Adult and Pediatric Epilepsy and Sleep Flashcards
1
Q
Name Characteristics of PNES
A
-Tendency for episodes to occur when in front of witnesses
-Tendency not to occur during sleep
-Unresponsiveness with motor features mimicking a generalized tonic-clonic seizure or focal seizures with impaired awareness (most common)
-Asynchronous movements, pelvic thrusting, side-to-side head movements or body movements
-Eye closure during episode
-Absent post-ictal confusion
2
Q
PNES is common in what type of patient population?
A
Often seen in patients who have comorbid psychiatric disorders and social stressors.
3
Q
PNES presents to ED for evaluation of episodes, management?
A
Initial reassurance, outpatient follow-up, prolonged EEG monitoring to capture spells.
4
Q
Simple Febrile Seizure (length, frequency, Distribution)
A
Length: less than 15 minutes
Frequency: 1 in a 24 hour period
Distribution: generalized
5
Q
Complex Febrile Seizure (length, frequency, Distribution)
A
Length: equal to or greater than 15 minutes
Frequency: more than 1 in a 24 hour period
Distribution: focal
6
Q
Definition of Febrile Seizure
A
-Seizure with a temperature greater than 38°C
-between ages of 6 months and 5 years
-no signs of a central nervous system infection, electrolyte imbalance, or metabolic cause, and no history of afebrile seizures.
7
Q
What is management of simple febrile seizure?
A
-no further workup, treatment is counseling and education
- if seizure greater than 5 minutes
would treat as status epilepticus with 1st line benzodiazepines
8
Q
When does a febrile seizure warrant further work-up?
A
significant symptomatology (e.g. persistent vomiting), physical findings (e.g. neck stiffness), abnormal neurological exam, or complex febrile seizures would warrant further workup
9
Q
What cytochrome enzyme are oral hormone contraceptives (OCPs) metabolized (inactivated) by in the liver?
A
cytochrome CYP3A4
10
Q
A) What anti-seizure medications are cytochrome CYP3A4 enzyme inducers?
B) how does this effect levels of OCPs?
A
A) Phenytoin, phenobarbital, and carbamazepine
B) reduce the level of circulating hormonal contraceptives
11
Q
List anti-seizure medications that are non-enzyme–inducing, and will not effect levels of circulating hormonal contraceptives
A
-acetazolamide
-brivaracetam, levetiracetam
-cannabidiol
-clobazam
-clonazepam
-ethosuximide
-gabapentin, pregabalin
-lacosamide
- lamotrigine
-sodium valproate
-stiripentol
-tiagabine
-vigabatrin
-zonisamide
12
Q
What is the most frequently encountered and most common form of generalized idiopathic epilepsy?
A
Juvenile myoclonic epilepsy (JME)
13
Q
What is the common age presentation for JME?
A
5 and 16 years old
14
Q
JME syndrome is characterized by what seizure types?
A
myoclonic jerking movements followed by generalized tonic-clonic seizures
15
Q
What are precipitating factors for JME?
A
sleep deprivation, excessive alcohol use, and stressors
16
Q
What is first-line treatment for JME?
A
-Best treated with valproic acid.
