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Kathie Teta, RN, CPNP

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spastic hemiplegia: involves both extremities on one side (upper greater than lower) ... only if physically or psychosocially disabling (start with Clonidine or Tenex) ... – PowerPoint PPT presentation

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Title: Kathie Teta, RN, CPNP


1
Pediatric Neurology Overview
  • Kathie Teta, RN, CPNP
  • Child Neurology Associates


2
Objectives
  • Describe common neurological disorders seen in
    children and adolescents.
  • List imaging studies and lab tests needed for
    diagnosing and treating neurological disorders in
    children and adolescents.
  • Discuss when referral to a pediatric neurology
    specialist is indicated

3
Pediatric Neuroanatomy
4
Pediatric Neuroanatomy
5
Pediatric Neuroanatomy
6
Pediatric Neuroanatomy
7
Definition paroxysmal events thought to
represent abnormal electrical activity in
cerebral neurons
Seizures
  • Pathogenesis
  • - usually idiopathic
  • - other causes

8
Partial or focal seizures - simple - complex
- secondary generalized
Classification of seizures
  • Generalized seizures
  • - tonic-clonic (grand mal) - myoclonic
  • - tonic
    - atonic
  • - absence (petit mal) -
    infantile
  • - atypical absence
    spasms

9
Evaluating Patients with Seizures
  • Description of the seizure
  • Neurologic exam
  • Patient/family history
  • Precipitating factors
  • Imaging studies
  • EEG

10
Evaluating Patients with Seizures
  • Differential diagnosis
  • -Syncope
  • -Vertigo
  • -Tics
  • -Psychogenic symptoms
  • -Breath holding spells
  • -Reflux in infants

11
Pediatric Seizures
  • Plan/Management
  • -Status epilepticus requires transport to ED
  • -Refer to neurologist for accurate
    classification of seizure type
  • -Monotherapy over polytherapy
  • -Monitor CBC, LFTs, drug levels
  • -Treatment for 2 years (seizure free)

12
Seizure ManagementPharmacologic Treatment
  • Partial seizures
  • Oxcarbazepine
  • Carbamazepine
  • Phenytoin
  • Phenobarbital
  • Levetiracetam
  • Lamotrigine
  • Topiramate
  • Absence seizures
  • Ethosuximide
  • Valproic acid

13
Seizure ManagementPharmacologic Treatment
  • Tonic-Clinic Seizures
  • Phenytoin
  • Valproic acid
  • Carbamazepine
  • Topiramate
  • Levetiracetam
  • Myoclonic and Atonic Seizures
  • Clonazepam
  • Lamotrigine
  • - Levetiracetam
  • Infantile Spasms
  • ACTH
  • Topamax

14
Seizure ManagementPharmacologic Treatment
Diastat
15
Diastat
  • Used for seizures longer than 5 minutes in length
    or for clusters of seizures

16
Diastat Dosing
17
Seizure ManagementPharmacologic Treatment
  • Goal of pharmacologic treatment seizure control
    without adverse effects

18
Pediatric SeizuresEducation
  • Seizure first aid and precautions
  • Precipitants of seizures
  • Georgia driving laws
  • Avoid pertussis vaccine if seizures are not well
    controlled

19
Pediatric SeizuresEducation
  • Medication side effects
  • -Common side effects
  • -Serious side effects
  • -rash/Stephens Johnson syndrome
  • -anemia
  • -liver damage
  • -Interaction with oral contraceptives
  • -Interaction with other medications

20
Definition convulsion associated with temps
101 in a child under age 5 to 6 years
Febrile Seizures
  • Pathogenesis
  • - unclear etiology
  • - genetic factors
  • - family history of epilepsy

21
Febrile Seizures
  • Classification
  • Simple - less than 10 minutes
  • - short postictal period
  • - generalized
  • Complex - may reoccur within 24 hours
  • - focal features
  • Atypical - refer to neurologist for evaluation

22
Febrile Seizures
  • Diagnosis/Evaluation
  • Description of the seizure
  • Physical exam
  • Patient/family history
  • Precipitating factors
  • Lab work
  • Lumbar puncture if needed
  • EEG (rare)

