Neuro Flashcards
(35 cards)
seizure & epilepsy
-seizure = sudden, transient disturbance of brain activity manifested by involuntary motor, sensory, autonomic, or psychic phenomena, often accompanied by alteration of consciousness
-triggered by any factor that disturbs brain function (acute metabolic, traumatic, anoxic, or infectious insults)
-Unprovoked seizures -> chronic abnormalities/genetic mutations
-Epilepsy: 2 seizures separated by at least 24 hours
-single seizure w/ > 60% risk of recurrence, or dx of epilepsy syndrome
-Risk of 2nd seizure after unprovoked seizure -> 50%
-Risk of recurrence after 2nd unprovoked seizure -> 85%
-Epilepsy highest in newborn period, flattens after age 10-15 years
-70% have remission w/ 1st appropriate medication
classification of seizures/epilepsy
-Classified by The international League Against Epilepsy (ILAE)
-FOCAL, GENERALIZED, UNKNOWN
-Focal: With or w/o alteration of awareness, motor versus nonmotor (autonomic, emotional, or sensory)
-Generalized: Tonic-clonic (stiffening-shaking), absence, myoclonic (twitching/jerking), atonic (sudden loss of tone), myotonic atonic (jerk followed by sudden loss of tone), tonic, and clonic
-Epilepsy syndromes defined by nature of seizures, age of onset, EEG findings, and other clinical factors
seizure/epilepsy sx
-onset: Determines if its a seizure and if there is localized (focal) onset
-Events prior to, during, and after (even videos are useful)
-Generalized convulsions (observed by others), aura (alterations in behavior or feelings of extreme fear, happiness/anxiety, odd or unusual taste/smell, rising feeling in abdomen)
-Specific sx may help define location of seizure onset
-Behavior and movements are key in classifying
-Lateralized motor movements (eye deviation to one side, dystonic posturing of a limb) w/o impaired awareness -> focal, motor seizures
(simple, partial seizures)
-Automatisms (blinking, chewing, or hand movements) assoc with “zoning out” (partially impaired awareness) -> focal, motor seizures
-Acute, complete LOC and “whole body” (generalized) motor activity -> generalized, convulsive seizures
-Tonic posturing, tonic-clonic activity, or myoclonus may occur
-Behavioral arrest w/ “staring” and automatisms -> absence versus focal motor
-Main differentiator: No postictal state w/ absence, myoclonic, or atonic seizures
-Many focal and most generalized seizures have postictal sleepiness state
seizures/epilepsy: dx and eval
-< 3yo with new onset of unprovoked seizures -> EEG and MRI (most have structural, genetic, or metabolic cause)
-Routine EEG useful primarily for defining interictal activity and/or recording clinical seizure (when seizures are easily provoked)
-EEG showing epileptiform activity may confirm and clarify dx -> not always dx
-Other dx used selectively:
-Routine labs -> unnecessary in well child w/ seizures (unless high clinical suspicion of serious medical conditions)
-Further workup for suspicion of acute systemic etiology (renal failure, sepsis, or substance abuse)
-Emergent imaging -> unnecessary if no trauma or acute abnormalities on exam
seizures/epilepsy: first aid tx
-Turn to side
-Do not place any objects in mouth or try to restrain tonic-clonic movements
-Prolonged seizures (> 5 mins) or seizure clusters (> 6 hrs): Acute home tx with benzos (rectal diazepam gel (Diastat) or intranasal midazolam!!!!!!!!!!!!!!!!)
seizures/epilepsy: tx: antiseizure meds (ASM)- just know ethosuximide name
-No ASM prevents or cures epilepsy
-Unnecessary until dx of epilepsy established
-Some are effective for focal seizures, but make generalized seizures worse (oxcarbazepine, carbamazepine)
-Some are effective for most and relatively safe (levetiracetam)
-!!!!Ethosuximide- absence seizures
-SE can help guide tx
-Ex: Topiramate tends to suppress appetite; valproic acid often precipitates weight gain
-If monotherapy unsuccessful -> a 2nd and sometimes 3rd med may be required
-Tx of acute, symptomatic (provoked) is correction of cause -> however short-term ASM may be necessary
-continue meds until no seizures for at least 1-2 years
-Most tapered off over 6-8 weeks
-Recurrent seizures affect 25% of kids who attempt tapering off from meds -> restart ASM and maintain for at least another 1-2 years
-Favorable prognosis: Younger age of onset, normal EEG, undetermined etiology, ease of controlling seizures
-Poor prognosis: Identified etiology, older onset, continued epileptiform EEG, difficulty in establishing seizure control, polytherapy, generalized tonic-clonic or myoclonic seizures, abnormal neurologic examination
seizures/epilepsy/ tx: alternative tx
-Ketogenic diet (unknown mechanism, requires close monitoring)
-ACTH and corticosteroids: Standard of care for infantile spasms (Vigabatrin as alternative)
-Implantable devices (pacemaker-like devices in brain)
-Epilepsy Surgery
-Option for those w/ resistant/”refractory” epilepsy: Failure of 2 drugs alone or as a combination therapy
-Identification and removal of epileptogenic focus (50-95% remission)