-Other options include: levetiracetam, lamotrigine, topiramate, and zonisamide
17
Q
Valproic Acid (mechanism of action)
A
- Increase GABA
- Sodium channel inactivation
18
Q
Valproic Acid (Specific conditions treated)
A
- Generalized tonic-clonic seizures
- Juvenile myoclonic epilepsy
- Partial seizures
- Myoclonic seizures
19
Q
Valproic Acid (Adverse effects)
A
- Weight gain
- Tremor
- Alopecia
- Sedation
- Ataxia
- Hepatotoxic
- Teratogenic
20
Q
Levetiracetam (mechanism of action)
A
- SV2A receptor modulator
21
Q
Levetiracetam (Specific conditions treated)
A
- Focal seizures
- Generalized seizures
22
Q
Levetiracetam (Adverse Effects)
A
- Psychosis
- Somnolence
23
Q
Carbamazepine (mechanism of action)
A
- Sodium channel inactivation
24
Q
Carbamazepine (conditions treated)
A
- Trigeminal neuralgia
- Focal seizures
25
Carbamazepine (adverse effects)
- Hyponatremia
- Agranulocytosis
- Stevens-Johnson syndrome
26
Oxcarbazepine (mechanism of action)
- Sodium channel inactivation
27
Oxcarbazepine (conditions treated)
- Trigeminal neuralgia
- Focal seizures
28
Oxcarbazepine (Adverse Effects)
- Hyponatremia
- Agranulocytosis
- Stevens-Johnson syndrome
29
Lamotrigine (mechanism of action)
- Sodium channel inactivation
30
Lamotrigine (conditions treated)
- Focal seizures
- Generalized seizures
31
Lamotrigine (adverse effects)
- Stevens-Johnson syndrome
- Blurred vision
32
Phenytoin (mechanism of action)
- Sodium channel inactivation
33
Phenytoin (conditions treated)
- Focal seizures
- Generalized seizures
34
Phenytoin (adverse effects)
- Gingival hyperplasia
- Stevens-Johnson syndrome
- Hirsutism
- Fetal hydantoin syndrome
35
Topiramate (mechanism of action)
- Blocks voltage-gated sodium channels
36
Topiramate (conditions treated)
- Focal seizures
- Generalized seizures
37
Topirmate (adverse effects)
- Glaucoma
- Nephrolithiasis
- Paresthesias
38
Ethosuximide (mechanism of action)
T-type calcium channel inhibition
39
Ethosuximide (conditions treated)
- Absence epilepsy
40
Ethosuximide (adverse effects)
- Gastrointestinal symptoms
41
Phenobarbital (mechanism of action)
- GABA-A agonist
42
Phenobarbital (conditions treated)
- First-line in neonatal seizures
- All seizure types
43
Phenobarbital (adverse effects)
- Sedation
- Dependence
44
Benzodiazepine (mechanism of action)
- Indirect GABA-A agonist
45
Benzodiazepine (conditions treated)
- First-line for status epilepticus
46
Benzodiazepine (adverse effects)
- Sedation
- Dependence
47
Vigabatrin (mechanism of action)
- Inhibits GABA transaminase
48
Vigabatrin (conditions treated)
- Refractory seizures
49
Vigabatrin (adverse effects)
- Vision loss
50
Zonisamide (mechanism of action)
- Sodium-channel inhibition
51
Zonisamide (conditions treated)
- Generalized epilepsy
- Focal seizures
52
Zonisamide (adverse effects)
- Oligohidrosis
- Sedation
- Lightheadedness
53
What is Burst suppression typically characteristic of?
neuronal injury from an anoxic injury but can be a nonspecific finding in the setting of hypothermia or sedation
54
What is recommended for patients with witnessed cardiac arrest?
goal temperature of 32°C to 36°C for at least 24 hours following arrest. During this monitoring, continuous EEG is recommended to evaluate for possible epileptiform activity.
55
Characterize Myoclonus
brief, shock-like, involuntary movements caused by muscle contractions or inhibitions
56
Myoclonic status epilepticus
A) localization
B) Timing of onset
C) duration of symptoms
D) response to treatment
E) Description of myoclonus
F) Neurophysiologic symptoms
G) Prognosis
A) Cortical (more common, or subcortical)
B) Within 72 hours of cardiac arrest
C) Days to weeks
D) poor
E) -Stimulus-sensitive myoclonus
-Spontaneous myoclonus
F) -Burst-suppression pattern
-Generalized periodic epileptiform discharges time-locked with myogenic artifact
-Alpha coma
G) 90%-100% mortality rate
Poor Note: Early post-anoxic myoclonic status epilepticus is no longer suggestive of a poor prognosis.
