Identification of Symptoms of Epilepsy in Children with Intellectual Disabilities

Identification of Symptoms of Epilepsy in Children with Intellectual Disabilities

                    Patients with intellectual disabilities often have complications additional diseases such as epilepsy, heart disease, movement disorder, self-control, complications of psychotropic drugs, anticonvulsants, etc. They may have epilepsy, movement disorder or behavior that occurs intermittently. Clear classification of symptoms is necessary for determining the cause, following up and arranging proper activities that support safety living.

                   Children refer to those from birth to 15 years of age. In case their actual age cannot be identified, all age ranges are accepted.

                    Children with intellectual and developmental disabilities refer to those with intellectual and developmental disabilities, cerebral palsy or autism.

                     Seizures means symptoms in which brain cells abnormally and continuously disperse a large-scale　of electrical discharge from one part to other parts of the brain causing patients to temporarily express abnormal symptoms like seizures, shocks, disorders of　consciousness, etc. Seizures include:

                     1. Partial seizure refers to seizures that abnormal electrical discharge originates on one part of the brain which can be categorized as follows.

                      2. Grand mal seizure refers to seizures caused by abnormal discharge from both sides of the body when the symptoms occur. This kind of seizures includes:

                           2.2 Spasm and Epilepsy A patient will be unconscious and contracted for less than 30 seconds followed by muscular jerks for 1-2 minutes. He may bite his tongue, urinate, feel nervous or fall asleep. These symptoms exist for no more than 5 minutes. He will feel headache or muscular pain when he is conscious.

                   3. Seizure does not fall on either item 1 or 2 above such as infantile spasms in new born baby.

Spasm with fever occurs from various causes such as;

                   1. Seizure from fever in children aged 6 months to 5 years old is mostly found in a child aged 1-3 years old. Fever is not caused by infection of central nervous system. Seizure occurred within the first 24 hours of fever is classified into:

                          1) Tonic-clonic seizure, convulsions or tetany exist for 15 minutes but not over 5 minutes. Abnormal nervous system is not found nor is recurrence observed within 24 hours.

                          2) Seizure may occur specifically or last longer than 15 minutes or have early abnormality of nervous system or repeated seizure within 24 hours.

Seizure from fever does neither affect the development of learning, behavior and intelligence of children different than their brethren (sibling) nor cause any cerebral palsy.

                  2. Seizure from infected nervous system like Meningitis or Encephalitis

                  3. Metabolic abnormality together with fever such as low sugar or calcium, or magnesium in blood or some toxics in the brain

                  4. First epileptic seizure

Epilepsy means recurrent seizures or 2-3 times of tentative recurrence and widely refers to seizures resulting from brain alterations.

                 In patients with acute disease such as drugs, brain infection, metabolic abnormality, no fever nor pathological conditions, this is not considered epilepsy.

Epilepsy with frequent seizures refers to apoplexy that patients have received medications or operations and have drug in blood circulation in the therapeutic level. However, they still have seizures more than once a month. Factors associated with these symptoms are like patients with continuous seizures, more than one type of seizures, convulsions or epilepsy group- infantile spasms, Lennox Gastaut syndrome or severe deficit of intelligence, etc. Twenty three percent of children with intellectual disabilities hardly controls seizures.

                 Status epilepticus refers to the conditions of continuous seizures for more than 30 minutes or series of seizures in which patients are not conscious in each interval. There are 2 categories as follows.

                 1. Continuous　seizure with convulsions is divided into;

                2. Continuous seizures without jerks while level of conscious altered and abnormal electrical discharge are found such as unconscious partial seizure and grand mal without contractions or jerks.

              Continuous seizures is he first sign of status epilepticus. Twelve percent of all ages of epilepsy patients have a chance to get continuous seizures for 5-10%. Patients aged less than 1 year, 5 years and 16 years have continuous seizures with 37, 85 and 16-24% respectively. Most of them have continuous seizure once. Children aged lower than 5 years show greater chance of recurrent seizures than other age ranges.

