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1
Q

What is hypotonia?

How is this different from weakness?

A
  • Decreased resistance of movement during passive stretching of muscles
  • Weakness is the decreased or less than normal force generated by the active contraction of muscles
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2
Q

Central vs. Peripheral Hypotonia

A
  • Central hypotonia
    • Dysfunction of the upper motor neurons
    • Cortical pyramidal neurons & their descending corticospinal pathways
  • Peripheral hypotonia
    • Dysfunction of the lower motor neurons
    • Spinal motor neurons & distally to muscle fibers
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3
Q

History in a hypotonic infant may reveal….

A
  • Antenatal or neonatal problems
  • Peripheral hypotonia: decreased fetal movements & breech presentation
  • Central hypotonia: seizures in the neonatal period
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4
Q

What are the physical exam findings in hypotonia?

A
  • Weak cry, decreased spontaneous movement, frog-leg posture, muscle contractures
  • Central hypotonia
    • Altered level of consciousness
    • Decreased DTRs, ankle clonus
  • Peripheral hypotonia
    • Consciousness unaffected
    • Muscle bulk & DTRs decreased
  • Congenital neuromuscular disorder pts
    • Bilateral ptosis, ophthalmoplegia, flat mid-face, fish-shaped mouth, high-arched palate, chest wall abnormalities (bell-shaped chest, pectus excavatum, carinatum), bilateral cryptorchidism
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5
Q

What is the etiology of hypotonia?

A
  • Systemic pathology
    • Sepsis, electrolyte abnormalities, hepatic or renal encephalopathy
  • Neural pathology
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6
Q

What is the differential diagnosis of central hypotonia?

A
  • Acquired
    • Electrolyte abnormality
    • Hypoxic-ischemic injury
    • Infection
      • Sepsis vs. Meningitis
    • Intracranial hemorrhage
    • Trauma (cerebral, cervical)
  • Congenital
    • Cerebral malformation
    • Chromosomal disorder
      • Down syndrome
      • Prader-Willi syndrome
    • Metabolic disorder
      • Urea cycle defects
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7
Q

What is the differential diagnosis of peripheral hypotonia?

A
  • Spinal cord
    • Spinal muscular atrophy
  • Peripheral nerves
    • Familial dysautonomia
  • Neuromuscular junction
    • Botulism
    • Neonatal myasthenia
    • Magnesium toxicity
  • Muscle
    • Congenital muscular dystrophy
    • Congenital myotonic dystrophy
    • Metabolic myopathy
      • Pompe’s disease
      • Phosphofructokinase deficiency
    • Structural myopathy
      • Central core disease
      • Nemaline rod myopathy
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8
Q

When evaluating hypotonia, _______________ must be ruled out.

A
  • acute life-threatening causes
  • sepsis, meningitis, acute metabolic disorder
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9
Q

When central hypotonia is suspected, what 3 things need to be done?

A
  • Head CT scan
    • Rule out acute CNS injury or congenital malformation
  • Serum electrolytes
    • Ca, Mg, ammonia, lactate, pyruvate
    • Rule out metabolic disorders
  • High-resolution chromosome studies
    • FISH tests for suspected genetic disorders
    • ex: Prader-Willi syndrome
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10
Q

When peripheral hypotonia is suspected, what 4 things need to be done?

A
  • Serum creatinine kinase (CK) levels
  • DNA tests for spinal muscular atrophy
  • EMG: nerve conduction studies crucial to identify myasthenic disorders
  • Muscle biopsy
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11
Q

Spinal muscular atrophy (SMA)

  • definition
  • epidemiology
A
  • Anterior horn cell degeneration
  • Hypotonia, weakness, tongue fasciculations
  • 1 in 10,000-25,000 live births
  • 2nd most common hereditary neuromuscular disorder after Duchenne’s
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12
Q

What is the classification system for spinal muscular atrophy?

A
  • Type I
    • Infantile form
    • Onset <6 mo
    • Werdnig-Hoffman disease
  • Type II
    • Intermediate form
    • 6-12 mo
  • Type III
    • Juvenile form
    • >3 YO
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13
Q

What is the etiology of spinal muscular atrophy?

A
  • Autosomal recessive
  • Mutations in survival motor neuron gene (SMN1) on chromosome 5
  • Pathology of spinal cord
    • Degeneration & loss of anterior horn neurons
    • Infiltration of microglia & astrocytes
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14
Q

What are the clinical features of spinal muscular atrophy?

A
  • Weak cry, tongue fasciculations
    • Difficulty sucking & swallowing
  • Bell-shaped chest
  • Frog-leg posture when in supine position
    • Generalized hypotonia, weakness, areflexia
  • Normal extraocular movements & normal sensory exam
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15
Q

How is spinal muscular atrophy diagnosed?

A
  • DNA testing
    • Diagnostic >90% of cases
  • Muscle biopsy
    • Atrophy of groups of muscle fibers that were innervated by damaged axons
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16
Q

What is the management & prognosis of spinal muscular atrophy?

A
  • Treatment is supportive
    • Gastrostomy tube feeding
    • Diligent surveillance & therapy of respiratory infections
    • Physical therapy to maintain ROM
  • No cure
  • Prognosis
    • SMA type I: survival >1 YO unusual
      • Death: respiratory insufficiency or pneumonia
    • SMA types II & III: survival until adolescence & adulthood
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17
Q

What is the definition of infantile botulism?

What is the etiology?

A
  • Bulbar weakness & paralysis during 1st yr
  • Secondary to ingestion of Clostridium botulinum species & absorption of botulism toxin
  • Source
    • Infected foods: honey, spores
    • Toxin prevents presynaptic release of ACh
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18
Q

What are the clinical features of infantile botulism?

A
  • Onset of symptoms 12-48 hrs after ingestion
  • Classic first symptom: constipation
  • Neurologic symptoms
    • Weak cry & suck
    • Loss of previously obtained motor milestones
    • Opthalmoplegia, hyporeflexia
  • Paralysis: symmetric & descending
    • Diaphragmatic paralysis can occur
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19
Q

How is infantile botulism diagnosed?

