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Presentation download of all 3
presentations:
Pain, Delirium or
Neuroirritability?
Child Neurology and
Integrative Pediatric Pain and
Palliative Care
http://NoNeedlessPain.org
Episodes of Inconsolability in Children
with Developmental Delay- Definitions,
Evaluation and Treatment
With Drs Julie Hauer and Stefan
Friedrichsdorf
Audrey Foster-Barber, MD,
PhD
May 2015
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A Case
Neuroirritability
•  An 8 year old boy is admitted with 3 hours of screaming
•  Define irritability
•  Diagnosis of adrenoleukodystrophy, failed bone marrow
transplant
•  What is the pathophysiology?
•  Model of neuroirritability- infantile colic
•  No vision, no language, spastic quadriparesis, g tube fed
•  Who is at highest risk?
•  Parents feel he is in pain
•  Distinguish from delirium
•  He has been doing this long crying jag each evening for a
few weeks, but this is longer than most
•  Clinical evaluation- Dr. Hauer
•  Treatment- Dr. Friedrichsdorf
•  No fever, elevated HR, elevated BP
•  Is this pain? What is the source? Is this delirium?
•  Is this Neuroirritability?
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What is Neuroirritability?
Crying is Communication
•  Google and Pubmed search, poll of Child Neurologists
•  Crying as part of a communication loop
•  Nonverbal expression of need
–  Neuro-irritability
–  Neuro-cry
•  Caregiver physiologic response- increased heart rate,
endocrine changes
–  Pathologic cry
–  Screaming of unknown origin
•  Caregiver tries to interpret and to meet the child’s need
–  Central Irritability
•  Successful interaction encourages communication,
provides feedback to caregiver- solidifies bond
–  Pain-like behavior
–  Pain or Irritability of Unknown Origin, PIOU
–  Irritabile insomnia
–  Light-Switch mental status change
–  Paroxysmal fussiness
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Crying differs in neurologic impairment
Crying is not the only sign of distress
•  f0- frequency of cry- speed of opening and closing of the
vocal cords
•  Facial features- grimace
•  Change in sleep pattern- increase or decrease
•  Typical f0 is 350-700 Hz, abnormal is 1000-2000
•  Increase in abnormal movements- more spasticity,
dystonia, stereotypy
•  higher f0 cries in prematurity, brain injury, chromosome
abnormality
•  Decrease in movements- freezing
•  Autism spectrum often have few facial expression
changes and cry modulation seen as atypical
•  Autonomic changes- HR, sweating, flushing
•  Self harm- biting, scratching, head banging
•  Higher f0 cry, atypical cry pattern is perceived as more
aversive, distressing
•  Atypical or idiosyncratic reactions- laughing
•  Inability to soothe leads to risk of caregiver distress
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Pathophysiology of Neuroirritability
Pathophysiology of Neuroirritability
•  Abnormal cognitive, communication and motor systems
•  Abnormal cognitive, communication and motor systems
•  Is it pain?
•  Is it emotional?
–  This population at high risk for pain (musculoskeletal, skin, gi,
dental, headache, csf shunt)
–  Overstimulation by the environment
–  Oversensitized to pain- from prior painful stimuli (gut dysmotility,
GERD and constipation, hydrocephalus, surgeries)
–  More Rapidly cycling irritability- seen in patients with
developmental delay and family history of bipolar disorder
–  Autistic patients- specific anxiety treatment often effective
–  Excessive response to mild discomfort- hyperalgesia
–  Caregiver distress- sometimes neuroirritable behavior varies
with provider; cause or effect?
–  Pain response to typically nonpainful stimuli- allodynia
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Pain Systems
•  Nociceptive
–  Normal response to
tissue damage
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Infantile Colic- Normal Neuroirritability?
•  Neuropathic
•  Paroxysmal fussiness in infancy
•  Irritable, cry, arch back, twist trunk, extend legs
–  Pain due to lesion or
disease of the
Peripheral or Central
Nervous system
•  Typically starts after 2 weeks of age, decreases by 3-4
months
•  > than 3 hrs of crying, >3 days per week, for > 3 weeks in
an infant
–  Genetic or acquired
–  Abnormal pain signals
without ongoing injury
•  Inconsolable
•  Higher risk of poor bonding, of caregiver frustration, of
abuse
–  Hyperalgesia, allodynia
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Colic- Infant Migraine or Marker of
Genetic Risk
•  Immature nervous system?- processing the sensory input
of the day
•  Mothers with migraine 2x more likely to have infant with
colic
•  Higher association with anxious parents- direction of
causality?