57
Lance-Adams Syndrome
A) localization
B) Timing of onset
C) duration of symptoms
D) response to treatment
E) Description of myoclonus
F) Neurophysiologic symptoms
G) Prognosis
A) Either subcortical or cortical
B) Hours to years after cardiac arrest
C) Days to years
D) Variable, usually better
E) -Stimulus-sensitive myoclonus
-Spontaneous myoclonus
-Intention myoclonus
F) Epileptiform activity in one-third of cases
Up to 20% are normal
Diffuse or focal slowing can be seen
G) Unclear but ~50% survival
58
Post-anoxic myoclonus was once thought to indicate poor neurologic prognosis in all cases, but what do studies show now?
multiple variables, including the length of advanced cardiovascular life support, quality of compressions, and other findings should be considered prior to prognostication
59
What Anti-seizure medications (ASMs) to avoid in JME?
-carbamazepine
-phenytoin
-tiagabine
-gabapentin.
60
Why avoid certain ASMs in treating JME?
aggravate myoclonus and increase or precipitate absence seizures in patients with myoclonic epilepsy.
61
Describe classic JME
usually starts in adolescence as isolated awakening myoclonic seizures (described as symmetrical, sudden jerks of hands, forearms, and shoulders causing clumsiness)
62
When do most JME patients present for evaluation?
After having GTC
63
What other seizure type is also common with JME?
Absence seizures can coexist in approximately 40% of cases.
64
JME EEG characteristics
-interictal EEG has 4- to 6-Hz spike or polyspike and slow-wave complexes
- ictal EEG with the myoclonic jerks typically shows 10- to 16-Hz polyspike discharges.
65
Why should Valproic Acid acid be avoided in certain patients?
avoided in females of childbearing age due to teratogenicity risk that is higher than with other antiepileptics (FDA pregnancy category D for seizures)
66
What are the types of focal seizures with retained awareness?
it can be motor or sensory or involve special senses (gustatory, auditory, visual, etc.).
67
Where does a focal nonmotor (or focal sensory) seizure originate in the brain?
originate from the postcentral gyrus, as well as the secondary sensory areas (Sylvian fissure anterior to the precentral gyrus), supplementary sensory area, and insular cortex.
68
a) What is a Jacksonian march and in b)what type of seizures can it occur?
a)spreading of the paresthesias
b) both focal motor and sensory seizures
69
Initial work-up for focal nonmotor (sensory) seizure?
-laboratory tests (electrolyte, glucose, and thyroid-stimulating hormone levels and a drug screen)
-EEG (the results of which may be normal or show interictal discharges)
-MRI of the brain with and without contrast to rule out any structural abnormalities (mass, abscess, cortical dysplasia, encephalomalacia, etc.). Prolonged EEG or video EEG may be needed but are not part of the initial workup.
70
Focal seizures (Frontal Lobe)
-Uncontrolled motions
-Purposeful movements (pointing, grimacing, etc.)
-laughing
-repetitive talking
71
Focal Seizures (parietal lobe)
-Physical sensation (tingling, prickling, etc)
-Sensory disturbances
(hallucinations, dizziness, etc)
72
Focal seizures (occipital lobe)
-Visual irregularities (flashing lights, shimmering lines, visual hallucinations)
73
Focal Seizures (temporal lobe)
-temporary memory loss
-heightened emotional states (anxiet, euphoria, etc)
-Repetitive movements (chewing, pulling clothes, etc)
-Deja vu
74
Describe benign myoclonus of early infancy (benign nonepileptic infantile spasms)
-repetitive jerks of the neck or upper limbs, leading to flexion/rotation of the head, and extension/abduction of the limbs without a change in consciousness, "Shudders"
-clusters and during mealtime
-occur while the child is awake and can be precipitated by excitement or frustration.
75
What is age of onset and disappearance of benign myoclonus of early infancy?
Usually the movements begins at 3-8 months of age, increase in intensity and severity over weeks to months, and then remit spontaneously at about 24 months.
76
Is EEG normal in benign myoclonus of early infancy, is there need for treatment?
