Acpnp Neurologic Disorders

Front 1

Cranial Nerves

Back 1

I: olfactory (smell)

II: optic (sight, visual acuity)

III: oculomotor (extraocular movements, eye-lid opening, pupil constriction)

IV: trochlear (superior oblique muclse that in torts eye)

V: trigeminal (chewing, touch, pain and temp in face, corneal reflex)

VI: abducens (lateral rectus muscle that abducts eye)

VII: facial (eye-lid closure, sensation/taste anterior 2/3 of tongue, tear and salivary glands, corneal reflex )

VIII: vestibulocochlear ( equilibrium/hearing)

IX: glossopharyngeal (sensation and taste in post 1/3 of tongue, swallowing, salivary glands, viscera in thorax/abdomen)

X: vagus (movements in pharynx, larynx, palate)

XI: accessory (swallowing/ movement of head)

XII: hypoglossal (speech/swallowing)

Front 2

AVM

Back 2

• can be assoc w/ sturge-weber, neurofibromatosis, von Hippel-Lindau
• congenital: occurs in 3rd week of fetal development
• Vein of Galen: infant population, >30% of AVMs in gen-peds pop.
• port-wine stain spans cutaneous distribution of trigeminal cranial nerve (CN V) = suspicious for AVM
• ICH 80% of time
• s/sxs increased ICP: HA, N/V, sz, focal neuro defecits, hydrocephalus/macrocephaly, high output cardiac failure, audible intracranial bruit, cardiomegaly
• ruptured AVM = medical emergency
• CT initially
• cerebral angiography = gold standard -> hematoma/edema will block or hide vasculature if done too early -> done when clinically stable and "cooling off" pd for resorption of hematoma
• assume increased ICP and tx accordingly

Front 3

increased ICP control

Back 3

• positioning (HOB elevated and midline)
• hyperosmolar tx
• mild hyperventilation (PaCO2 ~30-35 mmHg)
• temp control
• sz control
• sedation/analgesia
• pharm. paralysis prn

Front 4

Botulism

Back 4

• sxs: constipation, poor feeding, lethargy, increasing weakness, hypotonia, symmetrical CN palsies, neonates: weak cry, expressionless face, ptosis, sluggish pupillary response, G/S/S reflexes diminished
• stool sample + bolulinum toxin
• supportive care + BIG-IV (only through CA dpt of health)

Front 5

Brain abscess

Back 5

• S. aureus, enterobacter, streptococcus most common -> anaerobic and microaerophilic cocci and gm- and gm+ anaerobic bacilli
• clinical manifestations typically nonspecific = leading to undiagnosed (HA, mental status change, focal neuro defects, fever, sz, n/v, nuchal rigidity, papilledema)
• MRI or CT of head
• Tx: antimicrobial tx, control ICP if present, surgical resection, aspiration or drainage if more than 1 area involved

Front 6

CVA (stroke)

Back 6

• risk fx: congenital or acquired heart dis, arteriopathies, sickle cell, hypercoagulable conditions
• ischemic: (neonates) sz, decreased responsiveness, focal weakness, (kids) focal neuro deficit, aphasia, visual disturbances, HA
• Hemorrhagic: increased ICP -> HA, vomiting; irritability, sz, hemiparesis
• CT or MRI w/ diffusion-weighted imaging
• supportive care: prevent fever, normoglycemia, normovolemia
• Ischemic: heparin (unfractionated or LMW) for up to 1 week; aspirin 3-5 mg/kg prophylaxis

Front 7

Delirium

Back 7

• pCAM-ICU assessment tool

Front 8

Encephalopathy

Back 8

• Types: actue toxic/metabolic, hypoxic-ischemic, infectious, post infectious, vascular, static, progressive
  
• s/sxs: mental status change, cognitive decline, concentration probs, sz, nystagmus, tremor, myoclonus, abnml movements
• Dx approach broad and specific to pt risk fx
• CT or MRI, EEG
• correct electrolyte imbalances, antimicrobials if infectious, anti epileptics for sx, steroids w/ post infectious or autoimm.

