(1)
Department of Emergency Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
How common is it to have a single seizure among the general population? |
About 5 % |
What are the two terms you can use for a seizure affecting just one part of the body? |
Focal (old term) or
Partial (new term) |
What differentiates a “simple” from a “complex” seizure? |
Simple means no change in level of consciousness
Complex means there was a change |
What is a “generalized” seizure? |
Seizure affecting both hemispheres from the start |
What does it mean if a seizure is “secondarily generalized?” |
Started in one location (partial seizure) then spread to both hemispheres |
If you see an EEG on the boards, how hard should you work to read it? |
Not hard at all – treat the patient, not the EEG |
Which type(s) of seizure does not have a postictal phase? |
Absence & Simple Partial |
If the eye fields of the brain are involved in a seizure, do the eyes go toward or away from the seizure? |
Away
(Overactive frontal eye fields push the eyes away) |
If a child is getting into trouble for annoying behaviors or not paying attention, what sort of seizures should you consider? |
Absence or complex partial |
During a complex partial seizure with automatisms, will the child be able to respond to outside stimuli? |
Not normally, but some responsiveness |
What is the best way to test for any remaining level of consciousness in an impaired patient? |
Painful stimulation |
What is the most common sort of automatism to occur in an absence seizure? |
Blinking
(but remember that automatisms are most common with complex partial seizures) |
What is the best medication for absence seizures? |
Ethosuximide |
Should Benign Rolandic seizures be treated? |
Only if frequent/problematic |
What group of patients typically develops Rolandic seizures? |
School-aged kids who are otherwise fine |
What is a benign Rolandic seizure? |
A partial seizure that sometimes generalizes – usually occurs at night |
Why are they called “Rolandic” seizures? |
There is a characteristic spike on EEG over the Rolandic fissure area |
What is the other name for benign Rolandic seizures? |
Benign childhood epilepsy
with CentroTemporal Spikes
(BCECTS) |
What is the usual course of benign Rolandic seizures? |
Spontaneously resolve
(by age 15 years) |
Why is it important to know about benign Rolandic seizures? |
• Common cause of epilepsy (about 15 % of childhood cases)
• No treatment needed
• Spontaneous resolution expected |
The 3-Hz per second spike-and-wave pattern is famous for being associated with what type of seizure? |
Absence |
What are the two paths patients with absence seizures are likely to follow as they get older? |
• Resolution in adolescence
• Epilepsy (30 %) |
Which epilepsy syndrome is known for being induced by visual stimuli? |
Juvenile myoclonic epilepsy |
What sort of seizure will the patient have, if he/she has a visually induced seizure? |
Could be myoclonic only, or could be myoclonic with absence or tonic-clonic |
How do you treat visually induced myoclonic seizures? |
Avoid the stimuli (or wear dark glasses, if needed) |
“Incoordination” or jerking movements in the early morning are sometimes the first signs of which seizure disorder? |
Juvenile myoclonic epilepsy |
What do infantile spasms look like? |
Spasms of the flexors – especially head & trunk |
What is the special buzzword associated with the EEG appearance of infantile spasms? |
“Hypsarrhythmia” |
What does hypsarrhythmia mean? |
The EEG has a slow rhythm, and is disorganized
(Specifically – very high voltage, random, slow waves and spikes in all cortical areas) |
Which genetic disorder is especially associated with infantile spasms? |
Tuberous sclerosis
(although lots of others cause it, too) |
Which type of pediatric seizure has the worst prognosis? |
Infantile spasm |
What proportion of children with infantile spasm will go on to have epilepsy? |
Around 60 % |
When children with infantile spasms later develop epilepsy, which type of epilepsy often evolves? |
Lennox-Gastaut syndrome
(difficult to control, multiple types of seizures associated with intellectual disability)
Note: The term “mental retardation” while most familiar to many medical practitioners, has now been replaced by the term “intellectual disability” in many sources. Mental retardation has also been eliminated as a term in the 2013 revision of the Diagnostic & Statistical Manual of Mental Disorders, and in US federal legislation.
