Scott 4-22-08 Pediatric Epilepsy

Front 1

epilepsy definition

Back 1

two or more seizures/spells that are stereotypical in nature

Front 2

the rhythm seen in the occipital regions while awake with eyes closed

Back 2

Background Rhythm

Front 3

Normal background in adults

Back 3

10-12 hertz and is called an alpha rhythm

Front 4

2-3 hertz

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Delta rhythm

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5-6 hertz

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Theta

Front 6

12-14 hertz

Back 6

Beta rhythm and generally associated with medications (benzodiazepines).

Front 7

pediatric backgrounds are slower and not as well organized. A rule of thumb is the awake background should be 3-4Hz at 3 months, 5Hz by 6 months, 6-7Hz from 9-18 months, 7-8Hz by 24 months, 9Hz by seven and 10 Hz by 15 years of age. Consequentially, a background alpha rhythm in a 6 year old is abnormal.

Back 7

pediatric backgrounds are slower and not as well organized. A rule of thumb is the awake background should be 3-4Hz at 3 months, 5Hz by 6 months, 6-7Hz from 9-18 months, 7-8Hz by 24 months, 9Hz by seven and 10 Hz by 15 years of age. Consequentially, a background alpha rhythm in a 6 year old is abnormal.

Front 8

--usually single high voltage waves that are easily seen through the background

Back 8

Spike waves

Front 9

When are spike waves assoc with epilepsy

Back 9

By themselves they may be “normal”. But, when described with an after following slow wave, i.e spike and slow wave discharge are usually associated with an epilepsy syndrome.

Front 10

Posterior Driving Response ?

Back 10

—Seen with photic stimulation, should be symmetrical and is normal. A lack of posterior driving response is normal as long as it is symmetrical.

Front 11

"Rhythmic slowing" during _________________ stimulation can also be normal.

Back 11

"Rhythmic slowing" during hyperventilatory stimulation can also be normal.

Front 12

Hipsarrhythmia—? ********

Back 12

high voltage 1-3Hz multifocal asynchronous spike/sharp wave activity is the defining rhythm in Infantile Spasm.

Front 13

Rolandic Spikes occur at the central regions and are definitive of _______ Rolandic Epilepsy.

Back 13

Rolandic Spikes occur at the central regions and are definitive of Benign Rolandic Epilepsy.

Front 14

Photoparoxsymal (photoconvulsive) Response usually in the form of spike and slow wave discharges elicited with ______ stimulation. This can be either generalized (more common) or localized.

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Photoparoxsymal (photoconvulsive) Response usually in the form of spike and slow wave discharges elicited with photic stimulation. This can be either generalized (more common) or localized.

Front 15

for the most part seizures during the neonatal period are due to structural causes or metabolic derangements associated with ______ _______ of metabolism

Back 15

for the most part seizures during the neonatal period are due to structural causes or metabolic derangements associated with inborn errors of metabolism

Front 16

There are three specific syndromes during the neonatal period that I want you to be aware of

Back 16

Benign Neonatal Familial Convulsions
Epilepsy Associated with Hypocalcaemia
Withdrawal Seizures

Front 17

Benign Neonatal Familial Convulsions
This generally occurs 7-14 days after birth, and consists of Myoclonic like jerking. What differentiates this from the other more sinister types is a _______ _______ of similar spells. This generally resolves within a brief period.

Back 17

Benign Neonatal Familial Convulsions
This generally occurs 7-14 days after birth, and consists of Myoclonic like jerking. What differentiates this from the other more sinister types is a family history of similar spells. This generally resolves within a brief period.

Front 18

Epilepsy Associated with Hypocalcaemia
Can be seen from birth up to 14 days of age and associated with hypocalcaemia of any cause. These seizures respond to what?.

Back 18

Epilepsy Associated with Hypocalcaemia
Can be seen from birth up to 14 days of age and associated with hypocalcaemia of any cause. These seizures do not respond to anticonvulsant medication but to calcium supplementation.

Front 19

Withdrawal Seizures
Again can occur from birth out to _-_ weeks of age depending on the agent. Most of these children will later manifest symptoms of developmental delay. Most commonly seen with substances of abuse including, alcohol, barbiturates, benzodiazepines, cocaine, narcotics, and stimulants. In a number of states mothers found to be using illicit drugs during pregnancy are subject to prosecution for child endangerment/abuse.

