Transcript Introduction To Epilepsy Semiology diagnosis Treatment QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture. M.

Introduction To Epilepsy
Semiology diagnosis
Treatment
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
M. Scott Perry, M.D.
Emory University
April 18, 2007
September 18, 2006
Objectives
• Recognize different types of seizures.
• Discuss workup for new onset seizures
• Learn classification of epilepsy types based
on history, seizure type, MRI, and EEG
findings
• Review common treatments used in epilepsy
• Learn prognosis based on epilepsy type
• Briefly review some frequently asked
questions
Spells
Seizure Equivalents
GERD
Breath Holding
Infantile Masturbation
Syncope
Benign Sleep Myoclonus
Recurrent
(Epilepsy)
Seizure
Symptomatic
Electrolytes
Trauma
Ingestion
Case 1
QuickTime™ and a
Sorenson Video 3 decompressor
are needed to see this picture.
QuickTime™ and a
Sorenson Video 3 decompressor
are needed to see this picture.
Seizure Imitators
• Benign Neonatal Sleep Myoclonus
• Myoclonic jerks are focal, multifocal,
unilateral or bilateral
• 1-5 hz, distal>proximal
• Begins in first weeks, diminishes by 2nd
month, generally gone by 6 months
• Episodes may be exacerbated by benzos
Seizure Imitators
Breath Holding Spells
• Incidence: 4.6% (population study,
N=4980)
• Onset: 6-18 months
• 90% resolve by age 6y
• cyanotic and pallid
CYANOTIC BREATHHOLDING SPELLS
• 60 % are cyanotic
• stimulus triggered (anger, frustration)
• short cry
• breathing interrupted in expiration
• cyanotic, limp, LOC
• +/- sleep
COMPLICATED
BREATH-HOLDING
SPELLS
• Breath-holding spells + seizure-like
activity
• usually more prolonged
• 15% have complicated features
• clonic activity follows LOC
• stiffening
Seizures:
What information is useful?
• What was the patient doing when it started?
Unresponsive?...are you sure? Asleep or
awake?
• Tell us exactly what you saw:
• E.R.B.S.A.O?
• Does it make anatomical sense? Same side,
both sides, just arms, etc.
• How long did it last?
Clinical Characteristics of
Seizures in Neonates (Scher, et al
1989)
•No accepted classification for neonatal seizures
•80 neonates with suspicious movements. Only 8
had electrographic seizures.
•Focal/multifocal clonic: 44% epileptic
•“Subtle seizures”-roving eye movements, arrest of
behavior, lip smacking, autonomic-30%
•Tonic(focal or generalized) 8%
•Myoclonic 7%
Clinical Characteristics of
Seizures in Neonates (Scher, et al.
1993)
•92 neonates with electrographic seizures (345
EEG recordings)
•48% had electroclinical event
•Subtle 71%, clonic 41%, myoclonic 20%, tonic
9%
•34% with only electrographic events
•17/90 (19%) of paralyzed neonates had
electrographic events
Clinical Seizures in
Neonates
•Gen. Tonic Clonic seizures don’t happen in
neonates.
•69 infants, 101 seizures, only 4 resembled
GTCS, none truly were. (Nordli, et al.)
•Duration- average duration 2.25 minutes.
Usually shorter, rarely longer. Intertictal recovery
8 minutes (Clancy and Legido, 1987)
•Status Epilepticus- clinical SE is rare, electrographic
may not be
•487 seizures, only 2 SE (Clancy, et al)
•33% FT infants, 9% PT (Scher, et al)
Seizure Semiology of
Neonates
Focal/Multifocal
Tremors
Subtle
Tonic Clonic
QuickTime™ and a
QuickTime™
Sorenson Video 3 decompressor
Sorenson
picture.
are needed
needed to see this picture.
are
Seizure Types
Partial
Simple Partial
Complex Partial
Partial Secondarily
Generalized
Generalized
Simple partial
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
•Preserved consciousness
•Isolated motor/sensory
•Complex partial involves loss of consciousness
Partial Secondarily
Generalized
QuickTime™ and a
YUV420 codec decompressor
are needed to see this picture.
