Nursing children with neurological
concerns
Nursing 315
October 2011
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Reading:
Review A&P of brain and spinal cord (refer to what you learned
in Bio 251 & 252)
Principles and practices of neuro assessment (refer to what you
learned in N275)
Perry, Hockenberry et al Chapters:
42: Cognitive and sensory impairment
51: Cerebral dysfunction
55: Neuromuscular or muscular dysfunction
COGNITIVE AND SENSORY
IMPAIRMENT
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Autism spectrum disorders
Genetic disorders (e.g. Down syndrome, fragile X
syndrome
Disorders of auditory or visual perception or processing.
`There are many ways of being smart`
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Children with cognitive and sensory impairments learn
differently than typical children. The role of those who
help them grow up is to try to understand and support
those pathways, and promote normalization and
integration where appropriate. Atraumatically.
Step-by-step processes of learning (how to dress, how to feed
oneself, how to read, how to speak) require task analysis of what
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is involved – and assistance with the fraction of the task is
problematic.
e.g. Many visually impaired children CAN learn to read… but there
needs to be a system of substitution for the visual input of letters
on a page. Many children with Down syndrome CAN learn basic
self-care, but perhaps velcro can be used a substitute for buttons.
DOWN SYNDROME
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Approx 95% of children with Down Syndrome have an extra
chromosome 21.
A minority have MOSAIC Down, wherein some cells have the
trisomy, and other cells have the typical 46 chromosomes.
Although women aged 40-and-over have a 1:110 chance of having
a baby with Down syndrome, MOST children with Down are born to
parents in their 20s-30s.
Prenatal diagnosis possible – with parents then having to make the
tough choice about termination of pregnancy.
Characteristics of children with Down syndrome:
Head/face:
--rounded, small head (bradycephaly)
--speckling of irides (brushfield’s spots)
--short pinna, small ears
--depressed nasal bridge
--inner epicanthal folds
--high, arched narrow palate
--protruding tongue
--short, broad neck
Body:
--shortened rib cage, short stature
--congenital heart defects common
--hypotonia (“like a rag-doll”)
--wide space b/w big and second toe
--transverse palmar (“simian”) crease
--increase risk of hypothyroidism and leukemia
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Intellectual
--average IQ around 50
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Incidence: 1 per 800-1000 live births.
Lifespan: more than 80% survive to age 55 years and
beyond.
No treatment exists to change the genetic makeup; but
there are treatments and approaches to the multiple
associated health issues: increased rates of congenital
heart disease, hypotonia, hypothyroidism.
You are working with an Early Intervention service and 5year-old Justin has just been referred to you. You want to
help him develop his muscle tone, to encourage expression
and to build his vocabulary. How will you do that.
FRAGILE X SYNDROME
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Caused by an abnormal gene on the long arm of the X
chromosome.
Women can be carriers (the healthy X chromosome dominates) but
men exhibit the trait.
Classic physical findings include a long face with prominent jaw
(prognathism), large protruding ears, and behavioral
manifestations such as outbursts, hyperactivity, short attention
span
Speech and language therapies
 Modifications in teaching
 Occupational therapy
 Rx: Serotonin agents such as Carbamazepine (Tegretol)
or
Fluoxetine (Prozac) to control outbursts
* Rx: CNS stimulants to improve attention span and
decrease hyperactivity
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AUTISM SPECTRUM DISORDERS
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Immune and-or environmental factors interact with a genetic
disorder of prenatal or postnatal brain development.
What we see: some combination of difficulties in regulating motion
and some parts of memory, delayed speech, atypical social
interaction often starting with difficulties establishing eye contact,
persistence of infantile reflexes, epileptic seizures.
Incidence: 1 in 150 children are living with ASD (CDC, 2007).
Screening for normal speech:
12 months… at least—babbling, gesturing
18 months… at least—a few single words
24 months… at least some two-word phrases
A group of diverse disorders including:
autism
Asperger`s
Pervasive Developmental Disorder Not Otherwise
Specified (PDD-NOS).
Diagnostic criteria (APA, DSM-4) includes:
marked impairment in social interaction
qualititative impairments in communication
restricted repetitive and stereotyped patterns of
behavior, interests, and activities
(see p. 1200 of Perry et al text)
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The majority – but not all – children with autism have
some degree of intellectual impairment, but also may have
atypical abilities… and may excel in music, puzzle-building,
mathematics.
Most children with autism will require lifelong supports.
Families need support.
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Videos of children and families living with autism.