23
Febrile Seizures
  • Differential Diagnosis
  • Epilepsy
  • Meningitis/Encephalitis
  • Metabolic disorder

24
Plan/Management
Febrile Seizures
  • Aggressive treatment of fever and illness
  • First aid for seizures

25
Syncope
  • Definition transient loss of consciousness with
    inability to maintain postural tone followed by
    spontaneous recovery

26
Neurocardiogenic syncope (Vasovagal syncope)
  • benign (and the most frequent) cause of fainting.
  • common in children and adolescents.
  • blood pressure drops, reducing circulation to the
    brain and causing loss of consciousness.
  • typically occurs while standing, often preceded
    by a sensation of warmth, nausea, lightheadedness
    and visual "grayout."
  • If the syncope is prolonged, it can trigger a
    seizure. Placing the person in a reclining
    position will restore blood flow and
    consciousness

27
Syncope
  • Diagnostic testing
  • - EKG
  • - EEG if warranted

28
Noncardiac SyncopeCauses
  • Sudden changes in position (orthostatic
    hypotension)
  • Dehydration
  • Hypoglycemia
  • Growth spurts
  • Vestibular disease
  • Seizures

29
Syncope
  • Treatment
  • High salt diet
  • Increased fluid intake
  • Slow change in positions

30
Breath Holding Spells
  • infantile syncope
  • involuntary
  • initiated by a noxious stimulus
  • associated with crying
  • consciousness and posture are lost
  • occurs from infancy through age 5
  • pallid or cyanotic
  • may be associated with convulsive movements

31
Breath holding spells
  • Differential diagnosis
  • Seizures

32
Breath holding spells
  • Treatment
  • Place child flat
  • Try to interrupt the event
  • Do not give extra attention after the event

33
Headaches
  • chronic or intermittent
  • migraine, tension, sinus, or rebound
  • progressive or nonprogressive

34
Definition chronic, recurring headache often
accompanied by nausea, vomiting, photophobia,
and phonophobia
Migraine Headaches
35
Migraine Headaches
  • Pathogenesis
  • - genetic
  • - inflammation of vessels and
    meninges
  • - vascular dilation
  • - excessive muscle contraction
  • - trigeminovascular activation (due to
    decreased serontonin levels)

36
Migraine HeadachesClassification
  • Common no obvious warning period before the
    headache starts
  • Classic 10 to 30 minute warning period
    immediately before the headache

37
Migraine Headaches Characteristics -
unilateral/bilateral - throbbing/pressure
- nausea/vomiting - vertigo -
visual changes - phono/photophobia -
worsens with activity
38
Migraine Headaches
  • Incidence
  • - 1 in 20 children
  • - More common in boys before puberty
  • - more common in girls after puberty

39
Migraine Headaches
  • Headache Triggers
  • Medications -Caffeine overuse
  • Hypoglycemia -OTC med overuse
  • Hormonal changes -Depression
  • Poor fluid intake -Anxiety
  • Stress -Weather changes
  • Sleep (too much/little) -Illness
  • Smells/light/noise -Injury

40
Migraine Headaches

Food Triggers
41
Migraine Headaches
  • Diagnosis/Evaluation
  • History onset, frequency, symptoms, pattern, use
    of medications, family history
  • Physical/neuro exam

42
Migraine Headaches
  • Diagnostic tests
  • - CT scan if warranted
  • - Lab work if concerns with infection, fatigue
  • - Ophthalmology exam

43
Migraine Headaches
  • Differential Diagnosis
  • Infection -Tension headaches
  • Intracranial tumor - Rebound
    headaches
  • Sinus disease - Pseudotumor
  • Depression - Chiari
  • Anxiety Malformation

44
Migraine Headaches
  • Plan/Management
  • -Mild attacks NSAIDs
  • -Moderate/severe attacks
  • - Triptan use
  • - Midrin
  • - Refer to neurologist

45
Migraine Headaches
  • Plan/Management
  • Preventive therapy when child has 2 or more
    headaches per week
  • -Cyproheptadine (Periactin)
  • 0.25mg /kg/day divided, every 8 to 12 hours
  • -Adrenergic blocking agents (Inderal)
  • -Tricyclic antidepressants
  • -Calcium channel blocking agents
  • -Anticonvulsants