In 2015, however, the ICD-11 international classification of diseases will adopt the term “intellectual developmental disorder” to replace both the terms “mental retardation” & “intellectual disability.” This term will then replace intellectual disability in many sources. |
What is the main danger in treating seizure disorders? |
Respiratory arrest – (more patients die of medication complications than die of seizures!) |
If a child has had a febrile seizure, is there risk of developing epilepsy increased? |
Yes – it’s doubled, but still very small
(0.5 vs. 1.0 – exact numbers quoted vary, some recent studies suggest it might be as high as 2.4)
The number traditionally quoted, and most likely to occur on exams, is the doubling to 1 %. |
If you think you have a child with breath holding spells, what can reassure you that it is, in fact, breath holding? |
The sequence –
Pain/anger/fear →
Crying →
Breath holding |
If a child has jerking movements after losing consciousness, does that make it less likely that the episode was really a breath holding spell? |
No – the child may jerk, be limp, or stiff after losing consciousness |
Children with breath holding spells have a higher than average likelihood of also having what physiological disorder? |
Anemia/iron deficiency |
Why do Klippel-Feil patients have limited neck motion? |
Some or all of their cervical vertebra are fused |
What is the “triad” of Klippel-Feil syndrome? |
Short neck
Limited neck motion
Low occipital hairline
(all neck related!) |
Risk for serious neurological problems in Klippel-Feil patients is highest if the fusion occurs in what part of the c-spine? |
Occipito-C1 vertebral junction |
Is it common for Klippel-Feil patients to have associated anomalies? |
Yes |
What is lissencephaly? |
Smooth brain (gyri are missing) |
With which congenital syndrome is lissencephaly most associated? |
Miller-Dieker |
What is schizencephaly? |
Clefts in the cerebral hemispheres that weren’t supposed to be there
(remember “schizo” means split, so schizencephaly is extra “splits” of the brain) |
What sorts of clinical problems does schizencephaly create, if any? |
Seizures, severe intellectual disability, quadriparesis |
What is porencephaly? |
Holes (pores) within the brain |
How would a child develop porencephaly?
(2 ways) |
Usually occurs due to damage from stroke or infection after birth
Can also be congenital, due to developmental error(s) |
What is acquired porencephaly?
(formerly called pseudoporencephaly!) |
Special name for acquired holes in the brain, not due to developmental processes |
How can pseudoporencephaly be distinguished from congenital porencephaly? |
Congenital porencephalic cysts communicate with the ventricle or subarachnoid space, acquired cysts generally do not
&
Acquired porencephaly is not associated with other CNS malformations |
How will acquired porencephaly patients present? |
Focal findings, seizures, or sometimes asymptomatic |
Do congenital porencephaly patients present differently from acquired porencephaly? |
Congenital patients often have more global problems, such as intellectual disability, cerebral palsy, and blindness |
If you’re born without a corpus callosum, what sorts of problems should you expect to experience? |
Some neuropsychiatric difficulties, but often subtle
(75 % of these patients have normal intelligence & otherwise normal appearing function) |
Why would someone fail to develop a corpus callosum? |
1. Inherited – both AD and AR
2. In utero injury prior to the 20th week of pregnancy
3. Association to some in utero exposures, especially alcohol |
Holoprosencephaly is highly associated with what very unusual facial abnormality? |
Cyclopia (single eye) |
What is the most severe form of holoprosencephaly? |
“Alobar”
(this means that no lobes formed in the front part of the brain at all) |
What is the most obvious structural finding if you scan a patient with alobar holoprosencephaly? |
Single ventricle |
How long do alobar holoprosencephaly patients usually survive? |
A few months |
Use of which recreational drug are associated with agenesis of the corpus callosum? |
Alcohol & cocaine |
What is static encephalopathy? |
Cerebral dysfunction that doesn’t worsen over time (it’s allowed to improve) |