A
  • Suggestive hx, neuro exam, toxin in stool
  • EMG: brief, small-amplitude muscle potentials
    • Incremental response during high-frequency stimulation
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20
Q

How is infantile botulism managed?

A
  • Treatment is supportive
    • Nasogastric feeding
    • Assisted ventilation
  • Botulism immune globulin
  • Antibiotics CONTRAINDICATED
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21
Q

What is congenital myotonic dystrophy?

What is the epidemiology?

A
  • Myotonia: inability to relax contracted muscles
  • Autosomal dominant muscle disorder
  • Presents in newborn period
  • Weakness & hypotonia
  • 1 in 30,000 live births
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22
Q

What is the etiology of congenital myotonic dystrophy?

A
  • Trinucleotide repeat disorder
  • Autosomal dominant, variable penetrance
  • Chromosome 19
  • Transmission through infected mothers (>90%)
  • Earlier onset in mother, more likely will transfer
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23
Q

Congential myotonic dystrophy

  • antenatal hx
  • neonatal hx
  • physical exam
  • adulthood
  • additional problems
A
  • Antenatal hx
    • Polyhydramnios: poor swalling in utero & decreased fetal movements
  • Neonatal hx
    • Feeding & respiratory problems
  • Physical exam
    • Facial diplegia (bilateral weakness), hypotonia, areflexia, arthrogryposis (multiple joint contractures)
  • Myotonia not always present in newborn
    • Later, by 5 YO
  • Adulthood
    • Myotonic facies (atrophy of masseter & temporalis muscles), ptosis, stiff straight smile, inability to release grip after hand shaking (myotonia)
  • Additonal problems
    • Mental retardation, cataracts, cardiac arrhythmias, infertility
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24
Q

How is congenital myotonic dystrophy diagnosed?

A
  • Should be suspected in all infants w/ hypotonia
    • Mother should also be examined
  • DNA testing
    • EMG & muscle biopsy no longer indicated
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25
Q

Congential Myotonic Dystrophy

management

prognosis

A
  • Treatment is supportive
    • Infants may require assisted ventilation & gastrostomy tube feedings
  • Outlook is guarded
    • Infant mortality up to 40% (resp problems)
    • All survivors have mental retardation
    • Average IQ 50-65
    • Feeding problems subside w/ time
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26
Q

What is Hydrocephalus?

A
  • Increased CSF under pressure w/i ventricles
  • Results from:
    • Blockage of CSF flow
    • Decreased CSF absorption
    • Increased CSF production (rare)
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27
Q

What are the 3 types of hydrocephalus?

A
  • Noncommunicating hydrocephalus
    • Enlarged ventricles
    • Obstruction of CSF flow through ventricles
    • ex: aqueductal stenosis
  • Communicating hydrocephalus
    • Enlarged ventricles
    • Increased production of CSF (tumors)
    • Decreased absorption of CSF (bacterial meningitis)
  • Hydrocephalus ex vacuo
    • ​Ventricular enlargement caused by brain atrophy
      *
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28
Q

What are the congenital causes of hydrocephalus?

A
  • Chiari Type II malformation
  • Dandy-Walker malformation
  • Congenital acqueductal stenosis
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29
Q

What are the acquired causes of hydrocephalus?

A
  • Intraventicular hemorrhage (preterm infants)
  • Bacterial meningitis
  • Brain tumors
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30
Q

What is Chiari type II malformation?

A
  • Downward displacement of the cerebellum & medulla through the foramen magnum
  • Blocks CSF flow
  • Associated w/ lumbosacral myelomeningocele
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31
Q

What is Dandy-Walker malformation?

A
  • Absent or hypoplastic cerebellar vermis
  • Cystic enlargement of the 4th ventricle
  • Blocks flow of CSF
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32
Q

What is Congenital Aqueductal Stenosis?

A
  • X-linked trait
  • Thumb abnormalities
  • CNS abnormalities: spina bifida
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33
Q

The main clinical feature of hydrocephalus is _____.

A

increasing head circumference

>97% for age

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34
Q

What are the clinical features of hydrocephalus for infants?

A
  • Infants w/ open cranial sutures
  • Large anterior & posterior fontanelles & split sutures
  • Sunset sign
    • Tonic downward deviation of both eyes
    • Pressure from enlarged 3rd ventricles on the upward gaze center in the midbrain
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35
Q

What are the clinical features of hydrocephalus for older children?

A
  • Closed cranial sutures
  • Increased ICP
    • Headache
    • Nausea & vomiting
    • Unilateral 6th nerve palsy
    • Papilledema
    • Brisk DTRs but w/ a usually downward plantar response
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36
Q

How is hydrocephalus evaluated & managed?

A
  • Increasing head circumference & signs/symptoms of increased ICP
    • Urgent head CT scan
  • Surgical placement of ventriculoperitoneal shunt
    • Divert flow of CSF
    • Complications: shunt infection/obstruction
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37
Q

What is the prognosis of hydrocephalus?

A
  • Pts w/ aqueductal stenosis
    • Best cognitive outcome
  • Pts w/ Chiari type II malformation
    • Low-normal intelligence
    • Language disorders
  • Pts w/ X-linked hydrocephalus
    • Severe mental retardation
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38
Q

What is spina bifida?

A

Any failure of bone fusion in the posterior midline of the vertebral column

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39
Q

Definitions

  • Neural tube defects
  • Myelomeningocele
  • Meningocele
  • SB occulta
A
  • Neural tube defect
    • All forms of failure of neural tube closure
    • Anencephaly to sacral meningocele
  • Myelomeningocele
    • Herniation of spinal cord tissue & the meninges through a bony cleft
    • Commonly the lumbosacral region
    • 20X more than meningocele
  • Meningocele
    • Herniation of the meninges only
    • Not associated w/ neural deficits
  • SB occulta
    • No herniation of tissue through vertebral cleft
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40
Q

The highest incidence of neural tube defects is in _______ and the lowest is in _____.