46
Migraine Headaches
  • Education
  • Reduce precipitating factors
  • Reduce intake of caffeine
  • Consistent sleep
  • Increased fluids
  • Exercise

47
Migraine Headaches
  • Refer to Neurologist for
  • New onset severe headaches
  • Headaches with vomiting, especially in AM
  • Change in headache pattern type, location,
    frequency or intensity
  • Chronic progressive headaches
  • Chronic nonprogressive headaches

48
Café au Lait Spots
  • Can be marker for neurofibromatosis I or tuberous
    sclerosis
  • May increase in number/size with age

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Neurofibromatosis
  • Genetic disorder of the nervous system that
    primarily affect development and growth or neural
    (nerve) cell tissues.
  • Tumors grow on nerves and cause skin changes and
    bone deformities.
  • 30 50 of new cases arise spontaneously

51
Neurofibromatosis
  • 5 or more lesions greater than 5 mm in
    prepubertal child
  • 6 or more lesions greater than 15 mm in post
    pubertal child
  • Axillary or inguinal freckling
  • May have iris hamartomas or optic glioma
  • Often associated with developmental delay

52
Neurofibromatosis
  • Diagnosis MRI
  • Treatment supportive
  • Treat seizures
  • Surgery for accessible tumors

53
Tuberous Sclerosis
  • Genetic disease that causes tumors to grow in the
    brain and on other vital organs (heart, lungs,
    eyes, kidneys, skin)
  • Lesions develop late infancy/early childhood
  • Shagreen patch (raised lesion) on lower back or
    nape of neck in 50 of affected children by age 15

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Tuberous Sclerosis
  • Usually presents as developmental delay or
    seizures
  • Shortens life expectancy

56
Tuberous Sclerosis
  • Diagnosis CT/MRI
  • ECHO
  • Renal ultrasound
  • Treat symptoms

57
Café au Lait Spots
  • Refer if
  • child has multiple areas of hyperpigmentation
  • child develops new café au lait spots or
    fibromas

58
Definition inclusive term to designate a number
of static neuromotor disorders due to a previous
central motor system insult
Cerebral Palsy
  • Pathogenesis often unknown
  • -prenatal insults genetic, malnutrition,
    infection, anoxia, toxemia
  • -perinatal insults prematurity, anoxia,
    hyperbilirubinemia, IVH
  • -postnatal infections, trauma, toxins

59
Cerebral Palsy
  • Incidence
  • Affects 2.5 in 1000 live births in the United
    States

60
Cerebral Palsy
  • Classifications
  • -Spastic cerebral palsy
  • -spastic quadriplegia involves all 4
    extremities
  • -spastic hemiplegia involves both extremities
    on one side (upper greater than lower)
  • -spastic diplegia involves all 4 extremities
    (lower greater than upper)
  • -spastic paraplegia involves both legs with
    normal upper extremities

61
Cerebral Palsy
  • Classifications
  • -Athetoid cerebral palsy
  • - associated with lesions of the basal ganglia
    or extrapyramidal tract
  • - uncontrolled and uncoordinated movements

62
Cerebral Palsy
  • Classifications
  • Ataxic cerebral palsy
  • - associated with a cerebellar lesion
  • - early hypotonia and decreased tendon
    reflexes
  • -Mixed cerebral palsy

63
Cerebral PalsyClinical Presentation
  • asymmetry of movement
  • hypotonia
  • hypertonia
  • early handedness
  • suck/swallow difficulties
  • prolonged drooling
  • persistent toe walking
  • falls easily
  • delayed milestones
  • decreased spontaneous movement

64
Cerebral Palsy
  • Diagnosis/Evaluation
  • -history prenatal, perinatal and postnatal
  • onset of symptoms
  • -clinical evaluation
  • - general
  • - developmental
  • - strength/tone