If a patient is encephalopathic due to high ammonia levels, would that be considered static encephalopathy? |
No –
whatever caused the encephalopathy must be a completed process leaving changed cerebral function to use this label |
What type of problem does the term “cerebral palsy” refer to? |
Motor |
Technically, what is a convulsion? |
A seizure due to a cortical lesion (most seizures have this mechanism) |
Is cerebral palsy a type of static encephalopathy? |
Yes – motor only |
What aspects of motor function can cerebral palsy affect? |
Posture, tone, & movement (of course) |
Do the problems that cause cerebral palsy occur during, before, or just after birth? |
Any of those
(often the timing of the event is not clear) |
Are sensory problems a part of cerebral palsy? |
No |
Is perinatal asphyxia the major causal factor in development of cerebral palsy? |
No
(<10 % of cases are thought to be related to perinatal asphyxia) |
What is the perinatal asphyxia-related factor that gives a high likelihood of later CP? |
Metabolic acidosis
(umbilical artery pH < 7 and base deficit > 12 at time of delivery) |
What are three known risk factors for the development of CP? |
Prematurity/low birth weight
Intrauterine infection
Congenital malformations |
Is low birth weight associated with CP? |
Yes
(but the causal relationship is not clear) |
Low birth weight is especially associated with what sort of CP? |
Spastic diplegia
(mainly affects the legs, but arms can be involved too) |
Infection is most associated with what sort of CP? |
Spastic quadriplegia
(all four extremities are affected) |
Which aspects of the immune system have been implicated in CP? |
Immune mediators, e.g., interleukins, interferons, and TNF factors
(higher than normal levels may disrupt developing neural circuitry) |
What does “spastic CP” mean? |
Same types of findings as upper motor neuron lesions – hypertonic & hyperreflexic, clonus, & weakness |
What are the subtypes of spastic CP? |
Hemiplegia (one side)
Quadriplegia (both sides – all four extremities)
Diplegia (both sides, but legs more affected than arms or face) |
Dyskinetic CP is another type of CP. What is its presentation? |
Purposeless movements that disappear during sleep – often choreoathetoid and dystonic movements
(truncal twisting, grimacing, etc.) |
What is the underlying problem in dyskinetic CP? |
Abnormal basal ganglia |
Is it possible to have both dyskinetic and spastic CP? |
Yes, unfortunately |
Which sort of CP has the fewest associated problems? |
Dyskinetic |
Which children with CP are most likely to have intellectual disability?
(2 types of CP) |
Mixed (meaning spastic & non-spastic types of CP in the same person)
&
Spastic quadriplegic |
Is intellectual disability common among kids with CP? |
Yes – about 50 % |
Which types of CP are most associated with seizure disorder? |
Spastic hemiplegia & quadriplegia |
Has botulinum toxin been used successfully in spastic CP? |
Yes – for focal problem areas of spasm |
What relatively new treatment, involving a muscle relaxant, has been shown to be helpful in spastic CP? |
Intrathecal baclofen |
What oral meds are used to decrease the spasticity in CP? |
Baclofen
Dantrolene
Benzodiazepines (diazepam, clonazepam) |
If an infant had a stroke in utero, how will that child present (assuming it is detected clinically)? |
Hemiplegic CP or early hand dominance |
Complications from congenital heart disease account for what proportion of strokes in children? |
1/4 |
When is the most dangerous time for a child with congenital heart disease, in terms of stroke risk? |
Cardiac cath or surgery –
1/2 of the strokes happen within 3 days of one of these |
How important are pro-thrombotic factors in childhood strokes? |
Important – 1/3 to 1/2 of children who stroke have them |
If a child has antiphospholipid antibody, what will he/she be predisposed to develop? |
Clots – including stroke |
What does lupus anticoagulant put the patient at risk for? |
Clotting (it was a poor name choice) |
Which types of antiphospholipid antibodies are most commonly implicated in childhood stroke? |
Anticardiolipin antibody & lupus anticoagulant
(these are both types of antiphospholipid antibodies) |
Which other prothrombotic factors are known to be important contributors to stroke in children and young adults?