A

Ireland (1 in 250 live births)

Japan (1 in 3,000 live births)

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41
Q

What is the etiology of neural tube defects?

A
  • Multifactorial
    • Environmental
    • Genetic
    • Nutritional
    • Teratogenic factors
  • Mothers taking folic acid have decreased incidence of spina bifida
  • Teratogens that cause SB
    • Valproate, phenytoin, colchicine, vincristine, azathioprine, methotrexate
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42
Q

What are the clinical features of SB occulta?

A
  • Skin on the back epithelialized
  • Hairy patch or dimple covers the area
  • Usually lumbosacral region
  • No neurologic defects
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43
Q

What are the clinical features of Meningocele?

A
  • Fluctuant midline mass overlying the spine
  • Filled w/ CSF, transluminated
  • Neurologic deficits mild/absent
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44
Q

What are the clinical features of Myelomeningocele?

A
  • Fluctuant midline mass anywhere along spine
  • Commonly lumbosacral region
  • Neurologic defects present
    • Depend on level of lesion
    • Complete paraplegia (above L3) to preserved ambulation & variable bladder or bowel incontinence (S3 & below)
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45
Q

What are the 5 associated abnormalities & complications of Myelomeningocele?

A
  • Hydrocephalus
    • 90% of lumbosacral associated w/ Chiari type II malformations & hydrocephalus
  • Cervical hydrosyringomyelia
    • Accumulation of fluid w/i the central spinal cord canal & within the cord itself
  • Defects in neuronal migration
    • Gyral anomalies
    • Agenesis of corpus callosum
  • Orthopedic problems
    • Rib abnormalities, deformities of LE, LE fractures from loss of sensation
  • GU defects
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46
Q

How is spina bifida diagnosed prenatally?

A
  • Alpha fetoprotein
    • Elevated in amniotic fluid & maternal serum
    • At 16-18 wks gestation, detects 80% of spinal defects
  • Fetal sonography
    • Highly sensitive
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47
Q

How is spina bifida diagnosed after birth?

A
  • SB occulta
    • Skin abnormality overlying the spine
    • Confirmed by spinal radiographs
  • Meningocele
    • Physical exam findings
    • Confirmed by MRI of spinal cord & spine
  • Myelomeningocele
    • Physical exam
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48
Q

How is spina bifida managed?

A
  • SB occulta doesn’t require treatment
  • Meningocele: surgical repair
  • Myelomeningocele
    • Urgent surgical repair w/i 24 hrs of birth
    • Reduce morbidity & mortality from infection
    • Prevent further trauma to exposed neural tissue
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49
Q

What is the prognosis of spina bifida?

A
  • SB occulta & meningocele
    • Excellent prognosis
  • Myelomeningocele
    • 90% of pts survive to adolescence
    • Many handicapped
    • Associated problems:
      • Wheelchair dependency
      • Bladder/bowel incontinence
      • Mental retardation
      • Seizures
      • Precocious puberty
      • Pressure sores
      • Fractures
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50
Q

What is the definition of a coma?

A
  • State of unawareness of self & environment
  • Patient lies w/ eyes closed
  • Unarousable by external stimuli
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51
Q

What is the etiology of a coma?

Young vs. old children?

A
  • Children <5 YO
    • Nonaccidental trauma
    • Near-drowning
  • Older children
    • Drug overdose
    • Accidental head injury
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52
Q

What are the etiologies of focal lesions that lead to a coma?

A

Abnormal neuroimaging studies

  • Supratentorial lesions
    • Vascular: subarachnoid hemorrhage, multiple infarcts, thalamic infarct
    • Trauma: subdural hematoma, nonaccidental trauma
    • Tumors
    • Demyelination (postinfectious encephalitis)
  • Infratentorial lesions
    • Vascular: cerebellar hemorrhage
    • Trauma
    • Tumors
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53
Q

What are the etiologies of diffuse lesions that lead to a coma?

A

Normal neuroimaging studies

  • Ingestion
    • Drugs: atropine, scopolamine, benzodiazepines, barbiturates, ethanol, lithium, opiates, TCAs
    • Toxins: lead, mercury
  • Infection: encephalitis
  • Hypoxemia: near-drowning, CO
  • Abnormal metabolites
    • Metabolic: hypo/hyperglycemia, hypo/hypernatremia, thiamine deficiency
    • Endocrine: hypo/hyperthyroidism, hypo/hypercortisolism
  • Organ failure
    • Cardiac arrest, hepatic failure, uremia
  • Seizures
    • Nonconvulsive status epilepticus
  • Reye syndrome
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54
Q

What is the goal in the assessment of the comatose patient?

A
  • Determine the depth of the coma
  • Identify the neurologic signs that indicate the site & cause of the coma
  • Monitor the patient’s recovery
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55
Q

What are 6 ways to assess a comatose patient?

A
  • Glascow Coma Scale
  • Head & neck exam
  • Abnormal motor responses
  • Abnormal respiratory responses
  • Pupillary size & reactivity
  • Other brainstem reflexes
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56
Q

What is the Head & Neck exam for a comatose patient?

A
  • Scalp injuries
  • Breath odors (alcohol intoxication, DKA)
  • Nuchal rigidity (meningitis)
  • CSF or blood draining from auditory canal
    • Indicates a basilar skull fracture
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57
Q

What are some abnormal motor responses that can indicate brain damage in a comatose patient?

A
  • Flaccidity or no movement
    • Severe spinal/brainstem injury
  • Decerebrate posturing (extension of arms/legs)
    • Subcortical injury
  • Decorticate posturing (flexion of arms & extension of legs)
    • Bilateral cortical injury
  • Asymmetric responses
    • Hemispheric injury
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58
Q

What are some abnormal respiratory responses that can indicated injury in a comatose patient?

