Ch 22 Epilepsy and Seizure Disorders Flashcards
(152 cards)
1
Q
definition of seizure
A
transient occurrence of signs and sx due to abnormal excessive or synchronous neuronal activity in the brain
2
Q
what is ictal semiology
A
clinical manifestation, behaviors exhibited during seizure
3
Q
seizures are classified based on
A
observed behavior during the event
behavioral features at the onset is KEY to classification
4
Q
what are the 3 classifications of seizure onset?
A
focal onset
generalized onset
unknown onset
5
Q
focal seizure
A
onset originating in networks limited within one cerebral hemisphere
may be discretely localized within the hemisphere
widely distributed within the hemisphere or
originate in subcortical structure
6
Q
seizures can be caused by acquired and congenital conditions
A
true
7
Q
All conditions that cause seizure can lead to epilepsy, t or false
A
false, not all conditions may lead to epilepsy. some can be treated or resolved without recurrent seizures
8
Q
epilepsy
A
recurrent seizures
implies underlying condition that is not related to transient factors and is an intrinsic property of the brain where there is enduring predisposition to generate epileptic seizures
9
Q
diagnosis of epilepsy is made when there are
A
at least 2 unprovoked seizures
at least 24 hours apart
after a single unprovoked seizure when the risk for another is known to be high (>60%) based on other medical factors
epilepsy syndrome diagnosed in childhood
10
Q
etiology of epilepsy
A
structural genetic infectious metabolic immune unknown
11
Q
seizure types
A
focal
generalized
unknown
12
Q
epilepsy types
A
focal
generalized
combined generalized and focal
unknown
13
Q
what is the mechanism of epilepsy?
A
fundamental disruption of the balance between inhibitory and excitatory neuronal activity
development of recurrent excitatory networks
disruption can be caused by acquired or congenital genetic conditions affecting the brain
14
Q
epilepsy is a lifespan or acute disorder?
A
lifespan, may persist throughout lifespan
15
Q
most common site of pathology in adults and adolescents with seizures?
A
temporal lobe (hippocampus)
16
Q
seizures in temporal lobe would show
A
alteration of awareness = 2/3 of all cases
17
Q
most common pathology in adults
A
hippocampal sclerosis (HS) 65-75% of surgical cases
18
Q
what is hippocampal sclerosis
A
histopathologically defined pattern of cell loss and astrogliosis in the hippocampal formation
commonly seen in temporal lobe epilepsy
can also be seen in dementia, hypoxic injury
19
Q
astrogliosis
A
abnormal increase in the number of astrocytes due to the destruction of nearby neurons from central nervous system (CNS) trauma, infection, ischemia, stroke, autoimmune responses or neurodegenerative disease.
20
Q
Hippocampal sclerosis has how many subtypes?
A
3 subtypes
Type 1 - 60-80%: prominent cell loss in hippocampal subfields
Type 2, 3 - less common: cell loss more limited to CA1 and CA 4
21
Q
how is hippocampal sclerosis detected?
A
neuroimaging
appearance of atrophy
increased intensity on T2 and FLAIR
22
Q
new onset epilepsy in adults - common or uncommon
A
uncommon
usually asociated with primary CNS neoplasm, neurovascular event, trauma, infection, autoimmune disorders
23
Q
autoimmune mediated epilepsy may be related to
A
paraneoplastic syndrome
infectious etiology
24
Q
underlying pathology in pediatric epilepsy is dependent on?
A
age
more variable
more unknown
25
neonatal seizures are usually associated with
hypoxic ischemic encephalopathy 35-45% of cases
infarction/hemorrhage
genetic or syndromic epilepsy
brain malformation
26
childhood onset epilepsy variable or stable?
variable
27
cause of childhood onset epilepsy?
genetic variants
autoimmune epilepsy
malformation of cortical development (MCD)
low grade brain tumors
28
Malformation of cortical development (MCD) is a frequent cause of
epilepsy
| MCD can present in anywhere in the cerebral cortex
29
MCD vary in what ways?
