Title: Kathie Teta, RN, CPNP
1Pediatric Neurology Overview
- Kathie Teta, RN, CPNP
- Child Neurology Associates
2Objectives
- 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
3Pediatric Neuroanatomy
4Pediatric Neuroanatomy
5Pediatric Neuroanatomy
6Pediatric Neuroanatomy
7Definition paroxysmal events thought to
represent abnormal electrical activity in
cerebral neurons
Seizures
- Pathogenesis
- - usually idiopathic
- - other causes
8Partial 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 -
9Evaluating Patients with Seizures
- Description of the seizure
- Neurologic exam
- Patient/family history
- Precipitating factors
- Imaging studies
- EEG
10Evaluating Patients with Seizures
- Differential diagnosis
- -Syncope
- -Vertigo
- -Tics
- -Psychogenic symptoms
- -Breath holding spells
- -Reflux in infants
11Pediatric 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)
12Seizure ManagementPharmacologic Treatment
- Partial seizures
- Oxcarbazepine
- Carbamazepine
- Phenytoin
- Phenobarbital
- Levetiracetam
- Lamotrigine
- Topiramate
- Absence seizures
- Ethosuximide
- Valproic acid
13Seizure ManagementPharmacologic Treatment
- Tonic-Clinic Seizures
- Phenytoin
- Valproic acid
- Carbamazepine
- Topiramate
- Levetiracetam
- Myoclonic and Atonic Seizures
- Clonazepam
- Lamotrigine
- - Levetiracetam
- Infantile Spasms
- ACTH
- Topamax
14Seizure ManagementPharmacologic Treatment
Diastat
15Diastat
- Used for seizures longer than 5 minutes in length
or for clusters of seizures
16Diastat Dosing
17Seizure ManagementPharmacologic Treatment
- Goal of pharmacologic treatment seizure control
without adverse effects
18Pediatric SeizuresEducation
- Seizure first aid and precautions
- Precipitants of seizures
- Georgia driving laws
- Avoid pertussis vaccine if seizures are not well
controlled
19Pediatric 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
20Definition 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
21Febrile 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
-
22Febrile Seizures
- Diagnosis/Evaluation
- Description of the seizure
- Physical exam
- Patient/family history
- Precipitating factors
- Lab work
- Lumbar puncture if needed
- EEG (rare)
23Febrile Seizures
- Differential Diagnosis
- Epilepsy
- Meningitis/Encephalitis
- Metabolic disorder
24Plan/Management
Febrile Seizures
- Aggressive treatment of fever and illness
- First aid for seizures
25Syncope
- Definition transient loss of consciousness with
inability to maintain postural tone followed by
spontaneous recovery
26Neurocardiogenic 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
27Syncope
- Diagnostic testing
- - EKG
- - EEG if warranted
28Noncardiac SyncopeCauses
- Sudden changes in position (orthostatic
hypotension) - Dehydration
- Hypoglycemia
- Growth spurts
- Vestibular disease
- Seizures
29Syncope
- Treatment
- High salt diet
- Increased fluid intake
- Slow change in positions
30Breath 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
31Breath holding spells
- Differential diagnosis
- Seizures
32Breath holding spells
- Treatment
- Place child flat
- Try to interrupt the event
- Do not give extra attention after the event
33Headaches
- chronic or intermittent
- migraine, tension, sinus, or rebound
- progressive or nonprogressive
34Definition chronic, recurring headache often
accompanied by nausea, vomiting, photophobia,
and phonophobia
Migraine Headaches
35Migraine Headaches
- Pathogenesis
- - genetic
- - inflammation of vessels and
meninges - - vascular dilation
- - excessive muscle contraction
- - trigeminovascular activation (due to
decreased serontonin levels)
36Migraine 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
38Migraine Headaches
- Incidence
- - 1 in 20 children
- - More common in boys before puberty
- - more common in girls after puberty
39Migraine 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
40Migraine Headaches
Food Triggers
41Migraine Headaches
- Diagnosis/Evaluation
- History onset, frequency, symptoms, pattern, use
of medications, family history - Physical/neuro exam
42Migraine Headaches
- Diagnostic tests
- - CT scan if warranted
- - Lab work if concerns with infection, fatigue
