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Pediatrics Module 3 > Neurology > Flashcards

Neurology Flashcards

1
Q

headache history

A
  • history
    • what is the typical course?
    • how long does the headache last?
    • what makes the headache better/worse?
    • what precipitates the headache?
    • what other symptoms do you have?
    • how does this compare to past headaches?
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2
Q

headache: review stress-related factors, red flags that prompt futher exploration

A
  • review stress-related factors
    • school difficulty
    • family relationships
    • peer relationships
    • activity schedule
    • sleep hygiene
    • caffeine intake
  • red flags that prompt further exploration
    • dramatic increase in headache severity
    • headache that awakens a child from sleep
    • change in established headache pattern/disrupt school and daily activities
    • gradually increasing frequency and severity
      • suggests increasing intracranial pressure
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3
Q

headach physical exam, red flags in history or exam may set off further investigation

A
  • physical exam
    • growth, head circumference, blood pressure
    • intracranial hypertension (papilledema)
    • focal neurologic signs
    • general exam findings
      • rhinitis, dental abscess, bruit, head trauma, hematoma,
  • red flags in history or exam may set off further investigation
    • head imaging (CT for hemorrhage, MRI for tumor & cerebellar imaging)
    • electroencephalography (EEG)
      • +/- sleep deprivation
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4
Q

tension headache, cluster headache

A
  • tension headache
    • diffuse
    • symmetric
    • often related to fatigue
  • cluster headache
    • extreme deep pain in and around one eye
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5
Q

migraine headache

A
  • migraine
    • triggered by stress, vomiting, family history
  • classification
    • migraine with aura (visual or otherwise)
    • migraine without aura
    • complicated migraine (transient focal abnormality)
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6
Q

childhood migraine variants

A
  • paroxysmal torticollis
    • attacks of head tilt, sometimes with vertigo +/-vomiting
    • requires ruling out posterior fossa pathology
  • benign paroxysmal vertigo
    • attacks of unsteadiness with nystagmus and vomiting followed by sleep
    • may precede development of typical migraine
    • requires ruling out epilepsy and CNS tumor
  • cyclic vomiting
    • protracted attacks of vomiting, 1-4 times/hour, up to 5 days
    • requires ruling out epilepsy, GI disorder, urea cycle disorder
  • abdominal migraine
    • attacks of migraine lasting 1-72 hours, untreatable by other means
    • midline, dull, moderate to severe pain
    • associated with anorexia, nausea, vomiting and pallor
    • diagnosed often by response to anti-migraine therapy
    • requires ruling out other GI disorder
  • confusional migraine
    • episodic disorientation or combativeness, sometimes followed by headache
    • requires ruling out drug abuse, epilepsy, CNS ischemia
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7
Q

migraine therapies

A
  • migraine acute episode
    • sleep
    • acute treatment
      • acetaminophen, ibuprofen
      • sumatriptan, other triptans
    • rescue treatment
      • NSAIDs
      • promethazine
      • metoclopramide
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8
Q

migraine prophylaxis

A
  • create management plan to prevent stressors
  • biofeedback (stress reduction)
  • physical modalities (massage, PT, exercise)
  • medications (usually involves a neurologist)
    • cyproheptadine
    • antihypertensives
      • propranolol, verapamil
    • tricyclic antidepressants
      • amitriptyline, nortriptyline
    • anticonvulsants
      • valproate, topiramate, gabapentin
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9
Q

headache summary

A
  • summary
    • acutely rule out underlying pathology
    • address exacerbating factors
    • management plan
      • medications (start with acetaminophen, ibuprofen)
      • abortive agents
      • daily prophylaxis (neurologist)
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10
Q