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E.g Autism every day. 7-minute youtube video
PERCEPTUAL DISORDERS
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Visual impairment
Hearing impairment
Dyslexia, dyscalculia, dysgraphia
Auditory processing disorders
Visuo-spatial processing disorders (nonverbal learning
disabilities)
VISUAL IMPAIRMENTS
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Can be caused by
Genetic conditions such as: albinism, TaySachs disease
Prenatal and perinatal infections such as: CMV,
toxoplasmosis, herpes, chlamydia
Ocular trauma
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Retinoblastoma. Unilateral (usually) or bilateral ocular tumors.
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Retinopathy of prematurity
Amblyopia (strabismus, or e.g., or any cause of unilateral vision
impairment leads the brain to receive images only from the
stronger eye… so that the weaker eye is essentially unused … and
corresponding visual centers in the brain also do not develop.
HEARING IMPAIRMENTS
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Common – an estimated 3:1000 infants have some degree
of hearing loss
Types:
 Conductive hearing loss
 Sensorineural hearing loss
 Mixed
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Causes: genetic predisposition, anatomic malformations,
perinatal infection such as CMV or toxoplasmosis,
ototoxicity from certain antibiotics such as gentamycin
used in treating pediatric infections, chronic ear infections,
Down syndrome.
Conductive hearing loss can be partially remedied with hearing
aids, that amplify sound.
Sensorineural hearing loss – cochlear implants. Children with
sensorineural hearing loss have damaged the auditory nerves of
the inner ear. The cochlear implants bypass these and stimulate
surviving deeper auditory nerve fibers to send signals to the brain.
Working with visually impaired children:
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dependance on auditory and tactile stimuli for
orientation to time and space
educational adaptations
CEREBRAL DYSFUNCTION
(chap 51)
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Trauma to the head, brain tumors, seizure disorders, and
CNS infections all have neurological effects and sequelae.
Head trauma remains a significant cause of death and
permanent disability in children.
Neurological assessment --
pupillary response
eye opening in response to stimulus
best motor response to stimulus
best response to auditory or visual stimulus
hand grip: equal, unequal
LOC: alert; sleepy; irritable; disoriented; combative; lethargic; agitated…
Muscle tone: normal; arching; spastic; flaccid; weak; decorticate; decerebrate…
Fontanel: soft; flat; sunken; tense; bulging; closed
Mood: content; quiet; withdrawn; flat; hostile
Eye movement: normal; nystagmus; strabismus…
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Pediatric versions of Glasgow coma scale are available.
Similar, but small children may not typically be oriented to
time and place though verbal children usually can state
their first name.
See p. 1553 of text.
TRAUMAS
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Head traumas
acceleration & deceleration traumas
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subdural and epidural hematomas
concussion
skull fracture
Post-traumatic syndromes
post-concussion syndrome
cranial nerve palsies
seizure disorders
Infants and young children have soft and not-quite-fused
skulls. This presents a special plasticity to the brain, and
overall, children can survive brain injury better than adults.
However, their still-developing brains are especially
vulnerable. Also, their comparatively large head size in rt
the rest of their bodies means the head is particularly
vulnerable to trauma.
Underlying causes of symptoms is often related to not only to the actual injury
or disease process and bruising/bleeding, but to the swelling that occurs, or
blockage in the normal flow of cerebral spinal fluid through the ventricles of
the brain… in the closed bony cranium.
All of these events result in INCREASED INTRACRANIAL PRESSURE (IIP)…
irritable, high-pitched unconsolable cry; tense and bulging fontanels;
distended scalp veins; vomiting unrelated to feeding; headache; seizures;
bradycardia; drowsiness; flexion or extension posturing… coma.
Blunt trauma to the head in acceleration/deceleration
impact results in bruising of the brain and tissues at the
point of impact (coup), and directly opposite this
(contrecoup).
Trauma may also cause deformation of the head, with
destruction of bone, brain, and other tissue.
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Care of children with head injury involves sustaining vital signs,
possibly neurosurgery , possibly corticosteroid medication to
reduce swelling.
Support for families. While extent of tissue damage can be
described, neurological outcomes are often unclear.
Post-traumatic syndromes include hydrocephalus from damage to
structures, cranial nerve palsies, sensory or motor deficits,
emotional lability, behavior disturbances.
CONCUSSION
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The most common head injury is concussion, a transient neuronal
dysfunction, with instantaneous loss of awareness and
responsiveness that lasts minutes to hours. May result in loss of
consciousness – but not necessarily.
Concussion is defined as a traumatically induced alteration in
mental status. Confusion and amnesia follow.