(5) |
Protein C
Protein S
Antithrombin III
Elevated homocysteine
Factor 5 Leiden |
If a child has a stroke between birth and age two, what are the two most common presentations? |
Seizure or hemiplegia |
If a child presents with an apparent hemiplegia, what two other diagnoses should you consider in addition to structural damage to the brain? |
Complicated migraine
&
Todd’s paralysis
(following a seizure) |
What bizarre possibility should you consider when a child suddenly develops hemiplegia and no cause can be found? |
“alternating hemiplegia”
(Very rare disorder with hemiplegia that switches sides, and seizures between episodes of hemiplegia. Course is progressive neuro deterioration) |
What is ataxic CP? |
Damage to cerebellum results in coordination & gait problems |
How important is sickle cell disease, as a cause of stroke in children? |
Important – ≥10 % |
In what age range is stroke most common for SC kids? |
2–5 years
(rare <2 years) |
Higher levels of hemoglobin F are helpful to sickle cell patients, in general. Does it protect the patient from stroke? |
No |
Is it common for sickle cell children to have asymptomatic strokes? |
Yes – 1 in 5
(the brain is very adaptable in young children, and if the area is small and/or non-critical, there may not be any symptoms) |
Which are more common in children – ischemic strokes or hemorrhagic strokes? |
Equally common |
Which are more fatal in children – ischemic strokes or hemorrhagic strokes? |
Hemorrhagic (>25 %!) |
For sickle cell patients, how can you predict which ones are at special risk for stroke? |
Transcranial Doppler flow studies – high flow means increased risk |
What is moyamoya disease? |
It’s really a description, not a disease – chronic occlusion of small cerebral vessels causes collaterals to form |
What is the buzzword for appearance of moyamoya on scans or angiography? |
“Puff of smoke” – a dense area of wispy vessels is seen |
In children, moyamoya is associated with which disorders?
(4) |
Sickle cell
Trisomy 21
Neurofibromatosis (type 1)
& Radiation treatment |
Stroke symptoms are occasionally caused by another sort of problem, not thrombus, embolus, or hemorrhage. What problem is that? |
Arterial dissection – usually of the internal carotid |
What is the most common cause of CNS vasculitis in children? |
Bacterial meningitis |
What percentage of children who have had bacterial meningitis also have evidence of a CNS infarct? |
About 10 % |
When chickenpox is associated with stroke, when does the stroke develop? |
Within 12 months of the rash
(recent data suggests the risk may be limited to the first 6 months, but this is still under investigation) |
Which kids are most likely to develop chicken pox associated strokes? |
<10 years old |
Where do infarcts usually occur, when stroke follows chicken pox? |
Internal capsule or basal ganglia
(think of it as subcortical motor stuff) |
Which two rheumatologic diseases are rare causes of CNS vasculitis in children? |
Lupus & Takayasu arteritis (usually seen in Asian females) |
If a child has a stroke without a clear etiology, what metabolic disorders should you consider? |
↑ homocysteine
MELAS disease
Fabry disease |
What is MELAS disease? |
Mitochondrial myopathy
Encephalopathy
Lactic Acidosis
Stroke-like episodes |
Why does Fabry disease predispose children to stroke? |
Ceramide builds up in the vascular endothelial cells, narrowing the vessels
(specifically, it is globotriaosylceramide that builds up)
(α-galactosidase deficiency) |
If a child does not have homocystinuria, should you still consider elevated homocysteine as a possible contributor to a stroke? |
Yes, if the stroke was ischemic –
We have recently learned that many things increase homocysteine levels (meds, cigarettes, renal disease, thyroid disease, vitamin deficiency, etc.) |
Why do elevated homocysteine levels lead to stroke, anyway? |
High levels damage vascular endothelium, promoting thrombus formation |
Is neonatal stroke more common in full-term or preterm infants? |
Full term |
Are neonatal strokes usually embolic or thrombotic? |
Embolic
(the placenta is thought to be the most common source)
Note: Ischemic & hemorrhagic strokes are equally common in children – ischemic stroke is more common in neonates |