Front 9

GBS (Gillian- Barre Syndrome)/Acute Inflamm demyelinating polyradicuoneuropathy

Back 9

• peripheral neuropathy; specific cause unknown
• progressive extremity weakness, usually ascending; distal to proximal muscles; max weakness 1-2 wks from onset
• Tx: IVIG, plasmapheresis, supportive care
• ** assess functional vital capacity, negative inspiratory force serially -> to determine trajectory and need for MV

Front 10

HIE (Hypoxic-Ischemic Encephalopathy)

Back 10

• occurs before, during or after birth 2/2 lack of O2 or inadeq. tissue perfusion = cerebral ischemia/hypoxia
• consider serum AA & urine OA if suspect IEM; thyroid function and cortisol if endocrine disorder suspected
• CT initially then MRI/MRA; EEG
• supportive care -> restore oxygenation/ventilation; ABCs, monitor for increased ICP; hypothermia for neonates, anti epileptics, multidisciplinary approach

Front 11

intracranial hypertension

Back 11

• brain 80%, CSF 10%, blood, 10%; fixed space in skull -> increase in any 1 of these requires decrease in another
• ICP nml values -> vary w/ age: <20-25 cm H2O in older kids; <15-20mmHG in <1 yo
• ICP peaks 24-72 hrs after trauma injury
• Cerebral perfusion pressure (CPP) = MAP-ICP
• Goals (mmHg): infant >40, kids >50, adolescents >60
• MAP = 1/3 SBP & 2/3 DBP -> CPP can be decreased from increase in ICP, decrease in systemic BP or combo.
• low CPP assoc w/ poor outcomes in trauma lit.
• pseudomor cerebri = idiopathic increased ICP -> no evidence of space occupying lesion or vascular abnormality

Front 12

intracranial hypertension

Back 12

• TBI most common cause
• sxs: loss/ altered LOC, vomiting, incr. HC, tense fontanel, sz, status epilepticus, coma
• Cushing triad = irreg resp, widening pulse pressure, bradycardia -> impending herniation = medical emergency
• CT initially
• insert ICP monitoring device -> external ventriculostomy or parenchymal catheter
• Maintain adequate CPP (goals as previous)

Front 13

Pseudotumor cerebri: idiopathic intracranial htn

Back 13

• often overwt/obese females w/ persistent HA and visual loss or diplopia + papilledema
• DX: opening pressure w/ LP; MRI (to document nml results); opthalmology exam
• risk fx: obesity, hypernatremia, tetracylclines, fluoroquins, OCPs, vit A, isotretinoin, sulfamethoxazole, growth hormone, lithium
• Tx: manage HA & diamox; repeat LPs to drain CSF; GOAL: prevent or reverse visual loss

Front 14

Bacterial meningitis

Back 14

• peak incidence 3-12 mos of age; late fall/early winter
• < 1 mo: GBS, e.coli, Listeria, Klebsiella
• 1-2 mos: S. pneumo, Hib, N. meningitidis, GBS, e.coli
• 2m-5y: S. pneumo, Hib, N. meningitis
• 5y: N. meningitidis, S. pneumo
• CSF: increased ^WBC (PMN leaks), ^protein,
• +Kernig/Brudzinski signs
• Traumatic tap: conservative approach -> count total # WBCs and disregard RBCs -> if >WBCs than nml for age then treat
• Tx: antibiotics; basilar skull fx and recent VP shunt -> add VANC
• Dexamethasone for +H.flu B (

Front 15

Normal CSF

Back 15

Newborn

• protein: 20-170 (mg/dl)
• glucose: 34-119 ( mg/dL)
• WBC: 0-22 (mm3)

Child >1 mo

• protein: 5-40
• glucose: 40-80
• WBC: 0-7

Front 16

Viral Meningitis

Back 16

• enterovirus >80% of cases; echovirus, coxsackie, arbovirus, mumps, EBV, CMV, varicela, adeno, HSV
• late summer/early fall
• pleocytosis, PMN leukocytosis -> 1st 24-48 hrs -> later increasing mono/lymphocytes
• glucose nml to sl decreased; prot sl ^; WBC mildly ^ w/ neutrophil predom. early then lyphocytosis
• CSF for HSV gold standard for HSV dx -> tx w/ Acyclovir
• HSV: EEG -> sharp waves on slow background in temporal region; CSF w/ RBCs; CT/MRI temporal hypodensity or edema
• self-resolves 7-10 d