pseudotumor cerebri

A
  • increased intracranial pressure in the absence of identifiable intracranial mass or hydrocephalus
  • postulated to be due to impaired CSF reabsorption
  • risk factors
    • obesity
    • female
    • sinus thrombosis
    • head injury
    • chronic CO2 retention
    • systemic lupus erythematosus
  • acute
    • headache
    • pulse synchronous tinnitus
    • pain behind the eye
    • pain with eye movements
    • transient visual obscurations
    • blurred vision or double vision
    • CN VI paresis
    • vomiting
    • macrocephaly
    • altered behavior
  • chronic
    • growth impairment
    • optic atrophy
    • visual field loss
    • total blindness
  • diagnosis (one of exclusion)
  • CT
    • rules out hydrocephalus
  • MRI
    • rules out intracranial mass
    • rules out hydrocephalus
  • ophthalmologic exam
    • papilledema
    • optic nerve changes
  • lumbar puncture
    • measurement of opening pressure
    • normal CSF panel and culture
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11
Q

pseudotumor cerebri treatment

A
  • treatment of underlying causes
    • weight loss is mainstay of therapy
    • treat anemia
  • medical treatment
    • diuretics
      • acetazolamide (Diamox) - carbonic anhydrase inhibitor
    • glucocorticoids
    • lumbar puncture
  • surgical treatment
    • optic nerve sheath decompression
    • lumboperitoneal shunt
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12
Q

seizures

A
  • a sudden, transient disturbance of brain function, manifested by involuntary, motor, sensory, autonomic, or psychic phenomena, alone or in any combination often accompanied by alteration of loss of consciousness
  • epilepsy
    • repeated seizures without evident cause
    • recurrent, unprovoked seizures
  • classification
    • symptomatic
      • cause is identified or presumed
    • idiopathic
      • cause is unknown or presumed to be genetic
  • symptomatic (examples)
    • infection (meningitis/encephalitis)
    • trauma
    • metabolic (hypoglycemia, hyponatremia)
    • hypoxic
    • tumor
    • malformation (hydrocephalus)
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13
Q

nonepileptic paroxysmal events

A
  • benign nocturnal myoclonus
  • shudder attacks
  • tics & Tourette’s syndrome
  • sleep orders
    • night terrors
    • sleepwalking/talking
    • cataplexy, narcolepsy
  • nightmares
  • migraine
    • benign paroxysmal vertigo
    • paroxysmal torticollis
  • conversion reaction & pseudoseizure
  • gastroesophageal reflux
  • masturbation
  • hypoglycemia
  • temper tantrums & breath-holding
  • syncope and vasovagal events
  • paroxysmal dystonia or choreoathetosis
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14
Q

seizure exam, treatment, abortive meds

A
  • history
    • events prior (aura, what was person doing?)
    • events during and after
  • exam
  • laboratories based on clinical suspicion
    • CBC, metabolic abnormalities
  • CT scan/MRI - indicated if seizure focus suspected
  • EEG
    • not sensitive and not specific
    • may help classify seizure type to determine therapy
    • may reveal subclinical seizures
  • treatment
    • ABCs
      • most seizures are brief and ABCs are of utmost importance in brief seizures
      • protect against self-injury, airway clearance, monitor ABCs
    • anticonvulsants - if seizures are prolonged or are hindering ABCs
    • education
      • seizure precautions
      • medication (side effect monitoring, compliance)
      • daily life
        • swimming
        • adequate sleep
        • driving
        • pregnancy
  • abortive medications
    • benzodiazepines
      • lorazepam parenterally
      • diazepam orally or rectally
      • midazolam nasally, orally, rectally
      • When giving benzos, you need to be careful of respiratory depression
    • phenobarbital and fosphenytoin
  • long-term anticonvulsants
    • different drug classes used for different types of epilepsy
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15
Q