origin
| presentation can be macro or microscopic
30
MCD classification is based on
genetic
embryologic
pathologic, histologic, imaging criteria
31
4 classifications of MCD
MCD caused by abnormal neuronal and glial proliferation or apoptosis
MCD caused by abnormal neuronal migration
MCD caused by abnormal cortical organization
MCD not otherwise classified
32
neonatal period and epileptic syndromes
neonatal seizures
familial neonatal epilepsy
ohtahara syndrome
early myoclonic encephalopathy
33
infancy and epileptic syndromes
```
febrile seizures
self limited infantile epilepsy
self limited familial infantile epilepsy
West syndrome
Dravet syndrome
Myoclonic epilepsy of infancy
Myoclonic encephalopathy in non progressive disorders
Epilepsy of infancy with migrating focal seizures
```
34
Childhood and epileptic syndromes
febrile seizures
early onset childhood occipital epilepsy
epilepsy with myoclonic atonic seizure
childhood absence epilepsy
self limited epilepsy with centrotemporal epilepsy
late onset childhood occipital epilepsy
autosomal dominant nocturnal frontal lobe epilepsy
epilepsy with myoclonic absences
lennox gastaut syndrome
epileptic encephalopathy with continuous spike and wave during sleeo
landau-kleffner syndrome
35
Adolescence/Adult and epileptic syndrome
juvenile absence epilepsy
juvenile myoclonic epilepsy
epilepsy with generalized tonic clonic seizures
autosomal dominant epilepsy with auditory features
other familial temporal lobe epilepsy
36
Variable at age of onset
familial focal epilepsy with variable foci
new onset refractory status epilepticus
febrile illness related epilepsy syndrome
progressive myoclonus epilepsy
reflex epilepsy
37
Distinctive constellations/surgical syndrome
mesial temporal lobe epilepsy with hippocampal sclerosis
rasmussen syndrome
gelastic seizure with hypothalamic hamartoma
hemiconvulsive hemiplegic epilepsy
38
epilepsy attributed to structural metabolic causes
MCD
neurocutaneous syndromes (tuberous sclerosis complex, Sturge weber)
tumor, infection, trauma, vascular, perinatal insults
39
Genes associated with epilepsy can be grouped into
monogenic
neurodevelopment-related genes
epilepsy related genes
potential epilepsy associated genes
40
of people worldwide have epilepsy
50 million people
41
majority of epilepsy occurs in what regions? developing or non developing
developing (possible 2/2 malaria, birth related injuries, lack of access to care)
42
USA incidence
50/100,000 individuals
43
prevalence of epilepsy
5-10/1000
| more than 3 million
44
most common onset at what age?
younger than 5 years or over 65 years
45
majority of seizures with alteration of consciousness or awareness occur before
age 15
46
which population has the fastest growing number of new onset cases?
elderly population
47
causes for mortality of epilepsy
```
neurological disease
neurovascular disease or neoplasm
SUDEP (suddenly unexplained)
unintentional injury
suicide
```
48
mortality rate increase in epilepsy for cases without a known cause OR in cases with symptomatic epilepsy
cases with symptomatic epilepsy
49
mortality risk factors higher for male or female?
same
50
mortality risk factors highest in which age group
younger
51
severity of epilepsy increases with
seizure recurrence
| frequency
52
known risk factors for seizure recurrence
prolonged febrile convulsions that leads to
- neurological injury
- neoplasm
- disorders of neuroblast migration
- TBI
53
cognitive deficits related to seizures are determined by
localized brain regions associated with specific functions (e.g. memory in temporal lobe epilepsy)
54
generalized cognitive deficits (e.g. attention, psychomotor speed) associated with
seizures themselves
| treatment of seizures with AED
55
which factor is the strongest determinant for cognitive impairment in epilepsy?
seizure frequency!
56
other determinants of cognitive impairment in epilepsy?
tumor, CNS infection, trauma
younger age of onset
no of AEDs
57
genetic epilepsy more associated with what kind of developmental disorders?
ASD
| ID
58
temporal lobe epilepsy with hippocampal sclerosis is associated with a history of
infantile seizures as an infant or toddler
59
what are febrile seizures?