EEG and neurologic development are normal. There is no need to treat with an antiseizure medication.
77
What is the triad of epileptic infantile spasms?
spasms, arrest of psychomotor development, and hypsarrhythmia on EEG
78
What is Sandifer syndrome? And treatment?
-paroxysmal spells of generalized stiffening and posturing caused by gastroesophageal reflux in infants
-happens within 30 minutes of a meal. Treatment of GERD disease reduces the frequency and severity of attacks.
79
What is paroxysmal dystonia?
-duration of attacks is usually longer
-sustained contracture of agonist and antagonist muscles
80
Typical cause of paroxysmal dystonia in an infants?
common etiology of dystonia is an acute reaction to a drug, such as metoclopramide or haloperidol.
81
What is Benign neonatal sleep myoclonus or benign sleep myoclonus of infancy?
-benign sleep myoclonus of infancy
-usually presents in the first few weeks of life and resolves by age 2-3 months -movements stop when baby is aroused
-normal EEG & normal findings on neurologic examination
82
What is the FDA approval purpose for Vagal Nerve Stimulator (VNS)?
-add-on therapy for adults and children 4 years and older to treat focal or partial seizures that do not respond after trying at least 2 appropriate antiseizure medications
83
Typical criteria for VNS recommendation?
VNS is typically used if a person is not able to have epilepsy surgery or if surgery does not work.
84
What is the goal of VNS treatment?
-usually to decrease breakthrough seizures by at least 50% and can often be used to decrease the maintenance antiseizure medication dose required for seizure control.
85
Epileptic encephalopathy
Early Infantile Epileptic Encephalopathy
(Ohtahara syndrome)
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A) infancy
B)Structural, genetic
C)Burst Suppresion
D) tonic spasms
86
Epileptic encephalopathy
Early Myoclonic Encephalopathy
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A) Infancy
B) Inborn errors of metabolism (e.g., nonketotic hyperglycinemia)
C) Burst suppresion
D) myoclonic seizures
87
Epileptic encephalopathy
Epilepsy of infancy with migrating focal seizures
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A) infancy
B) Epilepsy of infancy with migrating focal seizures
C) Focal seizures with migratory pattern
D) Focal tonic or clonic seizures
88
Epileptic encephalopathy
Infantile spasms (West syndrome)
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A) 3-12 months
B) Structural, genetic, or metabolic
C) Hypsarrhythmia
D) Spasms
89
Epileptic encephalopathy
Lennox-Gastaut syndrome
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A). Early childhood
B). Structural, genetic, or metabolic
C). Slow spike and wave
D). Multiple seizure types
90
Epileptic encephalopathy
Landau Kleffner Syndrome (presents with a rapidly progressive acquired auditory agnosia)
A)Age of onset
B)Common Etiology
C)EEG
D)Seizure Type
A) Childhood
B) Unknown
C) Electrical status epilepticus in sleep
D) Mild focal seizures
91
What is myoclonic status in nonprogressive encephalopathies (MSNE)? And in what condition is it most often seen?
-rare condition characterized by the early onset (birth to 5 years of age) of absence seizures and myoclonic seizures most frequently seen in Angelman syndrome
92
What is myoclonic status in nonprogressive encephalopathies (MSNE) an example of? Treatment?
-example of epileptic encephalopathy, a group of conditions where ongoing seizure activity leads to developmental delays.
-Benzodiazepines, as well as the ketogenic diet, can be used to treat episodes of myoclonic status, but overall the prognosis is poor.
93
Describe Angelman syndrome
-rare condition caused by the loss of the UBE3A gene on the maternal chromosome
- associated with myoclonic status in nonprogressive encephalopathies (MSNE)
- delayed development, problems with speech and balance, intellectual disability, and, sometimes, seizures. People with Angelman syndrome often smile and laugh frequently, and have happy, excitable personalities
94
Describe Lance Adams
-post hypoxic myoclonus, is seen in patients following cardiorespiratory arrest who develop myoclonus days or weeks after the event. Myoclonus is triggered by intentional actions and has no reliable correlation with EEG.