Front 17

Encephalitis

Back 17

• inflammatory process of brain parenchyma -> infectious or hyperimmune
• often assoc w/ meningitis: bacterial ->Borrelia burgdorferi (Lyme); Bartonella (cat scratch), treponema (syphillis)
• fungal-> cryptococcus ->immunocompromised
• viral -> rabies, HSV
• DX: constellation of sxs
• EEG: nonspecific; abnml 90%
• MRI: preferred over CT ->early lesion detection
• LP: delay if altered mental status
• ABCs, intubate GCS =8, anticonvulsants if sz, antibiotic/antiviral tx until infectious etiology excluded

Front 18

Myesthenia Gravis

Back 18

• chronic neuromusc dis 2/2 autoimm destruction of acetylcholine receptors
• Transient neonatal (transplacental transfer of maternal Ab) -> lasts 1 wk-2 mo, congenital (acetylcholine receptor Ab neg = not autoimm), Juvenile (3mo-16 yrs onset) -> ocular or generalized
• hallmark = muscle weakness worse w/ exercise, better w/ rest; progressive hypotonia, poor suck, tongue fasciculations
• DX: molecular genetic testing; based on history, presentation, PE -> 80% + AChR antibodies, 30-40%
• bedside test = edrophonium or Tensilon test -> rapid onset (30-90 sec), short acting (5 min) anticholinesterase = temporarily blocks degradation of acetylcholine
• r/o thymoma
• TX: anticholinesterase (pyridostigmine or neostigmine); immunosuppressants; IVIG
• myasthenia Crisis = life-threatening emergency; 2/2 infection or adverse rxn to meds; severe weakness of muscles = resp failure

** most kids w/ MG will lead normal lives w/ treatment, w/ temp or permanent remission

Front 19

NF1

Back 19

• neurocutaneous inherited disorder -> tumors in brain, spine, skin, eye
• presents birth -10 yrs
• DX: 6 or > cafe au lair spots >5mm in diameter in kids and >15 mm in adolescents; 2 or > neurofibromas or one plexiform neurofibroma; freckling in axilla or inguinal region; 2 or > lisch nodules; abnml development of spine, tibia or sphenoid; 1st degree relative w/ NF1
• TX: nonspecific; surgery for symptomatic or malignant tumors; formal neurodevelopment and behavioral eval -> increased risk for neurocognitive and behavioral deficits (ADHD, ASD, language/motor deficits;
• increased risk for malignancies (pheochromocytomas, CML)

Front 20

NF2

Back 20

• less common
• presents early childhood
• hallmark finding = HL or ringing in ears 2/2 slow-growing tumors on 8th CN (vestibular schwannoma
• may have cutaneous schwannoma (sm flesh-colored skin flaps)
• DX: Bilat CN VIII mass on MRI/CT; 1st degree relative w/ NF2 + unilat vestib schwannoma <30yo or any 2 of: glioma/miningioma/schwannoma/cataract
• TX: annual neuro and optho eval + audiometry and brain MRI

** at risk for developing on nervous system tumors (gliomas, meningiomas, schwannomas, neurofibromas)

** early onset of sxs and presence of meningioma assoc w/ poor prognosis

Front 21

tuberous sclerosis complex

Back 21

• neurocutaneous disorder -> development of benign tumors (harmartomas) in brain, skin, and kidneys mostly
• usu >2 yo (~5yo) for diagnosis
• infants p/w infantile spasms or sz, hypomelanotic skin lesions or cardia rhabdomyomas
• sz common -> refractory to AEDs

DX:

• definite TSC = 2 major features or 2 major + 2 minor;
• probable = 1 major + 1 minor
• possible either 1 major or 2 or > minors
• TX: no cure; inter professional approach
• AEDs to control sz

Front 22

Neuropathy

Back 22

• not a disease, but a symptom of disease
• motor (weakness or paralysis), sensory (numbness, dysesthesia, ataxia); autonomic (arrhythmia, hypotsn, bowel/bladder probs, abnml sweating); mixed
• Congenital: spinal musc atrophy, Friedreich ataxia, mitochondrial disorder, cong musc dystrophy, Charcot-Marie-Tooth
• Autoimm: MG, GBS
• Acquired: postinfectious (transverse myelitis), vit deficiencies, toxicities, trauma
• Cardinal sign = decreased or absent reflexes
• DX: comprehensive history + detailed neuro exam
• Tx: acute = symptom management
• chronic & acute = opioids, NSAIDS, gabapentin