A
  • Hypoventilation
    • Opiate or sedative overdose
  • Hyperventilation
    • Metabolic acidosis (Kussmaul respirations)
    • Neurogenic pulmonary edema
    • Midbrain injury
  • Cheyne-Stokes breathing
    • Alternating apneas & hyperpneas
    • Bilateral cortical injury
  • Apneustic breathing
    • Pausing at full inspiration
    • Pontine damage
  • Ataxic or agonal breathing
    • Irregular respirations w/ no pattern
    • Medullary injury
    • Impending brain death
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59
Q

How do pupillary size & reactivity provide clues to brain injury in a comatose patient?

A
  • Unilateral dilated nonreactive pupil
    • Uncal herniation
  • Bilateral dilated nonreactive pupils
    • Topical application of a dilating agent
    • Postictal state
    • Irreversible brainstem injury
  • Bilateral constricted reactive pupils
    • Opiate ingestion
    • Pontine injury
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60
Q

What is the Oculocephalic maneuver (doll’s eyes)?

A
  • When turning the head of an unconscious patient, the eyes normally look straight ahead & then slowly drift back to the midline position
  • Intact vestibular apparatus senses a change in position
  • Injured brainstem
    • Movement of the head does not evoke any eye movement
    • Negative oculocephalic maneuver
    • “negative doll’s eyes”
61
Q

What is Caloric Irrigation?

A
  • Performed when oculocephalic response negative or can’t be performed
  • Angle the head at 30o
  • Irrigate each auditory canal w/ 10-30 mL of ice water
  • Intact (normal) cold caloric response
    • Eye deviation to the irrigated side
  • Abnormal response
    • Pontine injury
62
Q

Abnormal _____ & _____ reflexes indicate significant brainstem injury.

A

corneal, gag

63
Q

How is a comatose patient diagnostically worked up?

A
  • Glucose check immediately
  • Urine toxicology screen, serum electrolytes, metabolic panel
  • Head CT scan
    • Identify mass lesions or trauma
  • Lumbar puncture
    • Rule out meningoencephalitis if CT neg
  • Urgent EEG
64
Q

What is a seizure?

A
  • Transient, involuntary alteration of consciousness, behavior, motor activity, sensation or autonomic function
  • Excessive discharge from population of cerebral neurons
65
Q

What is epilepsy?

What is status epilepticus?

A
  • Epilepsy
    • 2 or more spontaneous seizures
    • No obvious precipitating cause
  • Status epilepticus
    • Seizure _>_30 min
    • Pt doesn’t regain consciousness
66
Q

____% of children have a single afebrile seizure before 16 years of age.

Fewer than ____ of children who have a single seizure go on to develop epilepsy.

Epilepsy has an incidence of _____% during childhood.

A

4-6%

1/3

0.5-0.8%

67
Q

What is the etiology of seizures?

A
  • Imbalance btwn excitatory & inhibitory input within the brain
  • Abnormalities in the membrane properties of individual neurons
  • In some children, cause of seizures is known
  • 60-70% of cases, cause is unknown
68
Q

What are the 7 causes of acute seizures during childhood?

What are some examples of each?

A
  • Head trauma
    • Cerebral contusion, subdural hematoma
  • Brain tumor
    • Astrocytoma, meningioma
  • Toxins
    • Amphetamines, cocaine
  • Infections
    • Meningitis, encephalitis, brain abscess, neurocysticercosis
  • Vascular
    • Cerebral infarction, intracranial hemorrhage
  • Metabolic disturbances
    • Hypocalcemia, hypoglycemia, hypomagnesemia, hypo/hypernatremia, pyridoxine deficiency
  • Systemic diseases
    • HTN, hypoxic-ischemic injury, inherited metabolic disorder, liver disease, renal failure, neurocutaneous disorders (tuberous sclerosis)
69
Q

What are the criteria for the classification of seizures?

A
  • Presence or absence of fever
  • Extent of brain involvement
  • Whether consciousness is impaired
  • Nature of the movements
70
Q

What is the classification of seizures?

A
  • Febrile
    • Simple
    • Complex
  • Afebrile
    • Partial (one hemisphere)
      • Simple: consciousness not impaired
      • Complex: consciousness impaired
    • Generalized (both hemispheres)
      • Tonic-clonic
      • Tonic
      • Clonic
      • Myoclonic
      • Absence
      • Atonic
71
Q

What are tonic-clonic seizures?

A
  • Most common type of generalized seizure
  • Characterized by:
    • Increased thoracic & abdominal muscle tone, followed by clonic movements of the arms & legs
    • Eyes rolling upward
    • Incontinence
    • Decreased consciousness
    • Postictal state of variable duration
72
Q

What are absence seizures?

A
  • Brief staring spells
  • Occur w/o loss of posture
  • Minor motor manifestations
    • Eye blinking or mouthing movements
  • <15 seconds
  • No postictal state
73
Q

What are partial (focal) seizures?

What are the symptoms?

A
  • Discharge of group of neurons in one hemisphere
  • Motor, sensory, psychomotor features
  • Simple partial seizures
    • Consciousness not impaired
  • Complex partial seizures
    • Consciousness decreased
74
Q

How is epilepsy classified?

A
  • Predominant seizure type
  • Site of origin of epileptic discharge
75
Q

What is the differential diagnosis of seizure-like events?

A
  • Breath-holding spells (infants)
  • GERD (Sandifer syndrome)
  • Syncope
  • Migraine
  • Vertigo
  • Movement disorder (tics, chorea)
  • Sleep disturbances (night terrors, somnambulism)
  • Transient ischemic attack
  • Rage attacks
  • Psychogenic seizures
76
Q

How are seizures diagnosed?

A
  • EEG
    • Identifies focus & pattern of epilepsy
    • Abnormal EEG not required for diagnosis
  • Video-EEG monitoring
    • Useful when clinical info inadequate
    • Pts <3 YO, sleep seizures, poor historians
  • Neuroimaging studies
    • Should be performed in all children except those w/ absence seizures or benign rolandic epilepsy
77
Q

What are the steps in evaluating a seizure patient?

What are some important labs?