Causes of continuous seizures include:

               1. Brain pathology such as cerebral palsy, congenital brain abnormality.

             2.Suddenly stop anticonvulsants or drugs affecting nervous system such as benzodiazepine for sleep problem or anxiety

                3. High fever in children

                4. Metabolic abnormalities such as hyperglycemia hypoglycemia, abnormalities of sodium or calcium blood level, etc.

                5. Acute brain pathology such as stroke, head injuries, encephalitis, etc

                 Patients who have more than 5 minutes of seizures or over 2 times or more of unconscious seizures instantaneously, require emergency medications. Preliminary care include arranging a patient in recovery position-turn on side, removing dentures, foods or glasses and releasing tight dress to allow good breath. Have him lie in a place safe from hitting solid objects. Do not put a stick or object into his mouth or lever his mouth while he has seizures with grinding teeth. Immediately take him to the nearest hospital.

                   Prevalence rates of epilepsy in mild moderate and severe intellectual disabled children are 24, 23 and 53 percent respectively. The prevalence rates have direct association with brain pathology causing cerebral palsy and autism. The epilepsy prevalence in cerebral palsy and autism are 25% and 10% accordingly. Patients with abnormalities of brain structure such as cerebral dysgenesis and Schizencephaly, etc., also possess high rate of epilepsy.

                   Some people with intellectual disabilities may not be able to give information about seizure scenarios. Information is often obtained from a person who does not get used to symptoms. The diagnosis of epilepsy includes patients’ histories and other diseases, physical examination and nervous system, video clipping while shocking or similar symptoms, additional inspection like sugar, sodium and calcium level in blood, brain CT scan, EEG, MRI, etc. Less than 50% is found abnormal in each EEG performing,yet it always shows abnormal EEG after the seizure is present within 24 hours.

                   Children with intellectual disabilities and epilepsy are likely to have behavioral problems such as naughty, restlessness, sad and autistic more than children with other chronic diseases. Factors involving behavioral problems are like difficulties in controlling seizures, lack of support, family stress and so on.

                   Average age of children with intellectual disabilities that shows the first seizure is 1 year and 3 months. Children with mild and severe grade of intellectual disabilities express the first seizure when they are 3 years one month and 8 months respectively. The younger they show the first seizure, the more severe symptoms they have. The seizure symptoms are even more severe in children with cerebral palsy. While the first seizure if occurred when they are adult, it is likely to be less severe.

Characteristics of epilepsy and group of epilepsy in children with intellectual disabilities come in different forms.

                   For infants, infantile spasms are the cause of epilepsy most prevalent in children in first year (90%). Symptoms are like a series of infantile spasms. Most of children who begins to have seizures since infancy to 2 years will express symptoms since 3-7 months. Most of them are likely to have severe abnormality of nervous system within 3 types of spasms which are body bending seizure, body stretching and both. A child will cry while having seizures or stretching contractions followed by a series of spasms. Sometimes he will have eyes open wide with shock. Seizures occur partially or unequally in both sides of the body. For those whose causes can be identified such as brain abnormalities arising out of infection during pregnancy , Down syndrome , Tuberous Sclerosis , Phenylketonuria, Maple syrup urine disease, and urea cycle defect, etc.

                   In early childhood, if a group of Lennox – Gastaut syndrome is found, a child is likely to have seizures from 1-10 years of age and often have lower development or cognitive. Various seizers are like convulsions, tonic-clonic seizure (grand mal), atonic seizure, typical absence, atypical absence, etc. Brainwaves show partial characteristics for seizures present while awake or sleep. These seizures are hard to control since a child may earlier have infantile spasms.

                   Factors arousing spasms in children with intellectual disabilities include fever seizures. Causes frequently found are from ear infection, sinusitis, and diseases in upper respiratory system or urinary path and so on. Or seizures occur when stop taking anticonvulsive drugs.