Back 19

Withdrawal Seizures
Again can occur from birth out to 4-6 weeks of age depending on the agent. Most of these children will later manifest symptoms of developmental delay. Most commonly seen with substances of abuse including, alcohol, barbiturates, benzodiazepines, cocaine, narcotics, and stimulants. In a number of states mothers found to be using illicit drugs during pregnancy are subject to prosecution for child endangerment/abuse.

Front 20

INFANTIAL SYNDROMES occur during what age period?

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6-9 months of age through early adolescents.

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BENGIN INFANTILE EPILEPSY SYNDROMES
There are four in which I feel you should be aware of.

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BENIGN INFANTILE MYOCLNIC EPILEPSY
CHILDHOOD ABSENCE EPILEPSY
FEBRILE SEIZURES
BENIGN ROLANDIC EPILEPSY

Front 22

BENIGN INFANTILE MYOCLNIC EPILEPSY usually begins in the _ year of life. Spells are that of brief Myoclonic jerks. Generally they are associated with a positive family history. They can later, 10-12 years of age, have generalized tonic clonic seizures. These are generally easy to control and frequently go into remission. There can be mild developmental delay. EEG can show generalized spike waves while awake, usually disappears with sleep.

Back 22

BENIGN INFANTILE MYOCLNIC EPILEPSY usually begins in the 2 year of life. Spells are that of brief Myoclonic jerks. Generally they are associated with a positive family history. They can later, 10-12 years of age, have generalized tonic clonic seizures. These are generally easy to control and frequently go into remission. There can be mild developmental delay. EEG can show generalized spike waves while awake, usually disappears with sleep.

Front 23

CHILDHOOD ABSENCE EPILEPSY typically begins at _-_years of age and consists of starring spells with speech arrest. They last seconds and can occur frequently through out the day. Teachers will often say the child appears to be always “day dreaming”. This also tends to run in families. There also can be occasional generalized tonic clonic seizures.

Back 23

CHILDHOOD ABSENCE EPILEPSY typically begins at 4-6years of age and consists of starring spells with speech arrest. They last seconds and can occur frequently through out the day. Teachers will often say the child appears to be always “day dreaming”. This also tends to run in families. There also can be occasional generalized tonic clonic seizures.

Front 24

CHILDHOOD ABSENCE EPILEPSY
For the most part this also goes into remission in later adolescents. Due to problems with school performance these are typically treated. The drug of choice is __________. These children are usually normal and more commonly seen in females. Can be induced with hyperventilation showing 3-6Hz generalized spike and slow wave discharges.

Back 24

CHILDHOOD ABSENCE EPILEPSY
For the most part this also goes into remission in later adolescents. Due to problems with school performance these are typically treated. The drug of choice is ethosuximide. These children are usually normal and more commonly seen in females. Can be induced with hyperventilation showing 3-6Hz generalized spike and slow wave discharges.

Front 25

FEBRILE SEIZURES, typically begin at _-_ years of age. Child is normal when for no reason other than a high fever has a generalized tonic clonic seizure. These are typically brief in duration and can reoccur with fevers but are out grown quickly. This is also frequently seen as a familial disorder. Other than treating the fever anticonvulsants are not indicated. Routine EEGs are normal.

Back 25

FEBRILE SEIZURES, typically begin at 5-6 years of age. Child is normal when for no reason other than a high fever has a generalized tonic clonic seizure. These are typically brief in duration and can reoccur with fevers but are out grown quickly. This is also frequently seen as a familial disorder. Other than treating the fever anticonvulsants are not indicated. Routine EEGs are normal.

Front 26

BENIGN ROLANDIC EPILEPSY generally begins at age _-__ years. These are very commonly _________ seizures frequently at the onset there is a yell, shout, or other form of verbalization. These frequently secondarily go into a generalized tonic clonic seizure. There is a male predominance with this syndrome. EEG shows characteristic Rolandic spikes in the centrotemporal region (hence the other name Centrotemporal Epilepsy) with a horizontal dipole recorded during sleep. These may or may not require treatment depending on the frequency. These can be out grown by the late teenage years.

