Neuro Flashcards

(35 cards)

1
Q

seizure & epilepsy

A

-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

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2
Q

classification of seizures/epilepsy

A

-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

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3
Q

seizure/epilepsy sx

A

-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

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4
Q

seizures/epilepsy: dx and eval

A

-< 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

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5
Q

seizures/epilepsy: first aid tx

A

-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!!!!!!!!!!!!!!!!)

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6
Q

seizures/epilepsy: tx: antiseizure meds (ASM)- just know ethosuximide name

A

-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

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7
Q

seizures/epilepsy/ tx: alternative tx

A

-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)

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8
Q

seizures/epilepsy: complications/sequelae

A

-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

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9
Q

seizure/epilepsy: Education

A

-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

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10
Q

febrile seizures

A

-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

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11
Q

sleep disorders

A

-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

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12
Q

sleep disorders: developmental phenomenon (dont memorize #s)

A

-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

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13
Q

sleep disorders: parasomnias: night terrors

A

-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)

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14
Q

sleep disorders: parasomnias: somnambulism

A

-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

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15
Q

sleep disorders: parasomnias: nightmares

A

-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

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16
Q

sleep disorders: insomnia

A

-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

17
Q

sleep disorders: sleep disordered breathing/obstructive sleep apnea

A

-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

18
Q

sleep disorders: restless leg syndrome (RLS) & periodic limb movement disorder (PLMD)