Pathogenesis of concussion is still a bit unclear, but may
be a result of shearing forces that cause stretching,
compression, and tearing of nerve fibers.
POST-CONCUSSION SYNDROME can last for weeks to
months. Involve symptoms of impaired memory,
headache, behavioral disturbances.
NEUROLOGICAL INFECTIONS
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Menigitis
Encephalitis
Reye’s syndrome
Rabies
MENINGITIS
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An acute inflammation of the meninges and CSF. Most common
among under-fives.
Recent immunizations against Hib (Haemophilus influenza type B)
has successfully reduced the incidence of meningitis. However,
meningitis can be caused by other organisms as well—bacterial or
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viral, such as Staphylococccus aureus, Escherichia coli, Neisseria
meningitidis (meningococcus).
The most common route of infection is vascular dissemination from
foci of infection elsewhere.
Baby with meningococcal infection
Symptoms: sudden illness; arching; nuchal rigidity;
irritability; vomiting unrelated to feeding; thermo
instability; mottling; alterations in behavior and
consciousness.
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Meningitis is a medical emergency.
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Dx: lumbar puncture for CSF
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Treatment:
Sustain vital signs
Isolation precautions
IV access and hydration
IV antibiotics (start with two broad-spectrum antibiotics; once
culture and sensitivity results are back, one may suffice)
Corticosteroids (e.g. dexamethasone) to reduce swelling
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10 – 15% of bacterial meningitis is fatal.
Outcomes depend on duration of illness before antibiotic
therapy; type of organism; age of child; and adequacy of
treatment.
ENCEPHALITIS
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Can involve direct inflammation of the brain by a virus (e.g.
herpes) or postinfectious inflammation after infection.
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Early symptoms are vague… fever, irritability… but these evolve to
headache, dizziness, nuchal rigidity, changing levels of
consciousness and possibly seizures
Mild to severe forms.
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Tx: antiviral medications such as Acyclovir
Prognosis is highly variable. Some children recover
completely. Others have lifelong neurological sequelae.
SEIZURE DISORDERS
(EPILEPSY)
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Seizures are caused by excessive and disorderly neuronal
discharges in the brain.
Many forms…from subtle and transient to severe and continuous.
Multiple possible causes – acute trauma or infection, toxins,
tumors; metabolic disorders such as hypoglycemia or
hypocalcemia; residual damage from past traumas…
Types:
simple partial seizures with motor signs (tonic/clonic movements of part of
the body)
simple partial seizures with sensory signs (numbness, tingling, prickling,
pain, or paresthesia in part of the body)
complex partial seizures (psychomotor seizures – such as transient amnesia
or confusion; inability to respond to environment
tonic-clonic seizures (formerly known as grand mal) involving a tonic phase
(stiffening; eyes rolling up; apnea…); a clonic phase (from a few seconds to a
half hour or longer, usually about 30 seconds) of violent jerking,
incontinence…), and a post-ictal phase.
Status epilepticus
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Series of seizures at intervals too brief to allow the child to
regain consciousness between the time one event ends
and another begins.
A medical emergency as it can lead to respiratory arrest.
Absence seizures (formerly called petit mal) involving brief
loss of consciousness and may go unrecognized or be
confused with “daydreaming”.
Atonic and akinetic seizures (“drop attacks” with sudden
loss of tone)
Infantile spasms (sudden muscle spasms, possible rolling
of eyes upwards in an infant)
Videos
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Babyblue video of partial motor seizures
http://www.bing.com/videos/search?q=video+child+seizur
e&qpvt=video+child+seizure&FORM=VDRE#
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Video of child having tonic-clonic seizure
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What happens in children with seizure disorders?
In response to physiological stimuli, such as cellular dehydration,
severe hypoglycemia, electrolyte disturbances, sleep deprivation,
emotional stress, or endocrine changes… hyperexcitable cells
activate normal cells, and the impulse spreads thoughout… causing
changes in sensorimotor perception and level of consciousness.
Caring for a child having a seizure:
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Do not move or forcibly restrain him/her
Support airway with positioning
Remain calm, stay with the child, protect from injury.