Back 26

BENIGN ROLANDIC EPILEPSY generally begins at age 8-12 years. These are very commonly nocturnal seizures frequently at the onset there is a yell, shout, or other form of verbalization. These frequently secondarily go into a generalized tonic clonic seizure. There is a male predominance with this syndrome. EEG shows characteristic Rolandic spikes in the centrotemporal region (hence the other name Centrotemporal Epilepsy) with a horizontal dipole recorded during sleep. These may or may not require treatment depending on the frequency. These can be out grown by the late teenage years.

Front 27

INFANTILE SPASMS
These usually began at about 12 months of age. The two most common types are ____ Syndrome which can be inherited and _________ _________ (__) which is associated with congenital hamartomas.

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INFANTILE SPASMS
These usually began at about 12 months of age. The two most common types are West Syndrome which can be inherited and Tuberous Sclerosis (TS) which is associated with congenital hamartomas.

Front 28

Tuberous Sclerosis is generally is an Autosomal __________ inherited disorder with a prevalence of 1: 9700.

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Tuberous Sclerosis is generally is an Autosomal Dominant inherited disorder with a prevalence of 1: 9700.

Front 29

INFANTILE SPASMS
Typically one finds lesions or hamartomas in the periventricular white matter. Infantile spasms are generalized spasm which on EEG demonstrates a _______________ pattern.

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INFANTILE SPASMS
Typically one finds lesions or hamartomas in the periventricular white matter. Infantile spasms are generalized spasm which on EEG demonstrates a Hipsarrhythmia pattern.

Front 30

INFANTILE SPASMS
Treatment at diagnosis is with IV ____. Prognosis is very poor.

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INFANTILE SPASMS
Treatment at diagnosis is with IV ACTH. Prognosis is very poor.

Front 31

LENNOX-GASTAUT SYNDROME
generally begins in the first year of life.
usually profoundly _________.
multiple different seizures including absence, Myoclonic, drop attacks and generalized tonic/clonic seizures.
EEG shows 1-2 Hz generalized spike and slow wave discharges.
very malignant syndrome with ____ response to the anticonvulsants.

Back 31

LENNOX-GASTAUT SYNDROME
generally begins in the first year of life.
usually profoundly retarded.
multiple different seizures including absence, Myoclonic, drop attacks and generalized tonic/clonic seizures.
EEG shows 1-2 Hz generalized spike and slow wave discharges.
very malignant syndrome with poor response to the anticonvulsants.

Front 32

LENNOX-GASTAUT SYNDROME
For patients with significant drop attacks ________________________ can be performed.

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LENNOX-GASTAUT SYNDROME
For patients with significant drop attacks corpus callosotomy can be performed.

Front 33

LANDAU-KLEFFNER SYNDROME
Also referred to as Acquired ________ with Epilepsy. Onset is at about five years of age with a male predominance. The symptoms begin with ________ agnosia and loss of speech. These children at times are misdiagnosed as deaf or hearing impaired. While awake the EEG is generally normal, but during sleep there is continuous spike and slow wave discharges predominantly in the temporal-parietal regions.

Back 33

LANDAU-KLEFFNER SYNDROME
Also referred to as Acquired Aphasia with Epilepsy. Onset is at about five years of age with a male predominance. The symptoms begin with auditory agnosia and loss of speech. These children at times are misdiagnosed as deaf or hearing impaired. While awake the EEG is generally normal, but during sleep there is continuous spike and slow wave discharges predominantly in the temporal-parietal regions.

Front 34

LANDAU-KLEFFNER SYNDROME
Seizures while awake occur in 70% of patients early on but remit usually by the age of 15 years. For the most part the seizures are nocturnal. Early recognition and treatment with IV _____ can stop or reverse the language loss. Treatment with conventional _______________ can control the seizures but not the language loss. A surgical treatment called Multiple SubPial Transectioning seems to halt the progression of the language loss.

Back 34

LANDAU-KLEFFNER SYNDROME
Seizures while awake occur in 70% of patients early on but remit usually by the age of 15 years. For the most part the seizures are nocturnal. Early recognition and treatment with IV ACTH can stop or reverse the language loss. Treatment with conventional anticonvulsants can control the seizures but not the language loss. A surgical treatment called Multiple SubPial Transectioning seems to halt the progression of the language loss.