children aged 3 months to 6 years
who may have tonic-clonic seizures when they have a high fever
good outcomes
60
when there are recurrent episodes of febrile seizures, it presents a greater risk for
future development of temporal lobe epilepsy
61
increased risk for hippocampal sclerosis
history of perinatal complications
hypoxic ischemic injury
CNS infections
limbic encephalitis
62
treatment of TLE
very difficult to treat - 1/3 of TLE patients develop medically intractable seizures
anticonvulsant medications
surgery (temporal lobectomy - remove epileptogenic temporal lobe and mesial temporal structures)
thermal laser ablation
neurostimulation system (neurospace)
63
Behaviors associated with TLE
auras (mostly with GI symptoms, psychological phenomenon)
disorganized behaviors
repetitive movements of hands, tongue, mouth, lips, usually ipsilateral to size of seizure onset
post ictal confusion/fatigue (min to hours)
secondary generalization to tonic clonic seizure 50% of the time
64
seizure onset of TLE characteristics
alteration of awareness
gradual alteration of awareness
may not have LOC
65
Speech patterns and TLE
if pt can continue to speak clearly is associated with non language dominant temporal lobe onset in most cases
if patient is aphasic or has dysnomic speech after TLE, it's usually lateralizing, meaning, it's associated with language dominant TLE
66
surgeries of TLE
standard temporal lobectomy
modified anterior temporal lobectomy
selective amygdalahippocampectomy
67
risks of TLE surgery
cognitive morbidity
- decreased efficiency in learning and memory
- word finding
- visual field deficits
68
newer TLE treatment
thermal laser ablation applied to mesial temporal lobe structures
69
childhood absence epilepsy
primary generalized epilepsy
widespread neural networks at seizure onset
self limiting, does not persist past adolescence
70
Childhood absence epilepsy accounts for % of childhood seizures?
15%
71
which is the most common epilepsy syndromes of childhood?
Childhood absence epilepsy
72
imaging findings of Childhood absence epilepsy?
typically negative
73
age of onset of Childhood absence epilepsy
3- 8 years
| peak at 6 years
74
neurological implications of Childhood absence epilepsy ?
neurologically normal usually
| comorbid learning and attention problems
75
Childhood absence epilepsy EEG characterized by ? Hz?
3 Hz spike wave discharges
lasts 5-10 seconds
as short as 3, or as long as 20 seconds
76
untreated Childhood absence epilepsy?
very frequent seizures on a daily basis
77
behaviors of Childhood absence epilepsy
sudden behavior arrest
alteration of awareness (STARING)
minor motor automatisms (eyelid fluttering, lip movements)
begin and end suddenly
usually pt unaware
briefly disoriented and need prompting to resume activity
78
Medical tx of Childhood absence epilepsy failure rate
variably effective, but failure rate 47%
79
common meds for Childhood absence epilepsy
lamotrigine
valporic acid
ethosucimide
80
best meds for seizure relief in Childhood absence epilepsy?
Zarontin/ethosuximide
81
Is Childhood absence epilepsy benign or malicious?
benign
82
when do children with Childhood absence epilepsy have remission?
during adolescence most cases
| 15% persist into adolescence, adulthood, develop into juvenile myoclonic epilepsy
83
other epilepsy syndromes
Landau Kleffner syndrome
Lennox Gastaut syndrome
Rasmussen syndrome
(Left-right-left)
84
Landau Kleffner syndrome LKS
progressive encephalopathy
AFFECTS LANGUAGE
normal language prior to onset of seizures
85
onset of Landau Kleffener
ages 3- 7 years
86
deficits in Landau kleffner
progressive aphasia:
- development of receptive language impairment
- verbal auditory agnosia
- then gradual development of expressive language deficits
```
general cognitive impairment
regression of IQ
deficits in attention
sociability
autistic behaviors
```
87
cause of landau kleffner syndrome
no known cause
| 20% of cases show variation in GRIN2A
88
localization of Landau kleffner syndrome
language dominant perisylvian region or bilateral
| frontal lobe pathology
89
recovery and treatment of landau kleffner syndrome
no sponatneous recovery of language
tx does not help with cognitive impairment
speech and language interventions are critical (or use sign language)
90
Lennox Gastaut syndrome (LGS)
encephalopathic generalized epilepsy syndrome
| progressive and severe cognitive impairment
91
possible cause of lennox gastaut syndrome
possibly genetic
92
clinical presentation of lennox gastaut syndrome
multiple seizure types
- atypical absence seizure
- tonic seizure
- atonic seizure
93
Risk factor for lennox gastaut syndrome
```
history of infantile spasm
MCS
neurocutaneous disorder (e.g. tuberous sclerosis complex)
CNS infection (meningitis, encephalitis)
hypoxic ischemic injury
no single underlying pathology
```
94
how is lennox gastaut syndrome characterized?
using EEG pattern
- bursts of fast activity (During slow wave sleep)
- background EEG is usually slow activity and disorganized
cognitive impairment and behavior impairment
95
Rasmussen syndrome is characterized by
progressive unilateral encephalopathy
| medically refractory seizures
96
age and presentation of rasmussen syndrome
children in 3-14 years
premorbid cognitive and behaviors NORMAL
after seizures begin:
- loss of cognitive skills related to the side of seizure onset
- ultimately complete loss of function in the affected hemisphere (HEMIPARESIS!)