Which cerebral artery is most often involved in neonatal stroke? |
Left MCA
(middle cerebral artery) |
What is the best diagnostic for finding a stroke in a neonate? |
MRI
(Remember, US is not going to show you much in an ischemic stroke) |
How do neonates with stroke usually present? |
Seizures |
If a neonate seizes as a result of a cerebrovascular accident, is he/she likely to have a long-term seizure disorder? |
No – antiseizure medication may be needed for a few months, but usually not long-term |
In addition to emboli, another common cause of stroke in neonates is _________? |
Hypoxic-ischemic insult |
What do you expect to see in a full-term infant who has hypoxic-ischemic encephalopathy? |
Quadriparesis clinically – Parasagittal damage on imaging |
What do you expect to see in a preemie who has hypoxic-ischemic encephalopathy? |
Lower extremity weakness clinically – periventricular leukomalacia on imaging |
How does hypoxic-ischemic encephalopathy cause damage to the brain? |
Multiple mechanisms:
Hypoxia & asphyxia
Ischemia & acidosis |
What is the most common cause of intracranial bleeding in children? |
Trauma |
What are the other common reasons for intracranial bleeding in children? |
Coagulopathy
Vascular malformation
Aneurysm |
Typical signs of intraparenchymal bleeding are ______? |
Seizure
Focal neuro problem
Headache
Change in mental status |
Typical signs of subarachnoid bleeding are ________? |
“Worst headache of my life” & change in mental status |
What is the best diagnostic choice for acute intracranial bleeding in anyone who doesn’t have a fontanelle? |
Head CT
(no contrast – new blood is bright) |
What is the most common type of intracranial vascular malformation in children? |
Venous angiomas – usually benign, no treatment needed |
Which type(s) of intracranial malformations usually require surgical intervention? |
AVMs
(some may be embolized, others require surgical removal) |
Which disorders are associated with saccular aneurysms? |
Aortic coarctation
Ehlers-Danlos
Marfan’s
Autosomal dominant polycystic kidney disease
(many other disorders also have some association with saccular aneurysms) |
What is the other term for saccular aneurysms? |
Berry aneurysms |
Where are berry aneurysms usually found? |
Anterior half of the circle of Willis |
Should intracranial aneurysms be routinely removed? |
No – it depends on the aneurysm.
(If it becomes symptomatic, it definitely needs to be removed) |
The main risk for patients with berry aneurysms is _______? |
Subarachnoid hemorrhage |
Can a thrombotic event lead to headache, papilledema, nausea, vomiting, and 6th cranial nerve palsy – all without other localizing signs? |
Yes – Superior sagittal sinus thrombosis
(CSF return to the venous system is blocked) |
Proptosis, chemosis, and ophthalmoplegia can result from what thrombotic event? |
Cavernous sinus thrombosis
(usually related to cavernous sinus infection from nearby facial sinuses) |
The sagittal sinus and cavernous sinus are examples of what general type of structure? |
Cerebral veins |
What are the main risk factors for cerebral vein thrombosis? |
Dehydration &
Prothrombotic disorders
(which are also called “thrombophilias”) |
What is the most common presentation for a neonate with a cerebral vein thrombosis? |
Seizure |
What is the most common presentation for children with cerebral vein thrombosis? |
Headache with nausea/vomiting |
If you are worried about cerebral vein thrombosis, what will a screening head CT usually show? |
Often nothing – contrast is usually needed to see the clot |
What is the diagnostic test of choice to diagnose cerebral vein thrombosis? |
MRI/MRA with contrast |
How worried should you be about a patient who has a cerebral vein thrombosis? |
Worried – 20 % bad M&M
(about 5 % mortality & 15 % with long term sequelae) |
An adolescent female is diagnosed with optic neuritis on the board exam. What disorder do you suspect she has/will have? |
Multiple sclerosis
(about 50 % of optic neuritis patients will develop MS) |
Band-like sensory abnormalities, incoordination, oculomotor disturbance, and vision abnormalities are typical presenting symptoms for which disorder? |
Multiple sclerosis |
The best way to diagnose multiple sclerosis is ______? |
Clinically!
