language deficit is undeniably present, but the mechanisms involved have yet to be unraveled.

In the developing brain, epilepsy may cause the language areas to be structurally altered leading to developmental difficulties. In turn, a child may have trouble acquiring communication skills at a normal rate.[9] This delay may in some children be resolved by compensatory mechanisms or alleviated by medication and therapy, but in some children with persistent epilepsy, the delay may remain or worsen as they age.

In the case of temporal lobe epilepsy (TLE), studies have shown that there is structural compromise to the fiber tracts associated with memory and language, providing some explanation for the impairments in patients with epilepsy.[10]

Language abilities in pediatric epilepsy cases are evaluated using electrical cortical stimulation (ECS) language mapping, electrocorticography (ECoG), fMRI, Wada testing, and magnetoencephalography (MEG).[8]

fMRI has been shown to offer a promising strategy for defining language activation patterns as well as laterization patterns.[11]

It is important to identify language regions involved in epilepsy, particularly temporal lobe epilepsy, before surgical resection in order to reduce the risk of postoperative language deficits. Currently, ECS mapping is the standard of care in localization of areas involved in focal seizure onset and pre surgical planning.

Many pediatric and adult epilepsy patients develop atypical language lateralization due to the reorganization of connections in the epileptic brain. There have been documented cases of interhemispheric and intrahemispheric reorganization of language areas.[12] Several factors may be involved in the extent to which reorganization occurs.

"Table 1: Variables Associated With Interhemispheric and Intrahemispheric Reorganization Found in fMRI and ECS Studies."[8]

Variable Effect on Language Organization Interhemispheric/Intrahemispheric/Both
Early age of seizure onset Yes Both
Left-sided seizure focus Yes Both
Left-handedness Yes Both
Cortical dysplasia (vs discrete tumors) Yes Intrahemispheric
Lower IQ scores Yes Intrahemispheric
Gender No
Age at mapping No
Treatment with antiepileptic medications No
Ictal zone location No
Duration of epilepsy No
Seizure frequency No
Seizure propagation patterns No

The effects of epilepsy on language may be impacted by location of epileptiform activity, severity and duration of electrical discharges, age of onset, treatment method, and surgical resection areas.

In some cases, language impairment may be the first indicator of epileptiform activity in the brain of children. A study done at the University of Gothenburg showed that language impairments were more common in children with epileptic brain activity than children without.[9] They then investigated whether the epileptic activity was the cause of the language deficit or whether there were other factors involved. They found the greatest impairments in language in the children with misfiring on the left side of the brain, the side that controls linguistic abilities. This likely indicates that epileptic activity leads to language difficulties and suggests that in children with language impairments of unknown etiology, evaluations for epilepsy should be considered.

Causes

The causes of epilepsy in childhood vary. In about ⅔ of cases, it is unknown.[4]:44

  • Unknown 67.6%
  • Congenital 20%
  • Trauma 4.7%
  • Infection 4%
  • Stroke 1.5%
  • Tumor 1.5%
  • Degenerative .7%

Treatment

Most children who develop epilepsy are treated conventionally with anticonvulsants. In about 70% of cases of childhood epilepsy, medication can completely control seizures.[4]:6 Unfortunately, medications come with an extensive list of side effects that range from mild discomfort to major cognitive impairment. Usually, the adverse cognitive effects are ablated following dose reduction or cessation of the drug.[13]

Medicating a child is not always easy. Many pills are made only to be swallowed, which can be difficult for a child. For some medications, chewable versions do exist.[3]:43

The ketogenic diet is used to treat children who have not responded successfully to other treatments. This diet is low in carbohydrates, adequate in protein and high in fat. It has proven successful in two thirds of epilepsy cases.[14][7]:100

In some cases, severe epilepsy is treated with the hemispherectomy, a drastic surgical procedure in which part or all of one of the hemispheres of the brain is removed.[15]

See also

References
  1. "Epilepsy in Children: Diagnosis & Treatment". HealthyChildren.org. Retrieved 2019-12-16.
  2. Minardi C, Minacapelli R, Valastro P, Vasile F, Pitino S, Pavone P, et al. (January 2019). "Epilepsy in Children: From Diagnosis to Treatment with Focus on Emergency". Journal of Clinical Medicine. 8 (1): 39. doi:10.3390/jcm8010039. PMC 6352402. PMID 30609770.
  3. Leppik IE (2007). Epilepsy : a guide to balancing your life. Demos Medical Publishing. ISBN 978-1-932603-20-0.
  4. Devinsky O (2008). Epilepsy : patient and family guide (3rd ed.). Demos Medical Publishing. ISBN 978-1-932603-41-5.
  5. Wyllie E, Gupta A, Lachhwani DK (2015). Wyllie E, Gidal BE, Goodkin HP, Loddenkemper T, Sirven JI (eds.). Wyllie's treatment of epilepsy : principles and practice (Sixth ed.). Philadelphia: Wolters Kluwer. p. 1203. ISBN 978-1-4963-0054-6.
  6. Gay K, McGarrahan S (2007). Epilepsy : the ultimate teen guide (1st pbk. ed.). Lanham, Md: Scarecrow Press, Inc. ISBN 978-0-8108-5835-0.
  7. Wilner AN (2010). Epilepsy : 199 answers : a doctor responds to his patients' questions (3rd ed.). Sydney, Australia: Accessible Publishing Systems PTY, Ltd. ISBN 978-1-4587-5607-7.
  8. Chou N, Serafini S, Muh CR (January 2018). "Cortical Language Areas and Plasticity in Pediatric Patients With Epilepsy: A Review". Pediatric Neurology. 78: 3–12. doi:10.1016/j.pediatrneurol.2017.10.001. PMID 29191650.
  9. Rejnö-Habte Selassie G (March 2010). Speech and language dysfunction in childhood epilepsy and epileptiform EEG activity. Institute of Neuroscience and Physiology. Department of Clinical Neuroscience and Rehabilitation. (Doctor of Philosophy (Medicine) thesis). University of Gothenburg. Sahlgrenska Academy. ISBN 978-91-628-8034-7. Lay summary – ScienceDaily.
  10. McDonald CR, Ahmadi ME, Hagler DJ, Tecoma ES, Iragui VJ, Gharapetian L, et al. (December 2008). "Diffusion tensor imaging correlates of memory and language impairments in temporal lobe epilepsy". Neurology. 71 (23): 1869–76. doi:10.1212/01.wnl.0000327824.05348.3b. PMC 2676974. PMID 18946001.
  11. Adcock JE, Wise RG, Oxbury JM, Oxbury SM, Matthews PM (February 2003). "Quantitative fMRI assessment of the differences in lateralization of language-related brain activation in patients with temporal lobe epilepsy". NeuroImage. 18 (2): 423–38. doi:10.1016/s1053-8119(02)00013-7. PMID 12595196.
  12. Kadis DS, Iida K, Kerr EN, Logan WJ, McAndrews MP, Ochi A, et al. (May 2007). "Intrahemispheric reorganization of language in children with medically intractable epilepsy of the left hemisphere". Journal of the International Neuropsychological Society. 13 (3): 505–16. doi:10.1017/s1355617707070397. PMID 17445300.
  13. Greener M (May–June 2013). "Beyond seizures: understanding cognitive deficits in epilepsy". Progress in Neurology and Psychiatry. 17 (3): 31–32. doi:10.1002/pnp.285.
  14. "Ketogenic Diet FAQ". Charlie Foundation.
  15. Kenneally C (July 2006). "The Deepest Cut". The New Yorker.
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