97
cause of rasmussen syndrome
autoimmune basis
| no specific marker
98
treatment for rasmussen syndrome
immunotherapy
| BEST is surgical intervention (hemispherectomy)
99
NP results IQ in epilepsy
low IQ
| IQ is a proxy for outcome, disease severity, extent of pathology
100
Risk factor for low IQ in epilepsy
age of seizure onset
earlier onset - lower IQ
no progressive decline in IQ in recurrent seizures
101
risk factor for progressive decline in IQ
seizure severity
treatment with multiple meds
comorbid LD
102
epilepsy syndromes that have effect on IQ
MCD - severe cog impairment and very low IQ
| encephalopathic generalized epilepsy (Lennox Gastaut and landau kleffner) has extremely low IQ
103
Attention and epilepsy
30-50% have difficulties with attention
ADHD-I more common than other types
ADHD-C related to severity of epilepsy, early onset
affect boys and girls the same way
104
which type of seizure more associated with attention problems?
absence seizure
- impaired attention network comprising anterior insulafrontal operculum and medial frontal cortex
nocturnal seizure
- affect attention b/c of sleep patterns
localization-related partial epilepsy
- interictal discharges/transient cognitive impairment
105
how does attention problems in epilepsy differ from adhd?
epilepsy kids - more sustained attention difficulties
| ADHD kids - more complex and divided attention problems
106
treatment for attention problems in epilepsy
- AED can produce attention problems as side effect
- not often use stimulants to treat ADHD because of possible increase in seizure in some cases
- most research suggest AED does NOT lead to more seizure
107
comorbid epilepsy and ADHD brain structures
```
Decreased gray matter volume in
- sensorimotor
- supplmental motor
- prefrontal
decreased brainstem volumes
frontal cortical and subcortical systems regulating attention
```
108
processing speed in epilepsy changes due to...
1. side effects of AED
2. structural or neuroanatomical basis
- decrease in white matter volume
- frontal lobe epilepsy
- interictal epileptiform discharges
109
how does AED work on seizures?
- reduce hyperexcitabilty and neuronal transmission by increasing inhibitory action (of GABA)
- reduce availability and function of excitatory NT (e.g. glutamate)
110
language in epilepsy
common deficit
dependent on onset of seizure
- if onset is during critical language period, it can disrupt important networks involved in language
111
language deficits in epilepsy can be associated with some types of epilepsy
frontal and TLE involving dominant hemisphere
| - TLE have problems with word finding and semantic knowledge
112
Visuospatial is associated with which types of epilepsy?
associated with:
- primary generalized
- generalized nonconvulsive epilepsy
- localization-related epilepsy (non dominant)
113
Visuospatial and epilepsy deficits in?
```
impaired object recognition
spatial organization
visuomotor integration
visuospatial learning
complex perception
```
114
crowding effect
early onset lesions can lead to reorgnization of language from L to R or become bilateral
preferential neural resources allocated for language development
shifting resources to back up systems in homologous area in contralateral hemisphere
115
learning and memory and TLE
lateralized seizure onset
- one temporal lobe is more extensively involved in seizure onset and propogation
more verbal for dominant TLE
nonverbal for nondominant TLE
116
type 1 hippocampal sclerosis and memory
impaired (esp list learning)
117
memory outcome following temporal lobectomy is predicted by
baseline level of hippocampal function
- WADA testing, memory test performance
low baseline -> decreased risk of exacerbating existing memory impairment
high baseline -> increased risk of decline in memory
118
Intracarotid amobarbital hemispheric suppression =
WADA testing
119
gold standard for predicting catastrophic memory loss
WADA testing
120
Wada testing is useful for
predicting memory loss
| predicting common declines associated with TL surgery by analyzing functional adequacy and functional reserve
121
TLE memory declines can also be predicted by
fMRI findings of increased activation ipsilateral of seizure focus during memory testing
122
memory problems in other epilepsy syndrome besides TLE
CAE - attention problems affecting encoding and sotrage, more VS memory problems
frontal lobe epilepsy - problems with poor organization and strategies for learning and memory
123
predictors of more impaired executive function in epilepsy include
early onset
longer duration of seizures
involvement of frontal subcortical systems
124
frontal lobe epilepsy and EF
```
deficits in planning
mental flexibility
impulse control
motor coordination
less lateralized deficits in FLE
```
125
Frontal lobe epilepsy characteristics and EF
spread rapidly
engage bilateral malformation of cortical development (MCD)
more widespread abnormalities
126
TLE and EF
variability in cortical morphometric variations in TLE
extratemporal structures
- frontal and subcortical regions
127
Sensorimotor functions and Epilepsy
motor skills deficit (hemiparesis - Rasmussen syndrome)
| if epilepsy involves frontal and parietal sensorimotor cortex
128
social cognition and epilepsy
ID, ASD
| deficits in social cognition
129
social cognition and epilepsy type
TLE/FLE
- social cognition involves frontal cortex (anterior cingulate, temporal lobes, amygdala)
- other psychosocial factors that affect social cognition and skills
130
emotion and personality and epilepsy
mood d/o
- anxiety, depression (shows differently in diff ages)
- poor QOL
131
treatment of depression in epilepsy
SSRI SNRI
132
PVT SVT
20-25% fail PVT
133
atonic seizure problem?