A

-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

19
Q

sleep disorders: narcolepsy

A

-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

20
Q

sleep disorders: management

A

-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

21
Q

cerebral palsy- know spastic

A

-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

22
Q

cerebral palsy dx and tx

A

-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

23
Q

cerebral palsy: prognosis

A

-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

24
Q

Tics

A

-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

25
tics: tx
-Educate pt, family, and school -Tx underlying anxiety, inattention, or sleep difficulties -Pharm tx if tics are physically painful, disrupt social relationships, or prevent pt from focusing on school -1st-line: Clonidine or guanfacine -SE: Drowsiness, lightheadedness -2nd-line: Topiramate -FDA-approved: Haloperidol, pimozide, and aripiprazole – typically reserved for refractory tics
26
spina bifida
-Defective closure of caudal neural tube (end of week 4 of gestation) resulting in anomalies of lumbar and sacral vertebrae or spinal cord -Anomalies range in severity from clinically insignificant defects of L5 or S1 vertebral arches to major malformations -> leaving spinal cord uncovered by skin or bone on infant’s back -Myelomeningocele: Flaccid paralysis and loss of sensation in legs, incontinence of bowel/bladder -Extent and degree of deficit dependent on location -Usually have assoc Chiari type II malformation -> results in hydrocephalus and weakness of face/swallowing -Meningocele: Spinal canal and cystic meninges exposed on back, but underlying spinal cord is anatomically and functionally intact -Spina bifida occulta: Skin of back is intact, but defects of underlying bone or spinal cord present -Meningoceles/spina bifida occulta may: -Be assoc w/ lipoma, dermoid cyst, or dimple/tuft of hair over affect area -Have dermoid sinus: Epithelial tract extending from skin to meninges -> increased risk of meningitis -Have weakness/numbness in feet (ulcerations) or difficulties controlling bowel/bladder function (UTIs, reflux nephropathy, renal insufficiency)
27
spina bifida dx and tx
-Dx: -Myelomeningocele suggested by elevated a-fetoprotein (AFP) in mother’s blood and confirmed by US and high concentrations of AFP and acetylcholinesterase in the amniotic fluid -After birth: Screening US w/ MRI to confirm less dramatic spinal abnormalities -Tx/Prevention: -Myelomeningocele -Operative closure of open spinal defects -Tx of hydrocephalus by placement of VP shunt -Toddlers/children w/ lower spinal cord dysfunction require PT, bracing of lower extremities, and intermittent bladder catheterization -Most survivors have normal intelligence -Learning problems and epilepsy are more common than general population -Prevention via folate administration in pregnant mother
28
CNS infections
-Pts with CNS infections (bacteria, viruses, or other microorganisms) present with similar manifestations -Systemic signs of infection: Fever, malaise, chills, and organ dysfunction -CNS signs of infection: HA, stiff neck, fever/hypothermia, AMS, seizures, focal sensory and motor deficits -Meningeal irritation: Kernig and Brudzinski signs -In young infants: -Signs of meningeal irritation may be absent -Temperature instability/hypothermia more common than fever -Bulging fontanelles, increased head circumference -Older children/adolescents: Papilledema -CN palsies -RF: Infections involving sinuses, open head injuries, neurosurgical procedures, immunodeficiency, presence of mechanical shunt
29
CNS infections: Dx
-Dx: -CBC, chemistry panel, blood cultures, CSF (most important) -Start antibiotics even if LP is delayed -CSF labs: RBCs, WBCs, protein, glucose, bacteria, and other microorganisms; sample should be cultured -Serologic, immunologic, and PCR tests may be performed -Neuroimaging w/ CT/MRI w/ and w/o contrast may show brain abscess, meningeal inflammation, or 2ndary problems (vascular/effusions) -EEGs -Aseptic Meningitis: -Meningitis with neg CSF bacterial cx -Viral, specific infectious organisms (Lyme, syphilis, TB), parameningeal infections, chemical exposure, autoimmune disorders, Kawasaki disease
30
CNS infections: bacterial meningitis
-Can be acute, subacute, or chronic -Brain parenchyma usually inflamed and blood vessel walls infiltrated by inflammatory cells -> endothelial cell injury -> vessel stenosis -> secondary ischemia/infarction -Tx: -While waiting for results -> empiric tx w/ broad-spectrum antibiotic coverage (varies w/ age) -VP shunt: MC coagulase-negative staphylococci -> MRSA -Severely ill pts: Vancomycin + 3rd-generation cephalosporin (S. aureus and gram-negative rods MC) -After specific organisms identified -> antibiotic tailored based on sensitivity patterns -N. meningitidis: 5-7 days of tx -H. influenzae: 7-10 days of tx -> !Adjuvant dexamethasone -> significantly decrease hearing loss!!!! -S. pneumoniae: 10-14 days of tx -Gram-negative bacilli: 21 days of tx
31
bacterial meningitis complications and sequelae
-Complications: -Abnormalities in water/electrolyte balance -Seizures (20-30% of children) -Subdural effusions (33% of children w/ S. pneumoniae) -Cerebral edema -Sequelae: -Focal motor/sensory deficits, visual impairment, hearing loss, seizures, hydrocephalus, CN deficits -SN hearing loss may not be noted until months later -Early addition of dexamethasone to antibiotic regimen may decrease risk of hearing loss in some children -Some pts develop mild to severe cognitive impairment or behavioral issues
32
CNS infections: poliovirus
-Asymptomatic in 90-95% -acute febrile illness in ~ 5% -aseptic meningitis w/ or w/o paralysis in ~1-3% -Eliminated from >99% of world pop -Most protected from vaccination or previous silent infection -Initial sx: Fever, myalgia, sore throat, HA for 2-6 days -<5% of infected kids: Sx-free days followed by recurrent fever and signs of aseptic meningitis (HA, nuchal rigidity, and nausea) -1-2% have high fever, severe myalgia, and anxiety -> herald progression to loss of reflexes and subsequent acute flaccid asymmetrical paralysis -Proximal limb muscles -> distal limbs; lower limbs -> upper -Bladder distention and marked constipation accompany lower limb paralysis -Paralysis complete by time temp normalizes -Atrophy apparent by 4-8 weeks -Improvement of muscle paralysis w/in 6mo -Sensation remains intact, hyperesthesia of skin overlying paralyzed muscles is pathognomonic -Bulbar involvement affects swallowing, speech, and cardiorespiratory function, accounting for most deaths
33
CNS infection: poliovirus: dx and tx
-Labs: -Meningeal symptoms: CSF w/ lymphocytic pleocytosis, normal glucose, mildly elevated protein -Poliovirus growth in cell cx, readily differentiated from other enteroviruses -Rarely isolated from spinal fluid, but often present in throat and stool for several weeks following infection -PCR is preferred for detection -Complications: -Result of permanent destruction of anterior horn cells and paralysis -Respiratory, pharyngeal, bladder, and bowel malfunction are most critical -Death usually from respiratory dysfunction -Limbs injured near time of infection -> most severe and have worst prognosis for recovery -Tx/Prognosis: -Supportive: Bed rest, fever and pain control, careful attention to progression of weakness -No IM injections during acute phase -> may enhance paralysis -Intubation or tracheostomy, enteral feeding, catheter drainage of bladder -Post-polio muscular atrophy in 30-40% of paralyzed limbs 20-30 years later -Increasing weakness, fasciculations in previously affected, partially recovered limbs
34
CNS infection: tetanus
-Caused by Clostridium tetani (anaerobic, gram-positive bacillus) -contamination of wound by soil containing clostridial spores from animal manure -produces potent neurotoxin -> spread to CNS -> increases reflex excitability in neurons of spinal cord (muscle spasms) -2/3rd of U.S. cases -> minor puncture wounds of hand or feet -IV drug use/diabetes increase risk -In U.S. -> young children -> inadequate immunization -Newborns in underdeveloped countries -> contamination of umbilical cord -Incubation typically 3-21 days; in neonates, 4-14 days
35
tetanus sx, dx, tx
-1st sx- mild pain at wound site, followed by hypertonicity and spasm of regional muscles -Trismus (difficulty opening mouth) w/in 48hrs -Newborns: 1st signs are irritability and inability to nurse -> stiffness of jaw/neck, increasing dysphagia, and generalized hyperreflexia w/ rigidity and spasms of all muscles of the abdomen and back (opisthotonos) -Facial distortion/grimacing (risus sardonicus) -Convulsions triggered by minimal stimuli (sound, light, or movement) -Individual spasms last secs or mins -Temp- normal -typically conscious and lucid -Profound circulatory disturbance assoc w/ sympathetic overactivity may occur on days 2-4 > contributes to mortality rate -Tx: -Surgical debridement of wound -Human TIG x 1 dose (3000-6000U), IM with infiltration of part of dose around wound -If unavailable -> equine tetanus antitoxin may be available -Age-appropriate tetanus toxoid containing vaccine (different limb from TIG administration site) -Antibiotics (to decrease bacterial load) -> 1st-line: IV/PO metronidazole x 7-10 days -IV PCN G alternate -Complications: Sepsis, malnutrition, PNA, atelectasis, asphyxia spasms, decubitus ulcers, fractures in spine (from contractions) -Prognosis: -high fatality in newborns- injection drug users and pts w/ DM -Overall mortality in U.S- 6% -Many deaths due to PNA or respiratory failure -Complete recovery may take months -Prevention: -Active immunization w/ tetanus toxoid -Tetanus immunoglobulin (TIG) to prevent tetanus in those w/ < 3 doses who have tetanus-prone wounds (or those who are immunocompromised w/ tetanus-prone wounds regardless of immunization hx) -Tetanus toxoid booster at time of injury if: -None given w/in 10 years -None given w/in 5 years for heavily contaminated wounds