95
Describe Dravet syndrome, also called severe myoclonic epilepsy of infancy
-usually presents within the first year of life with a prolonged seizure in the setting of fever.
- mutations in the SCN1A gene.
-associated with developmental delays
-recent meds approved for tx: cannabidiol, stiripentol, and fenfluramine
96
Describe Tuberous sclerosis (TS), or Bourneville's disease
mutation in either TSC 1 (chromosome 9) or TSC 2 (chromosome 16).
cortical tubers (causes seizures), subependymal nodules, subependymal giant cell astrocytomas, ash leaf spots, Shagreen patches, and cardiac rhabdomyomas among other findings
97
Describe Prader-Willi syndrome
-loss of the paternal copy of the UBE3A gene on chromosome 15
-delayed development, short stature, and insatiable appetite with resulting obesity during childhood
- not associated with myoclonic status in nonprogressive encephalopathies (MSNE)
98
Myoclonic status in nonprogressive encephalopathies (MSNE) is seen most frequently in which syndrome?
Angelman syndrome
99
What is most common form of reflex epilepsy?
photosensitive epilepsy
100
What is photosensitive epilepsy?
flashing lights trigger seizures
101
What is a term often used to describe photosensitive epilepsy, particularly in childhood?
Sunflower syndrome
102
Typical auras in temporal lobe epilepsy
emotional (fear) aura, déjà vu, or an autonomic feature (epigastric sensation).
103
typical temporal lobe semiology
behavioral arrest, impaired awareness, and automatisms
104
Visual aura involving amaurosis localizes to to which lobe?
occipital
105
ictal piloerection (goose bumps) is reported in which lobe?
temporal lobe
106
Name initial signs of phenytoin toxicity
incoordination
ataxia
horizontal nystagmus
107
What are the pharmacokinetics of phenytoin?
as the serum concentration increases, it reaches a point within the recommended therapeutic range after which the half-life begins increasing. Beyond that point, the phenytoin plasma level increases disproportionately with an increase in the dose-- risk of toxicity
108
What chemotherapeutic agent decreased seizure threshold?
-Busulfans
- recommended that patients being treated with busulfan be prophylactically started on an antiepileptic agent, although, even with appropriate prophylaxis, there is still a risk of seizure
109
Describe lateralized periodic discharges?
-stereotyped, repetitive, sharply contoured discharges
-well-described abnormality on EEG that may be ictal or interictal in nature
110
When do you treat lateralized periodic discharges?
- if they correlate with a clinical event concerning for a seizure, a trial of an antiepileptic is mandatory
111
Describe Epilepsy with myoclonic-atonic seizures (EMAS)
-previously myoclonic-astatic epilepsy or Doose syndrome
- generalized seizures with either a myoclonic or an astatic (drop attack) component
-Age of onset is 1 to 5 years, and most patients develop normally prior to presentation.
- The EEG may initially be normal.
-Interictal EEG typically shows 2 to 5 Hz generalized spike and wave complexes.
-Family history of epilepsy is common. Prognosis is variable, with some children having normal development and others develop intellectual disability.
-Seizures can be resistant to medication.
112
Lateralizing Features in Epilepsy
-Early forced head version
hemisphere contralateral to the direction of the head version (ie, if the head turns to the right, the seizure onset is in the left hemisphere)
-Unilateral irritative stimulation of the frontal eye fields often causes conjugate gaze contralateral to the stimulation.