                  Children with cerebral palsy with both arms and both legs contractions have more frequency of seizures than those present only with half body while those with dyskinetic cerebral palsy show less seizures. First seizure in children with cerebral palsy occur in infancy based on brain pathology causing cerebral palsy. They have all kinds of seizures and frequency such as grand mal seizure, convulsion, unconscious seizure follow by tonic seizure or atonic seizure. Group of seizures like infantile spasms, Lennox-Gastaut syndrome while and typical absence seizures are scarcely found.

Autism,

                   Children with severe intellectual disabilities will show more severe seizure than other group. Some of the patients with language delay development have epilepsy and/ or abnormalities of brainwave. Some patients get better after being treated with steroid and/ or antiepileptic drugs.

                     Epilepsy is accounted for 6 percent of Down syndrome of which 1 percent is infantile spasms. The prognosis of this group is good. Half of them are grand mal seizure or infantile spasms. Down syndrome with cerebral palsy is likely to have epilepsy for 10-15 years prior to expressing symptoms.

                     Most of Angelman patients will have infantile spasms, typical absence, atonic seizure, stretching grand mal seizure and crooked torso. Patients may have partial seizure followed by grand mal seizure at the age of 1 year. Fever shock may be an initial symptom and fever will stimulate more severe seizure. Continuous seizure frequently occurs at the age of 1 month to 1 year and always with severe seizure. The less age the seizure occurs, the more and difficult to control.When a child is growing, seizures are less. In teenagers, caregivers often misunderstand that a patient has no seizures. Abnormalities of brainwave are still found. In addition, seizures are difficult to distinguish from abnormal motion.

                       Rett syndrome is the cause of severely developmental and intellectual disabilities and epilepsy in girl. Particular characteristics are regressive growth and development since infancy. A child will have autistic  like symptoms together with recurrent motions, especially hand movements. About 25-30% of patients have seizures. Most have grand mal seizure and unconscious partial spasms. A little portion of them have infantile spasms or tonic spasm; however, recurrent behaviors are hard to differentiate or somehow diagnose as seizures like pointless staring, periodic breathing stop, etc.

                       Other chromosomal abnormalities that have seizure are Tuberous sclerosis , Fragile X syndrome, chromosome 15 inversion-duplication syndrome, Wolf- Hirschorn syndrome, 4p deletion, ring chromosome 20 syndrome, deletion of the long arm of chromosome 1, trisomy 9p, etc.

                       1. There are many symptoms similar to seizures in children of various ages (see table below), for example, a child cclashes himself against the floor, screams, kicks, bites himself or attacks a person close to him (temper tantrum), breath holding spell in children aged 7 – 18 months who are stimulated from scared, hurt or angry. There are 2 types of breath holding spell: a) Cyanotic type- a child holds breath for 30 seconds and turns green and contracted, some may shock; b) Fainting type a child is often aroused by a blow on his head or pressing on his eye socket, scream and breath holding are not explicit as observed in the first type.

                       2. Syncope or fainting

                       3. Behaviors similar to seizures in children with intellectual disabilities

                       4. Other symptoms like sleep disorders, myoclonic jerk, sleep myoclonus

Symptoms Similar to Seizures in Children

Differential Fainting from Grand mal Seizure

Reference: WWW .stacommunications.com / journal /cme / 2004 / September / pdf / 072.pdf.

                1. Children with severe spasm, acute spasm, symptom common to grand mal

                2. Children with cerebral palsy and sever grand mal caused by chronic constipation, pain

                3. Patients with gastroesophageal reflux, some may have stretching grand mal

                4. Children ignore stimuli; for example, being too quiet, still eye, no facial expressions, slow response and symptoms similar to typical absence spasms.

                5. Repeated motions include nodding, flouncing in/ out, turning body around , waving and clapping hands, etc.

                6. Complications from psychiatric medications like eyelid retraction, abnormal motions

Seizure in intellectually disabled children that are often missed to identify.

                Symptoms of seizures in children with intellectual disabilities are not probably identified- spasms without jerks and partial seizure. Certain amount of time in EEG recording and video clipping may help differential diagnosis among absence spasm, continuous myoclonic seizure without jerk, seizure with periodical headache, seizure with dizziness, seizure with failure of self-control, conscious partial seizure, unconscious partial seizure with other symptoms and postictal effects.