•Starts partial, rapidly spreads
•You have to ask the questions to get the answers
Partial Seizure Clues
• Contralateral
• Head Deviation, Eye Deviation, Dystonic
Posturing, Unilateral Clonic Activity,
Postictal Paralysis
• Ipsilateral
• Automatisms, Eye Blinking, Nose Wiping
Head and Eye
Deviation
Differentiating Seizure
Types - Semiology
Partial Seizures
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
What do you see?
1. Head Deviation
2. Automatism
3. Eye Deviation
4. Unilateral
Dystonic/Clonic
Activity
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
Seizure Types
Partial
Generalized
Simple Partial
Complex Partial
Partial Secondarily
Generalized
Generalized Tonic Clonic
Tonic
Clonic
Atonic
Myoclonic
Absence
Generalized Seizure
Myoclonic
• Characterized by quick, arrhythmic, and
symmetric/asymmetric movements
•Often not reported by patients.
•Ask about sudden falls or dropping
objects
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
See the Difference?
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
Myoclonic-fast,
jerking motion
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
Clonic-rhythmic
Semiology
Typical Absence Seizures
•Characterized by brief, abrupt impairment of
consciousness associated with EEG
demonstrating 3 Hz spike and slow wave
complexes with normal interictal background
•May also demonstrate:
•mild clonic, tonic, or atonic components
Panayiotopoulos “The Epilepsies:
•automatisms
Seizures, Syndromes, and
Manangement
•autonomic components
Generalized Seizure
Semiology
Infantile Spasms
QuickTime™ and a
DV/DVCPRO - NTSC decompressor
are needed to see this picture.
Review So Far
• Common seizure imitators in pediatrics
• Seizures come in two basic types. You have
to ask the right questions to distinguish them
• Now...how do you diagnose epilepsy (i.e.
when is EEG/MRI necessary) and why do we
care?
Epilepsy Types
Partial
Generalized
Cryptogenic
Idiopathic
Benign Rolandic
Epilepsy
Benign Occipital
Epilepsy
Cryptogenic
Symptomatic
Idiopathic
Symptomatic
Childhood Absence
Juvenile Absence
Juvenile Myoclonic
Grand Mal Upon
Awakening
West Syndrome
Lennox-Gastaut
•Primary = Idiopathic = presumed genetic
•Secondary = Symptomatic=underlying cerebral cause (i.e. injury, dyplasia, etc.)
Idiopathic Partial Epilepsy
Benign Rolandic Epilepsy
(Benign Childhood Epilepsy with Centro-Temporal Spikes)
•Onset 1-14 years, 75% between 7-10 years of age
•Prevalence is 15% of children with seizures
•Characterized by infrequent, often single, focal seizures consisting of
unilateral facial sensorimotor symptoms, oropharyngolaryngeal
manifestations, speech arrest, or hypersalivation lasting 1-2 minutes
•1/3 -2/3 will have secondarily generalized seizures
•75% are nocturnal
•MRI normal
•Typical EEG
Benign Rolandic Epilepsy
Prognosis/Treatment
•2-3% school age children have CT spikes
with <10% having BRE
•Remission usually within 2-4 years from
onset and before the age of 16 years