It is the only way. |
What is the basic clinical criterion for an MS diagnosis? |
Deficits that vary in time and place (location within the brain) |
What imaging test is often helpful in making a multiple sclerosis diagnosis? |
MRI – looking for areas of demyelination |
If you suspect MS, but the patient’s MRI is normal, does that rule-out the diagnosis? |
No |
If you’ve ordered an MRI on a patient with focal neurological abnormalities not consistent with MS, and the MRI report comes back with focal areas c/w MS, does that mean the patient has MS? |
No – A small but significant proportion of the normal population has these “spots” on MRI |
What lab test can be helpful to making an MS diagnosis? |
CSF with high IgG or “oligoclonal bands” |
It is rare to develop MS before what age? |
10 years old |
Traditional treatment for MS exacerbations relies mainly on what two medications? |
Steroids & ACTH |
What is the most common type of childhood seizure? |
Generalized (60 %) |
The buzzwords for the EEG appearance of a generalized seizure are ______ & _______? |
Bilateral & synchronous |
Do patients with generalized seizures typically have an aura before the seizure? |
No – it would be better if they did, so they could go sit down! |
Generalized seizures in very young children are often different in presentation from those of older children. How? |
Usually tonic or clonic, rather than a mix of both |
What is the deal with myoclonic seizures – is there a change of consciousness or not? |
Depends on length of seizure – very brief ones don’t affect mental status. Long ones often do. |
What is special about the relationship of myoclonic seizures to other seizure types? |
Myoclonic sometimes occurs with absence or tonic-clonic seizures |
Myoclonic seizures should classically have what characteristics? |
Short episode of quick bilateral muscle contractions – may be one or multiple jerks involved |
Do myoclonic seizures put the patient at risk for falls? |
Yes |
Patients with myoclonic seizure disorders are more likely than average to also have what sort of chronic diseases? |
Neurodegenerative |
Which seizure disorder runs in families, and is especially likely to appear with sleep deprivation or alcohol use? |
Juvenile myoclonic epilepsy |
A sudden loss of muscle tone, with loss of consciousness, sometimes preceded by one or two myoclonic jerks is what type of seizure disorder? |
Atonic or akinetic seizure disorder (both names are used)
The common name for these is “drop attacks” |
Which children are most likely to develop atonic seizures? |
Static encephalopathy/Lennox-Gastaut syndrome kids |
What is the treatment for akinetic seizures? |
No good one |
A patient of normal intelligence is presented who complains of bilateral jerking movements in the morning. Episodes began in the preteen years. What is the disorder? |
Juvenile myoclonic epilepsy |
In addition to bilateral involuntary jerking movements in the morning, the patient’s girlfriend told him that sometimes he has an all-out seizure in the morning. What disorder is that? |
Still juvenile myoclonic epilepsy – some have generalized tonic-clonic morning seizures |
Janz syndrome is the other name for which seizure disorder? |
Juvenile myoclonic epilepsy (they both start with “J”) |
What is the drug of choice for treating juvenile myoclonic epilepsy? |
Valproate |
What is the main worry in terms of serious side effects with using valproate? |
1. Hepatotoxicity (especially in preschoolers)
2. Pancreatitis |
What are the more common, but less worrisome, side effects of valproate?