113
Lateralizing Features in Epilepsy
- Ictal aphasia and postictal dysphasia
the dominant hemisphere
114
Lateralizing Features in Epilepsy
-Ictal speech and preserved awareness during ictal automatisms
non-dominant hemisphere
115
Lateralizing Features in Epilepsy
- Post-ictal nose wiping
The hemisphere ipsilateral to the hand used for nose-wiping
116
Lateralizing Features in Epilepsy
-Unilateral eye-blinking
Unilateral eye-blinking
117
Lateralizing Features in Epilepsy
- ictal vomiting
non-dominant hemisphere
118
What seizure type is associated with Tuberous Sclerosis Complex (TSC)?
all seizure types except for Absence
119
Mnemonic for TSC (tuberous sclerosis complex)
A mnemonic for manifestations of TSC is HAMARTOMA
H: Hamartoma
A: Adenoma sebaceum
M: Mitral regurgitation
A: Ash-leaf spots
R: Rhabdomyoma
T: Tuberous sclerosis
O: dOminant inheritance
M: Mental retardation (intellectual disability)
A: Angiomyolipoma (of kidney)
120
Mechanism of action for Vigabatrin
irreversible gamma-aminobutyric acid transaminase inhibitor
121
Infantile Spasm treatment associated with TSC?
Vigabatrin
122
What is a major side effect of Vigabatrin?
it can lead to visual field loss, which can be monitored with perimetry.
123
What portion of newly diagnosed epilepsy patient's have cognitive or behavioral issues ?
-Approximately half of newly diagnosed patients with epilepsy have demonstrable cognitive or behavioral difficulties on neuropsychological testing
-refer for baseline cognitive assessment at epilepsy onset for routine screening of cognitive or behavioral difficulties.
124
Cannabidiol (brand name epidiolex; a plant-based formulation of CBD) is approved by (FDA) for which epilepsy conditions?
Dravet syndrome, Lennox-Gastaut syndrome, and tuberous sclerosis complex
125
EEG characteristics of Lennox-Gastaut Syndrome
atonic seizures associated with generalized polyspike and wave, generalized voltage attenuation, and runs of low-voltage fast activity
126
Vertex waves
-synchronous, episodic, sharply contoured potentials that are maximal over the central regions
- typically in stage 1 but can persist into stage 2 sleep
127
Stage 2 of sleep consists of what structures on EEG?
K-complexes and spindles.
128
What is often seen in focal seizures with impaired awareness/ complex partial seizures?
Repetitive movements involving the distal extremities, mouth, or tongue are automatisms
129
What is Transient global amnesia?
-period of anterograde amnesia with no other focal neurologic deficits lasting < 24 hours.
-Epilepsy and stroke should be ruled out, and this is a diagnosis of exclusion
130
What is TGA imaging and timing?
-Diffusion-weighted imaging on MRI of the brain has revealed punctate foci of ischemia within the hippocampi in cases of TGA.
-timing can influence chances of detecting abnormalities, typically peaking between 24 and 72 hours and often disappearing after a month or longer.
131
Stage 2 sleep EEG characteristics
-more delta frequency background begins to emerge,
-and the defining features of sleep spindles, K-complexes, and posterior occipital sharp transients of sleep (POSTS) are seen
132
What is the origin of sleep spindles?
Sleep spindles are thought to reflect the synchronous activity mediated by thalamocortical neuronal networks.
133
Mesial temporal sclerosis
(a) medically refractory?
(b) resection curative?