A
  • ABCs
  • Laboratory studies
    • Afebrile seizures
      • 1st time in healthy child w/ normal neuro exam is fine
      • Serum electrolytes & neuroimaging in child w/ prior afebrile seizures
    • Febrile seizures
      • CNS infection MUST be ruled out
      • Examination of CSF
      • CBC, CXR, urine & blood cultures
78
Q

How is status epilepticus treated?

A
  • IV anti-convulsants
  • Short-acting benzodiazepine
    • Lorazepam, diazepam
  • Loading dose of phenobarbital or phenytoin
79
Q

How is epilepsy treated?

A
  • Pharmacotherapy
    • Single drug therapy
    • Generalized epilepsy: valproic acid or phenobarbital
    • Absence epilepsy: ethosuximide
    • Partial epilepsy: carbamazepine or phenytoin
  • Surgery
    • Medically intractable epilepsy: surgery to remove epileptic tissue
    • Best prognosis: temporal lobe lesions (75% complete control/remission)
80
Q

What are some treatment alternatives for epilepsy? (besides drugs/surgery)

A
  • Vagal nerve stimulator
    • Pacemaker-sized device that sends electrical impulse to the vagus nerve
    • Side effect: hoarseness
  • Ketogenic diet
    • High fat, low carb diet
    • State of ketosis suppresses seizure activity
81
Q

What is the prognosis of epilepsy?

A
  • Epilepsy is not a lifelong disorder
  • 70% of children can be weaned off their meds
    • After 2 yr seizure free period & normalization of EEG
82
Q

Febrile Seizures

  • definition
  • epidemiology
  • etiology
A
  • Any seizure accompanied by a fever owing to a non-CNS cause in patients 6 mo to 6 YO
  • Common, 3% of all children
  • Pathophysiologic mechanism unknown
  • Can be inherited (several gene mutations found)
83
Q

Simple vs. Complex Febrile Seizures

A
  • Simple febrile seizure
    • <15 min, generalized
  • Complex febrile seizure
    • >15 min, focal, recurs w/i 24 hrs
84
Q

How are febrile seizures diagnosed?

A
  • History, normal neurologic exam, exclusion of any CNS infection
  • Lumbar puncture only if meningitis suspected
  • Neither neuroimaging or EEG needed unless neurologic exam abnormal
85
Q

How are febrile seizures managed?

A
  • First-time or occasional
    • Not treated
  • Aggressive anti-pyretic treatment of subsequent febrile illnesses may help prevent febrile seizures
  • Frequent, recurrent
    • Daily anticonvulsant prophylaxis
      • Valproic acid, phenobarbital
    • Abortive treatment w/ rectal diazepam
86
Q

Approximately ____% of pts w/ one febrile seizure will have a recurrence. Recurrence risk _____ with increasing patient age. The risk of epilepsy is ____%.

A

30%, decreases, 2% (low)

87
Q

What are epileptic syndromes?

How are they classified?

A
  • Epileptic conditions characterized by a specific age of onset, seizure characteristics, EEG abnormality
  • 3 of the most common
    • Infantile spasms
    • Absence epilepsy of childhood
    • Benign rolandic epilepsy
88
Q

Infantile spasms (West syndrome)

age of onset

etiology

A
  • Age of onset: 3-8 mo (rare in children >2 YO)
  • Tuberous sclerosis is the most commonly identified cause of infantile spasms
    • PKU, hypoxic-ischemic injury, intraventricular hemorrhage, meningitis, encephalitis
89
Q

What are the clinical features of infantile spasms?

How is it diagnosed?

A
  • Brief, myoclonic jerks, lasting 1-2 sec each
    • Clusters of 5-10 seizures over 3-5 min
  • Sudden arm extension or head & trunk flexion
    • “jackknife seizures”, “salaam seizures”
  • EEG
    • Hypsarrhythmia pattern
    • Highly disorganized pattern of high amplitude spike & waves in both cerebral hemispheres
90
Q

How are infantile spasms managed?

What is the prognosis?

A
  • ACTH IM injections
    • 4-6 wks, effective in >70% pts
  • Valproic acid
    • Second-line drug of choice
  • Vigabatrin
    • Most effective for those w/ tuberous sclerosis
  • Outlook is poor
    • Moderate-severe mental retardation
91
Q

Absence epilepsy of childhood

age of onset

etiology

A
  • Age of onset: 5-9 YO
  • Female:Male = 3:2
  • Autosomal dominant inheritance
  • Age-dependent penetrance
92
Q

What are the clinical features of absence epilepsy of childhood?

A
  • Absence seizures 5-10 sec
  • Frequent, tens to hundreds of times per day
  • Accompanied by automatisms
    • Eye blinking, incomprehensible utterances
  • Loss of posture, urinary incontinence, postictal state do NOT occur
93
Q

Absence epilepsy of childhood

  • diagnosis
  • management
  • prognosis
A
  • EEG
    • Generalized 3 Hz spike & wave discharge
    • Both hemispheres
  • Treatment
    • Ethosuximide (1st line) or valproic acid
  • Outlook is very good
    • Seizures resolve in adolesence
    • No cognitive impairment
94
Q

Benign rolandic epilepsy

(benign centrotemporal epilepsy)

  • definition
  • epidemiology
  • etiology
A
  • Involves nocturnal partial seizures w/ secondary generalization
  • Most common partial epilepsy during childhood
    • 15% of epilepsy
  • Presents at 3-13 YO
  • Peak incidence: 6-7 YO
  • Boys > girls
  • Autosomal dominant, variable penetrance
95
Q

What are the clinical features of benign rolandic epilepsy?

A
  • Seizures in early morning hours
  • Oral-buccal manifestations
    • Moaning, grunting, pooling of saliva
  • Seizures spread to face & arm
  • Generalize to tonic-clonic seizures
96
Q

Benign rolandic epilepsy

  • diagnosis
  • management
  • prognosis
A
  • EEG
    • Biphasic spike & sharp wave disturbance in the mid-temporal & central regions
  • Treatment
    • Valproic acid (1st line), carbamazepine
  • Outcome is excellent
    • Seizures remit spontaneously during adolescence, no effects on development
97
Q

What are the intracranial causes of a headache?