(5) |
Nausea or weight gain, hair loss, thrombocytopenia, sleepiness |
Which antiseizure medication can be used with any of the common seizure types? |
Valproate |
Are absence seizures generalized or focal? |
Generalized |
How long does an absence seizure usually last? |
A few seconds
(not more than 10) |
Are there any movements that often accompany absence seizures? |
No big movements – flickering eyelids are common, though! |
What is the old (French) name for absence seizures? |
Petit mal |
How can you tell an absence seizure from a complex partial seizure? |
Absence is:
Short (<10 s)
No aura
No postictal phase |
Is it possible to induce an absence seizure in children who don’t normally have them? |
Yes, with 3–4 min of hyperventilation
(even gives you classic EEG 3 cycles/second waves) |
Is there a familial form of absence epilepsy, and if so, how is it inherited? |
Yes – multifactorial
The children grow out of it in adolescence |
What are simple partial seizures? |
Seizures that affect only one area of the cortex – usually motor, but can be sensory or cognitive (e.g., hallucinations) |
What is the most common type of simple partial seizure? |
Motor |
How long do simple partial seizures last? |
Short – 10 to 20 s |
Do you expect a postictal phase, or aura, with simple partial seizures? |
No – neither |
Complex partial seizures usually come from what part of the brain? |
Limbic system
(names like amygdala, hippocampus, and “mesial” temporal lobe) |
What is the most common specific anatomic abnormality seen in children with complex partial seizures? |
Hippocampal sclerosis |
Is a postictal phase expected with complex partial seizures? |
Yes |
What is the likelihood that a complex partial seizure patient will become seizure free on meds? |
<1/3 |
What are the drugs of choice for complex partial seizures in pediatrics?
(2 preferred, and 2 backup) |
Carbamazepine & oxcarbazepine
Phenytoin & valproate are also appropriate first line agents, but the two above are generally preferred
(many antiseizure medication options, and combinations of medications, are possible) |
Which type of seizure patient is most often a candidate for surgical intervention? |
Complex partial
(due to poor control with meds) |
Can someone have an “emotional” seizure? |
Yes – complex partial temporal lobe seizures can consist mainly of an intense emotional experience |
What is the other name for infantile spasms? |
West syndrome
(Think of facing West to salaam, the position of an infantile spasm – Lennox-Gastaut comes later) |
Infantile spasms typically occur in what age group? |
Less than 12 months old, of course! (that’s why they call it infantile) |
What does an infantile spasm look like? |
The politically incorrect way to remember it is as a “salaam” attack – the child suddenly flexes head and trunk |
How many spasms usually occur at a time? |
Anywhere from several to hundreds |
What is the eponymic name sometimes used for infantile spasm? |
West syndrome |
Is the DTaP vaccine related to the development of infantile spasms? |
No |
What is the most common treatment for infantile spasms? |
ACTH
(vigabatrin is also used) |
How successful is treatment for infantile spasms? |
Not very good |
What is the natural course of infantile spasms? |
Spontaneously resolves by 5 years old, but the children develop severe intellectual disability & other seizure types develop (Lennox-Gastaut) |
What defines Lennox-Gastaut syndrome?
(3 features) |
• Severe & multiple types of seizures
• Intellectual disability
• Characteristic EEG |
What is the characteristic EEG for Lennox-Gastaut patients? |
Long runs of bilaterally synchronous sharp and slow-wave complexes – cycles at 2 per second
(commonly known as “atypical spike & wave pattern”) |
What is the clinical presentation of a child with Lennox-Gastaut syndrome? |
A mix of tonic, atonic, absence seizures, and tonic-clonic seizures – most children have two or more different seizure types each day |
What is the drug of choice for Lennox-Gastaut syndrome? |
There isn’t one – it is refractory to treatment |
Older children with Lennox-Gastaut are more likely to have which seizure type? |
Tonic-clonic |
Will children outgrow Lennox-Gastaut? |
No |
With closed head trauma, what major risk factors make the child more likely to have a lasting seizure disorder? |
Structural damage (cerebral contusion, hematoma, & penetrating head injury) or unconsciousness lasting >24 h
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