- - Ophthalmology exam
43Migraine Headaches
- Differential Diagnosis
- Infection -Tension headaches
- Intracranial tumor - Rebound
headaches - Sinus disease - Pseudotumor
- Depression - Chiari
- Anxiety Malformation
-
44Migraine Headaches
- Plan/Management
- -Mild attacks NSAIDs
- -Moderate/severe attacks
- - Triptan use
- - Midrin
- - Refer to neurologist
45Migraine 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
46Migraine Headaches
- Education
- Reduce precipitating factors
- Reduce intake of caffeine
- Consistent sleep
- Increased fluids
- Exercise
47Migraine 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
48Café au Lait Spots
- Can be marker for neurofibromatosis I or tuberous
sclerosis - May increase in number/size with age
49(No Transcript)
50Neurofibromatosis
- 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
51Neurofibromatosis
- 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
52Neurofibromatosis
- Diagnosis MRI
- Treatment supportive
- Treat seizures
- Surgery for accessible tumors
53Tuberous 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
54(No Transcript)
55Tuberous Sclerosis
- Usually presents as developmental delay or
seizures - Shortens life expectancy
56Tuberous Sclerosis
- Diagnosis CT/MRI
- ECHO
- Renal ultrasound
- Treat symptoms
57Café au Lait Spots
- Refer if
- child has multiple areas of hyperpigmentation
- child develops new café au lait spots or
fibromas
58Definition 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
59Cerebral Palsy
- Incidence
- Affects 2.5 in 1000 live births in the United
States
60Cerebral 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
61Cerebral Palsy
- Classifications
- -Athetoid cerebral palsy
- - associated with lesions of the basal ganglia
or extrapyramidal tract - - uncontrolled and uncoordinated movements
62Cerebral Palsy
- Classifications
- Ataxic cerebral palsy
- - associated with a cerebellar lesion
- - early hypotonia and decreased tendon
reflexes -
- -Mixed cerebral palsy
63Cerebral PalsyClinical Presentation
- asymmetry of movement
- hypotonia
- hypertonia
- early handedness
- suck/swallow difficulties
- prolonged drooling
- persistent toe walking
- falls easily
- delayed milestones
- decreased spontaneous movement
64Cerebral Palsy
- Diagnosis/Evaluation
- -history prenatal, perinatal and postnatal
- onset of symptoms
- -clinical evaluation
- - general
- - developmental
- - strength/tone
-
65Cerebral Palsy
- Differential Diagnosis
- -progressive CNS lesions
- -metabolic disorders (with hypotonia)
- -mitochondrial disorders
- -Prader-Willi syndrome
- -Rett syndrome
- -myotonic dystrophy
- -developmental delay
66Cerebral Palsy
- Plan/Management
- -Referral to neurology for complete evaluation
- -Therapy services Early Intervention/Babies
Cant Wait - -Audiological evaluation if speech delays
67Cerebral Palsy
- Neurological Plan/Management
- -metabolic testing
- -chromosomes/genetic testing
- -adaptive equipment/orthotics
- -medications for spasticity
- -education
- -referral to orthopedics if warranted
- -imaging studies
68Definition Failure to reach developmental
milestones within an accepted time range
Developmental Delay
- Pathogenesis
- - often unknown
- - prematurity
- - hypotonia
- - cerebral palsy
- - chromosomal abnormalities
-
69Developmental Delay
-history prenatal, perinatal and postnatal
onset of symptoms -clinical evaluation -
general (any regression?) - developmental
- strength/tone
70Developmental 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
71Developmental Delay
- Plan/Management
- -Referral to neurology for complete evaluation
- -Therapy services Early Intervention/Babies
Cant Wait - -Audiological evaluation if speech delays
72Developmental Delay
- Neurological Plan/Management
-metabolic testing -chromosomes/genetic
testing -adaptive equipment/orthotics -therapy
services -imaging studies -education
73Cranial disorders
- - Macrocephaly- Microcephaly- Plagiocephaly-
Crainiosynostosis
74Macrocephaly
- Monitor head circumference
- Check fontanelle full, soft, bulging
- Signs of increased ICP vomiting, sunset eyes,
irritability - Genetic disposition?