febrile seizure

A
  • convulsion associated with a temp > 38.0° Celsius
  • child 6 months to 5 years of age
  • Not caused by CNS illness or metabolic abnormality
  • no history of previous afebrile seizures
  • most common childhood seizure
  • incidence 2-5% of children under 5 years of age
  • simple febrile seizure – meets all criteria:
    • duration under 15 minutes
      • total duration less than 30 minutes if in series
    • no recurrence in 24 hours
    • no focal features
  • complex febrile seizure – meets one or more criteria:
    • duration over 15 minutes
    • recur in series for total duration > 30 minutes or more than seizure in a 24 hour period
    • focal features
  • 65-90% of all febrile seizures are simple type
  • Inciting agent
    • Viral infections
    • immunization-related fever
    • predisposing factors
      • heredity - SCN1a mutations - Dravet, GEFS+
      • maternal alcohol intake and smoking during pregnancy raises risk 2-fold
  • degree of fever is widely variable
    • height of fever does not seem to correlate with onset of seizure
  • often the first sign of illness
  • simple febrile seizures most commonly are generalized and tonic-clonic
  • reassurance
    • if fever work up unremarkable
    • if neurologic exam unremarkable
  • treat fever to treat fever
  • treat underlying illness
    • decreasing fever in febrile seizure patient does not decrease chance of another febrile seizure
    • antiepileptic medication if in status (status epilepticus)
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16
Q

febrile seizure differential diagnoss

A
  • differential diagnosis
    • chills/rigors because of fever
    • meningitis
    • encephalitis
    • intracranial tumor
    • metabolic disorder
    • neurologic disorder (developmental delay)
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17
Q

diagnostic evaluation

A
  • diagnostic evaluation
    • EEG usually not indicated for simple febrile seizure if history and physical benign
    • neuroimaging red flags
      • macrocephaly or microcephaly
      • focal features of seizure or abnormal neurologic exam
      • signs of increased intracranial pressure
      • history of signs of trauma
    • electrolytes, glucose, calcium, BUN should be measured if history and physical indicate (vomiting, diarrhea, edema, dehydration) or with complex febrile seizure
    • Search for underlying cause of fever based on clinical suspicion
    • AAP recommends stronger consideration of LP
      • infants younger than 12 months who have had first seizure with fever
      • febrile seizures occurring on or after second day of illness
18
Q

febrile seizure: prevalence of meningitis

A
  • the prevalence of meningitis among patients with febrile seizures was 1-2%
    • the absence of any remarkable findings on the history or physical examination makes bacterial meningitis unlikely as the cause of the fever and seizure
19
Q

febrile seizures ABCs

A
  • ABCs
  • anticonvulsants
    • if seizure is prolonged (>10 minutes)
    • watch for respiratory compromise
    • drugs in office or ED
      • benzodiazepine (midazolam, lorazepam)
      • fosphenytoin
    • rectal diazepam (option for home use)
20
Q

febrile seizures role of preventative tx

A
  • role of preventive therapy
    • antipyresis
      • around-the-clock use not shown to be of benefit to all children with febrile seizures
      • unknown if some subset benefits
      • intermittent use warranted
    • anticonvulsants
    • “based on the risk and benefits of effective therapies, neither continuous nor intermittent anticonvulsive therapy is recommended for children with one or more simple febrile seizures.” (AAP recommendations)
21
Q

febrile seizures: recurrence risk

A
  • recurrence risk
    • young age at onset
    • history of febrile seizure in a first-degree relative
    • low degree of fever while in emergency department
    • brief duration between the onset of fever and the initial seizure
  • all 4 factors – recurrence risk is 70%
  • 0 of 4 factors – recurrence risk is <20%
  • Risk of epilepsy:
    • Simple Febrile Seizures: 1-2%
    • Complex Febrile Seizures: 5-10%
  • risk of recurrent febrile seizures is 10%
    • higher in children with first febrile seizure under age 1 year
22
Q

infantile spasms (west syndrome)