-Generalized seizures: Corpus callosotomy (10% reduction)
seizures/epilepsy: complications/sequelae
-Psychosocial impact:
-Mood disturbances, especially anxiety/depression, anger, feelings of guilt and inadequacy
-School-aged kids- increased risk of suicide
-Actual/perceived stigmas (limiting activities at school adds to this)
-Cognitive Impairment:
-epilepsy, untreated/poorly controlled -> can develop reduced cognition and memory
-Some ASMs have reversible cognitive impairment effects (phenobarbital, topiramate, and zonisamide)
-Psychosis following seizures or as a SE of meds
-Injury/Death:
-Direct physical injuries frequent with atonic seizures, at times necessitating protective headgear
-Must promote water safety (monitoring, showers versus baths)
-Most deaths are related to underlying neurologic disorder, not the seizures
seizure/epilepsy: Education
-Supervision while swimming, protective headgear
-No absolute CI to sports
-Some literature suggests exercise decreases overall seizure burden
-Avoid sleep deprivation/alcohol -> triggers
-Driving: Varies by state
-Most states - learner’s permit or driver’s license if seizure free for at least 6-12 months
-Pregnancy: Discuss interactions of oral contraceptives w/ ASMs, potential teratogenic effects of ASMs, and management of pregnancy as soon as appropriate w/ adolescents
-School intervention
-Schools required by federal law to work w/ guardians to establish a seizure action plan for child w/ epilepsy
-School authorities should be encouraged to avoid needless restrictions and to address emotional/educational needs of children
febrile seizures
-Criteria: 6mo-6yrs, fever > 38.8C, non-CNS infection
->90% are generalized, last < 5 mins, and occur early in the illness
-Dx:
-evaluate for source of fever (to exclude CNS infections)
-Always consider meningitis/encephalitis!
-Routine labs/imaging/EEG seldom helpful (unless warranted based on H&P)
-Tx:
-Prophylactic ASMs not recommended
-Control of fever is ineffective at preventing recurrence (not recommended solely for prevention of seizure)
-Prognosis:
-Recurrent febrile seizures in 30-50%, as well as other forms of seizures
-Cognitive function not significantly different from that of siblings w/o febrile seizures
sleep disorders
-Impact quality of life -> public health problem
-assoc w/ maladaptive daytime behaviors, poorer development, greater parental stress, obesity, insulin resistance, alterations in sympathetic tone, and immune dysfunction
-20-40% of kids have sleep disturbance at some point in first 4 years -> 10-12% in school-aged kids
-MC disorder = insomnia -> others include parasomnias, sleep-disordered breathing, restless legs syndrome, narcolepsy, and circadian rhythm disturbances
-2 major sleep stages: Rapid eye movement (REM) and nonrapid eye movement (NREM)
-REM occurs throughout night, but increases in latter half
-NREM divided into 3 stages (N1, N2, N3)
-Deepest NREM sleep during first 1-3hrs of sleep
sleep disorders: developmental phenomenon (dont memorize #s)
-Infants are not born w/ sleep-wake cycles
-REM > NREM in newborns, decreases by 3-6mo
-Newborns sleep 10-19 hours/day in 2- to 5-hour blocks
-1st year: Consolidation of sleep into a 9- to 12-hour block, naps x 1/day
-3-5 years -> no more naps
-Recommendations for hours of sleep by National Sleep Foundation
-1-2 yrs: 11-14 hours/day
-3-5 yrs: 10-13 hours/day
-6-13 yrs: 9-11 hours/day
-Adolescents: 9-9.5 hours/day
-Often get only 7-7.25 hours due to 1-3 hour sleep delay phase (from changes in hormonal regulation of circadian rhythm)
-Some school districts begin at later start times for high schoolers
sleep disorders: parasomnias: night terrors
-Night Terrors
-w/in 2 hrs of sleep, during deepest stage of NREM sleep
-3% of kid affected- 3-8yo
-sit up in bed screaming, thrashing about, rapidly breathing, tachycardic, and sweating
-incoherent and unresponsive to comforting
-Episode may last 30 minutes
-goes back to sleep w/o memory of event next day
-Tx:
-Parental reassurance
-Measures to avoid stress, irregular sleep schedule, or sleep deprivation (prolongs deep sleep)
sleep disorders: parasomnias: somnambulism
-sleepwalking
-Also occurs during slow-wave/deep sleep
-4-8yo
-Typically benign -> but pt may injure themselves while walking around
-create safe environment (lock doors, bells on bedroom door)
-Tx: Avoid stress, sleep deprivation
sleep disorders: parasomnias: nightmares
-Frightening dreams during REM sleep (later in night)
-typically followed by awakening (alert)
-3-5yo -> incidence 25-50%
-describes frightening images, recall the dream, and talk about it during the day
-Seeks and responds well to parental comfort
-wants to stay w/ parents for rest of the night
-Treatment: Self-limited; may be assoc w/ stress, trauma, anxiety, sleep deprivation
sleep disorders: insomnia
-Difficulty initiating sleep and nighttime awakenings
-daytime fatigue for both parents and child, parental discord about management, and family disruption