Front 35

RASMUSSEN’S SYNDROME (ENCEPHILITIS)
This is an idiopathic syndrome generally occurring from 2-14 years of age with the average at 6.8 years of age. Frequently presenting as partial seizures which can be in the form of status Epilepticus (continuous seizures) which is compounded with a gradual ____plegia and __________ loss.

Back 35

RASMUSSEN’S SYNDROME (ENCEPHILITIS)
This is an idiopathic syndrome generally occurring from 2-14 years of age with the average at 6.8 years of age. Frequently presenting as partial seizures which can be in the form of status Epilepticus (continuous seizures) which is compounded with a gradual hemiplegia and intellectual loss.

Front 36

RASMUSSEN’S SYNDROME (ENCEPHILITIS)
The etiology appears to be related to a viral inducement of antibodies to the glutamate receptor GluR3. Initially it starts with seizures, then neurological deterioration, finally the seizures abate but the neurological deterioration persists. The mainstay of treatment has been _________________ once hemiplegia is present. Prognosis is very poor.

Back 36

RASMUSSEN’S SYNDROME (ENCEPHILITIS)
The etiology appears to be related to a viral inducement of antibodies to the glutamate receptor GluR3. Initially it starts with seizures, then neurological deterioration, finally the seizures abate but the neurological deterioration persists. The mainstay of treatment has been hemispherectomy once hemiplegia is present. Prognosis is very poor.

Front 37

For the most part there is only one seizure disorder which starts in the later teenage years. What is it?

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JUVINILE MYOCLONIC EPILEPSY (OF JANZ) OR JME

Front 38

JUVINILE MYOCLONIC EPILEPSY (OF JANZ) OR JME
This is a primary generalized epilepsy which is characterized with brief __________ jerks upon awakening, absence seizures and later grand mal seizures. __% of patients with absence epilepsy will evolve into this. For the most part the Myoclonic jerks are just attributed to the child/teenage being “clumsy”. Most cases are actually diagnosis when the patient has a grand mal seizure.

Back 38

JUVINILE MYOCLONIC EPILEPSY (OF JANZ) OR JME
This is a primary generalized epilepsy which is characterized with brief Myoclonic jerks upon awakening, absence seizures and later grand mal seizures. 30% of patients with absence epilepsy will evolve into this. For the most part the Myoclonic jerks are just attributed to the child/teenage being “clumsy”. Most cases are actually diagnosis when the patient has a grand mal seizure.

Front 39

JUVINILE MYOCLONIC EPILEPSY (OF JANZ) OR JME
Onset is usually at about age 16-18 years of age in males and 18-21 in females. Sleep deprivation and _______ are precipitating factors. EEG will demonstrate 3 Hz generalized spike and slow wave discharges better seen on a sleep deprived EEG. Treatment of choice is _________ with lamictal being suggested as first line therapy by some experts. While considered to one of the more benign epilepsies this requires lifelong treatment

Back 39

JUVINILE MYOCLONIC EPILEPSY (OF JANZ) OR JME
Onset is usually at about age 16-18 years of age in males and 18-21 in females. Sleep deprivation and alcohol are precipitating factors. EEG will demonstrate 3 Hz generalized spike and slow wave discharges better seen on a sleep deprived EEG. Treatment of choice is depakote with lamictal being suggested as first line therapy by some experts. While considered to one of the more benign epilepsies this requires lifelong treatment

Front 40

Lupus cerebritis in adolescents typically presents with altered mental status/psychosis, ______ and seizures. Treatment is aimed at the underlying cause, SLE with symptomatic use of AEDs.

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Lupus cerebritis in adolescents typically presents with altered mental status/psychosis, ataxia and seizures. Treatment is aimed at the underlying cause, SLE with symptomatic use of AEDs.

Front 41

HERPES SIMPLEX ENCEPHALITIS
Frequently the presenting sign/symptom of HSV is ________. As this initially effects the temporal lobes the seizures tend to be complex partial with temporal lobe like auras. This generally requires lifelong AED therapy along with the treatment of the HSV.