Isolate from view of curious bystanders
911 for status epilepticus
ER treatment of status epilepticus involves
support of respirations and vital signs (ABC)
rectal diazepam / valium
IV access
IV antiepileptics: lorazepam/ ativan
or diazepam/ valium
or fosphenytoin/ cerebrex
or phenobarbitol / phenobarb
Treatment:
Usually pharmacological, but it may take numerous drug
adjustments to find the right dose of the right drug to
control seizures while limiting side-effects.
e.g. phenytoin (dilantin)
valproic acid (depakene)
Neuromuscular dysfunctions
(chapter 55)
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Cerebral palsy
Myelomeningocoele
Congenital hydrocephalus
Anencephaly
Spinal muscular atrophy
Werdnig-Hoffman Disease
Kugelberg-Welander Disease
Duchenne Muscular Dystrophy
CEREBRAL PALSY
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http://www.youtube.com/watch?v=uFaw8etxMw8
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YouTube: TomRogers123
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8 minute video wherein a 13 year boy teaches us about
what CP is.
CEREBRAL PALSY (CP)
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A group of permanent disorders of the development of movement and
posture, causing activity limitation, that are caused by nonprogressive
disturbances that occurred in the fetal or infant brain.
In addition to motor disturbances, CP also involves some combination of
differences in sensation, perception, communication, cognition, secondary
musculoskeletal conditions, and epilepsy
Many children with CP have normal-or-better intellectual abilities.
Causes: prenatal or perinatal asphyxia; low birth weight and
prematurity; intrauterine infections; meningitis; shaken baby
syndrome…
Improvements in survivorship of very low birth weight babies has
also resulted in a rise in the number of children with CP.
Different types:
spastic or pyramidal (>70%). involving hypertonicity and poor
control of posture, balance, and coordinated movement.
dyskinetic. Writhing and twisting movements
ataxic or extrapyramidal. Wide gait; rapid repetitive movements
mixed.
Goals of care:
To establish locomotion and communication.
To gain optimal appearance
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To correct associated defects such as contractures as soon as
possible
To adapt educational opportunities
To provide for social interaction with other affected and
unaffected children.
Orthopedic devices to aid locomotion include:
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Physical therapy
Orthopedic surgery to correct contractures
Pharmocotherapy for muscle relaxation
Pharmacotherapy for seizure control
Voice-activated computer technology
Voice synthesizers for children who cannot speak
Dental hygiene
Protective headgear
Recreational activities
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Supporting children and families…
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You are a nurse at a children’s hospital Ortho Unit. Bethany, age 6, who has spastic
quadriplegic CP, is admitted for ortho surgery to correct some bony contractures of her lower
limbs. Her voice is dysarthric. She is of normal intellect, and is frustrated when you cannot
make out what she is saying. Her mother understands her words. No sibs. The dad left the
family two years ago to start another family, and mom quit her job to be a full-time caregiver.
The family receives social assistance.
Plan? For Bethany? For her mom?
NEURAL TUBE DEFECTS
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Normally, the spinal cord is covered by meninges and a column of
bone, fusing in early fetal life.
MYELODYSPLAGIA refers to any malformation of the spinal canal
and spinal cord. NEURAL TUBE DEFECTS result from
myelodysplagia and can occur at any point along the spinal tract,
involving some or all of it.
Can involve:
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Spina bifida occulta
Meningocoele
Myelomeningocoele
Anencephaly
MYELOMENIGOCOELE
(spina bifida)
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What we see is some degree of motor and sensory loss in
areas innervated at or below the lesion, affecting – to
varying degrees – movement, sensation, and/or
continence.
Depending on the degree of downward-herniation of the
spinal contents, children with spina bifida commonly also
have congenital noncommunicating hydrocephalus.
The downward traction tends to obstruct the flow of CSF
between the ventricles of the brain.
HYDROCEPHALUS
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http://www.youtube.com/watch?v=Tk6HyxSI4Ag
ANENCEPHALY
DUCHENNE MUSCULAR DYSTROPHY
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YouTube: iangriff2007
3 minute video wherein a teenager with Duchenne teaches
us about his condition.
Duchenne Muscular Dystrophy is the most severe and most
common form.
An X-linked recessive disorder affecting males almost exclusively…
females are carriers.
Boys with DMD usually have apparently normal development
through infancy and early childhood… with evidence of muscle
weakness appearing b/w ages 3-7.
Early signs: waddling gait, lordosis, frequent falls, inability to ride
a bicycle…
Loss of independent ambulation by age 9-11.
Slow but relentless progressive muscle weakness throughout the
teenage years.
Death occurs from failure of respiratory muscles or infection.
Best lifespan: into early adulthood.
Survival can be extended by respiratory support such as
tracheostomy and suctioning…
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How to maximize independence in teenage boys with DMD?
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Genetic counseling for families affected.
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YOUR feelings about caring for children with serious
neurological disorders?
Societal stigmas?
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Countering societal stigmas?