                In addition, self injury is frequently found in patients with psychosis. They will also have seizure easily. The differential diagnosis of epilepsy that caused by follows is required.

                1) Abnormalities in temporal lobe- symptoms are like chest choke, autonomic nervous system disorder, psychoneurosis symptoms such as déjà vu, jamais vu, etc.- abnormalities in smelling or tasting or illusion. Seizures symptoms frequently found in this type of epilepsy in this specific brain area are fix eyes absence mind, autonomic nervous symptoms like chewing mouth, inattentive hands movement, showing strange manners and so on. Patients will be confused after having seizures.

                2) Abnormalities in frontal lobe contain conscious partial seizure, short term unconscious partial seizure and may develop into grand mal seizure. During seizure patients may flounce or show irregular manners unconsciously, for example, pretend to cycle, hip up or grasp reproductive organ. They may often fall down if they have convulsions or seizure with acute muscle weakness.

                1. Improve treatment guidelines for patients with epilepsy, prevention measures and treatment planning, and provide information for parents and staffs.

                2. Develop and publicize manuals on treatment of epileptic patients.

                3. Provide practical trainings on how to assist children when seizures occur for educational and medical personnel.

                4. Provide supports for all levels of human resource development.

                5. Watch medications’ errors and maintain sufficient supply of medicines.

                6. Record data of seizures, prevent factors arousing spasms and arrange activities suitable for children’s routines.

                7. Provide knowledge and develop skills required for treating patients with epilepsy as well as care for parents, caregiver and patients’ mental health and follow up the following aspects.

                           1) Information from portfolio such as seizure symptom, stimuli, frequency, abnormality of nervous system, past medications, dose and consistent use of drugs, and follow up on a child’s development and central nervous system’s abnormalities.

                           2 ) Additional examinations to uncover causes dependent upon the age of patients, records of seizures and abnormalities found when examination is taken place.

                           3) Notify significant treatments such as selection of medications, side-effects, effectiveness of treatments, and practices.

                           4 ) Predictions like a chance of recurrence of spasms, follow up, advice common to those for children in general, reduction of factors affecting spasms, for instance, being exhausted while playing and not getting enough sleep. While seizures are present, try to avoid the risks of dangers from swimming or climbing a tree. Wait until seizure is under control.

                          5) Advice how to help children with Spasms

                 1. Improve knowledge and skills in specifying symptoms and treatments of patients while seizure occurs.

                 2. Coordinate with other professions or provide proper transfer.

                 3. Communicate with parents so that a patient will receive continued services.

                 4. Provide activities suitable for a patient and create understanding among other students.

Roles of Parents and Caregiver

               1. Identify spasms, assist and prevent risk factors triggering seizures.

               2. Receive consistent medications and provide information of spasms, adverse side-effects to curators.

               3. Decide daily activities for a patient.

References

Authors Panel
Ruenkaew Kanokpongsak, MD. แพทย์หญิงเรือนแก้ว กนกพงศ์ศักดิ์
Ubonwan Wattanadilokkul, MD. แพทย์หญิงอุบลวรรณ วัฒนดิลกกุล
Sahas Liamsuwan, MD. นายแพทย์สหัส เหลี่ยมสุวรรณ
Veerayuth Prapanpoj, MD. นายแพทย์วีรยุทธ ประพันธ์พจน์
Thaweesak Siriratrekha, MD. นายแพทย์ทวีศักดิ์ สิริรัตน์เรขา
Sansanee Sudprasert, Mrs. นางศันสนีย์ สุดประเสริฐ
Juthamas Chuasikaew, Mrs. นางชุฑามาศ เชื้อสีห์แก้ว
Pornpan Khanduang, Mrs. นางพรพรรณ ขันดวง
Ratana Wongsang, Mrs. นางรัตนาพร วงษ์สังข์