A
  • Primary headaches
    • Primary dysfunction of neurons
      • Migraine headache
    • Primary dysfunction of muscles
      • Tension headache
  • Secondary headaches
    • Increased ICP
      • Hydrocephalus, brain tumor, subdural hematoma
    • Meningeal irritation
      • Meningitis, subarachnoid hemorrhage
98
Q

What are the extracranial causes of a headache?

A
  • Local causes
    • Ears: otitis media
    • Eyes: refractive rror, glaucoma
    • Nose: sinusitis
    • Mouth: toothache, abscess
    • TMJ dysfunction
  • Systemic causes
    • Anemia (children)
    • Depression (adolescents)
    • Hypoglycemia (children)
    • HTN (adolescents)
    • Psychogenic
    • CO poisoning
99
Q

What are the 4 pieces of clinical information that help determine the cause of a headache?

A
  • Quality of pain
    • Migraine: throbbing/pounding
    • Tension: aching/pressure
  • Site & radiation
    • Migraine: unilateral, periorbital to forehead & occiput
    • Tension: generalized/bitemporal
  • Time of onset
    • Tension: end of day
    • Increased ICP: morning
  • Duration
    • Shorter duration, less likely serious
100
Q

What is a migraine?

A
  • Prolonged (>1 hr), unilateral headaches
  • Nausea, vomiting, visual changes
  • Caused by changes in cerebral blood flow
101
Q

Migraines are the most common cause of headaches in children & adolescents, occuring in up to ___% of school-age children.

Age of onset is younger than ____yrs in _____%.

Before puberty, (M/F) dominant.

After puberty, (M/F) dominant.

A

5%

5 years, 20%

M>F

F>M

102
Q

What is the etiology of migraines?

A
  • Autosomal dominant
    • >80% of children have at least 1 affected parent
  • Changes in cerebral blood flow secondary to release of serotonin (5-HT), substance P & VIP from changes in neuronal activity
103
Q

How are migraines classified?

A
  • Migraine w/o aura
    • Most common form in children
    • No warning symptoms
  • Migraine w/ aura
    • Preceded by transient visual changes
    • Blurred vision, small areas of decreased vision (scotomata), streaks of light, hemianopsia, unilateral pressure/weakness
  • Migraine equivalent
    • Young children, headache absent
    • Prolonged, transient alteration of behavior
    • Cyclic vomiting, cyclic abd pain, paroxysmal vertigo
104
Q

Migraines are associated w/ what focal neurologic signs?

A
  • Ophthalmoplegic migraine
    • Unilateral ptosis or cranial nerve III palsy
  • Basilar artery migraine
    • Vertigo, tinnitus, ataxia, dysarthria
105
Q

What are the precipitating factors in migraines?

A
  • No obvious precipitating cause
  • Many sufferers sensitive to vasoactive substances in certain wines, cheeses, preserved meats & chocolate
  • Some patients have headaches induced by stress, fatigue, menstruation, exercise
106
Q

What are the 7 main clinical features of migraines?

A
  • Prolonged, throbbing, unilateral headache
    • Supraorbital area & radiates to occiput
    • Young children: bifrontal
  • Nausea & vomiting
    • Motion sickness common
  • Visual disturbances
    • Blurred vision, scotomata, jagged streaks of light that outline old forts (fortifications)
  • Photophobia or phonophobia
    • Treat by lying in dark, quiet room
  • Over-the-counter analgesics ineffective
  • Symptoms improved by sleep
  • Neurologic exam normal
107
Q

Migraines

  • diagnosis
  • management
  • prognosis
A
  • Hx & normal neuro exam
  • Rest & elimination of known triggers
  • Abortive treatment
    • Sumatriptan: selective 5-HT agonist
    • Injectable, intranasal, oral
  • Prophylaxis
    • Propranolol
  • Lifelong disorder w/ waxing & waning course
108
Q

What is a tension headache?

What age is most common?

A
  • Bifrontal or diffuse, dull, aching headaches
  • Associated w/ muscle contraction
  • Unusual during childhood
  • Extremely rare in children <7 YO
109
Q

What are the clinical features of a tension headache?

A
  • Dull, aching, rarely throbbing
  • Increases in intensity during the day
  • Pain usually bifrontal, can be diffuse
  • Isometric contraction
    • Temporalis, masseter, trapezius
  • No vomiting, visual changes, paresthesias
110
Q

How are tension headaches diagnosed?

How are they managed?

A
  • Tension headaches are very rare in childhood
  • Other diagnoses (migraines) should be preferentially considered
  • Treatment
    • Reassurance & pain control
    • Acetaminophen, ibuprofen
    • Stress & anxiety reduction
111
Q

What is a cluster headache?

What are the clinical features?

How is it treated?

A
  • Extremely rare during childhood
  • Unilateral frontal or facial pain
    • Conjunctival erythema
    • Lacrimation
    • Nasal congestion
  • <30 minutes, several times a day
  • May not occur for wks-mo
  • Treatment
    • Abortive therapy: oxygen, sumatriptan
    • Prophylaxis: Ca2+ blockers, valproic acid
112
Q

What is ataxia?

A
  • Inability to coordinate muscle activity during voluntary movement
  • Involves trunk or limbs
  • Caused by cerebellar or proprioceptive dysfunction
113
Q

What is the differential diagnosis of unsteady gait?

A
  • Cerebellar dysfunction
    • Unsteady wide-based stance
    • Irregular steps & veering to one side/other
  • Weakness
    • Muscle weakness: spinal cord lesions, acute disorders of motor unit (Guillain-Barre)
  • Encephalopathy
    • Infection, drug overdose, recent head trauma (decreased consciousness)
  • Seizures
    • During a seizure or postictal period
  • Vision problems
  • Vertigo
    • Migraines, acute labyrinthitis, brainstem tumors
114
Q

What is the differential diagnosis of cerebellar ataxia?