- CT scan to rule out hydrocephalus
75Microcephaly
- Monitor head circumference
- Check fontanelle premature closure?
76(No Transcript)
77(No Transcript)
78Plagiocephaly- flattening of one side of the
skull
79(No Transcript)
80Craniosynostosis
- Premature closing of the sutures of the skull
81(No Transcript)
82Definition 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
83Movement 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)
84Movement DisordersClassifications
- Spasticity
- Dystonia
- Athetosis
- Chorea
- Myoclonus
- Tic disorder
- Tremor
- Ataxia
- Rigidity
85Movement 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
86Movement DisordersChorea - Common Causes
- Cerebral palsy
- Medication-induced
- Sydenhams chorea
- Check for strep infection
87Movement 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
88Movement 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
89Tic 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
90(No Transcript)
91Movement 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
92Movement Disorders
- Diagnosis/Evaluation
- History
- Physical/neuro evaluation
- Direct observation/videotape review
- EEG to rule out seizures
- Refer to neurology for further evaluation and
diagnosis
93Movement 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)
95Brachial Plexus Injury (Erbs Palsy)
- Types
- Avulsion
- Rupture
- Neuroma
- Stretch most common
96(No Transcript)
97Brachial 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
99Myopathy
- Can be genetic, inflammatory, progressive
- Muscular dystrophy
- Congenital myopathies
- Mitochondrial disorder
- Metabolic disorder
- Endocrine disorder
100(No Transcript)
101Myopathy
- Diagnostic tests
- MRI of the Brain
- Metabolic testing
- EMG
- Muscle biopsy
102Myopathy
- Treatment
- Treat symptoms
- Therapies
- Adaptive equipment
103Neuropathy
- Any disease that affects any part of the nervous
system - Peripheral neuropathy most common, affects feet
and legs - Causes 30 70 idiopathic
104Neuropathy
- Description
- Symmetric/asymmetric
- Proximal or distal
- Acute/chronic
- Rapid or slow onset
105Neuropathy
- Symptoms
- Pain
- Weakness
- Cramping
- Decreased reflexes
- Parasthesias
- Allodynias
106Neuropathy
- Diagnostic test EMG
- Treatment start with NSAIDs
107 Concussion/Head Injury
108Head Injury
- Mild head injury - temporary loss of
consciousness (concussion) - Moderate head injury obtunded for several hours
- Severe in a coma
109(No Transcript)
110ConcussionGrading Scale
- Grade 1
- Transient confusion
- No loss of consciousness
- Symptoms resolve in less than 15 minutes
111ConcussionGrading Scale
- Grade 2
- Transient confusion
- No loss of consciousness
- Symptoms last more than 15 minutes
112ConcussionGrading Scale
- Grade 3
- Any loss of consciousness, brief or prolonged
113Concussion
- Symptoms
- Fatigue
- Amnesia regarding events around the injury
- Headache
- Dizziness
- Irritability (behavior changes)
- Memory disturbances
- Seizures (rarely associated with later epilepsy)
114Concussion
- Diagnostic testing
- CT of head
- EEG if suspicion of seizure
- Neuropsychological evaluation (for memory issues)
- Management treat symptoms
115Concussion
- Refer for
- Prolonged headaches/dizziness
- Memory disturbances
- Seizures
116ConcussionReturn to Sports
117Grade 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
-
118(No Transcript)
119(No Transcript)