A
  • term used to describe seizures that have characteristic clinical findings in age group 4 months to 8 months
    • sudden adduction and flexion of limbs
    • sudden adduction and flexion of the head and trunk
    • usually occurs in clusters when patient is irritable or fatigued
  • categories
    • cryptogenic (40%)
      • no etiology evident, normal development before seizures
    • symptomatic (60%)
      • association with perinatal and prenatal event or other identifiable cause
      • have poor responses to anticonvulsants and poor intellectual prognosis
  • EEG
    • waking state EEG reveals chaotic high-voltage slow waves, random spikes & background disorganization
  • treatment
    • anticonvulsant therapy
    • ACTH may be helpful
    • ketogenic diet
23
Q

neurocutanous disorders

A
  • Neurofibromatosis
  • Tuberous Sclerosis
  • Sturge-Weber syndrome
  • disorders of tissue arising from neuroectoderm
    • skin findings
    • brain, spinal cord, eye manifestations
    • hamartomas
      • normal tissue growing at abnormal sites or abnormally rapidly
24
Q

neurofibromatosis

A
  • two types of neurofibromatosis
    • NF 1 and NF 2
  • NF-1 epidemiology
    • 50% of cases due to new mutations in the NF1 gene
    • 40% of patients will develop medical complications of the disorder
25
Q

neurofibromatosis type 1

A
  • NF-1 is more prevalent than NF-2
    • clinical criteria
      • six or more café au lait spots
      • axillary or inguinal freckling
      • two or more Lisch nodules
      • two or more neurofibromas or one plexiform neurofibroma
      • distinctive osseous lesion
      • optic glioma
      • first degree relative with NF-1
26
Q

cafe au lait spots

A
  • present in nearly all neurofibromatosis patients
  • predilection for trunk and extremities
  • spares the face
  • common normal finding but in patients with increased number of spots and larger size characteristic of NF-1
27
Q

neurofibroma

A
  • small, rubbery, usually involve the skin
  • may be found viscerally and along peripheral nerves
  • often appear in adolescence
  • plexiform neurofibroma
    • diffuse thickening of nerve trunk
    • may produce overgrowth of extremity
28
Q

complications of neurofibromas

A
  • scoliosis
  • learning disabilities
  • hypertension (renal artery stenosis)
  • disorders of the hypothalmus
  • tumors
    • brain
    • eye (may manifest with strabismus)
29
Q

treatment of neurofibromatosis

A
  • treatment
    • management of complications
    • referral to a neurofibromatosis center
    • genetic counseling
      • prenatal testing is available if parent of possibly affected patient has known mutation
  • brain MRI if any hint of optic glioma
  • renal ultrasound with Doppler flow
  • school assessment (individualized education plan)
30
Q

neurofibromatosis 2

A
  • prevalence of about 1 in 40,000
  • criteria
    • unilateral eighth nerve mass, particularly bilateral acoustic neuroma
    • parent, sibling or child with NF-2 with either
      • unilateral eight nerve mass or
      • any two of the following
        • neurofibroma
        • meningioma
        • glioma
        • Schwannoma
        • juvenile posterior subcapsular lenticular opacities
31
Q

tuberous sclerosis

A
  • autosomal dominant disorder characterized by proliferation of tubers from abnormal production of protein hamartin and tuberin
  • prevalence of about 1 in 6,000
  • clinical manifestations
    • may present with seizures (infantile spasms)
    • calcified tubers in the periventricular areas
    • mental retardation
    • hypopigmented skin lesions
      • ash leaf macules
      • shagreen patch
      • adenoma sebaceum
  • ash leaf spot
    • hypopigmented macule or patch
    • Wood’s lamp highlights appearance
  • shagreen patch
    • roughened, raised lesion with an orange peel, leathery texture
    • often located in lumbosacral region
  • sebaceous adenomas
    • tiny, red, hyperpigmented nodules over the nose and cheeks
  • subungual fibroma
  • periungual fibroma
    • commonly appear during adolescence
  • treatment
    • seizure control
    • screening for complications
      • renal ultrasound
      • echocardiogram to look for rhabdomyomas
      • ophthalmologist referral to look for hamartomas
    • genetic counseling
32
Q