Back 41

HERPES SIMPLEX ENCEPHALITIS
Frequently the presenting sign/symptom of HSV is seizures. As this initially effects the temporal lobes the seizures tend to be complex partial with temporal lobe like auras. This generally requires lifelong AED therapy along with the treatment of the HSV.

Front 42

DRUG ABUSE
Most of the stimulant agents, cocaine, methamphetamines and even ___ drugs can and frequently cause seizures as a result of abuse and/or withdraw. Benzodiazepines, alcohol sedatives and narcotics can cause seizures as a result of withdraw and can be life-threatening. Treatment is aimed a detoxifying the patient with symptomatic use of AEDs.

Back 42

DRUG ABUSE
Most of the stimulant agents, cocaine, methamphetamines and even ADD drugs can and frequently cause seizures as a result of abuse and/or withdraw. Benzodiazepines, alcohol sedatives and narcotics can cause seizures as a result of withdraw and can be life-threatening. Treatment is aimed a detoxifying the patient with symptomatic use of AEDs.

Front 43

WORK UP FOR NEW ONSET SEIZURE
First and foremost is the _______ of the event and _______ history of similar disorders. Some of the benign infantile/neonatal disorders do not require exhaustive testing. Next is the physical examination. If the child/infant is normal for age then brain tumors, etc are less likely. If not one has to be worried about possible causes

Back 43

WORK UP FOR NEW ONSET SEIZURE
First and foremost is the history of the event and family history of similar disorders. Some of the benign infantile/neonatal disorders do not require exhaustive testing. Next is the physical examination. If the child/infant is normal for age then brain tumors, etc are less likely. If not one has to be worried about possible causes

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WORK UP FOR NEW ONSET SEIZURE
Second, a through __________ evaluation including complete metabolic panels, urine drug screen, blood cell count and differential and an ___ panel when appropriate.

Back 44

WORK UP FOR NEW ONSET SEIZURE
Second, a through laboratory evaluation including complete metabolic panels, urine drug screen, blood cell count and differential and an ANA panel when appropriate.

Front 45

WORK UP FOR NEW ONSET SEIZURE
Third, ____________. In the case of febrile seizures most authorities suggest CT Scan/MRI is not necessary. Also, in the benign familial neonatal types further neuroimaging is not necessary. In all other cases in my opinion either CT scan or MRI of the brain is required, especially so if a structural lesion or infection is suspected.

Back 45

WORK UP FOR NEW ONSET SEIZURE
Third, neuroimaging. In the case of febrile seizures most authorities suggest CT Scan/MRI is not necessary. Also, in the benign familial neonatal types further neuroimaging is not necessary. In all other cases in my opinion either CT scan or MRI of the brain is required, especially so if a structural lesion or infection is suspected.

Front 46

WORK UP FOR NEW ONSET SEIZURE
Fourth, ___. This is again not necessary in a febrile seizure. However, in all other cases it is my opinion this testing is mandatory. The closer the EEG is done to the event the more likely it will be abnormal. Bare in mind between seizures the EEG can be normal in a lot of patients. If appropriate, a sleep deprived EEG can be obtained, and today with the digital technology available we can perform 72 hour ambulatory EEGs.
*Within 7 days*

Back 46

WORK UP FOR NEW ONSET SEIZURE
Fourth, EEG. This is again not necessary in a febrile seizure. However, in all other cases it is my opinion this testing is mandatory. The closer the EEG is done to the event the more likely it will be abnormal. Bare in mind between seizures the EEG can be normal in a lot of patients. If appropriate, a sleep deprived EEG can be obtained, and today with the digital technology available we can perform 72 hour ambulatory EEGs.
*Within 7 days*

Front 47

WORK UP FOR NEW ONSET SEIZURE
Lastly, ______ _________. A ______ _________ is mandatory if there is a clinical suspicion of infection. Again, in my opinion all patients should have a CT scan of the brain prior to ______ _________.

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WORK UP FOR NEW ONSET SEIZURE
Lastly, Lumbar Puncture. A lumbar puncture is mandatory if there is a clinical suspicion of infection. Again, in my opinion all patients should have a CT scan of the brain prior to lumbar puncture.

Front 48

Sandifer’s syndrome - explain

Back 48

neonates to one year. Myoclonic jerks. EEG normal. Reflux is cause - stimulation of vagal nerve causes the jerks. Quite common in children.