•Less than 2% will develop infrequent
generalized seizures in adulthood
•Treat or not to treat
Benign Occipital Epilepsy
Gastaut Type
•Onset 3-15 years
•Manifest as visual hallucinations,
blindness, or both-lasting seconds to <3
minutes
•Rarely terminate with hemiconvulsions
or generalized convulsion
•50% have postictal headache
•Similar manifestation to seizures from
occipital lesions - MRI needed
•Typical EEG
Benign Occipital Epilepsy
Fixation-Off EEG
Benign Occipital Epilepsy
Prognosis/Treatment
•Remission occurs 2-4 years from
onset for 50-60% of patients
•Dramatic response to carbamazepine
in >90%
•15% association with celiac disease
Symptomatic Partial
Epilepsy
• Abnormal MRI (stroke, dyplasia, etc.) or
abnormal EEG without classic pattern
• History not consistent with primary partial
epilepsy
• Prognosis varies
Secondary Partial Epilepsy MRI
Heterotopia
Mesial Temporal Sclerosis
Idiopathic Generalized
Epilepsy
Childhood Absence Epilepsy
•Onset 2-10 years, peak 5-6 years
•2/3 are females
•Abrupt cessation of activity or speech last
4-20 seconds followed by return to baseline
•Normal MRI
•Typical EEG with 3Hz SW often provoked
with HV
Idiopathic Generalized
Epilepsy
Childhood Absence Epilepsy
Idiopathic Generalized
Epilepsy
Childhood Absence Epilepsy
Prognosis/Treatment
•Remission
often occurs before 12 years of
age
•Less than 10% develop infrequent
generalized tonic clonic seizures in
adolescence or adult life
•Rarely will patients continue to have
absence seizures as adults
•Treatment with valproic acid, ethosuximide,
or lamotrigine will control absences in >80%
•Possible role for topiramate and
levetiracetam
Idiopathic Generalized
Epilepsy
Juvenile Absence Epilepsy
•Age of onset 9-13 years
•80% suffer from GTCS and 15-25% have
Myoclonic seizures with onset 1-10 years
after absences
•Frequent/severe absences
•Absence status in 20%
•Prognosis: 70-80% will be controlled,
though this is a lifelong disorder
•20% may have intractable absences and
GTCS
Idiopathic Generalized
Epilepsy
Juvenile Myoclonic Epilepsy
•Characterized by myoclonic jerks upon
awakening starting in adolescence
•GTCS (>90%) may begin a few months later,
occasionally earlier
•Absence seizures (33%), if present, begin
between 5-16 years
•M:F equal
•Seizure precipitants: Sleep deprivation,
alcohol, stress, video games
•EEG:
Idiopathic Generalized
Epilepsy
Juvenile
Myoclonic
Epilepsy
irregular generalized 3-6hz spike/polyspike-slow wave
discharges and generalized fragments. 33% have
photoparoxysmal responses
Epilepsy
Juvenile Myoclonic Epilepsy
Treatment/Prognosis
•Valproic Acid, levetiracetam most
commonly used monotherapy treatment
•Lamotrigine, clonazepam
•Prognosis: Seizures well controlled in up to
90% of patients. Treatment is lifelong, as
80% relapse after drug withdrawal
•Carbamazepine, oxcarbazepine, phenytoin,
gabapentin, tiagabine, and vigabatrin are
contraindicated
•Lifestyle management with regards to
alcohol use, sleep deprivation, etc.