-Several factors contribute to disturbances:
-Quantity and timing of feeds in 1st yr
-Most infants >6mo can go through night w/o feedings (waking for feeds is probably a learned behavior)
-Bedtime habits
-Child’s temperament
-Low sensory thresholds and less rhythmicity (regulatory disorder)
-Separation anxiety (9 months)
-Psychosocial stressors
-Complex medical conditions: Neurological, developmental, psychiatric disorders
sleep disorders: sleep disordered breathing/obstructive sleep apnea
-Obstructed breathing during sleep accompanied by loud snoring, chest retractions, morning headaches, dry mouth, and daytime sleepiness
-1-3% of preschoolers, peaks between 2-6yo
-Assoc w/ daytime behavioral disorders -> ADD/ADHD
sleep disorders: restless leg syndrome (RLS) & periodic limb movement disorder (PLMD)
-2% of kids
-RLS: Uncomfortable sensation in LE at night when trying to fall asleep, relieved by movement
-Described as “creepy-crawly” or “itchy bones”
-PLMD: Stereotyped, repetitive limb movements often assoc with partial arousal or awakening
-Both have been associated w/ iron deficiency and drugs (caffeine, nicotine, antidepressants)
-No studies on medications for children
sleep disorders: narcolepsy
-Chronic, inappropriate daytime sleep that occurs regardless of activity or surroundings and is not relieved by increased sleep at night
-Half of individuals experience their initial symptoms in childhood, typically around/after puberty (sometimes in early childhood)
-Dx: Short latency between sleep onset and transition into REM sleep
-Tx: Sleep hygiene/behavior modification
-CNS stimulants/antidepressants off-label
sleep disorders: management
-BEARS screening tool:
-Bedtime resistance
-Excessive daytime sleepiness
-Awakening during the night
-Regularity and duration of sleep
-Sleep-disordered breathing
-Medical psychosocial hx, PE
-Assess allergies, lateral neck films, polysomnography
-GER, dental pain, eczema, meds
-Tx: ABCs of SLEEPING
-Age-appropriate Bedtimes and wake times w/ Consistency, Schedules and routines, Location, Exercise and diet, no Electronic in bedroom or before bed, Positivity, Independence when falling asleep, Needs of child met throughout the day, equals Great sleep
-Little evidence to support use of melatonin/clonidine for sleep disorders
cerebral palsy- know spastic
-0.2% of live births
-Static impairment of motor development -> affecting tone, strength, coordination, or movements
-Causes: Vast range from perinatal brain injury (preterm birth), hypoxic ischemic injury, stroke, or infection to brain malformations and genetic conditions
-Nonprogressive, full degree of impairment may not be evident until 2-4yo when motor expectations are more robust
-Subtypes: !Spastic (80%), ataxic (15%), athetoid/dyskinetic (5%), persistent hypotonia w/o spasticity (<1%)
-!!Spastic: Described based on limb(s) affected: Monoplegia, hemiplegia, diplegia, and quadriplegia
-Affected extremity may be smaller/shorter than others
-Ataxic: Upper extremity predominantly involved
-Athetoid/dyskinetic: Choreoathetosis or dystonia
-MC comorbid conditions: Epilepsy (25%) and intellectual disability (50%)
-Others: Vision impairment, language delay, AS, learning disabilities
cerebral palsy dx and tx
-Dx:
-H&P guides workup
-MRI of brain is essential to understand extent of cerebral involvement and may reveal etiology or identify perinatal stroke/brain malformation
-Genetic testing, metabolic testing: Targeted based on clinical context
-Tx:
-Directed at maximizing child’s neurologic functioning w/ physical, occupational, and speech therapy
-Task-specific approach: Encourages neuroplasticity
-Intensive constraint-induced movement therapy: Benefits affected extremity
-Monitoring for orthopedic issues, neurophysiological testing to provide optimal school support, and treatment of spasticity and seizures
-Support/counseling for family
cerebral palsy: prognosis
-Depends on child’s cognitive abilities, severity of motor deficits, and etiology
-Mild CP: Improvement w/ age, some experience resolution of motor deficits by age 7
-Successful ambulation by age 12 > most predictive of adult functional ability
-Severely affected children can have shortened lifespan
-Infections such as PNA or sepsis MCC of death
-Many children have typical neurodevelopment and lifespans and lead productive, satisfying lives
Tics
-Nonrhythmic, involuntary movements that are stereotyped but evolve over time
-~20% of school-aged kids
-onset 6-8yo, M > F
-MC motor tic -> eye blinking
-Vocal tics - throat clearing, sounds, words
-Preceding urge or tension and brief susceptibility are classic features of tics
-Wax and wane in frequency,
-exacerbated by stress
-~90% have comorbidities -> anxiety, depression, sleep difficulties, OCD tendencies, ADHD, and learning disabilities
-(dont need to know below)
-Provisional tic disorder: Motor or vocal tics lasting < 1yr
-Persistent tic disorder: Motor or vocal tics lasting > 1yr
-Tourette syndrome: Motor and vocal tics lasting > 1yr
-All < 18yo