Kathie Teta, RN, CPNP

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Pediatric Neurology Overview

• Kathie Teta, RN, CPNP
• Child Neurology Associates

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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

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Pediatric Neuroanatomy
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Pediatric Neuroanatomy
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Pediatric Neuroanatomy
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Pediatric Neuroanatomy
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Definition paroxysmal events thought to
represent abnormal electrical activity in
cerebral neurons
Seizures

• Pathogenesis
• - usually idiopathic
• - other causes

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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

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Evaluating Patients with Seizures

• Description of the seizure
• Neurologic exam
• Patient/family history
• Precipitating factors
• Imaging studies
• EEG

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Evaluating Patients with Seizures

• Differential diagnosis
• -Syncope
• -Vertigo
• -Tics
• -Psychogenic symptoms
• -Breath holding spells
• -Reflux in infants

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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)

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Seizure ManagementPharmacologic Treatment

• Partial seizures
• Oxcarbazepine
• Carbamazepine
• Phenytoin
• Phenobarbital
• Levetiracetam
• Lamotrigine
• Topiramate

• Absence seizures
• Ethosuximide
• Valproic acid

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Seizure ManagementPharmacologic Treatment

• Tonic-Clinic Seizures
• Phenytoin
• Valproic acid
• Carbamazepine
• Topiramate
• Levetiracetam

• Myoclonic and Atonic Seizures
• Clonazepam
• Lamotrigine
• - Levetiracetam
• Infantile Spasms
• ACTH
• Topamax

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Seizure ManagementPharmacologic Treatment
Diastat
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Diastat

• Used for seizures longer than 5 minutes in length
  or for clusters of seizures

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Diastat Dosing
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Seizure ManagementPharmacologic Treatment

• Goal of pharmacologic treatment seizure control
  without adverse effects

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Pediatric SeizuresEducation

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

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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

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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

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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

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Febrile Seizures

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

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Febrile Seizures

• Differential Diagnosis
• Epilepsy
• Meningitis/Encephalitis
• Metabolic disorder

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Plan/Management
Febrile Seizures

• Aggressive treatment of fever and illness
• First aid for seizures

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Syncope

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

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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

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Syncope

• Diagnostic testing
• - EKG
• - EEG if warranted

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Noncardiac SyncopeCauses

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

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Syncope

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

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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

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Breath holding spells

• Differential diagnosis
• Seizures

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Breath holding spells

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

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Headaches

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

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Definition chronic, recurring headache often
accompanied by nausea, vomiting, photophobia,
and phonophobia
Migraine Headaches
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Migraine Headaches

• Pathogenesis
• - genetic
• - inflammation of vessels and
  meninges
• - vascular dilation
• - excessive muscle contraction
• - trigeminovascular activation (due to
  decreased serontonin levels)

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Migraine HeadachesClassification

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

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Migraine Headaches Characteristics -
unilateral/bilateral - throbbing/pressure
- nausea/vomiting - vertigo -
visual changes - phono/photophobia -
worsens with activity
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Migraine Headaches

• Incidence
• - 1 in 20 children
• - More common in boys before puberty
• - more common in girls after puberty

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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

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Migraine Headaches

Food Triggers
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Migraine Headaches

• Diagnosis/Evaluation
• History onset, frequency, symptoms, pattern, use
  of medications, family history
• Physical/neuro exam

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Migraine Headaches

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

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Migraine Headaches

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

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Migraine Headaches

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

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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

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Migraine Headaches

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

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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

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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

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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

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Neurofibromatosis

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

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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

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Tuberous Sclerosis

• Diagnosis CT/MRI
• ECHO
• Renal ultrasound
• Treat symptoms

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Café au Lait Spots

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

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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

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Cerebral Palsy

• Incidence
• Affects 2.5 in 1000 live births in the United
  States

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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

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Cerebral Palsy

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

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Cerebral Palsy

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

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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

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Cerebral Palsy

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

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Cerebral Palsy

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

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Cerebral Palsy

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

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Cerebral Palsy

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

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Definition Failure to reach developmental
milestones within an accepted time range
Developmental Delay

• Pathogenesis
• - often unknown
• - prematurity
• - hypotonia
• - cerebral palsy
• - chromosomal abnormalities

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Developmental Delay

• Diagnosis/Evaluation

-history prenatal, perinatal and postnatal
onset of symptoms -clinical evaluation -
general (any regression?) - developmental
- strength/tone
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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

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Developmental Delay

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

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Developmental Delay

• Neurological Plan/Management

-metabolic testing -chromosomes/genetic
testing -adaptive equipment/orthotics -therapy
services -imaging studies -education
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Cranial disorders

• - Macrocephaly- Microcephaly- Plagiocephaly-
  Crainiosynostosis

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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

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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

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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)

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Movement DisordersClassifications

• Spasticity
• Dystonia
• Athetosis
• Chorea
• Myoclonus

• Tic disorder
• Tremor
• Ataxia
• Rigidity

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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

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Movement DisordersChorea - Common Causes

• Cerebral palsy
• Medication-induced
• Sydenhams chorea
• Check for strep infection

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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

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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

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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

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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

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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

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Brachial Plexus Injury (Erbs
Palsy)
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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

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Weakness/Myopathy
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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

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Myopathy

• Treatment
• Treat symptoms
• Therapies
• Adaptive equipment

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Neuropathy

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

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Neuropathy

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

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Neuropathy

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

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Neuropathy

• Diagnostic test EMG
• Treatment start with NSAIDs

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Concussion/Head Injury
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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

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ConcussionGrading Scale

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

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ConcussionGrading Scale

• Grade 3
• Any loss of consciousness, brief or prolonged

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Concussion

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

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Concussion

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

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Concussion

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

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ConcussionReturn to Sports
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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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