Front 23

Seizures

Back 23

• 1/3 w/ febrile sz will have another in future
• general: sz originates & engages bilat hemispheres
• absence: brief, 15-20 sec pds of impaired conscious or confusion
• myoclonic: brief muscle movements <1sec
• Clonic: rhythmic, repetitive movements of head, trunk and extrems
• tonic: sustained extension or flexion of head, trunk & extrems
• atonic: loss of muscle tone
• focal: originates w/ in 1 hemisphere
• post-ictal focal deficit = Todd paralysis
• MRI if Todd paralysis, prolonged sz, or not returned to baseline w/in several hrs of sz
• TX: stabilize ABCs, IV, Lorazepam 1st line (may repeat dose), fospheny or phenobarb for persistent sz
• Family education: rectal diazepam, sz recognition, AED therapy, seek med attention: 1st time sz, lasting >5 min, high fever, risk for head injury, compromised CR function

Front 24

Status Epilepticus

Back 24

• single seizure lasting >30 min OR 2 or > consecutive sz w/out return to baseline
• refractory = ongoing sz after 2 doses anticonvulsant meds
• complications: rhabdomyolysis, hyperthermia, cerebral edema, resp failure

Front 25

Shunt malfunction/infection

Back 25

• young age and principle dx or obstructive hydrocephalus assoc w/ high risk for shunt failure
• highest incidence <6 mos old
• lifespan of shunt before replacement = 2 yrs in child <2 yrs and 8-10 yrs in >2 y.o
• VP shunts: differential pressure valves open when IV pressure >predetermine threshold
• most common malfunction 2/2 obstruction
• > 1/2 infections 2/2 coag- staph (S. epidermidis most common)
• fever, HA, N/V, upgaze paralysis, papilledema **drowsiness best predictive sxs for malfunction
• US for infants w/ open fontaneles, CT or MRI; ant/lat X-ray for continuity of shunt (** neuroimaging has low sensitivity for IDing shunt malfunction independently)
• shunt malfunction = med emergency -> may need EVD, treat ^ICP

Front 26

spinal muscular atrophy

(Type 1, 2, 3)

Back 26

• autosomal recessive disorder of anterior horn cells in brainstem and spinal cord
• degenerative -> progressive hypotonia and muscle weakness
• P/W: p. 203 table
• DX: EMG = denervation 2/2 malfunction of motor neurons, but motor and sensory conduction velocities normal ; genetic testing
• TX: no cure & nonspecific; aggressive tx of sxs & assoc complications (resp insuff/failure, feeding difficulties, ortho issues)

Front 27

Drug Levels

Back 27

• Fospheny (10-20 ug/ml), phenobarb (15-40 ug/ml), valproic acid (50-100 ug/ml), carbamazepine (4-12 ug/ml
• indications: initiation of rx, breakthrough sz, monitor adherence, changes in level w/ addition of 2nd rx w/ known interactions
• trough levels should be drawn 30 min prior to dose

Front 28

MRI vs CT

Back 28

CT:

• bony abnorms, hydrocephalus, ICH, cerebral edema, space-occupying lesions, intracranial calcifications
• more sensitive detecting bld = trauma eval
• quick = perform on conscious pt

MRI

• ischemia or infarct, degenerative dis, congenital anomalies, arteriovenous malformations, lesions in posterior foss and spinal cord
• no radiation
• ^gray/white differentiation
• higher-resolution detail at skull bases and orbit
• longer imaging required = sedation often

Front 29

LP

Back 29

• contraindications: ^ICP, bleeding/plt dysfunction/disorder, resp/hemodynam instab, skin infect near site of entry, spinal cord trauma/compression, post spinal cord fusion in lumbar region, focal neuro deficit
• head CT on any pt w/ suspected ^ICP or space-occupying lesion prior to LP -> negative does not completely exclude ^ICP

Front 30

ventriculostomy

Back 30

• preferred device for monitoring ICP, can be used for drainage
• for TBI, ^ICP; CPR w/ abnml head CT on admission and/or GCS =8
• Maintain ICP <20-25 mmHg older kids; <15-20 in <1 y.o
• maintain CPP = adequate MAP