a)most common cause of medically refractory epilepsy b) can be cured by surgical resection
134
Pre-Surgical study for Epilepsy; name utility of study
-T1/T2/Contrast-enhanced MRI
-Demonstrates location/characteristics of the lesion
-Resolution dependent on strength of the magnet
135
Pre-Surgical study for Epilepsy; name utility of study
-Susceptibility weighted imaging/Gradient recalled echo
-Highlights paramagnetic properties of iron in hemoglobin
-Useful for identifying small vascular lesions such as cavernous malformations
136
Pre-Surgical study for Epilepsy; name utility of study
-Diffusion tensor imaging
-Highlights white matter tracts by measuring areas of increased water diffusion
-Useful for identifying key tracts near the lesion, predicting deficits, and determining plans to minimize the risk of deficits
137
Pre-Surgical study for Epilepsy; name utility of study
-Positron emission tomography
-Injection of 18F-fluorodeoxyglucose tracks the relative blood flow to areas of the brain
-Identification of hypermetabolic areas during ictal periods or hypometabolic areas during interictal periods
138
Pre-Surgical study for Epilepsy; name utility of study
-Single-photon emission computed tomography
-Injection of a radiolabeled tracer leads to binding of the tracer on the first pass in the brain - allows for determination of regional blood flow at time of injection
-Identification of hypermetabolic areas during ictal periods or hypometabolic areas during interictal periods
139
Pre-Surgical study for Epilepsy; name utility of study
- Magnetoencephalography
-Identifies pathological high-frequency oscillations without the signal/location degradation seen in EEG
-Helps identify epileptogenic lesions in patients with multiple intracranial lesions
140
Pre-Surgical study for Epilepsy; name utility of study
-Carotid amobarbital test (Wada)
-Injection of sodium amobarbital into the internal carotid artery leads to temporary suppression of the injected hemisphere
-Neurological testing after injection allows for localization of key functions such as language
141
Name Characteristics of VNS pathology
-Understood pathology
-Natural history of the type of epilepsy is expected to be refractory
-Presurgical evaluation is able to be done
-Surgery offers a chance of seizure freedom/improvement
142
Examples of pathology in Epilepsy for VNS consideration
-Mesial temporal epilepsy with hippocampal sclerosis
-Lesional epilepsy
-Catastrophic cause (ie, static encephalopathy)
-Rasmussen encephalitis
-Sturge-Weber syndrome
-Medically refractory epilepsy with drop attacks
143
VNS in pediatric patients
-good outcomes in multiple seizure types, including both partial and generalized seizures, and in the treatment of Lennox-Gastaut syndrome.
-Robust data for the treatment of drop-attack, in particular
144
Most common side effect of VNS
- cough and voice alterations (temporary)
145
Forced normalization phenomenon in epilepsy
phenomenon in which sudden effective control of seizures can precipitate psychotic symptoms, with a return to normal mentation after a breakthrough seizure.
146
Psychosis of epilepsy is associated with which type and what lobe?
focal epilepsy of temporal and frontal lobe origin
147
What can be done therapy-wise to manage forced normalization psychosis?
psychosis can be incompletely responsive to antipsychotic therapies or antidepressants but may respond to decreasing antiepileptic medications to the lowest effective dose
148
EEG difference between West Syndrome and benign myoclonus of infancy?
- ictal EEG normal in benign myoclonus, abnormal in West syndrome
- interictal EEG can be normal in both West Syndrome and benign myoclonus
-awake EEG may still be normal for a brief period after the onset of clinical spasms in West Syndrome
149
Typical pathognomic EEG findings in West Syndrome
hypsarrhythmia, which is generalized, high-amplitude, chaotic, asynchronous slowing, often with admixed epileptiform discharges.
-West syndrome typically develop this pattern over time as their disease progresses
150
Juvenile Myoclonic Epilepsy (JME) clinical profile, classic triad
classic triad of absence seizures, myoclonic seizures, and generalized tonic-clone. seizures
151
Name JME gene involved in disease causing mutation
-Mutations of EFHC1 gene have been most commonly identified
-most common genetic epilepsy
152
Temporal lobe seizures-- localizing symptoms
-contralateral dystonic limb posturing
-contralateral head version
-automatisms (lip smacking, chewing, eyelid fluttering)
-ipsilateral limb automatisms (distal)
-autonomic features
-sensations of fear or deja vu, and postictal nose wiping
153
Parietal lobe seizures-- localizing symptoms
-unilateral paresthesias due to involvement of the somatosensory cortex
-Classically, these seizures progress in a gradual but step-wise fashion, spreading sequentially from one body part to the other, ie, hand to arm to face to leg, called the Jacksonian march
154
EEG characteristic of Vertex waves
-sharply contoured, short-duration waves with maximal expression over the central head regions occurring during non-rapid eye movement stage 1 sleep (N1)
-attenuation of the posterior dominant rhythm and transition to mixed-frequency activity, with predominantly theta-range frequency
155
EEG characteristics of sleep spindles
-sleep spindles (0.5- to 1.5-second bursts of 10- to 15-Hz sinusoidal oscillations), which occur during stage N2 sleep.