A
  • Brain tumors
    • Cerebellar astrocytoma
    • Cerebellar primitive neuroectodermal tumor (medulloblastoma)
    • Neuroblastoma
  • Trauma
    • Cerebellar contusion
    • Subdural hematoma
  • Toxins
    • Ethanol
    • Anticonvulsants
  • Vascular
    • Cerebellar infarction/hemorrhage
  • Infections
    • Meningitis
    • Encephalitis
  • Inflammatory
    • Acute cerebellar ataxia of childhood
  • Demyelination
    • Acute disseminated encephalomyelitis
    • Multiple sclerosis
115
Q

What is Acute Cerebellar Ataxia of Childhood?

What is the age of onset & etiology?

A
  • Unsteady gait secondary to a presumed autoimmune or postinfectious cause
  • Most common cause of ataxia in children
  • Age of onset: 18 mo to 7 yrs
    • Rarely in children >10 YO
  • Etiology
    • Varicella, influenza, EBV, mycoplasma
    • Ataxia follows viral illness by 2-3 wks
    • Immune complex deposition in the cerebellum
116
Q

What are the clinical features of acute cerebellar ataxia of childhood?

A
  • Truncal ataxia
    • Deterioration of gait
    • Young children refuse to walk (fear of fall)
  • Slurred speech & nystagmus
    • Hypotonia & tremors less common
  • Fever is absent
117
Q

How is acute cerebellar ataxia of childhood diagnosed?

A
  • Hx & physical exam
  • Exclusion of other causes of ataxia
  • Urgent neuroimaging study necessary in all pts
    • Rule out life-threatening causes
    • Tumors/hemorrhage in posterior fossa
  • Head CT scan is normal
118
Q

How is acute cerebellar ataxia of childhood managed?

A
  • Treatment is supportive
  • Complete resolution of symptoms: 2-3 mo
  • Physical therapy may be useful
119
Q

Guillain-Barre Syndrome

(acute inflammatory demyelinating polyneuropathy)

  • definition
  • etiology
A
  • Demyelinating polyneuritis
  • Ascending weakness, areflexia, normal sensation
  • Campylobacter jejuni
    • Prodromal gastroenteritis
    • CMV, EBV, HSV, influenza, varicella, Coxsackie virus
120
Q

What is the pathophysiology of Guillain-Barre Syndrome?

A
  • Principal sites of demyelination
    • Ventral spinal roots
    • Peripheral myelinated nerves
  • Cell-mediated immune response to infectious agent that cross-reacts to antigens on the Schwann cell membrane
121
Q

What are the 4 main clinical features of Guillain-Barre Syndrome?

A
  • Ascending, symmetric paralysis
    • May progress to respiratory arrest
  • No sensory loss occurs
    • Low back or leg pain (50%)
  • Cranial nerve involvement
    • Facial weakness (40-50%)
  • Miller-Fischer syndrome (variant)
    • Ophthalmoplegia
    • Ataxia
    • Areflexia
122
Q

How is Guillain-Barre Syndrome diagnosed?

A
  • Lumbar puncture
    • Albuminocytologic dissociation
    • Increased CSF protein in the absence of an elevated cell count
    • 1 wk after symptom onset
  • EMG
    • Decreased nerve conduction velocity or conduction block
  • Spinal MRI
    • Children <3 YO
    • Rule out compressive lesions of spinal cord
123
Q

How is Guillain-Barre Syndrome managed?

What is the prognosis?

A
  • IVIG
    • 2-4 days
    • Preferred treatment for children
  • Plasmapheresis
    • 4-5 days
    • Removes patient’s plasma along w/ presumed anti-myelin Ab
  • Complete recovery is the rule in children
    • Physical therapy may be necessary
124
Q

What is Sydenham chorea?

(St. Vitus’ dance)

A
  • Self-limited autoimmune disorder
  • Associated w/ rheumatic fever
  • Presents with:
    • Chorea
      • Uncontrolled, restless proximal limb movements
    • Emotional lability
125
Q

Sydenham chorea occurs in approximately ___% of patients w/ rheumatic fever.

Onset is common btwn ___ & ___ yrs of age.

A

25%, 5-13 YO

126
Q

What is the pathophysiology of Sydenham chorea?

A

Ab cross-react w/ membrane antigens on both group A ß-hemolytic streptococcus & basal ganglia cells

127
Q

What are the 8 clinical features of Sydenham chorea?

A
  • Immunologic response (2-7 mo)
  • Children appear restless
    • Face, hands, arms
    • Movements continuous, quick, random
  • Speech jerky or indistinct
  • Chameleon tongue
    • Patients unable to sustain protrusion of the tongue
  • Choreic hand
    • Wrist flexed & hyperextended at the metacarpal joints
  • Milkmaid’s grip
    • Pts unable to maintain grip on examiner’s fingers
  • Emotional lability
  • Gait & cognition not affected
128
Q

What is the differential diagnosis of Sydenham chorea?

A
  • Other conditions may cause chorea
    • Acquired & congenital conditions
  • Encephalitis
  • Kernicterus
  • SLE
  • Huntington’s disease
  • Wilson’s disease
129
Q

How is Sydenham chorea diagnosed?

A
  • No single confirmatory test
  • Elevated ASO or anti-DNase B titer
    • Recent strep infection
  • Neuroimaging
    • Head MRI
      • Increased signal intensity in the caudate & putamen on T2 weighted
    • Single-photon emission computed tomography (SPECT)
      • Increased perfusion to thalamus & striatum
130
Q

How is Sydenham chorea managed?

What is the prognosis?

A
  • Haloperidol, valproic acid, phenobarbital
  • Symptoms: several months to 2 yrs
  • Generally, all patients recover
131
Q

What is Tourette Syndrome?

What is the prevalence?

What is the etiology?

A
  • Chronic, lifelong movement disorder
  • Motor & phonic tics <18 YO
  • Tics are brief, stereotypical behaviors that are initiated by an unconscious urge that can be temporarily suppressed
  • 1 in 1,000 live births; 3% of children
  • Cause is unknown
132
Q

What are the clinical features of Tourette syndrome?

