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5/30/15
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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116-20
Desena AD, Greenberg BM, Graves D. “Light switch” mental status changes and irritable insomnia are two
particularly salient features of anti-NMDA receptor antibody encephalitis. Pediatr Neurol. 2014 Jul; 51(1):151-3.
Esposito G., Venuti P., Bornstein M. H. Assessment of Distress in Young Children: A Comparison of Autistic
Disorder, Developmental Delay, and Typical Development. Res Autism Spectrum Disord. 2011; 5(4): 1510-1516.
•  Thank you!
Hadden KL, von Baeyer CL. Global and specific behavioral measures of pain in children with cerebral palsy. Clin J
Pain. 2005 Mar-Apr; 21 (2): 140-6.
•  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
Hauer J. Identifying and managing sources of pain and distress in children with neurologic impairment. Pediatr Ann.
2010 Apr; 39 (4): 198-205.
Holbein M. The Crying Child. Palliative Care Case of the Month. May 2014, Vol 14, No. 36. University of Pittsburgh.
Jan JE, Abroms IF, Freeman RD, Brown GM, Espezel H, Connolly MB. Rapid cycling in severe multidisabled
children a form of bipolar affective disorder? Pediatr Neurol. 1994 Feb; 10 (1): 34-9.
•  [email protected]
Kheddache Y, Tadj C. Characterization of pathologic cries o f newborns based on fundamental frequency
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Schwantes S, O’Brien HW. Pediatric palliative care for children with complex chronic medical conditions. Pediatr
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•  IP3 415-353-1328
Siden H B, Carleton BC, Oberlander TF. Physician variability in treating pain and irritability of unknown origin in
children with severe neurologic impairment. Pain Res Manag. Sept/Oct 2013; 18(5): 243-248.
Sillanpaa M, Saarinen M.Infantile colic associated with childhood migraine: A prospective cohort study.
Cephalalgia. 2015. Epublish ahead of print
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