156
What seizure medication increases risk if polycystic ovarian syndrome (PCOS)?
Valproate
157
Benign familial neonatal seizure typical diagnosis
evaluation for seizures fails to reveal an etiology and there is a family history of neonatal seizures with subsequent normal growth and development
158
Benign familial neonatal seizure mutations in which genes
mutations in genes encoding voltage-gated potassium channels. (KCNQ2 and KCNQ3)
- autosomal domintant
159
Benign familial neonatal seizure clinical presentation
-typically presents in the first few weeks of life with focal, multifocal, or generalized seizures.
-seizures are brief but occur 20 to 30 times per day and may be difficult to control.
-Interictally, the infant will act normally and should have a normal neurologic examination.
-EEG does not have specific features that help distinguish BFNS from other neonatal epilepsies
160
Benign familial neonatal seizure outcome
generally favorable, with resolution of seizures typically in early to midinfancy and normal neurodevelopment
161
Describe classic EEG findings, known as hypsarrhythmia
high voltage with random spike and waves with varying duration involving the cortical regions.
162
West Syndrome Triad
infantile spasms, psychomotor arrest, and EEG findings of hypsarrhythmia
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First Line treatment for infantile spasms for West Syndrome vs Tuberous Sclerosis
West Syndrome corticotropin (ACTH)
Tuberous Sclerosis Vigabatrin
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Describe infantile spasm characteristics
spasms of the trunk, neck, and extremities that are more common after arousals and tend to occur in clusters
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Glomus tumor Epidemiology
-0.8:100,000 in US
-Generally presents in 40s-50s
-No sex predilection
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Glomus tumor pathophysiology
-Tumor arises from glomus cells - paraganglia near parasympathetic ganglia
-Tumors are generally benign with symptoms due to local mass effect
-Commonly found along carotid and aortic bodies
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Glomus tumor evaluation
CT or MRI with contrast to identify the lesion
Consider catheter-directed angiogram to evaluate vascularity of lesion
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Glomus tumor management
-Surgical resection to remove mass effect
-Alpha-adrenergic blockade prior to removal for functioning tumors
-Consider pre-operative embolization to reduce blood loss
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Paragangliomas-- location and clinical course
-located near the carotid body arise from the parasympathetic paraganglia which are non-chromaffin, glomus cells.
-these tumors are benign and generally non-functional but can lead to carotid body syndrome due to local mass effect
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Occipital lobe seizure semiology
-elementary visual hallucinations, ictal blindness, nystagmoid eye movements, and contralateral eye movements
-if involving ictal blindness then will be contralateral to the seizure focus
-Typical visual hallucinations can include contralateral flashing lights or flashing colors. When seizure spreads to the posterior temporal lobe, more-complex experiential hallucinations can occur
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Epileptic nystagmus is divided into 3 main proposed pathogenic types depending on the clinical features of nystagmus.
Type 1 : activation of the cortical saccade region, producing a contraversive fast-beating nystagmus, with the slow phase not crossing the midline
Type 2: activation of the cortical pursuit region, with a slow ipsiversive saccade that crosses the midline with linear velocity. The quick (contraversive) phase is generated reflexively.
Type 3: activation of cortical optokinetic region, with the ipsiversive slow phase associated with reflexive quick phases.
- also, associated vertigo due to connections with the vestibular nucleus in the brainstem.
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Describe most epileptic nystagmus
fast beating nystagmus contralateral to the seizure focus
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Posterior reversible encephalopathy syndrome preferentially affects what circulation?
posterior circulation in the parietal/occipital lobe
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In medically refractory MTS epilepsy, what is next step after medical therapy failure?
- Mesial temporal sclerosis can be associated with additional epileptogenic foci; therefore, seizure localization should be undertaken prior to surgical resection.