high risk for falls
134
psychosocial issue with epilepsy
driving restriction
underemployment
reduced independence
135
employment
unemployment and underemployment
- education issues at early age, absences, LD, reduced work choices
- safety reasons
- distance
136
driving in epilepsy
if history of sustained control, can drive
recurrent seizures despite AED, cannot drive
seizure free and discontinuation of AED, cannot drive during drug withdrawal period
137
focal onset seizures has 2 types
aware
| impaired awareness
138
focal onset - aware
person is awake and aware during a seizure
139
focal onset - impaired awareness
person is confused or their awareness is affected in some way during a focal seizure
140
focal motor onset seizures
cause a change in muscle activity (jerking of a finger, stiffening of one part of the body, or weakness in specific muscles)
The movements may spread from one area and involve one side of the body or extend to muscles on both sides of the body.
can affect specific muscles or affect speech
141
examples of focal motor seizures
automatisms - involuntary, automatic, coordinated, and repetitive movements of the person’s hands, feet, vocal chords, lips and face, or other areas.
atonic - sudden loss in muscle tone
clonic -rhythmic jerking of one limb or one side of body
epileptic spasm - sudden flexion or extension of muscles
hyperkinetic -sudden, large, irregular movements by limbs or one side of the body
myoclonic - brief jerks or muscle contractions, usually in single bursts
tonic - brief increase in muscle tone for the duration of the seizure
142
focal non motor onset seizures
changes in any one of the senses.
person remains alert and able to interact
generally last seconds to less than two minutes.
143
examples of focal non motor seizures
autonomic -changes in the part of the nervous system that automatically controls bodily functions (heart rate, sensation)
behavior arrest - decrease in amplitude or outright arrest of ongoing motor activity for the duration of the seizure,
cognitive/emotional - Change how people think, feel, or experience things
sensory - affect any of an individual’s five senses
144
Generalized onset has 2 types of seizures
motor
| nonmotor (absence)
145
Generalized onset motor tonic clonic seizure
grand mal seizure
loses consciousness
muscles stiffen
jerking movements
generally last 1 to 3 minutes
146
convulsive status epilepticus
1 seizure that lasts > 10 minutes
| or 3 seizures without a normal period in between
147
clonic seizures
rapidly alternating contraction and relaxation of a muscle (repeated jerking)
movements cannot be stopped by restraining or repositioning the arms or legs
rare occurence
148
tonic seizures
causes a sudden stiffness or tension in the muscles of the arms, legs or trunk.
stiffness lasts about 20 seconds
usually happen during sleep.
may fall if standing
149
Generalized onset non motor (absence) seizure
“blanking out” or staring into space
usually so brief that they frequently escape notice.
petit mal
150
Generalized non motor seizures has 2 types
atypical
| typical
151
generalized nonmotor typical seizure
a very brief, usually less than 10 second, interruption of ongoing activities.
person staring off into or possibly a brief upward deviation of the eyes
152
generalized nonmotor atypical seizure
change in muscle tone
make some kind of movement in addition to staring into space (e.g. blinking, chewing, or hand gestures).
may last up to 20 seconds.