Sturge-Weber disease

A
  • etiology
    • anomalous development of the vascular bed during early cerebral vascularization
    • overlying leptomeninges richly vascularized and the brain beneath becomes atrophic and calcified
  • clinical manifestations
    • facial nevus (port-wine stain)
    • seizures
    • mental retardation
    • intracranial calcifications
    • hemiparesis
  • port wine stain
    • facial nevus involving upper face, eyelid
    • glaucoma of the ipsilateral eye common complication
  • radiologic diagnosis
    • intracranial calcifications on CT scan
    • unilateral cortical atrophy and ipsilateral dilatation of the lateral ventricle
  • CT scan findings
    • cerebral atrophy
    • intracranial calcifications
  • treatment
    • conservative management if seizures and development problems mild
    • moderate to severe disease
      • recalcitrant seizures – lobectomy
        • controversial decision
      • risk of glaucoma - ophthalmologist intraocular pressure monitoring
      • port wine stain - laser therapy
      • developmental delay – special education
33
Q

Guillain-barre syndrome

A
  • acute inflammatory demyelinating polyradiculoneuropathy
    • Different subtypes exist including axonal GBS, Miller Fischer variant
  • disorder is thought to result from a post-infectious immune mediated process that predominantly affects motor nerves
  • pathophysiology
    • most likely grouping of disorders with peripheral nerve demyelination as a common mechanism
    • occurs after infection in two-thirds of cases
      • Campylobacter sp.
      • CMV, EBV
      • Haemophilus influenzae
      • Mycoplasma pneumoniae
      • viruses
  • clinical manifestations
    • fine paresthesias & tingling of toes and fingertips
    • pain may be a prominent feature
    • ascending weakness/paralysis
      • lower extremity symmetric weakness
      • ascends to arms and cranial nerves
  • exam
    • diminished or absent deep tendon reflexes
    • weakness
    • paralytic ileus
    • poor respiratory effort
    • bladder dysfunction
    • autonomic dysregulation
      • hypertension
      • hypotension
      • tachycardia/bradycardia
      • abnormal sweating
  • diagnosis
    • MRI showing nerve root enhancement frequently seen
    • electrophysiologic studies
    • Specific autoantibodies can be sent
    • CSF
      • elevated protein
  • Treatment
    • IVIG
    • plasmapheresis
    • physical therapy
  • watch for complications
    • respiratory failure
    • nutrition
    • autonomic disturbance
34
Q

transverse myelitis

A
  • inflammation of the spinal cord
  • thought to have a immunologic basis
  • pathogenesis (proposed)
    • cell-mediated immune response
    • direct invasion of the spinal cord
    • autoimmune vasculitis
  • clinical manifestations
    • back pain at level of lesion
    • abrupt onset of progressive weakness; legs weak and flaccid
    • areflexia
    • sensory disturbances in the lower extremities below level of spinal lesion
    • history of preceding viral infection
    • sphincter disturbances
  • laboratory findings
  • lumbar puncture - non-specific
    • increased protein
    • pleocytosis with lymphocytes
  • MRI (often diagnostic)
    • localized edema of area of spinal cord
  • treatment
    • medications (controversial benefit)
      • corticosteroids
      • IVIG
      • plasmapheresis
    • supportive care
35
Q