A
  • Motor tics
    • Simple: eye blinking, head/shoulder shaking
    • Complex: bouncing, jumping, kicking
  • Phonic tic
    • Simple: cough, groan, bark
    • Complex: echolalia (repetition of heard words/phrases)
  • Tics present _>_1 yr
  • Absence of signs of neurodegenerative disorder
  • Coprolalia
    • Utterance of obsence words (15%)
  • Associated findings
    • Learning disabilities
    • Attention deficit/hyperactivity disorder
    • Obsessive compulsive traits
133
Q

What is the differential diagnosis of Tourette syndrome?

A
  • Disorders that may cause tics
    • Wilson’s disease
    • Sydenham chorea
    • Partial seizures
    • Pediatric autoimmune neuropsych disorders associated w/ streptococcal infection (PAN-DAS)
    • Simple habits
  • Habits are situation-dependent & under voluntary control
134
Q

How is Tourette syndrome diagnosed?

A
  • Clinical diagnosis: hx & neuro findings
  • No lab or imaging tests
135
Q

How is Tourette syndrome managed?

What is the prognosis?

A
  • Pimozide (drug of choice)
    • Effective, minimal extrapyramidal SE
  • Clonidine
    • Less effective than pimozide
    • Major SE: sedation
  • Haloperidol
    • Risk of tardive dyskinesia
  • Hyponotherapy
    • Effective in some patients
  • Tics decrease in adulthood
    • Pharmacotherapy successful, SE limiting
136
Q

Duchenne & Becker Muscular Dystrophies

  • definition
  • epidemiology
  • etiology
A
  • Progressive, X-linked myopathies
  • Myofiber degeneration
  • DMD more severe than BMD
  • Deletion in dystrophin gene
  • All ethnic groups
    • 1 in 25,000 live births
    • Onset 2-5 YO
137
Q

What is the pathophysiology of DMD & BMD?

A
  • Dystrophin
    • High molecular weight cytoskeletal protein
    • Associates w/ actin & other structural membrane elements
  • Absence of dystrophin
    • Weakness & rupture of plasma membrane
    • Injury & degeneration of muscle fibers
138
Q

How do DMD & BMD appear on light microscopy?

A
  • Degeneration & regeneration of muscle fibers
  • Infiltration of lymphocytes into the injured area & replacement of damaged muscle fibers w/ fibroblasts & lipid deposits
139
Q

What are the clinical features of DMD & BMD?

What are the similarities/differences?

A
  • Slow, progressive weakness (legs first)
  • Loss of ability to walk
    • DMD: 10 YO
    • BMD: _>_20 YO
  • Psuedohypertrophy of calves
    • Excess accumulation of lipids
    • Replace degenerating muscle fibers
    • DMD > BMD
  • Gower’s sign
    • Weakness of pelvic muscles
    • Pts extend each leg & “climb up” each thigh
  • Cardiac involvement (50%)
    • Cardiomegaly, tachycardia, cardiac failure
  • Cognition
    • DMD: mild cognitive impairment
    • BMD: normal intelligence
140
Q

How are DMD & BMD diagnosed?

A
  • Presence of enlarged calf muscles in a young boy w/ muscle weakness suggests diagnosis
  • CK level elevated
  • EMG
    • Small, polyphasic muscle potentials
    • Normal nerve conductions
  • Muscle biopsy
    • Typical dystrophic pattern
  • Absent/decreased dystrophin levels
    • IHC, Western blot
  • DNA testing
    • Gene deletion (90%)
141
Q

How are DMD & BMD managed?

A
  • No cure
  • Oral steroids improve strength transiently
  • Not successful in clinical trials
    • Gene replacement
    • Myoblast transplantation
    • Dystrophin replacement
142
Q

What is the prognosis of DMD? BMD?

A
  • DMD
    • Wheelchair dependent by 10 YO
    • Die in late teens from respiratory failure
    • Assisted ventilation may help
  • BMD
    • Wheelchair dependent in 20s
    • Life expectancy in 50s
143
Q

Myasthenia Gravis

  • definition
  • etiology
  • boys vs. girls
A
  • Autoimmune disorder
  • Progressive weakness or diplopia
  • Ab against ACh receptor at NMJ
  • Girls 2-6X more than boys
144
Q

What is the classification system of Myasthenia Gravis?

A
  • Neonatal myasthenia
    • Transient weakness in the newborn period
    • Secondary to transplacental transfer of maternal AChR ab from the mother with myasthenia gravis
  • Juvenile myasthenia
    • Presents in childhood
    • Secondary to AChR ab formation
145
Q

What are the clinical features of myasthenia gravis?

A
  • Neonatal myasthenia
    • Hypotonia, weakness, feeding problems
  • Juvenile myasthenia
    • Bilateral ptosis (most common)
    • Characteristic increasing weakness later in the day w/ sustained muscle activity
    • Diplopia secondary to decreased extraocular movements
    • DTRs preserved
    • Other autoimmune disorders (JRA, DM, thyroid disease)
146
Q

How is myasthenia gravis diagnosed?

A
  • Tensilon test
    • IV injection of edrophonium chloride
    • Rapidly acting cholinesterase inhibitor
    • Transient improvement of ptosis
  • Decremental response to low freq (3-10 Hz) repetitive nerve stimulation
  • Presence of AChR ab titers
147
Q

How is myasthenia gravis managed?

Neonatal vs. Juvenile?

A
  • Neonatal
    • Treatment symptomatic
    • Compromised respiration: cholinesterase inhibitors or IVIG
  • Juvenile
    • Cholinesterase inhibitors
      • Pyridostigmine bromide
    • Immunotherapy
      • Corticosteroids
      • Plasmapheresis lowers AChR ab
        • When symptoms worse, respiratory effort compromised, pt unresponsive
      • IVIG
    • Thymectomy
148
Q

What is the prognosis of Myasthenia Gravis?

A
  • Neonatal
    • Symptoms mild & resolve 1-3 wks
  • Juvenile
    • Remission after thymectomy (60%)

Pediatrics (24 decks)

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