Symptomatic Generalized
Epilepsy
Infantile Spasms
West Syndrome
“...these bobbings...they come on whether sitting or
lying; just before they come on he is all alive and in
motion...and then all of a sudden down goes his head
and upwards his knees; he then appears frightened
and screams out. --W.J. West (1841)
Symptomatic Generalized
Epilepsy
Infantile Spasms
West
Syndrome
•Onset between 3-12 months, peak at 5
months
•Incidence: 3-5/10,000
•Spasms are flexor, extensor, or combined
•Clusters with 20-150 seizures per day,
occurring most often on awakening or prior to
sleep
•Developmental delay preceeds spasms in
2/3
•Classified as symptomatic, probably
symptomatic, and cryptogenic
Symptomatic Generalized
Epilepsy
Infantile Spasms
West
Syndrome
•80% symptomatic with most caused by pre-,
peri-, or post-natal insults (i.e. HIE, ICH,
dysplasias, trauma)
•50% of patients with TS have spasms
•3% of patients with Trisomy 21
•Aicardi’s syndrome (spasms, agenesis of
the corpus callosum, and retinal lacunes
•EEG demonstrates hypsarrhythmia
•High voltage, chaotic, arrhythmic and asynchronous
which becomes more synchronous in NREM sleep
•Multifocal independent spike wave discharges
•Periods of electrodecrement
Symptomatic Generalized
Epilepsy
Infantile Spasms
West Syndrome
Symptomatic Generalized
Epilepsy
Infantile Spasms
Prognosis
•Spasms typically will remit, even without
treatment, by 18 months of age
•60% of patients develop other seizure
types, CPS and Lennox-Gastaut syndrome
are most common
•90% of patients have developmental
delay, 66% are severely cognitively
impaired
Symptomatic Generalized
Epilepsy
Infantile Spasms
•ACTH - 50% remission,
all or none. No
Treatment
proven dosing regimen, no clear reason
why it works
•Topiramate - similar efficacy usually in
high doses (25-30mg/kg/d)
•Vigabatrin - especially useful in TS
(90%), beware of irreversible visual field
defects
•Pyridoxine, valproate, zonisamide,
levetiracetam, lamotrigine, felbatol, keto
diet
•Surgery
Symptomatic Generalized
Epilepsy
Lennox-Gastaut Syndrome
•Three criteria
•Multiple intractable seizures including
tonic (80-100%), atypical absence (66%),
and atonic (50%)
•cognitive and behavioral abnormalities
•Slow (<2.5 Hz) generalized spike wave
•Onset 1-7 years, peak 3-5
•10-30% develop from West syndrome or
other epileptic encephalopathies
Symptomatic Generalized
Epilepsy
Lennox-Gastaut Syndrome
Symptomatic Generalized
Epilepsy
Lennox-Gastaut Syndrome
Prognosis/Treatment
•5% die, 80-90% have seizures as adults, and
approximately 90% have severely impaired
cognition and behavior
•Treatment includes almost every AED with
polypharmacy common.
•Ketogenic diet, VNS, corpus callosotomy
Choosing an AED
• Type of epilepsy
Treatment of Epilepsy:
AEDs
Partial
Phenytoin
Phenobarbital
Valproic Acid
Carbamazepine
Oxcarbazepine
Gabatril
Gabapentin
Topiramate
Lamotrigine
Zonisamide
Levetiracetam
Generalized
Valproic Acid
Topiramate
Zonisamide
Lamotrigine
Levetiracetam
Ethosuximide
Felbamate
Choosing an AED
• Type of epilepsy
• Type of formulation (IV, capsule, sprinkle,
etc.)
Choosing an AED
Formulation
• IV: Benzos, phenytoin, phenobarbital, valproic
acid, levetiracetam
•
Sprinkles: valproate, topiramate
•
Liquids: carbazepine, oxcarb, levetiracetam,
valproate, dilantin.
zonegran,lamictal,topiramate will dissolve in
H20
•
Extended release: valproate, carbamazepine
Choosing an AED
• Type of epilepsy
• Type of formulation (IV, capsule, sprinkle,
etc.)
• Time to onset
Choosing an AED
Time To Onset
• Rapid onset: Any IV form
• Onset in 24 hours: Levetiracetam
• Onset in Days: carbamazepine, oxcarb,
dilantin, valproate, zarontin.
• Slow titration: Topiramate, zonisamide
• Really slow: Lamictal
Choosing an AED
• Type of epilepsy
• Type of formulation (IV, capsule, sprinkle,
etc.)