65
Cerebral Palsy
  • Differential Diagnosis
  • -progressive CNS lesions
  • -metabolic disorders (with hypotonia)
  • -mitochondrial disorders
  • -Prader-Willi syndrome
  • -Rett syndrome
  • -myotonic dystrophy
  • -developmental delay

66
Cerebral Palsy
  • Plan/Management
  • -Referral to neurology for complete evaluation
  • -Therapy services Early Intervention/Babies
    Cant Wait
  • -Audiological evaluation if speech delays

67
Cerebral Palsy
  • Neurological Plan/Management
  • -metabolic testing
  • -chromosomes/genetic testing
  • -adaptive equipment/orthotics
  • -medications for spasticity
  • -education
  • -referral to orthopedics if warranted
  • -imaging studies

68
Definition Failure to reach developmental
milestones within an accepted time range
Developmental Delay
  • Pathogenesis
  • - often unknown
  • - prematurity
  • - hypotonia
  • - cerebral palsy
  • - chromosomal abnormalities

69
Developmental Delay
  • Diagnosis/Evaluation

-history prenatal, perinatal and postnatal
onset of symptoms -clinical evaluation -
general (any regression?) - developmental
- strength/tone
70
Developmental Delay
  • Differential Diagnosis
  • -progressive CNS lesions
  • -metabolic disorders (with hypotonia)
  • -mitochondrial disorders
  • -Prader-Willi syndrome
  • -Rett syndrome
  • -myotonic dystrophy
  • -cerebral palsy
  • -autism/pervasive developmental disorder

71
Developmental Delay
  • Plan/Management
  • -Referral to neurology for complete evaluation
  • -Therapy services Early Intervention/Babies
    Cant Wait
  • -Audiological evaluation if speech delays

72
Developmental Delay
  • Neurological Plan/Management

-metabolic testing -chromosomes/genetic
testing -adaptive equipment/orthotics -therapy
services -imaging studies -education
73
Cranial disorders
  • - Macrocephaly- Microcephaly- Plagiocephaly-
    Crainiosynostosis

74
Macrocephaly
  • Monitor head circumference
  • Check fontanelle full, soft, bulging
  • Signs of increased ICP vomiting, sunset eyes,
    irritability
  • Genetic disposition?
  • CT scan to rule out hydrocephalus

75
Microcephaly
  • Monitor head circumference
  • Check fontanelle premature closure?

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Plagiocephaly- flattening of one side of the
skull
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Craniosynostosis
  • Premature closing of the sutures of the skull

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Definition disturbance of central motor control
manifested in alterations of posture and
movement
Movement Disorders
  • -usually a secondary disorder that is, often
    associated with lesions of the brain such as
    cerebral palsy, brain injury or stroke than
    occurring as a primary disorder

83
Movement Disorders
  • Etiology
  • Can be inherited or acquired
  • Cerebral palsy most common etiology
  • 80 have movement disorders
  • -PANDAS (Pediatric Autoimmune Neuropsychiatric
    Disorders Associated with Strep infections)

84
Movement DisordersClassifications
  • Spasticity
  • Dystonia
  • Athetosis
  • Chorea
  • Myoclonus
  • Tic disorder
  • Tremor
  • Ataxia
  • Rigidity

85
Movement DisordersChorea
  • Hyperkinetic movement disorder with involuntary,
    random, quick jerking movements
  • Movements are abrupt, nonrepetitive, and
    arrhythmic with variable frequency and intensity
  • Most commonly seen in proximal limbs, neck,trunk
    and facial muscles
  • May worsen with voluntary movements

86
Movement DisordersChorea - Common Causes
  • Cerebral palsy
  • Medication-induced
  • Sydenhams chorea
  • Check for strep infection

87
Movement DisordersMyoclonus
  • Sudden shocklike involuntary muscle jerk that may
    affect a single body region, one side of the
    body, or the entire body
  • Can be repetitive
  • Can be normal (sleep myoclonus) or abnormal
  • May originate in the spinal cord, brain stem or
    cerebral cortex

88
Movement DisordersTics
  • Sudden, involuntary, repetitive, rapid, random,
    purposeless, highly stereotyped movements or
    abnormal sounds
  • Simple or complex
  • Most common primary movement disorder in
    childhood
  • Transient or chronic
  • Motor and vocal