infant botulism

A
  • acute, flaccid paralytic illness caused by the neurotoxin produced by soil organism Clostridium botulinum
    • Gram-positive spore forming anaerobe
    • found in fresh and cooked agricultural products
      • HONEY - no honey under age 1
    • botulinum toxin is heat labile
  • forms of botulism
    • wound
    • food-borne
    • infantile
  • 95% of cases are between 3 weeks and 6 months of age
  • almost half of cases are from California
    • probably due to soil mixture
    • history of parent working in/with soil - construction, landscaping etc
  • botulinum toxin is carried by the bloodstream to peripheral cholinergic synapses
  • binds irreversibly, blocking acetylcholine release and causing impaired neuromuscular and autonomic transmission
  • loss of motor endplate function
  • recovery requires re-growth of terminal unmyelinated motor neurons
  • symmetric, descending paralysis
  • cranial nerve palsies
    • poor feeding
    • weak suck
    • feeble cry
    • ptosis
    • obstructive apnea
  • laboratory confirmation
    • detection of botulinum toxin in serum
    • detection of Clostridium botulinum in feces
    • CSF normal
  • EMG defect in neuromuscular transmission
  • treatment
    • supportive care
    • tube enteral feeding
    • respiratory support
      • positioning to avoid aspiration
      • mechanical ventilation
  • botulism immune globulin (BIG)
    • reduces duration of illness if given early
36
Q

tics

A
  • quick, repetitive, irregular, briefly suppressible movements
    • facial
      • blinking
      • grimaces
      • twitches
    • trunk and extremities
    • tics occur in 20% or more of school children
    • tics that are chronic occur in 1% of mainstream children
    • usually last 1 month to 1 year
    • stress/anxiety tend to increase the amount of tics
  • treatment
    • teach parents natural history
    • disregard
    • monitor for co-morbidities
      • school problems
      • attention deficit hyperactivity disorder
      • obsessive-compulsive disorder
      • sleep difficulties
37
Q

Tourette’s syndrome

A
  • diagnostic criteria
    • tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than one year
      • during this period there was never a tic-free period of more than three consecutive months.
    • both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently
    • onset before 18 years of age
    • disturbance is not due to the direct physiological effects of a substance or a general medical condition (e.g. Huntington’s disease or post-viral encephalitis)
  • treatment
    • non-pharmacologic
      • counseling
      • adjustment of school and family members
    • medication
      • haloperidol
      • fluoxetine
      • clonidine
38
Q

Spinal muscular atrophy types I, II, III

A
  • group of disorders characterized by degeneration of motor neurons with compensation from re-innervation of an adjacent motor unit
  • upper motor neurons are not involved
39
Q

SMA type I, Wednig-Hoffman disease

A
  • clinical manifestations
    • severe hypotonia
    • generalized weakness
    • thin muscle mass
    • involvement of the tongue (fasciculations)
    • preservation of extraocular muscles
  • laboratory diagnosis
    • serum creatine kinase may be mildly elevated
      • different from muscular dystrophy
    • muscle biopsy - areas of atrophy, adjacent areas of hypertrophy
    • molecular genetic diagnosis
  • treatment
    • no treatment to delay progression
    • functional aids in type II or type III
    • supportive care
      • feeding NG feeds
      • respiratory support
        • oxygen
        • pulmonary toilet
40
Q

derangements of cranial size

A
  • Head size derangements
    • microcephaly
      • head circumference more than 2 standard deviations below the mean for age
    • macrocephaly
      • head circumference more than 2 standard deviations above the mean for age
41
Q

microcephaly

A
  • chromosomal/genetic/metabolic disorder
  • congenital malformation
  • placental insufficiency
  • perinatal hypoxia/trauma
  • toxins (fetal alcohol syndrome, medications, maternal phenylketonuria)
  • infections (TORCH)
  • radiation
  • familial
42
Q

macrocephaly

A
  • differential diagnosis
    • benign familial macrocephaly
    • increased intracranial pressure
      • with dilated ventricles - hydrocephalus
      • with mass effect - tumor, arachnoid cyst, porencephalic cyst
    • megalencephaly (large brain)
      • neurocutaneous disorder
      • gigantism
      • metabolic disorder
      • lysosomal storage disease
      • leukodystrophy – progressive white matter degeneration
    • thickened skull

Pediatrics Module 3 (10 decks)

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