• Time to onset
• Side Effects
Choosing An AED
Side Effects
Somnolence
•All
Rash
Renal Stones
•Topiramate
•Zonisamide
Hyponatremia
•Carbamazepine
•Oxcarbazepine
Cognitive
•Phenobarb
•Topiramate
Parasthesia
•Topiramate
•Zonisamide
Behavior
•Levetiracetam
Labs draws
•Carbamazepine
•Valproic Acid
•Phenytoin
Levels
•All
•Lamictal
•Phenytoin
•Phenobarb
Choosing an AED
• Type of epilepsy
• Type of formulation (IV, capsule, sprinkle,
etc.)
• Time to onset
• Side Effects
• Dosing Schedule
Choosing An AED
Dosing Schedule
• QD: Depakote ER, Zonisamide
• TID: Depakene, Neurontin, Tegretol,
Phenytoin (neonates)
• BID: Everything else
Febrile Seizure
•
3 types (simple, complex, status)
•
NIH consensus: Febrile seizure is an event in infancy or childhood, usually 3m-5 years,
associated with fever but without evidence of intracranial infection or defined cause.
Seizures with fever in children who have suffered a previous nonfebrile seizure are
excluded.
•
incidence- 4%: absolute risk increased with family
hx (1 relative 10%, 2-32%), daycare (7%), dev delay
(10%)
•
Risk of recurrence: 1 in 24
•
risk of future epilepsy: 2-10%
•
workup - MRI/EEG does not predict recurrence
• treatment
Practice Parameter
Febrile Seizures
•
Current Recommendations AAP [Pediatrics 97(5), May 1996, 769-71.]
•
Age 6-12 months with febrile seizure should strongly consider LP
•
Age 12-18 months should consider
• >18 months may use physical exam, associated symptoms to drive need
•
Based recommendations on 4 studies reporting 13-15% of children will
present with seizures as the initial manifestation of seizures with 30-35%
having no meningeal signs.
•
More recent reviews have suggested the presence of meningitis in the
absence of associated signs is rare (1/200), with a large percentage of such
patients with normal CSF at presentation. The introduction of the H.Flu
vaccine has significantly altered the epidemiology of infantile bacterial
meningitis making present treatment different from that 30 years ago
(which the AAP based their recommendations).
•
Most physicians would agree that LP in children outside the range of febrile
convulsions is necessary, as well as children within the range with sign or
symptoms of CNS infection, such as nuchal rigidity, altered mental status,
Practice Parameters
First Unprovoked Seizure
•
Laboratory investigations (CBC, CMP, tox screens) should be
considered based on historic and clinical findings
•
LP is of limited value in first unprovoked afebrile seizure
•
EEG is recommended to dx epilepsy syndromes and provide
for prognosis
•
MRI is preferred modality and should be considered in
children with cognitive/motor impairment that is unexplained,
focal onset seizures, or in children < 1y.
•
Emergenat imaging should be performed in children with
prolonged todd’s, or prolonged (several hours) postictal state.
•
Treatment: 46% have recurrence in 10years, 19% > 4
seizures, and 10%>10 seizures
FAQ (the ED)
• I have a 14 month old with a febrile seizure
and a “raging otitis,” do I need to do a LP?
• (3a.m.) Hey, how are you? I have a kid here
with known epilepsy that had a breakthrough
seizure (like he does once every 3 months or
so), do you want to increase his medicine?
• Do I need to CT this kid?
• I have a patient of Dr. Flamini’s here, how do
you want to treat him?
FAQ (the parents)
• Why does my child have seizures?
• Will my child be stupid?
• How long does my child need treatment?
• What do I do when my child has a seizure?
Case
• 16 year old female with first unprovoked
seizure, described as generalized tonic
clonic
•Focal signs at onset?
•Myoclonic or absence type episodes?
•Time of day?
•Previous workup?
•Treatment choices: Depakote, Keppra, Trileptal
•How long will she need medicine?
Case
• 8 y/o with frequent episodes of staring, at
times associated with lip smacking
•Focal signs at onset? Can they be stopped?
•Myoclonic or GTC episodes?
•Duration?
•Time of day?
•Previous workup?
•Treatment choices: Depakote, Keppra, Trileptal