•  Is it an infant migraine? Or sign of an abnormal
nociceptive system?
•  Hyper-reactive or hyper-sensitive child
•  Infantile colic raises likelihood of migraine, OR 5.6
•  Query gut pain? Though typical GI treatments (reflux, gas)
not shown to impact this behavior
•  Predictor of Pediatric Migraine
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Pediatric Migraine- Neuropathic pain?
An oversensitive brain?
•  Pediatric Migraine triad
•  Colic as a model of developmentally typical
Neuroirritability
–  tendency to motion sickness
–  Immature brain, based on a genetic tendency, associated with
neuropathic pain syndrome
–  tendency to ice-cream headaches
–  suffer from growing pains
•  Neuroirritability in Severe Neurologic impairment
•  Overzealous pain reaction to simple stimuli
–  A normal or excessive response to nociceptive pain?
–  dehydration
and/or
–  skipped meals
–  Pathological response to other non-pain stimuli?
–  poor sleep
–  hormonal changes
–  environmental changes- loud sounds bright light
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Who is at risk of Neuroirritability
•  Any child with severe neurologic impairment- cognitive,
communication, motor impairments
•  Degenerative neurologic disease- adrenoleukodystrophy,
mucopolysaccharoidosis, Krabbe
•  Especially those with dynamic CNS changesneurodegeneration, inflammation, intractable seizures
•  Inflammatory brain disease- NMDA-R encephalitis, opsoclonusmyoclonus-ataxia, infectious encephalitis
•  Acute brain injury- hypoxic ischemic injury, trauma, kernicterus
•  More severe at higher risk
•  Metabolic disease- mitochondrial disease, amino acidopathies
•  May depend also on underlying genetic tendency to
neuropathic pain
•  Chromosomal abnormalities- cri du chat (5p-), other
•  Severe impairment, subset of these patients- epileptic
encephalopathy, quadriplegic cerebral palsy, autism with intellectual
disability
•  May depend also on mood and environmental issues
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Neuroirritability or Delirium?
•  Delirium
•  Any seriously ill patient is at risk of delirium
•  Patients with severe neurologic impairment at high risk,
difficult to assess
–  Acute onset change in mental status
–  Fluctuations
•  May depend on context- is the patient ill? have there been
acute neurologic changes?
–  Inattention or confusion
–  May have other symptoms- hallucinations, change in sleep wake
cycle, autonomic instability
•  Neuroirritability often more prolonged, more chronic
•  Caused by acute disruption of brain centers controlling
attention, arousal, memory
–  Seizure, infection, new focal injury, metabolic derangement
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Medical Evaluation in Neuroirritability
Treatment of Neuroirritability
•  Clinical diagnosis, not a diagnosis of exhaustive
exclusion
•  Siden et al- PUP, Pathways for Unknown Pain
–  Standard analgesics
•  Take into context- parental report, relationship to feeding,
evacuation, moving, environmental changes, medications
–  Gabapentin, other antiepileptics
–  Antispasmodics
•  Good examination- teeth, ears, skin, diaper area, belly,
bones
–  Antiadrenergics, Benzodiazepines
–  Opioids
•  Neurologic status- worsened movement disorder, seizure
•  Nonpharmacologic interventions
•  Lab workup- infectious, inflammatory
–  Diaper change, vent g tube, change position
•  Imaging- brain, belly, bones
–  Decrease environmental stimulation
–  Music, massage, other sensory treatments
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Desena AD, Greenberg BM, Graves D. “Light switch” mental status changes and irritable insomnia are two
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Esposito G., Venuti P., Bornstein M. H. Assessment of Distress in Young Children: A Comparison of Autistic
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•  Thank you!
Hadden KL, von Baeyer CL. Global and specific behavioral measures of pain in children with cerebral palsy. Clin J
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•  Audrey Foster-Barber, MD, PhD
Hauer JM, Wical BS, Charnas L. Gabapentin successfully manages chronic unexplained irritability in children with
severe neurologic impairment. Pediatrics. 2007 Feb; 119(2): e519-522.
•  UCSF Child Neurology and Integrative Pediatric Pain and
Palliative Care Service
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Holbein M. The Crying Child. Palliative Care Case of the Month. May 2014, Vol 14, No. 36. University of Pittsburgh.
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