89
Tic Disorders
  • Tourette syndrome motor and vocal tics present
    for greater than one year
  • Treatment
  • - only if physically or psychosocially
    disabling (start with Clonidine or Tenex)
  • - do not draw attention to tics

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Movement DisordersTremor
  • Involuntary, rhythmic oscillation of at least one
    body region
  • Can occur during rest or action
  • Incidence in general population is 1.5 in 10,000
  • Check TSH and T4 free
  • Eliminate caffeine from diet

92
Movement Disorders
  • Diagnosis/Evaluation
  • History
  • Physical/neuro evaluation
  • Direct observation/videotape review
  • EEG to rule out seizures
  • Refer to neurology for further evaluation and
    diagnosis

93
Movement Disorders
  • Plan/Management
  • Pharmacologic approach
  • Oral pharmacotherapy
  • -Injectable neuromuscular blockade
  • Surgical approach
  • Orthopedic surgery
  • Selective dorsal rhizotomy
  • -Intrathecal baclofen pump
  • -Deep brain stimulation/stereotactic lesions

94
Brachial Plexus Injury (Erbs
Palsy)
95
Brachial Plexus Injury (Erbs Palsy)
  • Types
  • Avulsion
  • Rupture
  • Neuroma
  • Stretch most common

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Brachial Plexus Injury (Erbs Palsy)
  • Treatment
  • Physical therapy
  • Surgery - for avulsion and rupture injuries
    (needs to occur at young age)
  • Prognosis - depends on site and type of injury

98
Weakness/Myopathy
99
Myopathy
  • Can be genetic, inflammatory, progressive
  • Muscular dystrophy
  • Congenital myopathies
  • Mitochondrial disorder
  • Metabolic disorder
  • Endocrine disorder

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Myopathy
  • Diagnostic tests
  • MRI of the Brain
  • Metabolic testing
  • EMG
  • Muscle biopsy

102
Myopathy
  • Treatment
  • Treat symptoms
  • Therapies
  • Adaptive equipment

103
Neuropathy
  • Any disease that affects any part of the nervous
    system
  • Peripheral neuropathy most common, affects feet
    and legs
  • Causes 30 70 idiopathic

104
Neuropathy
  • Description
  • Symmetric/asymmetric
  • Proximal or distal
  • Acute/chronic
  • Rapid or slow onset

105
Neuropathy
  • Symptoms
  • Pain
  • Weakness
  • Cramping
  • Decreased reflexes
  • Parasthesias
  • Allodynias

106
Neuropathy
  • Diagnostic test EMG
  • Treatment start with NSAIDs

107
Concussion/Head Injury
108
Head Injury
  • Mild head injury - temporary loss of
    consciousness (concussion)
  • Moderate head injury obtunded for several hours
  • Severe in a coma

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ConcussionGrading Scale
  • Grade 1
  • Transient confusion
  • No loss of consciousness
  • Symptoms resolve in less than 15 minutes

111
ConcussionGrading Scale
  • Grade 2
  • Transient confusion
  • No loss of consciousness
  • Symptoms last more than 15 minutes

112
ConcussionGrading Scale
  • Grade 3
  • Any loss of consciousness, brief or prolonged

113
Concussion
  • Symptoms
  • Fatigue
  • Amnesia regarding events around the injury
  • Headache
  • Dizziness
  • Irritability (behavior changes)
  • Memory disturbances
  • Seizures (rarely associated with later epilepsy)

114
Concussion
  • Diagnostic testing
  • CT of head
  • EEG if suspicion of seizure
  • Neuropsychological evaluation (for memory issues)
  • Management treat symptoms

115
Concussion
  • Refer for
  • Prolonged headaches/dizziness
  • Memory disturbances
  • Seizures

116
ConcussionReturn to Sports
117
Grade of concussion Time until return
to play
  • Multiple grade 1 1 week
  • Grade 2 concussion 1 week
  • Multiple grade 2 2 weeks
  • Grade 3 brief loss 2 weeks
  • of consciousness (seconds)
  • Multiple grade 3 1 month or longer

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